MedWorm.com provides a medical RSS filtering service. Over 6000 RSS medical sources are combined and output via different filters. This feed contains the latest items from the 'Acta Neuropathologica' source. Sat, 20 Mar 2010 14:40:31 +0100 http://www.medworm.com/index.php?rid=3372019&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fm263606lq32j6281%2F Abstract  Previous studies have shown altered synuclein, increased oxidative stress damage and increased oxidative stress responses in patients with sporadic Parkinson’s disease (PD) without cognitive impairment. Yet no information exists about possible molecular alterations in the cerebral cortex in familial PD. The present study shows abnormal α-synuclein solubility and aggregation, and aggregated nitrated α-synuclein, in the cerebral cortex (area 8) in cases with long-lasting PD linked with the G2019S LRRK2 mutation, one of them with a few Lewy bodies (LBs) and the other two without LBs in the cerebral cortex. Increased expression of the oxidative stress marker malondialdehyde-lysine (MDAL), together with increased oxidative stress responses, AGE receptors (RAGE) and ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3372019 Mon, 15 Mar 2010 17:54:44 +0100 3372019 http://www.medworm.com/index.php?rid=3372018&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fm18005324606hgp2%2F Abstract  Altered levels of cerebrospinal fluid (CSF) peptides related to Alzheimer’s disease (AD) are associated with pathologic AD diagnosis, although cognitively normal subjects can also have abnormal levels of these AD biomarkers. To identify novel CSF biomarkers that distinguish pathologically confirmed AD from cognitively normal subjects and patients with other neurodegenerative disorders, we collected antemortem CSF samples from 66 AD patients and 25 patients with other neurodegenerative dementias followed longitudinally to neuropathologic confirmation, plus CSF from 33 cognitively normal subjects. We measured levels of 151 novel analytes via a targeted multiplex panel enriched in cytokines, chemokines and growth factors, as well as established AD CSF biomarkers (l... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3372018 Mon, 15 Mar 2010 17:54:44 +0100 3372018 http://www.medworm.com/index.php?rid=3372020&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fb652mv7k5w514468%2F Abstract  A retrospective autopsy-based study of the human submandibular gland, one of the three major salivary glands, together with anatomically related peripheral structures (cervical superior ganglion, cervical sympathetic trunk, vagal nerve at the level of the carotid bifurcation), was conducted on a cohort consisting of 33 individuals, including 9 patients with neuropathologically confirmed Parkinson’s disease (PD), three individuals with incidental Lewy body disease (iLBD), 2 individuals with neuropathologically confirmed multiple system atrophy (MSA), and 19 controls, using α-synuclein immunohistochemistry in 100 μm polyethylene glycol-embedded tissue sections. Lewy pathology (LP) was present in the submandibular glands and cervical superior ganglia in PD (9... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3372020 Mon, 15 Mar 2010 05:32:44 +0100 3372020 http://www.medworm.com/index.php?rid=3361028&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F962q202580878572%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-010-0668-zAuthors Paolo Nozza, Istituto Giannina Gaslini Unità Operativa di Anatomia Patologica Largo Gerolamo Gaslini, 5 16148 Genoa ItalyMaria Luisa Casciana, Istituto Giannina Gaslini Unità di Neuro-Oncologia, Dipartimento di Emato-Oncologia Genoa ItalyAndrea Rossi, Istituto Giannina Gaslini Unità Operativa di Neuroradiologia Genoa ItalyArmando Cama, Istituto Giannina Gaslini Unità Operativa di Neurochirurgia Genoa ItalyClaudia Milanaccio, Istituto Giannina Gaslini Unità di Neuro-Oncologia, Dipartimento di Emato-Oncologia Genoa ItalyAlessandro Raso, Istituto Giannina Gaslini Unità Operativa di Neurochirurgia Genoa ItalyMarcello Ravegnani, Istituto Giannina Gaslini Unità Operativa di Neurochirurgia Genoa Ita... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3361028 Thu, 11 Mar 2010 18:12:21 +0100 3361028 http://www.medworm.com/index.php?rid=3356446&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F680254j523k34v32%2F Abstract  Sural nerve biopsy is considered mandatory for diagnosing non-systemic vasculitic neuropathy (NSVN). This invasive technique may be associated with unpleasant sequelae and cannot easily be repeated. Skin punch biopsy from an affected area may be a less invasive and repeatable diagnostic method. Here we assessed the potential diagnostic value of skin punch biopsies in NSVN by analyzing skin biopsies in 20 patients with sural nerve biopsy-proven NSVN and in 11 patients with non-inflammatory axonal neuropathy. As further controls, skin biopsies were studied in nine healthy volunteers. Five millimeter skin punch biopsies were taken under local anesthesia from the distal lateral calf and T cells and macrophages were quantified after immunostaining. The diagnostic sensi... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3356446 Wed, 10 Mar 2010 12:47:29 +0100 3356446 http://www.medworm.com/index.php?rid=3356445&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fnv02t0782pj9m8r6%2F Abstract  In contrast to extracellular plaque and intracellular tangle pathology, the presence and relevance of intraneuronal Aβ in Alzheimer’s disease (AD) is still a matter of debate. Human brain tissue offers technical challenges such as post-mortem delay and uneven or prolonged tissue fixation that might affect immunohistochemical staining. In addition, previous studies on intracellular Aβ accumulation in human brain often used antibodies targeting the C-terminus of Aβ and differed strongly in the pretreatments used. To overcome these inconsistencies, we performed extensive parametrical testing using a highly specific N-terminal Aβ antibody detecting the aspartate at position 1, before developing an optimal staining protocol for intraneuronal Aβ detection in para... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3356445 Wed, 10 Mar 2010 12:47:29 +0100 3356445 http://www.medworm.com/index.php?rid=3356447&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fmg36u8875xx2334r%2F Abstract  The prevalence of Alzheimer disease (AD) and vascular dementia (VD) increases with advancing age, but less so after age 90 years. A retrospective hospital-based study of the relative prevalence of different disorders was performed in 1,110 consecutive autopsy cases of demented elderly in Vienna, Austria (66% females, MMSE <20; mean age 83.3 ± 5.4 SD years). It assessed clinical, general autopsy data and neuropathology including immunohistochemistry. Neuropathologic diagnosis followed current consensus criteria. Four age groups (7–10th decade) were evaluated. In the total cohort AD pathology was seen in 82.9% (“pure” AD 42.9%; AD + other pathologies 39.9%), VD in 10.8% (mixed dementia, MIX, i.e. AD + vascular encepha... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3356447 Wed, 10 Mar 2010 12:47:27 +0100 3356447 http://www.medworm.com/index.php?rid=3336931&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F65tv645812213554%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-010-0651-8 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 119 Journal Issue Volume 119, Number 3 / March, 2010 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336931 Wed, 03 Mar 2010 08:50:28 +0100 3336931 http://www.medworm.com/index.php?rid=3336932&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fm724484j385836w3%2F Abstract  Abnormal TDP-43 aggregation is a prominent feature in the neuropathology of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Mutations in TARDBP, the gene encoding TDP-43, cause some cases of ALS. The normal function of TDP-43 remains incompletely understood. To better understand TDP-43 biology, we generated mutant mice carrying a genetrap disruption of Tardbp. Mice homozygous for loss of TDP-43 are not viable. TDP-43 deficient embryos die about day 7.5 of embryonic development thereby demonstrating that TDP-43 protein is essential for normal prenatal development and survival. However, heterozygous Tardbp mutant mice exhibit signs of motor disturbance and muscle weakness. Compared with wild type control littermates, Tardbp +/− animals hav... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336932 Wed, 03 Mar 2010 08:50:27 +0100 3336932 http://www.medworm.com/index.php?rid=3336933&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F5468355p154u1183%2F Abstract  Human prion diseases can be sporadic, inherited or acquired by infection and show considerable phenotypic heterogeneity. We describe the clinical, histopathological and pathological prion protein (PrPSc) characteristics of a Dutch family with a novel 7-octapeptide repeat insertion (7-OPRI) in PRNP, the gene encoding the prion protein (PrP). Clinical features were available in four, neuropathological features in three and biochemical characteristics in two members of this family. The clinical phenotype was characterized by slowly progressive cognitive decline, personality change, lethargy, depression with anxiety and panic attacks, apraxia and a hypokinetic-rigid syndrome. Neuropathological findings consisted of numerous multi- and unicentric amyloid plaques througho... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336933 Wed, 03 Mar 2010 08:50:25 +0100 3336933 http://www.medworm.com/index.php?rid=3336935&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fkn6u3u426m2646h4%2F Abstract  Neuropathological diagnosis of neurodegenerative dementias evolved by adapting the results of neuroanatomy, biochemistry, and cellular and molecular biology. Milestone findings of intra- and extracellular argyrophilic structures, visualizing protein deposition, initiated a protein-based classification. Widespread application of immunohistochemical and biochemical investigations revealed that (1) there are modifications of proteins intrinsic to disease (species that are phosphorylated, nitrated, oligomers, proteinase-resistant, with or without amyloid characteristics; cleavage products), (2) disease forms characterized by the accumulation of a single protein only are rather the exception than the rule, and (3) some modifications of proteins elude present neuropatho... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336935 Wed, 03 Mar 2010 08:50:23 +0100 3336935 http://www.medworm.com/index.php?rid=3336934&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F4363r3h05748018g%2F Abstract  In Alzheimer’s disease (AD), Purkinje neurons in the cerebellum are spared, while, for instance, pyramidal neurons in the hippocampus are neuropathologically affected. Several lines of evidence suggest that the pathogenesis could be induced by the concentration-dependent polymerization of the amyloid β-peptide (Aβ) into extracellular oligomers. The role of intracellular Aβ is not fully investigated, but recent data indicate that also this pool could be of importance. Here, we use laser capture microdissection microscopy for isolation of Purkinje neurons from AD cases and controls, and quantify the low levels of intracellular Aβ using a novel and highly sensitive ELISA. Similar to Cornu Ammonis 1 pyramidal neurons, the intracellular levels of the most toxic v... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336934 Wed, 03 Mar 2010 08:50:23 +0100 3336934 http://www.medworm.com/index.php?rid=3336937&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj2751252n061n1ut%2F Abstract  Autism is characterized by a broad spectrum of clinical manifestations including qualitative impairments in social interactions and communication, and repetitive and stereotyped patterns of behavior. Abnormal acceleration of brain growth in early childhood, signs of slower growth of neurons, and minicolumn developmental abnormalities suggest multiregional alterations. The aim of this study was to detect the patterns of focal qualitative developmental defects and to identify brain regions that are prone to developmental alterations in autism. Formalin-fixed brain hemispheres of 13 autistic (4–60 years of age) and 14 age-matched control subjects were embedded in celloidin and cut into 200-μm-thick coronal sections, which were stained with cresyl violet and u... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336937 Wed, 03 Mar 2010 08:50:22 +0100 3336937 http://www.medworm.com/index.php?rid=3336936&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj53l7716262p34w4%2F Abstract  We have investigated alterations in myelin associated with Aβ plaques, a major pathological hallmark of Alzheimer’s disease (AD), in human tissue and relevant transgenic mice models. Using quantitative morphological techniques, we determined that fibrillar Aβ pathology in the grey matter of the neocortex was associated with focal demyelination in human presenilin-1 familial, sporadic and preclinical AD cases, as well as in two mouse transgenic models of AD, compared with age-matched control tissue. This demyelination was most pronounced at the core of Aβ plaques. Furthermore, we found a focal loss of oligodendrocytes in sporadic and preclinical AD cases associated with Aβ plaque cores. In human and transgenic mice alike, plaque-free neocortical regions showe... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3336936 Wed, 03 Mar 2010 08:50:22 +0100 3336936 http://www.medworm.com/index.php?rid=3309006&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F1m10x93m22n7m178%2F In this study, we performed clinical, pathological, and genetic analyses in infantile patients pathologically diagnosed as CFTD to evaluate CDM patients indistinguishable from CFTD. We examined CTG repeat expansion in DMPK in 28 infantile patients pathologically diagnosed as CFTD. Mutation screening of ACTA1 and TPM3 was performed, and we compared clinical and pathological findings of 20 CDM patients with those of the other cohorts. We identified four (14%) patients with CTG expansion in DMPK. ACTA1 mutation was identified in four (14%), and TPM3 mutation was found in two (7%) patients. Fiber size disproportion was more prominent in patients with ACTA1 or TPM3 mutations as compared to CFTD patients with CTG expansion. A further three patients among 20 CDM patients showed pathological f... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3309006 Wed, 24 Feb 2010 06:48:14 +0100 3309006 http://www.medworm.com/index.php?rid=3296378&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fx14h770406wg5107%2F Abstract  The neuropathology of solvent inhalation consists of patchy myelin loss with white matter macrophages that contain granular inclusions. It has been described only in a small number of cases. We sought to characterize the abnormalities in greater detail. In a retrospective study from 1995 to 2009, we encountered 88 autopsy cases with documented history of solvent abuse by inhalation and 1 with industrial exposure. Among these are 6 fetuses and infants with maternal exposure, 23 children (12–17 years), and 60 adults (18–66 years). Available brain samples from 75 cases were stained with solochrome cyanin (to demonstrate myelin) and periodic acid–Schiff (PAS) (to highlight the inclusions). Forty brains of ethanol and/or illicit drug exposed individuals ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3296378 Sun, 21 Feb 2010 06:54:26 +0100 3296378 http://www.medworm.com/index.php?rid=3274221&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fl12k573222970827%2F Abstract  Cerebral atherosclerosis (AS), small vessel disease (SVD), and cerebral amyloid angiopathy (CAA) are the most prevalent arterial disorders in the aged brain. Pathogenetically, AS and SVD share similar mechanisms: plasma protein leakage into the vessel wall, accumulation of lipid-containing macrophages, and fibrosis of the vessel wall. CAA, on the other hand, is characterized by the deposition of the amyloid β-protein in the vessel wall. Despite these differences between CAA, AS and SVD, apolipoprotein E (apoE) is involved in all three disorders. Such a pathogenetic link may explain the correlations between AS, SVD, CAA, and Alzheimer’s disease in the brains of elderly individuals reported in the literature. In addition, AS, SVD, and CAA can lead to tissue lesio... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3274221 Sun, 14 Feb 2010 06:50:28 +0100 3274221 http://www.medworm.com/index.php?rid=3256788&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fe17277107r318175%2F We report an unusual tauopathy in three Japanese patients presenting with Parkinsonism and motor neuron disease (neuroimaging revealed frontotemporal cerebral atrophy in two patients who were examined). At autopsy, all cases showed FTLD with the most severe neuronal loss and gliosis evident in the premotor and precentral gyri. Although less severe, such changes were also observed in other brain regions, including the basal ganglia and substantia nigra. In the spinal cord, loss of anterior horn cells and degeneration of the corticospinal tract were evident. In addition, the affected regions exhibited neuronal cytoplasmic inclusions resembling neurofibrillary tangles. Immunostaining using antibodies against hyperphosphorylated tau and 4-repeat tau revealed widespread occurrence of neu... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3256788 Sat, 06 Feb 2010 08:18:19 +0100 3256788 http://www.medworm.com/index.php?rid=3247989&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fu6v55w4724615416%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-010-0648-3Authors Wojciech Biernat, Medical University of Gdańsk Department of Pathology ul. Dębinki 7 80-211 Gdańsk PolandSonia Kaniuka, Medical University of Gdańsk Department of Endocrinology and Internal Medicine Gdańsk PolandMirosław Stempniewicz, Medical University of Gdańsk Department of Neurosurgery Gdańsk PolandDaniel Ręcławowicz, Medical University of Gdańsk Department of Neurosurgery Gdańsk PolandKrzysztof Sworczak, Medical University of Gdańsk Department of Endocrinology and Internal Medicine Gdańsk Poland Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3247989 Fri, 05 Feb 2010 17:53:21 +0100 3247989 http://www.medworm.com/index.php?rid=3241610&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F474074n44123xr24%2F Abstract  Diffusely infiltrating cerebral gliomas frequently carry point mutations in codon 132 of the isocitrate dehydrogenase 1 (IDH1) gene or in codon 172 of the IDH2 gene, which are both clinically important as diagnostic and prognostic markers. Here, we report on a method that allows for the rapid detection of IDH1 and IDH2 mutations based on pyrosequencing. The method is applicable to routinely processed tissue specimens and provides quantitative mutation data within less than one working day. Due to its high sensitivity, the technique may also be used for the diagnostic assessment of IDH1 or IDH2 mutation in tissue samples with low tumor cell content, such as the infiltration zone of diffuse gliomas. Using pyrosequencing and/or conventional cycle sequencing of IDH1 and... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3241610 Wed, 03 Feb 2010 17:55:36 +0100 3241610 http://www.medworm.com/index.php?rid=3241611&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fu19756771wxtjk4q%2F Content Type Journal ArticleCategory Letter of InvitationDOI 10.1007/s00401-010-0650-9Authors Herbert Budka, Institute of Neurology Vienna Austria Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3241611 Wed, 03 Feb 2010 17:55:35 +0100 3241611 http://www.medworm.com/index.php?rid=3241612&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F32k4u46384370320%2F Abstract  Activating β-catenin mutations with aberrant cytoplasmic and nuclear protein accumulation are hallmarks of adamantinomatous craniopharyngiomas (adaCP). These tumours tend to be associated with unfavourable and occasionally disastrous sequelae, as they invade adjacent brain structures such as the hypothalamus. The peculiar digitate growth pattern does not always allow gross surgical removal often leading to recurrence. The tips of invading adaCP epithelium harbour cell clusters with nuclear β-catenin accumulations, suggesting an influence of β-catenin-dependent signal transduction on the tumour migratory capacity. This hypothesis was tested by suppressing β-catenin expression in six primary human adaCP cell cultures using small interfering RNA (siRNA) directed ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3241612 Wed, 03 Feb 2010 17:55:34 +0100 3241612 http://www.medworm.com/index.php?rid=3241613&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fq057h84428303022%2F Abstract  Somatic mutations in the isocitrate dehydrogenase 1 gene (IDH1) occur at high frequency in gliomas and seem to be a prognostic factor for survival in glioblastoma patients. In our set of 98 glioblastoma patients, IDH1 R132 mutations were associated with improved survival of 1 year on average, after correcting for age and other variables with Cox proportional hazards models. Patients with IDH1 mutations were on average 17 years younger than patients without mutation. Mutated IDH1 has a gain of function to produce 2-hydroxyglutarate by NADPH-dependent reduction of α-ketoglutarate, but it is unknown whether NADPH production in gliomas is affected by IDH1 mutations. We assessed the effect of IDH1 R132 mutations on IDH-mediated NADPH production in glio... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3241613 Wed, 03 Feb 2010 06:48:43 +0100 3241613 http://www.medworm.com/index.php?rid=3233611&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fur07188w084t1312%2F Abstract  The mechanism of viral persistence, the driving force behind the chronic progression of inflammatory demyelination in canine distemper virus (CDV) infection, is associated with non-cytolytic viral cell-to-cell spread. Here, we studied the molecular mechanisms of viral spread of a recombinant fluorescent protein-expressing virulent CDV in primary canine astrocyte cultures. Time-lapse video microscopy documented that CDV spread was very efficient using cell processes contacting remote target cells. Strikingly, CDV transmission to remote cells could occur in less than 6 h, suggesting that a complete viral cycle with production of extracellular free particles was not essential in enabling CDV to spread in glial cells. Titration experiments and electron microscopy... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3233611 Mon, 01 Feb 2010 06:45:57 +0100 3233611 http://www.medworm.com/index.php?rid=3224122&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fg485201650767j73%2F Abstract  Amyotrophic lateral sclerosis (ALS) is a conformational disease in which misfolding and aggregation of proteins such as SOD1 (familial ALS) and TDP-43 (sporadic ALS) are central features. The conformations adopted by such proteins within motor neurons in affected patients are not well known. We have developed a novel conformation-specific antibody (USOD) targeted against SOD1 residues 42–48 that specifically recognizes SOD1 in which the beta barrel is unfolded. Use of this antibody, in conjunction with the previously described SEDI antibody that recognizes the SOD1 dimer interface, allows a detailed investigation of the in vivo conformation of SOD1 at the residue-specific level. USOD and SEDI immunohistochemistry of spinal cord sections from ALS cases resulting ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3224122 Thu, 28 Jan 2010 17:59:49 +0100 3224122 http://www.medworm.com/index.php?rid=3224123&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F1510517780852723%2F Abstract  The different histochemical ATPase properties of myosins separating the muscle fiber types have been utilized in diagnostic muscle biopsy routine for more than four decades. The ATPase staining method is rather laborious and has several disadvantages, such as weakening of staining over time and non-specific staining of capillaries, making the distinction of extremely atrophic muscle fibers difficult. We have developed a reliable and advanced immunohistochemical myosin double staining method for the identification of fiber types, including highly atrophic fibers in routine diagnostics. With this double staining method, we are able to distinguish among type I (ATPase type 1), IIA (ATPase type 2A), IIX (ATPase type 2B) and remodeled ATPase type 2C fibers expressing b... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3224123 Wed, 27 Jan 2010 20:26:08 +0100 3224123 http://www.medworm.com/index.php?rid=3224125&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F032t438h1301mp77%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-010-0641-xAuthors Elan D. Louis, Columbia University GH Sergievsky Center, College of Physicians and Surgeons New York NY USACordelia Erickson-Davis, Columbia University GH Sergievsky Center, College of Physicians and Surgeons New York NY USARajesh Pahwa, University of Kansas Medical Center Department of Neurology Kansas City KS USAKelly E. Lyons, University of Kansas Medical Center Department of Neurology Kansas City KS USAAnthony Garber, Molecular Diagnostic Laboratory Comprehensive Genetics Milwaukee WI USACarol B. Moskowitz, Columbia University Department of Neurology, College of Physicians and Surgeons New York NY USAArlene Lawton, Columbia University GH Sergievsky Center, College of Physicians and Surgeons New ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3224125 Wed, 27 Jan 2010 20:26:07 +0100 3224125 http://www.medworm.com/index.php?rid=3224124&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fg07404255t172722%2F Content Type Journal ArticleCategory Thanks to refereesDOI 10.1007/s00401-010-0640-y Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3224124 Wed, 27 Jan 2010 20:26:07 +0100 3224124 http://www.medworm.com/index.php?rid=3206536&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fg5014140m0k3r233%2F Abstract  The aim of this study was to characterize myelin loss as one of the features of white matter abnormalities across three common dementing disorders. We evaluated post-mortem brain tissue from frontal and temporal lobes from 20 vascular dementia (VaD), 19 Alzheimer’s disease (AD) and 31 dementia with Lewy bodies (DLB) cases and 12 comparable age controls. Images of sections stained with conventional luxol fast blue were analysed to estimate myelin attenuation by optical density. Serial adjacent sections were then immunostained for degraded myelin basic protein (dMBP) and the mean percentage area containing dMBP (%dMBP) was determined as an indicator of myelin degeneration. We further assessed the relationship between dMBP and glutathione S-transferase (a marker of ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3206536 Fri, 22 Jan 2010 03:00:32 +0100 3206536 http://www.medworm.com/index.php?rid=3195641&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fr83v126878678u84%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-010-0638-5 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 119 Journal Issue Volume 119, Number 2 / February, 2010 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3195641 Wed, 20 Jan 2010 06:44:28 +0100 3195641 http://www.medworm.com/index.php?rid=3195642&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F1w77125g83168082%2F Abstract  Ataxia-telangiectasia-like disorder (ATLD) is caused by mutations of the MRE11 gene and is characterized by cerebellar ataxia, increased frequency of chromosomal translocations and hypersensitivity to ionizing radiation. ATLD is a rare genetic disease and the associated pathological changes in the brain are unclear. Here, we report the neuropathological findings in the first cases of genetically confirmed ATLD in a pair of Japanese male siblings. Magnetic resonance imaging studies performed during infancy revealed that both subjects had cerebellar atrophy. They died of pulmonary cancer at 9 and 16 years. The siblings had the same compound heterozygous mutations of the MRE11 gene. Brain autopsy demonstrated mild and severe cerebellar atrophy in the vermis and me... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3195642 Wed, 20 Jan 2010 06:44:18 +0100 3195642 http://www.medworm.com/index.php?rid=3169352&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F660m58814w1g4n27%2F Abstract  Aberrant transcription and mRNA processing of multiple genes due to RNA-mediated toxic gain-of-function has been suggested to cause the complex phenotype in myotonic dystrophies type 1 and 2 (DM1 and DM2). However, the molecular basis of muscle weakness and wasting and the different pattern of muscle involvement in DM1 and DM2 are not well understood. We have analyzed the mRNA expression of genes encoding muscle-specific proteins and transcription factors by microarray profiling and studied selected genes for abnormal splicing. A subset of the abnormally regulated genes was further analyzed at the protein level. TNNT3 and LDB3 showed abnormal splicing with significant differences in proportions between DM2 and DM1. The differential abnormal splicing patterns for T... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3169352 Tue, 12 Jan 2010 06:44:31 +0100 3169352 http://www.medworm.com/index.php?rid=3169353&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fc660567352410569%2F Abstract  Compelling evidence identifies a link between cytotoxic effects of cytosolic phospholipase A2 (cPLA2) activity and neuron death in cell cultures. cPLA2 catalyzes the hydrolysis of membrane phospholipids to produce and release arachidonate, leading to plasma membrane injury, inflammatory response and subsequent cell death. To assess a role for cPLA2 in the pathomechanism of amyotrophic lateral sclerosis (ALS), we performed immunohistochemical, immunoblot, and densitometric analyses of cPLA2 and its active form phosphorylated at S505 (p-cPLA2) on spinal cords obtained at autopsy from ten sporadic ALS patients and ten age-matched controls. On sections, immunoreactivities for cPLA2 and p-cPLA2 were distinct and localized in almost all of the motor neurons, reactive astro... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3169353 Mon, 11 Jan 2010 18:20:16 +0100 3169353 http://www.medworm.com/index.php?rid=3154880&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F6rpn174153332053%2F Abstract  Prion disease is a neurodegenerative malady, which is believed to be transmitted via a prion protein in its abnormal conformation (PrPSc). Previous studies have failed to demonstrate that prion disease could be induced in wild-type animals using recombinant prion protein (rPrP) produced in Escherichia coli. Here, we report that prion infectivity was generated in Syrian hamsters after inoculating full-length rPrP that had been converted into the cross-β-sheet amyloid form and subjected to annealing. Serial transmission gave rise to a disease phenotype with highly unique clinical and neuropathological features. Among them were the deposition of large PrPSc plaques in subpial and subependymal areas in brain and spinal cord, very minor lesioning of the hippocampus and ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3154880 Tue, 05 Jan 2010 16:58:21 +0100 3154880 http://www.medworm.com/index.php?rid=3136256&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F928065m583n57n34%2F Abstract  Pilocytic astrocytoma (PA) is the most common glioma in the pediatric population. PAs can exhibit variable behavior that does not always correlate with location. Although oncogenic rearrangements of the BRAF gene have recently been described in PAs, it is not clear whether such alterations have an impact on outcome. An institutional cohort of 147 PAs (118 with outcome data) from both cerebellar and non-cerebellar locations (spine, diencephalon, midbrain, brainstem, and cortex) was utilized in this study. Parameters included quantification of characteristic morphologic variables as well as genes and molecular loci previously shown to be of relevance in high-grade gliomas, including 1p, 9p, 10q, 17p, 19q, and BRAF. Neither 1p, 9p, and 10q nor 19q showed significant a... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3136256 Thu, 31 Dec 2009 16:44:59 +0100 3136256 http://www.medworm.com/index.php?rid=3136255&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fh285536q4620m682%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0632-yAuthors Sandra Camelo-Piragua, Massachusetts General Hospital James Homer Wright Pathology Laboratories Boston MA USAMichael Jansen, Massachusetts General Hospital James Homer Wright Pathology Laboratories Boston MA USAAniruddha Ganguly, Massachusetts General Hospital James Homer Wright Pathology Laboratories Boston MA USAJ. ChulMin Kim, Massachusetts General Hospital James Homer Wright Pathology Laboratories Boston MA USADavid N. Louis, Massachusetts General Hospital James Homer Wright Pathology Laboratories Boston MA USACatherine L. Nutt, Massachusetts General Hospital James Homer Wright Pathology Laboratories Boston MA USA Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Sou... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3136255 Thu, 31 Dec 2009 16:44:59 +0100 3136255 http://www.medworm.com/index.php?rid=3134628&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fxv54645021332887%2F Abstract  Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant neurodegenerative disease caused by a small polyglutamine (polyQ) expansion (control: 4–20Q; SCA6: 20–33Q) in the carboxyl(C)-terminal cytoplasmic domain of the α1A voltage-dependent calcium channel (Cav2.1). Although a 75–85-kDa Cav2.1 C-terminal fragment (CTF) is toxic in cultured cells, its existence in human brains and its role in SCA6 pathogenesis remains unknown. Here, we investigated whether the small polyQ expansion alters the expression pattern and intracellular distribution of Cav2.1 in human SCA6 brains. New antibodies against the Cav2.1 C-terminus were used in immunoblotting and immunohistochemistry. In the cerebella of six control individuals, the CTF was detected in sucrose- and SDS-s... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3134628 Wed, 30 Dec 2009 16:44:36 +0100 3134628 http://www.medworm.com/index.php?rid=3131665&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F61687334ln857695%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0626-9 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 119 Journal Issue Volume 119, Number 1 / January, 2010 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3131665 Tue, 29 Dec 2009 17:51:31 +0100 3131665 http://www.medworm.com/index.php?rid=3127872&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F32q6wh2v40642647%2F Abstract  Axonal degeneration is a common pathologic feature in peripheral neuropathy, neurodegenerative disease, and normal aging. Oxidative stress may be an important mechanism of axonal degeneration, but is underrepresented among current experimental models. To test the effects of loss of the antioxidant enzyme Cu,Zn-superoxide dismutase (SOD1) on axon survival, we cultured dorsal root ganglion (DRG) neurons from SOD1 knockout mice. Beginning as early as 48–72 h, we observed striking degeneration of Sod1−/− axons that was prevented by introduction of human SOD1 and was attenuated by antioxidant treatment. To test susceptibility to increased superoxide production, we exposed wild-type DRGs to the redox-cycling herbicides paraquat and diquat (DQ). Dose-dependent ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3127872 Mon, 28 Dec 2009 19:13:34 +0100 3127872 http://www.medworm.com/index.php?rid=3125596&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fb127375u02737706%2F Abstract  In most subjects with Parkinson’s disease and dementia with Lewy bodies, α-synuclein (αS) immunoreactive pathology is found not only in the brain but also in the autonomic nuclei of the spinal cord. However, neither has the temporal course of αS pathology in the spinal cord in relation to the brain progression been established, nor has the extent of αS pathology in the spinal cord been analyzed in population-based studies. Using immunohistochemistry, the frequency and distribution of αS pathology were assessed semiquantitatively in the brains and spinal cord nuclei of 304 subjects who were aged at least 85 in the population-based Vantaa 85+ study. αS pathology was common in the spinal cord; 102 (34%) subjects had classic αS pathology in the thoracic and/o... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3125596 Sat, 26 Dec 2009 16:47:10 +0100 3125596 http://www.medworm.com/index.php?rid=3121503&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F92227488p7743504%2F Abstract  The choroid plexus is an epithelial–endothelial vascular convolute within the ventricular system of the vertebrate brain. It consists of epithelial cells, fenestrated blood vessels, and the stroma, dependent on various physiological or pathological conditions, which may contain fibroblasts, mast cells, macrophages, granulocytes or other infiltrates, and a rich extracellular matrix. The choroid plexus is mainly involved in the production of cerebrospinal fluid (CSF) by using the free access to the blood compartment of the leaky vessels. In order to separate blood and CSF compartments, choroid plexus epithelial cells and tanycytes of circumventricular organs constitute the blood–CSF–brain barrier. As non-neuronal cells in the brain and derived from neuroectode... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3121503 Wed, 23 Dec 2009 22:20:52 +0100 3121503 http://www.medworm.com/index.php?rid=3109498&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fm60310077xx14520%2F Content Type Journal ArticleCategory EditorialDOI 10.1007/s00401-009-0628-7Authors Werner Paulus, University Hospital Münster Institute of Neuropathology Domagkstr. 19 48129 Muenster Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3109498 Sat, 19 Dec 2009 16:43:51 +0100 3109498 http://www.medworm.com/index.php?rid=3106060&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj2w1888u374qu5x5%2F Abstract  The literature was reviewed to summarize the current understanding of the role of ciliated ependymal cells in the mammalian brain. Previous reviews were summarized. Publications from the past 10 years highlight interactions between ependymal cells and the subventricular zone and the possible role of restricted ependymal populations in neurogenesis. Ependymal cells provide trophic support and possibly metabolic support for progenitor cells. Channel proteins such as aquaporins may be important for determining water fluxes at the ventricle wall. The junctional and anchoring proteins are now fairly well understood, as are proteins related to cilia function. Defects in ependymal adhesion and cilia function can cause hydrocephalus through several different mechanis... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3106060 Fri, 18 Dec 2009 16:37:44 +0100 3106060 http://www.medworm.com/index.php?rid=3106061&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fd4k7857578w4x3k3%2F Abstract  Peptidylarginine deiminases (PADs)-mediated post-translational citrullination processes play key roles in protein functions and structural stability through the conversion of arginine to citrulline in the presence of excessive calcium concentrations. In brain, PAD2 is abundantly expressed and can be involved in citrullination in disease. Recently, we have reported pathological characterization of PAD2 and citrullinated proteins in scrapie-infected mice, but the implication of protein citrullination in the pathophysiology in human prion disease is not clear. In the present study, we explored the molecular and biological involvement of PAD2 and the pathogenesis of citrullinated proteins in frontal cortex of patients with sporadic Creutzfeldt-Jakob disease (sCJD). We... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3106061 Wed, 16 Dec 2009 07:01:22 +0100 3106061 http://www.medworm.com/index.php?rid=3086929&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fb0026l22p2347125%2F Abstract  The past 20 years have seen a gain in knowledge on microglia biology and microglia functions in disease that exceeds the expectations formulated when the microglia “immune network” was introduced. More than 10,000 articles have been published during this time. Important new research avenues of clinical importance have opened up such as the role of microglia in pain and in brain tumors. New controversies have also emerged such as the question of whether microglia are active or reactive players in neurodegenerative disease conditions, or whether they may be victims themselves. Premature commercial interests may be responsible for some of the confusion that currently surrounds microglia in both the Alzheimer and Parkinson’s disease research fields. A critic... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3086929 Sun, 13 Dec 2009 06:41:38 +0100 3086929 http://www.medworm.com/index.php?rid=3086930&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fwug1351u4747k163%2F Content Type Journal ArticleCategory CommentaryDOI 10.1007/s00401-009-0623-zAuthors Jason L. Eriksen, University of Houston Pharmacological and Pharmaceutical Science 521A SR2 4800 Calhoun Houston TX 77204 USA Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3086930 Sat, 12 Dec 2009 14:16:29 +0100 3086930 http://www.medworm.com/index.php?rid=3086931&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fyt3g342215343602%2F This article reviews (1) astrocyte functions in healthy CNS, (2) mechanisms and functions of reactive astrogliosis and glial scar formation, and (3) ways in which reactive astrocytes may cause or contribute to specific CNS disorders and lesions. Content Type Journal ArticleCategory ReviewDOI 10.1007/s00401-009-0619-8Authors Michael V. Sofroniew, University of California Department of Neurobiology, David Geffen School of Medicine 10833 Le Conte Avenue Los Angeles CA 90095-1763 USAHarry V. Vinters, University of California Division of Neuropathology, Department of Pathology and Laboratory Medicine, David Geffen School of Medicine Los Angeles CA 90095-1763 USA Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3086931 Thu, 10 Dec 2009 15:16:30 +0100 3086931 http://www.medworm.com/index.php?rid=3064965&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fp67813625358h53v%2F Abstract  The blood brain barrier (BBB) is formed by capillary endothelial cells with inter-endothelial cell tight junctions and other cells such as pericytes and astrocytes present. Previous studies have shown a role for tight junction abnormalities in BBB leakage in multiple sclerosis (MS) brain. This marks a key stage in the development of inflammatory demyelination in MS. The aim of this study was to identify aberrantly expressed genes involved in BBB changes in MS lesions. A focused endothelial cell biology microarray, capable of detecting changes in expression of 113 endothelial cell-specific genes, was employed to analyse endothelial cell mRNA extracted from post-mortem control white matter, MS normal appearing white matter (NAWM), chronic active or inactive lesions ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3064965 Sat, 05 Dec 2009 08:58:36 +0100 3064965 http://www.medworm.com/index.php?rid=3064966&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F36h5252221137751%2F Abstract  Mutations in the fused in sarcoma gene (FUS) were recently found in patients with familial amyotrophic lateral sclerosis (ALS). The present study aimed to clarify unique features of familial ALS caused by FUS mutation in the Japanese population. We carried out clinical, neuropathological, and genetic studies on a large Japanese pedigree with familial ALS. In six successive generations of this family, 16 individuals of both sexes were affected by progressive muscle atrophy and weakness, indicating an autosomal dominant trait. Neurological examination of six patients revealed an age at onset of 48.2 ± 8.1 years in fourth generation patients, while it was 31 and 20 years in fifth and sixth generation patients, respectively. Motor paralysis progres... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3064966 Fri, 04 Dec 2009 21:14:46 +0100 3064966 http://www.medworm.com/index.php?rid=3064967&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fmg096042872vk3m6%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0620-2Authors Sun-Ah Choi, Mayo Clinic Department of Neurology 13400 E. Shea Blvd. Scottsdale AZ 85259 USAVirgilio Gerald Hilario Evidente, Mayo Clinic Department of Neurology 13400 E. Shea Blvd. Scottsdale AZ 85259 USAJohn N. Caviness, Mayo Clinic Department of Neurology 13400 E. Shea Blvd. Scottsdale AZ 85259 USAHolly A. Shill, Banner Sun Health Research Institute The Cleo Roberts Center for Clinical Research 10515 W. Santa Fe Drive Sun City AZ USAMarwan N. Sabbagh, Banner Sun Health Research Institute The Cleo Roberts Center for Clinical Research 10515 W. Santa Fe Drive Sun City AZ USADonald J. Connor, Banner Sun Health Research Institute The Cleo Roberts Center for Clinical Research 10515 W. Santa Fe Drive Su... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3064967 Thu, 03 Dec 2009 12:49:24 +0100 3064967 http://www.medworm.com/index.php?rid=3054488&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fpt5nm46335147998%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0617-xAuthors Yasuo Miki, Institute of Brain Science, Hirosaki University Graduate School of Medicine Department of Neuropathology 5 Zaifu-cho Hirosaki 036-8562 JapanMakoto Nishie, Aomori Prefectural Central Hospital Department of Neurology Aomori JapanMio Ichiba, Kagoshima University Graduate School of Medical and Dental Sciences Department of Psychiatry Kagoshima JapanMasayuki Nakamura, Kagoshima University Graduate School of Medical and Dental Sciences Department of Psychiatry Kagoshima JapanFumiaki Mori, Institute of Brain Science, Hirosaki University Graduate School of Medicine Department of Neuropathology 5 Zaifu-cho Hirosaki 036-8562 JapanMasaya Ogawa, Aomori Prefectural Central Hospital Department of Neur... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3054488 Tue, 01 Dec 2009 08:29:50 +0100 3054488 http://www.medworm.com/index.php?rid=3041803&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F2v0r6175823x1103%2F Abstract  Progranulin (proepithelin) is a pleiotropic growth-factor associated with inflammation and wound repair in peripheral tissues. It also has been implicated in the response to acute traumatic brain injury as well as to chronic neurodegenerative diseases. To determine whether changes in progranulin expression also accompany acute spinal cord injury, C57BL/6 mice were subjected to mid-thoracic (T9 level) contusion spinal cord injury and analyzed by immunohistochemical and biochemical methods. Whereas spinal cord sections prepared from non-injured laminectomy control animals contained low basal levels of progranulin immunoreactivity in gray matter, sections from injured animals contained intense immunoreactivity throughout the injury epicenter that peaked 7–14 d... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3041803 Sat, 28 Nov 2009 07:03:14 +0100 3041803 http://www.medworm.com/index.php?rid=3032235&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fw120460368412026%2F Abstract  Neuroimaging with iron-sensitive MR sequences [gradient echo T2* and susceptibility-weighted imaging (SWI)] identifies small signal voids that are suspected brain microbleeds. Though the clinical significance of these lesions remains uncertain, their distribution and prevalence correlates with cerebral amyloid angiopathy (CAA), hypertension, smoking, and cognitive deficits. Investigation of the pathologies that produce signal voids is necessary to properly interpret these imaging findings. We conducted a systematic correlation of SWI-identified hypointensities to tissue pathology in postmortem brains with Alzheimer’s disease (AD) and varying degrees of CAA. Autopsied brains from eight AD patients, six of which showed advanced CAA, were imaged at 3T; foci corresp... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3032235 Tue, 24 Nov 2009 07:09:42 +0100 3032235 http://www.medworm.com/index.php?rid=3020838&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F2768427152q62187%2F Abstract  Primary melanocytic neoplasms of the central nervous system (CNS) are uncommon neoplasms derived from melanocytes that normally can be found in the leptomeninges. They cover a spectrum of malignancy grades ranging from low-grade melanocytomas to lesions of intermediate malignancy and overtly malignant melanomas. Characteristic genetic alterations in this group of neoplasms have not yet been identified. Using direct sequencing, we investigated 19 primary melanocytic lesions of the CNS (12 melanocytomas, 3 intermediate-grade melanocytomas, and 4 melanomas) for hotspot oncogenic mutations commonly found in melanocytic tumors of the skin (BRAF, NRAS, and HRAS genes) and uvea (GNAQ gene). Somatic mutations in the GNAQ gene at codon 209, resulting in constitutive activat... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3020838 Sat, 21 Nov 2009 09:08:35 +0100 3020838 http://www.medworm.com/index.php?rid=3020837&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F2165p33872281671%2F Abstract  Gliomas are a diverse group of brain tumors of glial origin. Most are characterized by diffuse infiltrative growth in the surrounding brain. In combination with their refractive nature to chemotherapy this makes it almost impossible to cure patients using combinations of conventional therapeutic strategies. The drastically increased knowledge about the molecular underpinnings of gliomas during the last decade has elicited high expectations for a more rational and effective therapy for these tumors. Most studies on the molecular pathways involved in glioma biology thus far had a strong focus on growth factor receptor protein tyrosine kinase (PTK) and phosphatidylinositol phosphatase signaling pathways. Except for the tumor suppressor PTEN, much less attention has b... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3020837 Sat, 21 Nov 2009 09:08:35 +0100 3020837 http://www.medworm.com/index.php?rid=3016356&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F78h7320m62356680%2F Content Type Journal ArticleCategory Consensus PaperDOI 10.1007/s00401-009-0612-2Authors Ian R. A. Mackenzie, Vancouver General Hospital and University of British Columbia Department of Pathology and Laboratory Medicine 855 West 12th Ave. Vancouver BC V5Z 1M9 CanadaManuela Neumann, University Hospital of Zurich Institute of Neuropathology Zurich SwitzerlandEileen H. Bigio, Northwestern University Feinberg School of Medicine Department of Pathology Chicago IL USANigel J. Cairns, Washington University School of Medicine Department of Pathology and Immunology St. Louis MO USAIrina Alafuzoff, Uppsala University Department of Genetics and Pathology Uppsala SwedenJillian Kril, The University of Sydney Department of Pathology Sydney NSW AustraliaGabor G. Kovacs, Medical University of Vienna I... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3016356 Wed, 18 Nov 2009 19:15:14 +0100 3016356 http://www.medworm.com/index.php?rid=3016357&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fn32131322342w747%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0613-1Authors Kurt A. Jellinger, Institute of Clinical Neurobiology Kenyongasse 18 1070 Vienna Austria Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3016357 Wed, 18 Nov 2009 19:15:13 +0100 3016357 http://www.medworm.com/index.php?rid=2997598&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fmu1548571x700304%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0607-z Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 118 Journal Issue Volume 118, Number 6 / December, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2997598 Sat, 14 Nov 2009 20:41:20 +0100 2997598 http://www.medworm.com/index.php?rid=2990864&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F15n240hv621344lq%2F In this report, we describe the clinical, histopathological and pathological prion protein (PrPSc) characteristics of two Dutch patients carrying novel adjacent stop codon mutations in the C-terminal part of PRNP, resulting in either case in hereditary prion protein amyloidoses, but with strikingly different clinicopathological phenotypes. The patient with the shortest disease duration (27 months) carried a Y226X mutation and showed PrP-CAA without any neurofibrillary lesions, whereas the patient with the longest disease duration (72 months) had a Q227X mutation and showed an unusual Gerstmann-Sträussler-Scheinker disease phenotype with numerous cerebral multicentric amyloid plaques and severe neurofibrillary lesions without PrP-CAA. Western blot analysis in the patient with... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2990864 Thu, 12 Nov 2009 18:38:27 +0100 2990864 http://www.medworm.com/index.php?rid=2986983&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F157632563rh76653%2F Abstract  The NBN (NBS1) gene belongs to a group of double-strand break repair genes. Mutations in any of these genes cause genome instability syndromes and contribute to carcinogenesis. NBN gene mutations cause increased tumor risk in Nijmegen breakage syndrome (NBS) homozygotes as well as in NBN heterozygotes. NBS patients develop different types of malignancies; among solid tumors, medulloblastoma (MB), an embryonal tumor of the cerebellum, has been reported most frequently. The majority of medulloblastomas occur sporadically, some of them manifest within familial cancer syndromes. Several signaling pathways are known to be engaged in hereditary and sporadic MB. The aim of our study was to identify mutations in selected exons of the NBN gene and to determine the frequency ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2986983 Wed, 11 Nov 2009 19:20:45 +0100 2986983 http://www.medworm.com/index.php?rid=2961215&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fc82737387208541p%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0606-0 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 118 Journal Issue Volume 118, Number 5 / November, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2961215 Tue, 03 Nov 2009 03:32:30 +0100 2961215 http://www.medworm.com/index.php?rid=2947978&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fb217705k34825064%2F We report the clinical, neuropsychological, imaging, genetic, and neuropathological features of GS, a patient with sporadic corticobasal syndrome. Genetic testing revealed no mutations in the microtubule associated protein tau or progranulin (PGRN) genes, but GS proved homozygous for the T allele of the rs5848 PGRN variant. Autopsy showed ubiquitin and TDP-43 pathology most similar to a pattern previously associated with PGRN mutation carriers. These findings confirm that FTLD-TDP should be included in the pathological differential diagnosis for sporadic CBS. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00401-009-0605-1Authors Maria Carmela Tartaglia, University of California Memory and Aging Center 1207 350 Parnassus Ave., Ste 905 San Francisco CA 94143-1207 USAMan... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2947978 Thu, 29 Oct 2009 19:38:46 +0100 2947978 http://www.medworm.com/index.php?rid=2924302&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fl3867v7741554q41%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0603-3Authors Marianna Baybis, University of Pennsylvania Medical Center Department of Neurology, PENN Epilepsy Center 3 West Gates Bldg., 3400 Spruce St. Philadelphia PA 19104 USAEleonora Aronica, University of Amsterdam Department of (Neuro)Pathology, Academic Medical Center Amsterdam The NetherlandsKatherine L. Nathanson, University of Pennsylvania Medical Center Division of Medical Genetics Philadelphia PA USAPeter B. Crino, University of Pennsylvania Medical Center Department of Neurology, PENN Epilepsy Center 3 West Gates Bldg., 3400 Spruce St. Philadelphia PA 19104 USA Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2924302 Thu, 22 Oct 2009 18:56:04 +0100 2924302 http://www.medworm.com/index.php?rid=2921972&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fg2563m786h662731%2F Abstract  Oligodendrocytes are the myelinating cells of the central nervous system (CNS). They are the end product of a cell lineage which has to undergo a complex and precisely timed program of proliferation, migration, differentiation, and myelination to finally produce the insulating sheath of axons. Due to this complex differentiation program, and due to their unique metabolism/physiology, oligodendrocytes count among the most vulnerable cells of the CNS. In this review, we first describe the different steps eventually culminating in the formation of mature oligodendrocytes and myelin sheaths, as they were revealed by studies in rodents. We will then show differences and similarities of human oligodendrocyte development. Finally, we will lay out the different pathways l... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2921972 Wed, 21 Oct 2009 19:27:14 +0100 2921972 http://www.medworm.com/index.php?rid=2921973&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F8v7162420t051231%2F Content Type Journal ArticleCategory CommentaryDOI 10.1007/s00401-009-0600-6Authors Werner Paulus, University Hospital Münster Institute of Neuropathology Domagkstr. 19 48129 Münster Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2921973 Wed, 21 Oct 2009 19:27:13 +0100 2921973 http://www.medworm.com/index.php?rid=2918161&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fxv6311153422vq3u%2F Content Type Journal ArticleCategory CommentaryDOI 10.1007/s00401-009-0602-4Authors Stephan Frank, Institute of Pathology Department of Neuropathology Schönbeinstrasse 40 4031 Basel SwitzerlandMarkus Tolnay, Institute of Pathology Department of Neuropathology Schönbeinstrasse 40 4031 Basel Switzerland Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2918161 Wed, 21 Oct 2009 09:05:32 +0100 2918161 http://www.medworm.com/index.php?rid=2918160&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fx6617531uj5261g2%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0593-1Authors Ida Elisabeth Holm, Aarhus University Hospital Laboratory for Experimental Neuropathology, Danish Neuroscience Center Aarhus DenmarkAdrian M. Isaacs, UCL Institute of Neurology MRC Prion Unit London WC1N 3BG UKIan R. A. Mackenzie, University of British Columbia Department of Pathology Vancouver BC Canada Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2918160 Wed, 21 Oct 2009 09:05:32 +0100 2918160 http://www.medworm.com/index.php?rid=2918162&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fe76050u4t0676960%2F This study implicates novel brain findings in Haddad syndrome mimicking those in murine Phox2b null mutants. This case suggests that abnormalities occur in CCHS in a network of sites critical to chemoreception. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00401-009-0599-8Authors Nestor D. Tomycz, Children’s Hospital Boston and Harvard Medical School Department of Pathology Boston MA USARobin L. Haynes, Children’s Hospital Boston and Harvard Medical School Department of Pathology Boston MA USAEdith F. Schmidt, Sunrise Hospital and Medical Center Las Vegas NV USAKate Ackerson, Children’s Hospital Boston and Harvard Medical School Department of Pathology Boston MA USAHannah C. Kinney, Children’s Hospital Boston and Harvard Medical School Department of Pathology B... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2918162 Wed, 21 Oct 2009 09:05:31 +0100 2918162 http://www.medworm.com/index.php?rid=2896705&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fb13851u7244145m2%2F We examined seven BIBD brains of patients with average age at onset 46 (range 29–57) and average duration of disease 8 years (range 5–12). Three cases presented with the behavioural variant of fronto-temporal dementia (FTD-bv) and one with FTD-bv combined with severe dysarthria. All four developed motor neuron disease/ALS syndrome (MND/ALS) several years later. In the other three cases, presentation was predominantly with motor symptoms, construed as MND/ALS in two, and progressive supranuclear palsy (PSP) in one. Severity of cortical degeneration varied, but all cases shared severe nigrostriatal atrophy and lower motor neuron pathology. In spared areas of cortex, FUS antibodies showed intense labelling of neuronal nuclei and weak positivity of cytoplasm, whereas, in affecte... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2896705 Wed, 14 Oct 2009 16:59:52 +0100 2896705 http://www.medworm.com/index.php?rid=2896706&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fp1j7561686n27m76%2F Abstract  In the present study, we have correlated plasma TDP-43 levels, as measured by ELISA, with the presence of TDP-43 pathological changes in the brains of 28 patients with frontotemporal lobar degeneration (FTLD) (14 with FTLD-TDP and 14 with FTLD-tau) and 24 patients with pathologically confirmed AD (8 with, and 16 without, TDP-43 pathological changes). Western blotting revealed full-length TDP-43, including a phosphorylated form, and a phosphorylated C-terminal fragment, in all samples examined. Both ELISA and immunohistochemistry were performed using phospho-dependent and phospho-independent TDP-43 antibodies for detection of phosphorylated and total TDP-43, respectively. Over all 52 cases, plasma levels of TDP-43, and scores of brain TDP-43 pathology, determined u... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2896706 Tue, 13 Oct 2009 08:16:33 +0100 2896706 http://www.medworm.com/index.php?rid=2884624&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fu652234274p05297%2F Abstract  Dementia in Parkinson’s disease (PD/PDD) is a common complication with a prevalence of up to 50%, but the specific changes underlying the cognitive decline remain undefined. Neuronal degeneration resulting in the dysfunction of multiple subcortical neurochemical projection systems has been described along with Lewy body-type pathology in cortical and limbic regions. Advanced alpha-synuclein (αSyn) pathology is not necessarily sufficient for producing dementia and concomitant Alzheimer’s disease (AD) change has also been proposed as a possible substrate of PDD. A lack of consensus in the extant literature likely stems from clinical heterogeneity and variable reliability in clinical characterisation as well as other historical and methodological issues. The con... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2884624 Sat, 10 Oct 2009 07:03:01 +0100 2884624 http://www.medworm.com/index.php?rid=2864181&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Ft2h42m3676ht16x2%2F Abstract  Clinical and autopsy studies have consistently reported cardiac sympathetic dysfunction in the left ventricular wall in patients with Parkinson’s disease (PD). Whether the nerve fibers of the cardiac conduction system or the atrial walls are equally affected in this disease process has not yet been well documented. Therefore, the aim of this study was to investigate sympathetic nerves in the cardiac conduction system as well as in the walls of all four heart chambers in patients with PD, in incidental Lewy body disease (iLBD), and in controls. Heart tissue from five PD patients, two iLBD cases, and seven controls were investigated immunohistochemically using antibodies directed against tyrosine hydroxylase (TH) and α-synuclein (syn-1). A marked diminution of TH... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2864181 Sat, 03 Oct 2009 06:29:52 +0100 2864181 http://www.medworm.com/index.php?rid=2864182&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj7787055q0p721w6%2F Abstract   IDH1 R132H mutations occur in approximately 70% of astrocytomas and oligodendroglial tumors. We developed a mouse monoclonal antibody targeting the IDH1 R132H mutation. Here, we show the high specificity and sensitivity of this antibody on Western blots and tissue sections from formalin fixed paraffin embedded tumor specimens. This antibody is highly useful for tumor classification, in detecting single infiltrating tumor cells and for the characterization of the cellular role of mutant IDH1 protein. Content Type Journal ArticleCategory Short ReportDOI 10.1007/s00401-009-0595-zAuthors David Capper, Ruprecht-Karls-University Heidelberg Department of Neuropathology, Institute of Pathology Im Neuenheimer Feld 220/221 69120 Heidelberg GermanyHanswalter Zentgraf, Ge... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2864182 Thu, 01 Oct 2009 18:32:00 +0100 2864182 http://www.medworm.com/index.php?rid=2833431&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fe016042t69215q83%2F In this study, we have chosen to study using different technical approaches (Q-RT-PCR, in situ hybridization and immunohistochemistry) the expression of five molecules involved in extracellular matrix degradation (cathepsin B, MMP2, MMP9, uPA and PAI-1) in glioblastomas in order to determine their prognostic impact among grade IV gliomas. Pilocytic astrocytomas were used as controls. Q-RT-PCR showed that transcripts of uPA, PAI-1, cathepsin B and MMP9 were significantly more expressed in glioblastomas (n = 52), in comparison to pilocytic astrocytomas (n = 17) (P = 0.049, P < 0.0001, P = 0.03 and P < 0.0001, respectively). On both univariate and multivariate analyses, cathepsin B and PAI-1 were strong predictors of overall... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2833431 Wed, 23 Sep 2009 06:01:23 +0100 2833431 http://www.medworm.com/index.php?rid=2811040&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fg6g0818367548801%2F Abstract  Multiple sclerosis (MS) is a chronic, inflammatory, demyelinating disease that affects the central nervous system and represents the most common neurological disorder in young adults in the Western hemisphere. There are several well-characterized experimental animal models that allow studying potential mechanisms of MS pathology. While experimental allergic encephalomyelitis is one of the most frequently used models to investigate MS pathology and therapeutic interventions, the cuprizone model reflects a toxic experimental model. Cuprizone-induced demyelination in animals is accepted for studying MS-related lesions and is characterized by degeneration of oligodendrocytes rather than by a direct attack on the myelin sheet. The present article reviews recent data co... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2811040 Thu, 17 Sep 2009 23:23:25 +0100 2811040 http://www.medworm.com/index.php?rid=2811041&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fv737w420h4218221%2F Abstract  Stress affects microglial function and viability during adulthood and early postnatal life; however, it is unknown whether stress to the pregnant dam might alter offspring microglia. The effects of prenatal stress on microglial development and distribution in the postnatal brain were studied using Wistar rats. Prenatal stress consisting of 20 min of forced swimming occurred on embryonic days 10–20. On postnatal days 1 and 10, stressed and control pups were killed. Microglia were identified using Griffonia simplicifolia lectin and quantified in the whole encephalon. In addition, plasma corticosterone was measured in dams at embryonic day 20, and in pups on postnatal days 1 and 10. At postnatal day 1, there was an increase in number of ramified microglia in the... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2811041 Wed, 16 Sep 2009 18:48:55 +0100 2811041 http://www.medworm.com/index.php?rid=3098449&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fu710835743754744%2F Kurt Jellinger Prize 2010 for Outstanding Scientific Writing in Neuropathology Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0587-z Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 118 Journal Issue Volume 118, Number 4 / October, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=3098449 Thu, 03 Sep 2009 06:25:17 +0100 3098449 http://www.medworm.com/index.php?rid=2767569&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fu710835743754744%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0587-z Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 118 Journal Issue Volume 118, Number 4 / October, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2767569 Thu, 03 Sep 2009 06:25:17 +0100 2767569 http://www.medworm.com/index.php?rid=2767570&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj50712r01q75r3x2%2F This report is based on a systematic reexamination of DRG, DR and ventral roots (VR) in 19 genetically confirmed cases of FRDA by immunocytochemistry and single- and double-label immunofluorescence with antibodies to specific proteins of myelin, neurons and axons; S-100α as a marker of satellite and Schwann cells; laminin; and the iron-responsive proteins ferritin, mitochondrial ferritin, and ferroportin. Confocal images of axons and myelin allowed the quantitative analysis of fiber density and size, and the extent of DR and VR myelination. A novel technology, high-definition X-ray fluorescence (HDXRF) of polyethylene glycol-embedded fixed tissue, was used to “map” iron in DRG. Unfixed frozen tissue of DRG in three cases was available for the chemical assay of total iron. Proli... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2767570 Thu, 03 Sep 2009 06:25:02 +0100 2767570 http://www.medworm.com/index.php?rid=2767571&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fhm551x3444146857%2F Abstract  In differentiated axons almost all microtubules (MTs) uniformly point their plus ends towards the axonal tip. The uniform polar pattern provides the structural substrate for efficient organelle transport along axons. It is generally believed that the mass and pattern of MTs polar orientation remain unchanged in differentiated neurons. Here we examined long-term effects of the MTs stabilizing reagent paclitaxel (taxol) over MTs polar orientation and organelle transport in cultured Aplysia neurons. Unexpectedly, we found that rather than stabilizing the MTs, paclitaxel leads to their massive polar reconfiguration, accompanied by impaired organelle transport. Washout of paclitaxel does not lead to recovery of the polar orientation indicating that the new pattern is se... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2767571 Thu, 03 Sep 2009 06:25:01 +0100 2767571 http://www.medworm.com/index.php?rid=2749406&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj8074q6255k7715k%2F Abstract  Sporadic inclusion body myositis (sIBM) is the most common myopathy in people over the age of 50 years. While immune-mediated inflammatory myopathies are well documented in dogs, sIBM has not been described. An 11-year-old dog with chronic and progressive neuromuscular dysfunction was evaluated for evidence of sIBM using current pathologic, immunohistochemical and electron microscopic diagnostic criteria. Vacuoles and congophilic intracellular inclusions were identified in cryostat sections of multiple muscle biopsies and immunostained with antibodies against amyloid-β peptide, amyloid-β precursor protein, and proteosome 20S of the ubiquitin–proteosome system. Cellular infiltration and increased expression of MHC Class I antigen were observed. Cytoplasmic ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2749406 Sat, 29 Aug 2009 09:49:13 +0100 2749406 http://www.medworm.com/index.php?rid=2749407&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F21552482u6761291%2F Abstract  Six subtypes of sporadic Creutzfeldt–Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenotypic features and concurrent PrPSc types has been reported but with inconsistencies among studies in both results and their interpretation. The issue currently complicates diagnosis and classification of cases and also has implications for disease pathogenesis. To explore the issue in depth, we carried out a systematic regional study in a large series of 225 cases. PrPSc types 1 and 2 concurrence was detected in 35% of cases and was higher in MM than in... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2749407 Sat, 29 Aug 2009 09:49:12 +0100 2749407 http://www.medworm.com/index.php?rid=2718078&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fk06k081173m1245t%2F We report the immunopathological analysis of the brain and tumor of two patients who died of anti-NMDAR-associated encephalitis, and of the tumor of nine patients who recovered. Findings included prominent microgliosis and deposits of IgG with rare inflammatory infiltrates in the hippocampus, forebrain, basal ganglia, and spinal cord. Detection of cells expressing markers of cytotoxicity (TIA, granzyme B, perforin and Fas/Fas ligand) was extremely uncommon. All tumors showed NMDAR-expressing neurons and inflammatory infiltrates. All patients’ NMDAR antibodies were IgG1, IgG2, or IgG3. No complement deposits were observed in any of the central nervous system regions examined. Overall, these findings coupled with recently reported in vitro data showing that antibodies downregulate th... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2718078 Wed, 19 Aug 2009 08:28:33 +0100 2718078 http://www.medworm.com/index.php?rid=2718079&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F3mv52vg07085740w%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0584-2Authors Yakov Fellig, Hadassah-Hebrew University Medical Center Department of Pathology Kiryat Hadassah P.O.B. 12000 91120 Jerusalem IsraelAndre M. Oliveira, Mayo Clinic Department of Laboratory Medicine and Pathology 200 First Street SW Rochester MN 55905 USAEmil Margolin, Hadassah-Hebrew University Medical Center Department of Neurosurgery Kiryat Hadassah P.O.B. 12000 91120 Jerusalem IsraelJ. Moshe Gomori, Hadassah-Hebrew University Medical Center Department of Radiology Kiryat Hadassah P.O.B. 12000 91120 Jerusalem IsraelMichele R. Erickson-Johnson, Mayo Clinic Department of Laboratory Medicine and Pathology 200 First Street SW Rochester MN 55905 USAMargaret M. Chou, University of Pennsylvania Department ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2718079 Wed, 19 Aug 2009 08:28:32 +0100 2718079 http://www.medworm.com/index.php?rid=2718080&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj315873020t541h3%2F Abstract  Radiolabeled Pittsburgh compound B (PIB) is a benzothiazole imaging agent that usually binds with high affinity, specificity, and stoichiometry to cerebral β-amyloid (Aβ) in patients with Alzheimer’s disease. Among a cohort of ten AD subjects examined postmortem, we describe a case of idiopathic, end-stage Alzheimer’s disease with heavy Aβ deposition yet substantially diminished high-affinity binding of 3H-PIB to cortical homogenates and unfixed cryosections. Cortical tissue samples were analyzed by immunohistochemistry, electron microscopy, ELISA, immunoblotting, MALDI-TOF mass spectrometry, in vitro 3H-PIB binding and 3H-PIB autoradiography. The PIB-refractory subject met the histopathological criteria for AD. However, cortical tissue from this case contai... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2718080 Wed, 19 Aug 2009 08:28:30 +0100 2718080 http://www.medworm.com/index.php?rid=2697710&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F8v340550h3208107%2F Abstract  Neuronal intermediate filament inclusion disease (NIFID) is an uncommon neurodegenerative condition that typically presents as early-onset, sporadic frontotemporal dementia (FTD), associated with a pyramidal and/or extrapyramidal movement disorder. The neuropathology is characterized by frontotemporal lobar degeneration with neuronal inclusions that are immunoreactive for all class IV intermediate filaments (IF), light, medium and heavy neurofilament subunits and α-internexin. However, not all the inclusions in NIFID are IF-positive and the primary molecular defect remains uncertain. Mutations in the gene encoding the fused in sarcoma (FUS) protein have recently been identified as a cause of familial amyotrophic lateral sclerosis (ALS). Because of the recognized ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2697710 Sun, 09 Aug 2009 11:04:06 +0100 2697710 http://www.medworm.com/index.php?rid=2686403&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fdxm627h08m41j3v6%2F Abstract  Mounting evidence suggests that glutamate excitotoxicity induces both enzymatic cleavage and nuclear translocation of apoptosis-inducing factor (AIF), which is involved in apoptosis-like programed cell death characterized by nuclear condensation without appearance of apoptotic bodies. Given the lack of apoptotic bodies in motor neurons in the spinal cord of patients with amyotrophic lateral sclerosis (ALS), the aim of the present study was to determine the role for AIF in this disease. We investigated the expression of AIF in spinal cords obtained at autopsy from ten sporadic ALS patients and ten age-matched, control subjects, using morphological and quantitative techniques. Immunohistochemical analysis showed that AIF immunoreactivity was localized in the nucleus ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2686403 Sun, 09 Aug 2009 09:33:47 +0100 2686403 http://www.medworm.com/index.php?rid=2683239&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Frp7r726838741620%2F Content Type Journal ArticleCategory ErratumDOI 10.1007/s00401-009-0579-zAuthors Karen Wolburg-Buchholz, University of Tübingen Institute of Pathology Liebermeisterstraße 8 72076 Tübingen GermanyAndreas F. Mack, University of Tübingen Institute of Anatomy 72076 Tübingen GermanyEsther Steiner, University of Bern Theodor Kocher Institute 3012 Bern SwitzerlandFriederike Pfeiffer, University of Bern Theodor Kocher Institute 3012 Bern SwitzerlandBritta Engelhardt, University of Bern Theodor Kocher Institute 3012 Bern SwitzerlandHartwig Wolburg, University of Tübingen Institute of Pathology Liebermeisterstraße 8 72076 Tübingen Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2683239 Fri, 07 Aug 2009 11:15:01 +0100 2683239 http://www.medworm.com/index.php?rid=2665636&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F2wm5378800401p41%2F In conclusion, GRN mutation carriers have increased levels of mRNA transcript from the normal allele in brain, and proliferation of microglia likely increases progranulin levels in affected regions of the FTLD-TDP brain, and whether or not these findings underlie the accumulation of TDP-43 pathology in FTLD-TDP linked to GRN mutations remains to be determined. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00401-009-0576-2Authors Alice S. Chen-Plotkin, University of Pennsylvania School of Medicine Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research 3rd Floor Maloney, 3600 Spruce Street Philadelphia PA 19104 USAJiping Xiao, University of Pennsylvania School of Medicine Department of Pathology and Laboratory Medicine, Center ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2665636 Sun, 02 Aug 2009 06:26:00 +0100 2665636 http://www.medworm.com/index.php?rid=2661688&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fg1254416v6n64726%2F Abstract  The underlying basis of carpal tunnel syndrome (CTS) and the basis of its increased incidence in diabetes are unknown. We have quantified pathology in an uncompressed nerve (posterior interosseous nerve, PIN) in the forearm between diabetic and non-diabetic patients with CTS and control subjects. In an age- and gender-matched series, 26 diabetic patients with CTS and 20 non-diabetic patients with CTS underwent biopsy of the PIN at the time of surgical carpal tunnel release. Control subjects consisted of ten PIN biopsies taken postmortem and three biopsies taken at the time of wrist surgery. We found PIN myelinated nerve fibre density significantly reduced in diabetic (mean 5,373/mm2 [95% confidence interval, 4,835–5,911]) and non-diabetic (6,617/mm2 [5,697–7,53... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2661688 Thu, 30 Jul 2009 08:09:37 +0100 2661688 http://www.medworm.com/index.php?rid=2651917&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Ft5n872p13x74m315%2F Content Type Journal ArticleCategory AbstractsDOI 10.1007/s00401-009-0573-5 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 118 Journal Issue Volume 118, Number 3 / September, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2651917 Tue, 28 Jul 2009 06:07:26 +0100 2651917 http://www.medworm.com/index.php?rid=2651918&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F9k64468w3w881556%2F Abstract  Alzheimer’s disease (AD) is characterized by pathological lesions such as amyloid-β (Aβ) plaques and cerebral amyloid angiopathy. Both these lesions consist mainly of aggregated Aβ protein and this aggregation is affected by macromolecules such as heparan sulfate (HS) proteoglycans. Previous studies demonstrated that HS enhances fibrillogenesis of Aβ and that this enhancement is dependent on the degree of sulfation of HS. In addition, it has been reported that these sulfation epitopes do not occur randomly but have a defined tissue distribution. Until now, the distribution of sulfation epitopes of HS has not yet been studied in human brain. We investigated whether a specific HS epitope is associated with Aβ plaques by performing immunohistochemistry on occi... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2651918 Mon, 27 Jul 2009 21:49:04 +0100 2651918 http://www.medworm.com/index.php?rid=2618325&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fa3n2522x12706596%2F Abstract  Pathogenic mutations in the gene encoding TDP-43, TARDBP, have been reported in familial amyotrophic lateral sclerosis (FALS) and, more recently, in families with a heterogeneous clinical phenotype including both ALS and frontotemporal lobar degeneration (FTLD). In our previous study, sequencing analyses identified one variant in the 3′-untranslated region (3′-UTR) of the TARDBP gene in two affected members of one family with bvFTD and ALS and in one unrelated clinically assessed case of FALS. Since that study, brain tissue has become available and provides autopsy confirmation of FTLD-TDP in the proband and ALS in the brother of the bvFTD-ALS family and the neuropathology of those two cases is reported here. The 3′-UTR variant was not found in 982 control su... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2618325 Sat, 18 Jul 2009 08:24:26 +0100 2618325 http://www.medworm.com/index.php?rid=2618326&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F22561n523jk14076%2F Content Type Journal ArticleCategory EditorialDOI 10.1007/s00401-009-0575-3Authors Werner Paulus, University Hospital Münster Institute of Neuropathology Domagkstr. 19 48129 Münster Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2618326 Sat, 18 Jul 2009 08:24:22 +0100 2618326 http://www.medworm.com/index.php?rid=2604153&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fk358572488768461%2F Abstract  The ubiquitin proteasome system (UPS) orchestrates the turnover of innumerable cellular proteins. In the process of ubiquitination the small protein ubiquitin is attached to a target protein by a peptide bond. The ubiquitinated target protein is subsequently shuttled to a protease complex known as the 26S proteasome and subjected to degradative proteolysis. The UPS facilitates the turnover of proteins in several settings. It targets oxidized, mutant or misfolded proteins for general proteolytic destruction, and allows for the tightly controlled and specific destruction of proteins involved in development and differentiation, cell cycle progression, circadian rhythms, apoptosis, and other biological processes. In neuropathology, alteration of the UPS, or mutations ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2604153 Tue, 14 Jul 2009 09:56:41 +0100 2604153 http://www.medworm.com/index.php?rid=2604152&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fu345070r225340k6%2F Abstract  Choroid plexus homogenates from 27 cases with Alzheimer disease-related pathology (AD), stages I/0 (n = 5), III–IV/0-B (n = 15) and V–VI/B-C (n = 7) and 3 age-matched controls (no clinical symptoms, no neuropathological lesions) were processed for gel electrophoresis and western blotting for oxidation markers carboxymethyl-lysine (CML) and N-carboxyethyl-lysine (CEL). Increased CEL and CML expression was seen in AD cases stages IVB, and V–VI/B-C when compared to controls and cases with AD-related pathology classified as I/0 and III/0. Variable stress damage was seen in stage III/B. Although lower stages of AD did not show β-amyloid deposition in the choroid plexus, the amount of β-amyloid was very variable at stages V/VI as reve... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2604152 Tue, 14 Jul 2009 09:56:41 +0100 2604152 http://www.medworm.com/index.php?rid=2604151&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F4w3734380106p6jv%2F Abstract  Camelids produce antibodies made of homodimeric heavy chains, and the antigen-binding region being composed of a single domain called VHH. These VHHs are much smaller than complete IgG. They are also more thermostable and more soluble in water; they should, therefore, diffuse more readily in the tissues. VHHs, expressed in bacteria, are easier to produce than conventional monoclonal antibodies. Because of these special characteristics, these antibody fragments could have interesting developments in immunohistochemistry and in the development of biomarkers. To test the possibility of their use in immunohistochemistry (IHC), we selected the glial fibrillary acidic protein (GFAP), a well-known marker of astrocytes. One alpaca (Lama pacos) was immunized against GFAP. L... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2604151 Tue, 14 Jul 2009 09:56:41 +0100 2604151 http://www.medworm.com/index.php?rid=2592442&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fe88126131x22430q%2F Abstract  Inflammatory myopathy with abundant macrophages (IMAM) and dermatomyositis (DM) are considered to represent related disorders, since they share inflammatory infiltrates and skin alterations. In order to get more insight into these disorders, we addressed the cellular composition of the inflammatory infiltrates in muscle biopsies of 11 patients with IMAM and DM. In IMAM, inflammatory infiltrates predominantly consisted of CD68+ MRP14+ macrophages which weakly expressed TNF-α, a few CD3+ T cells with a prominent IL-10 expression, and single CD123+ plasmacytoid dendritic cells. In DM, infiltrates were mainly composed of CD3+ CD4+ T cells which expressed IL-10, numerous CD123+ plasmacytoid dendritic cells, and CD20+ B cells. The low number of CD68+ macrophages was of... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2592442 Fri, 10 Jul 2009 08:10:13 +0100 2592442 http://www.medworm.com/index.php?rid=2592443&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F6165g6n20j884368%2F Abstract  Autism is a behaviorally defined disorder with deficits in social interaction, communication, atypical behaviors, and restricted areas of interest. Postmortem studies of the brain in autism have shown a broad spectrum of abnormalities in the cerebellum and neocortex, involving limbic regions such as anterior cingulate cortex (ACC, Brodmann’s area 24). Using stereological techniques, we analyzed quantitatively cytoarchitectonic subdomains of the ACC (areas 24a, b, c) with regard to cell packing density and cell size. Microscopic examination of the ACC was also done to identify any neuropathologies. Results showed a significant decrease in cell size in layers I–III and layers V–VI of area 24b and in cell packing density in layers V–VI of area 24c. Direct com... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2592443 Fri, 10 Jul 2009 08:10:12 +0100 2592443 http://www.medworm.com/index.php?rid=2592445&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F574q805004216823%2F Abstract  Duchenne muscular dystrophy (DMD) is characterized by variable alterations of the dystrophin gene and by muscle weakness and cognitive impairment. We postulated an association between cognitive impairment and architectural changes of the hippocampal GABAergic system. We investigated a major subpopulation of GABAergic neurons, the parvalbumin-immunopositive (PV-I) cells, in the dorsal hippocampus of the mdx mouse, an acknowledged model of DMD. PV-I neurons were quantified and their distribution was compared in CA1, CA2, CA3, and dentate gyrus in wild-type and mdx mice. The cell morphology and topography of PV-I neurons were maintained. Conversely, the number of PV-I neurons was significantly increased in the mdx mouse. The percent increase of PV-I neurons was from ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2592445 Thu, 09 Jul 2009 12:39:34 +0100 2592445 http://www.medworm.com/index.php?rid=2592444&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fd735h4p570735368%2F Abstract  A rosette-forming glioneuronal tumor (RGNT) was encountered in a 16-year-old Chinese girl. She experienced seizures with loss of consciousness for 1 month prior to diagnosis. A brain MRI revealed multifocal masses occupying all of the ventricular system associated with marked hydrocephalus. A biopsy was performed on the right lateral ventricle using a neuroendoscope and the patient was given postoperative radiotherapy. She was followed for 7 months, and there was no radiological or clinical evidence of tumor progression. Histological examination demonstrated two regions characterized by predominant neurocytic rosettes and scant low grade astrocytoma. No histological anaplasia was present. Immunohistological studies showed that the small round tumor cells... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2592444 Thu, 09 Jul 2009 12:39:34 +0100 2592444 http://www.medworm.com/index.php?rid=2590085&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Frn0362186206v376%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0553-9 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 118 Journal Issue Volume 118, Number 2 / August, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2590085 Tue, 07 Jul 2009 15:33:02 +0100 2590085 http://www.medworm.com/index.php?rid=2558234&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F1745268kt514p0m0%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0565-5Authors Sabrina Rossi, Regional Hospital Departments of Pathology, Neuroradiology, Pediatrics and Neurosurgery Treviso ItalyFausto J. Rodriguez, Mayo Clinic College of Medicine Department of Laboratory Medicine and Pathology Rochester MN 55905 USARenan A. Mota, Mayo Clinic College of Medicine Department of Laboratory Medicine and Pathology Rochester MN 55905 USAAngelo P. Dei Tos, Regional Hospital Departments of Pathology, Neuroradiology, Pediatrics and Neurosurgery Treviso ItalyFrancesco Di Paola, Regional Hospital Departments of Pathology, Neuroradiology, Pediatrics and Neurosurgery Treviso ItalyMatteo Bendini, Regional Hospital Departments of Pathology, Neuroradiology, Pediatrics and Neurosurgery Treviso... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2558234 Mon, 29 Jun 2009 10:07:03 +0100 2558234 http://www.medworm.com/index.php?rid=2558235&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fgm22g1756r852908%2F We describe the neuropathologic findings in a series of eight male PMD subjects with confirmed PLP1 mutations, including duplications, complete gene deletion, missense and exon-skipping. While PLP1 mutations have effects on oligodendrocytes that result in mutation-specific degrees of dysmyelination, our findings indicate that there are also unexpected effects in the central nervous system resulting in neuronal loss. Although length-dependent axonal degeneration has been described in PLP1 null mutations, there have been no reports on neuronal degeneration in PMD patients. We now demonstrate widespread neuronal loss in PMD. The patterns of neuronal loss appear to be dependent on the mutation type, suggesting selective vulnerability of neuronal populations that depends on the nature of t... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2558235 Mon, 29 Jun 2009 10:06:57 +0100 2558235 http://www.medworm.com/index.php?rid=2558236&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F004k130t15154162%2F Content Type Journal ArticleCategory EditorialDOI 10.1007/s00401-009-0566-4Authors Werner Paulus, University Hospital Münster Institute of Neuropathology Domagkstr. 19 48129 Munster Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2558236 Mon, 29 Jun 2009 10:06:50 +0100 2558236 http://www.medworm.com/index.php?rid=2549695&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fb8u7651125848hj1%2F Abstract  p62, also known as sequestosome1, is a shuttle protein transporting polyubiquitinated proteins for both the proteasomal and lysosomal degradation. p62 is an integral component of inclusions in brains of various neurodegenerative disorders, including Alzheimer disease (AD) neurofibrillary tangles (NFTs) and Lewy bodies in Parkinson disease. In AD brain, the p62 localized in NFTs is associated with phosphorylated tau (p-tau). Sporadic inclusion-body myositis (s-IBM) is the most common progressive muscle disease associated with aging, and its muscle tissue has several phenotypic similarities to AD brain. Abnormal accumulation of intracellular multiprotein inclusions, containing p-tau in the form of paired helical filaments, amyloid-β, and several other “Alzheimer-... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2549695 Fri, 26 Jun 2009 07:09:58 +0100 2549695 http://www.medworm.com/index.php?rid=2549696&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F9ht5377q212522w5%2F We report on an inverse association of IDH1 and IDH2 mutations in these gliomas and a non-random distribution of the mutation types within the tumor entities. IDH1 mutations of the R132C type are strongly associated with astrocytoma, while IDH2 mutations predominantly occur in oligodendroglial tumors. In addition, patients with anaplastic glioma harboring IDH1 mutations were on average 6 years younger than those without these alterations. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00401-009-0561-9Authors Christian Hartmann, Ruprecht-Karls-Universität Heidelberg Department of Neuropathology, Institute of Pathology Im Neuenheimer Feld 220/221 69120 Heidelberg GermanyJochen Meyer, German Cancer Research Center Clinical Cooperation Unit Neuropathology G380 69120 Heid... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2549696 Thu, 25 Jun 2009 06:52:39 +0100 2549696 http://www.medworm.com/index.php?rid=2492155&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fc027457238pk7611%2F Abstract  It is well established that only a fraction of Aβ peptides in the brain of Alzheimer’s disease (AD) patients start with N-terminal aspartate (Aβ1D) which is generated by proteolytic processing of amyloid precursor protein (APP) by BACE. N-terminally truncated and pyroglutamate modified Aβ starting at position 3 and ending with amino acid 42 [Aβ3(pE)–42] have been previously shown to represent a major species in the brain of AD patients. When compared with Aβ1–42, this peptide has stronger aggregation propensity and increased toxicity in vitro. Although it is unknown which peptidases remove the first two N-terminal amino acids, the cyclization of Aβ at N-terminal glutamate can be catalyzed in vitro. Here, we show that Aβ3(pE)–42 induces neurodegenerati... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2492155 Tue, 23 Jun 2009 06:02:38 +0100 2492155 http://www.medworm.com/index.php?rid=2492156&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F02894l3033173g3v%2F We examined a series of 120 astrocytomas including 70 pilocytic astrocytomas WHO grade I and 50 diffuse astrocytomas WHO grade II for both, BRAF–KIAA1549 fusion with a newly developed FISH assay and mutations in IDH1 and IDH2 by direct sequencing. Pilocytic astrocytomas contained the BRAF fusion in 49 cases (70%) but neither IDH1 nor IDH2 mutations. Astrocytomas WHO grade II exhibited IDH1 mutations in 38 cases (76%) but neither IDH2 mutations nor BRAF fusions. Thus, combined molecular analysis of BRAF and IDH1 is a sensitive and highly specific approach to separate pilocytic astrocytoma from diffuse astrocytoma. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00401-009-0550-zAuthors Andrey Korshunov, Ruprecht-Karls-Universität Heidelberg Department of Neuropatholo... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2492156 Fri, 19 Jun 2009 06:41:52 +0100 2492156 http://www.medworm.com/index.php?rid=2492157&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fp330723u7w6353h2%2F Abstract  In multiple sclerosis (MS), and its animal model experimental autoimmune encephalomyelitis (EAE), dysfunction of the blood–brain barrier (BBB) leads to edema formation within the central nervous system. The molecular mechanisms of edema formation in EAE/MS are poorly understood. We hypothesized that edema formation is due to imbalanced water transport across the BBB caused by a disturbed crosstalk between BBB endothelium and astrocytes. Here, we demonstrate at the light microscopic and ultrastructural level, the loss of polarized localization of the water channel protein aquaporin-4 (AQP4) in astrocytic endfeet surrounding microvessels during EAE. AQP4 was found to be redistributed over the entire astrocytic cell surface and lost its arrangement in orthogonal ar... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2492157 Wed, 17 Jun 2009 07:05:26 +0100 2492157 http://www.medworm.com/index.php?rid=2492158&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fc2u5337007615r43%2F In conclusion, the confirmation of the same pathways of cell death than previously described in experimental animal models encourages neuroprotective treatments acting on these mediators also in human stroke. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00401-009-0559-3Authors Tiina Sairanen, Helsinki University Central Hospital (HUCH) Department of Neurology P.O. Box 340 00029 Helsinki FinlandRita Szepesi, Biomedicum Helsinki Molecular Neurology Program P.O. Box 700 00029 Helsinki FinlandMarja-Liisa Karjalainen-Lindsberg, University of Helsinki Department of Pathology Haartmaninkatu 3 00029 Helsinki FinlandJani Saksi, Biomedicum Helsinki Molecular Neurology Program P.O. Box 700 00029 Helsinki FinlandAnders Paetau, University of Helsinki Department of Pathology Haa... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2492158 Tue, 16 Jun 2009 05:50:06 +0100 2492158 http://www.medworm.com/index.php?rid=2480505&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fl76554m771000674%2F Abstract  Pathological transactivation-responsive DNA-binding protein 43 (TDP-43) has been identified as a component of ubiquitinated inclusions in frontotemporal lobar degeneration with motor neuron disease, as well as in sporadic and some forms of familial amyotrophic lateral sclerosis. To clarify whether pathological TDP-43 is present in other neurodegenerative diseases involving the motor neuron system, we immunohistochemically examined the brain and spinal cord affected by two CAG repeat (polyglutamine) diseases, Machado–Joseph disease (MJD) and spinal and bulbar muscular atrophy (SBMA), using polyclonal antibody against TDP-43. In all the MJD cases, TDP-43-immunoreactive (ir) neuronal cytoplasmic inclusions (NCIs), although few in number, were found only in the lowe... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2480505 Sat, 13 Jun 2009 08:38:16 +0100 2480505 http://www.medworm.com/index.php?rid=2480506&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fx501342084x3v654%2F Content Type Journal ArticleCategory ErratumDOI 10.1007/s00401-009-0555-7Authors Tamas Revesz, University College London Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology Queen Square London WC1N 3BG UKJanice L. Holton, University College London Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology Queen Square London WC1N 3BG UKTammaryn Lashley, University College London Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology Queen Square London WC1N 3BG UKGordon Plant, UCLH NHS Foundation Trust The National Hospital for Neurology and Neurosurgery Queen Square London UKBlas Frangione, New York Uni... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2480506 Wed, 10 Jun 2009 10:02:55 +0100 2480506 http://www.medworm.com/index.php?rid=2480507&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F617u3408j6230377%2F Abstract  The role of microglial cells in the pathogenesis of Alzheimer’s disease (AD) neurodegeneration is unknown. Although several works suggest that chronic neuroinflammation caused by activated microglia contributes to neurofibrillary degeneration, anti-inflammatory drugs do not prevent or reverse neuronal tau pathology. This raises the question if indeed microglial activation occurs in the human brain at sites of neurofibrillary degeneration. In view of the recent work demonstrating presence of dystrophic (senescent) microglia in aged human brain, the purpose of this study was to investigate microglial cells in situ and at high resolution in the immediate vicinity of tau-positive structures in order to determine conclusively whether degenerating neuronal structures ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2480507 Wed, 10 Jun 2009 10:02:54 +0100 2480507 http://www.medworm.com/index.php?rid=2465911&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F5515413627841445%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0554-8Authors Wilson F. Abdo, Radboud University Nijmegen Medical Centre Institute of Neurology, Neuromuscular Centre Nijmegen P.O. Box 9101 6500 HB Nijmegen The NetherlandsTom van Mierlo, Radboud University Nijmegen Medical Centre Institute of Neurology, Neuromuscular Centre Nijmegen P.O. Box 9101 6500 HB Nijmegen The NetherlandsGerald J. Hengstman, Catherina Hospital Eindhoven, and Maxima Medical Centre Department of Neurology Eindhoven The NetherlandsH. Jurgen Schelhaas, Radboud University Nijmegen Medical Centre Institute of Neurology, Neuromuscular Centre Nijmegen P.O. Box 9101 6500 HB Nijmegen The NetherlandsBaziel G. van Engelen, Radboud University Nijmegen Medical Centre Institute of Neurology, Neuromuscu... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2465911 Sat, 06 Jun 2009 10:02:24 +0100 2465911 http://www.medworm.com/index.php?rid=2460837&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fp787113341wgxm72%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0551-yAuthors Jorge A. Bevilacqua, HCUCH Departamento de Neurología y Neurocirugía Santiago ChileMichel Fardeau, Groupe Hospitalier Pitié-Salpêtrière Association Institut de Myologie (AIM), Unité de Morphologie Neuromusculaire 75013 Paris FranceNorma Beatriz Romero, Groupe Hospitalier Pitié-Salpêtrière Association Institut de Myologie (AIM), Unité de Morphologie Neuromusculaire 75013 Paris France Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2460837 Tue, 02 Jun 2009 07:52:26 +0100 2460837 http://www.medworm.com/index.php?rid=2434736&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fv3237w7814627665%2F Abstract  Damage following ischemia and reperfusion (I/R) is common in the intestine and can be caused during abdominal surgery, in several disease states and following intestinal transplantation. Most studies have concentrated on damage to the mucosa, although published evidence also points to effects on neurons. Moreover, alterations of neuronally controlled functions of the intestine persist after I/R. The present study was designed to investigate the time course of damage to neurons and the selectivity of the effect of I/R damage for specific types of enteric neurons. A branch of the superior mesenteric artery supplying the distal ileum of anesthetised guinea pigs was occluded for 1 h and the animals were allowed to recover for 2 h to 4 weeks before tissu... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2434736 Sun, 24 May 2009 06:09:09 +0100 2434736 http://www.medworm.com/index.php?rid=2434735&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F5372462520h8ljw7%2F This study was aimed to characterise a Leigh-like subacute necrotising encephalopathy (SNE) in Yorkshire Terriers and to shed light on its enzymatic and genetic background. The possible resemblance to SNE in Alaskan Huskies and to human Leigh syndrome (LS) was another focus of interest. Eleven terriers with imaging and/or gross evidence of V-shaped, non-contiguous, cyst-like cavitations in the striatum, thalamus and brain stem were included. Neuropathological examinations focussed on muscle, brain pathology and mitochondrial ultrastructure. Further investigations encompassed respiratory-chain activities and the mitochondrial DNA. In contrast to mild non-specific muscle findings, brain pathology featured the stereotypic triad of necrotising grey matter lesions with relative preservati... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2434735 Sun, 24 May 2009 06:09:09 +0100 2434735 http://www.medworm.com/index.php?rid=2430004&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F53u57804636l8725%2F Abstract  Frontotemporal lobar degeneration (FTLD) can be classified as tau-positive (FTLD-tau) and tau-negative FTLD. The most common form of tau-negative FTLD is associated with neuronal inclusions that are composed of TAR DNA-binding protein 43 (TDP-43) (FTLD-TDP). Recent evidence suggests that FTLD-TDP can be further subdivided into at least three major histologic variants based on patterns of TDP-43 immunoreactive neuronal cytoplasmic inclusions (NCI) and dystrophic neurites (DN) in neocortex and hippocampus. The aim of this study was to extend the histologic analysis to other brain regions and to determine if there were distinct clinical and pathologic characteristics of the FTLD-TDP subtypes. Thirty-nine FTLD-TDP cases were analyzed (Mackenzie type 1 n = 24... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2430004 Wed, 20 May 2009 06:20:54 +0100 2430004 http://www.medworm.com/index.php?rid=2419808&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F67312708775403q3%2F We report the post-mortem neuropathologic findings of a patient with Wolfram syndrome and correlate them with his clinical presentation. In the hypothalamus, neurons in the paraventricular and supraoptic nuclei were markedly decreased and minimal neurohypophyseal tissue remained in the pituitary. The pontine base and inferior olivary nucleus showed gross shrinkage and neuron loss, while the cerebellum was relatively unaffected. The visual system had moderate to marked loss of retinal ganglion neurons, commensurate loss of myelinated axons in the optic nerve, chiasm and tract, and neuron loss in the lateral geniculate nucleus but preservation of the primary visual cortex. The patient’s inner ear showed loss of the organ of Corti in the basal turn of the cochleae and mild focal atro... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2419808 Sat, 16 May 2009 05:52:36 +0100 2419808 http://www.medworm.com/index.php?rid=2419807&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fe16624642078l0p3%2F Abstract  A K17I mutation in the ANG gene encoding angiogenin has been identified in a case that we previously published as ALS with neuronal intranuclear protein inclusions (Seilhean et al. in Acta Neuropathol 108:81–87, 2004). These inclusions were immunoreactive for smooth muscle α-actin but not for angiogenin. Moreover, they were not labeled by anti-TDP-43 antibodies, while numerous cytoplasmic inclusions immunoreactive for ubiquitin, p62 and TDP-43 were detected in both oligodendrocytes and neurons in various regions of the central nervous system. In addition, expression of smooth muscle α-actin was increased in the liver where severe steatosis was observed. This is the first neuropathological description of a case with an ANG mutation. Angiogenin is known to intera... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2419807 Sat, 16 May 2009 05:52:36 +0100 2419807 http://www.medworm.com/index.php?rid=2400831&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fr10874gxt3r675p1%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0543-yAuthors Rebecca Senetta, University of Turin Department of Biomedical Sciences and Human Oncology Via Santena 7 Turin ItalyElisa Trevisan, University of Turin Division of Neuro-Oncology Turin ItalyRoberta Rudà, University of Turin Division of Neuro-Oncology Turin ItalyFranco Benech, University of Turin Division of Neurosurgery Turin ItalyRiccardo Soffietti, University of Turin Division of Neuro-Oncology Turin ItalyPaola Cassoni, University of Turin Department of Biomedical Sciences and Human Oncology Via Santena 7 Turin Italy Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2400831 Wed, 06 May 2009 05:52:44 +0100 2400831 http://www.medworm.com/index.php?rid=2393624&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fv270wp08375v5767%2F Abstract  Autopsy findings of a patient, with sialidosis type I phenotype carrying V217M/G243R mutations in the lysosomal sialidase gene and biochemically defined isolated sialidase deficiency, who died of intractable lymphoma at the age of 32 years, are described. Perikaryal expansion of cytoplasm was evident, mostly in motor neurons (in the anterior horn and the brain stem), dorsal root ganglia, cerebellar dentate neurons and some neurons in the thalamus and nucleus basalis of Meynert. The stored material was lamellar in lysosomes and exhibited a specific affinity to wheat germ agglutinin at light and electron microscopy, which indicates the accumulation of terminal sialic acid at the non-reducing end of the sugar chain in this pathological structure. Neuronal loss i... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2393624 Tue, 05 May 2009 06:07:19 +0100 2393624 http://www.medworm.com/index.php?rid=2393625&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fcv1q80l216370621%2F This study supports an important role for iNOS-induced nitrosative stress in the reactive/inflammatory component of PVL. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00401-009-0540-1Authors Robin L. Haynes, Children’s Hospital Boston Departments of Pathology 300 Longwood Ave, Enders 1109 Boston MA 02115 USARebecca D. Folkerth, Children’s Hospital Boston Departments of Pathology 300 Longwood Ave, Enders 1109 Boston MA 02115 USAFelicia L. Trachtenberg, New England Research Institutes Watertown MA 02472 USAJoseph J. Volpe, Harvard Medical School Boston MA 02115 USAHannah C. Kinney, Children’s Hospital Boston Departments of Pathology 300 Longwood Ave, Enders 1109 Boston MA 02115 USA Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (So... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2393625 Tue, 05 May 2009 06:07:18 +0100 2393625 http://www.medworm.com/index.php?rid=2384460&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fr2274631v253g5n7%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0542-zAuthors J. Michael Schröder, Aachen University Hospital, RWTH Aachen Department of Neuropathology Pauwelsstr. 30 52074 Aachen Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2384460 Fri, 01 May 2009 06:42:28 +0100 2384460 http://www.medworm.com/index.php?rid=2384462&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj80467k136t37221%2F Abstract  Postencephalitic parkinsonism (PEP), a chronic complication of encephalitis lethargica, is a tauopathy characterized by multisystem neuronal loss and gliosis with widespread neurofibrillary lesions composed of both 3- and 4-repeat (3R and 4R) tau isoforms. Previous immunohistochemical studies in a small number of PEP cases demonstrated absence of Lewy bodies as well as the lack of other α-synuclein pathology, classifying PEP as a “pure” tauopathy. Neuropathologic examination of 10 brains with clinico-pathologically verified PEP confirmed widespread neurodegeneration in subcortical and brainstem areas associated with multifocal neurofibrillary pathology comprising both 3R and 4R tau. Very rare β-amyloid deposits were observed in two elderly patients, while Lew... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2384462 Thu, 30 Apr 2009 06:03:13 +0100 2384462 http://www.medworm.com/index.php?rid=2384461&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Ft5x428nw94j22054%2F Abstract  Brain edema continues to be a major cause of mortality after diverse types of brain pathologies such as major cerebral infarcts, hemorrhages, trauma, infections and tumors. The classification of edema into vasogenic, cytotoxic, hydrocephalic and osmotic has stood the test of time although it is recognized that in most clinical situations there is a combination of different types of edema during the course of the disease. Basic information about the types of edema is provided for better understanding of the expression pattern of some of the newer molecules implicated in the pathogenesis of brain edema. These molecules include the aquaporins, matrix metalloproteinases and growth factors such as vascular endothelial growth factors A and B and the angiopoietins. The p... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2384461 Thu, 30 Apr 2009 06:03:13 +0100 2384461 http://www.medworm.com/index.php?rid=2384463&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fh6596830h07t3439%2F Abstract  The two current major staging systems in use for Lewy body disorders fail to classify up to 50% of subjects. Both systems do not allow for large numbers of subjects who have Lewy-type α-synucleinopathy (LTS) confined to the olfactory bulb or who pass through a limbic-predominant pathway that at least initially bypasses the brainstem. The results of the current study, based on examination of a standard set of ten brain regions from 417 subjects stained immunohistochemically for α-synuclein, suggest a new staging system that, in this study, allows for the classification of all subjects with Lewy body disorders. The autopsied subjects included elderly subjects with Parkinson’s disease, dementia with Lewy bodies, incidental Lewy body disease and Alzheimer’s dise... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2384463 Tue, 28 Apr 2009 16:45:32 +0100 2384463 http://www.medworm.com/index.php?rid=2371582&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F635h2pq66532w77g%2F Abstract  Mild infection may trigger sudden death in the vulnerable infant by cytokine interactions with a compromised medullary serotonergic (5-HT) system, leading to disrupted cardiorespiratory regulation and sleep-related sudden death. The cytokine interleukin (IL)-6 is elevated in the cerebrospinal fluid in SIDS. We tested the hypothesis that the expression of IL-6 receptors (IL-6R) and/or gp130 (involved in IL-6R signaling) is altered in the medullary 5-HT system in SIDS. Immunohistochemistry of IL-6R and gp130 was performed on medullae from 25 SIDS infants, 20 infectious deaths, and 14 controls using a semi-quantitative grading system. In the SIDS cases, mean IL-6R intensity grade in the arcuate nucleus (major component of medullary 5-HT system) was significantly high... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2371582 Sun, 26 Apr 2009 05:46:14 +0100 2371582 http://www.medworm.com/index.php?rid=2367988&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fx532618235456893%2F Abstract  Synaptic loss is the major neurobiological substrate of cognitive dysfunction in Alzheimer’s disease (AD). Synaptic failure is an early event in the pathogenesis that is clearly detectable already in patients with mild cognitive impairment (MCI), a prodromal state of AD. It progresses during the course of AD and in most early stages involves mechanisms of compensation before reaching a stage of decompensated function. This dynamic process from an initially reversible functionally responsive stage of down-regulation of synaptic function to stages irreversibly associated with degeneration might be related to a disturbance of structural brain self-organization and involves morphoregulatory molecules such as the amyloid precursor protein. Further, recent evidence su... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2367988 Fri, 24 Apr 2009 06:54:29 +0100 2367988 http://www.medworm.com/index.php?rid=2366379&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F42g77m06645r2187%2F Abstract  Abeta accumulation has an important function in the etiology of Alzheimer’s disease (AD) with its typical clinical symptoms, like memory impairment and changes in personality. However, the mode of this toxic activity is still a matter of scientific debate. We used the APP/PS1KI mouse model for AD, because it is the only model so far which develops 50% hippocampal CA1 neuron loss at the age of 1 year. Previously, we have shown that this model develops severe learning deficits occurring much earlier at the age of 6 months. This observation prompted us to study the anatomical and cellular basis at this time point in more detail. In the current report, we observed that at 6 months of age there is already a 33% CA1 neuron loss and an 18% atrophy of the... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2366379 Thu, 23 Apr 2009 08:26:27 +0100 2366379 http://www.medworm.com/index.php?rid=2358290&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F63n490867677468p%2F Abstract  The lesions of Alzheimer disease include accumulation of proteins, losses of neurons and synapses, and alterations related to reactive processes. Extracellular Aβ accumulation occurs in the parenchyma as diffuse, focal or stellate deposits. It may involve the vessel walls of arteries, veins and capillaries. The cases in which the capillary vessel walls are affected have a higher probability of having one or two apoε 4 alleles. Parenchymal as well as vascular Aβ deposition follows a stepwise progression. Tau accumulation, probably the best histopathological correlate of the clinical symptoms, takes three aspects: in the cell body of the neuron as neurofibrillary tangle, in the dendrites as neuropil threads, and in the axons forming the senile plaque neuritic cor... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2358290 Tue, 21 Apr 2009 10:03:57 +0100 2358290 http://www.medworm.com/index.php?rid=2335214&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F5rv3705t52503g01%2F Content Type Journal ArticleCategory EditorialDOI 10.1007/s00401-009-0529-9Authors Kurt A. Jellinger, Institute of Clinical Neurobiology Kenyongasse 18 1070 Vienna Austria Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2335214 Fri, 10 Apr 2009 05:57:37 +0100 2335214 http://www.medworm.com/index.php?rid=2335213&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fd5231r31m4710n87%2F Abstract  Beta-amyloid (Aβ) is thought to be a key contributor to the pathogenesis of Alzheimer disease (AD) in the general population and in adults with Down syndrome (DS). Different assembly states of Aβ have been identified that may be neurotoxic. Aβ oligomers can assemble into soluble prefibrillar oligomers, soluble fibrillar oligomers and insoluble fibrils. Using a novel antibody, OC, recognizing fibrils and soluble fibrillar oligomers, we characterized fibrillar Aβ deposits in AD and DS cases. We further compared human specimens to those obtained from the Tg2576 mouse model of AD. Our results show that accumulation of fibrillar immunoreactivity is significantly increased in AD relative to nondemented aged subjects and those with select cognitive impairments (p&nbs... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2335213 Fri, 10 Apr 2009 05:57:37 +0100 2335213 http://www.medworm.com/index.php?rid=2335215&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fc856862532563491%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0534-zAuthors Elan D. Louis, Columbia University GH Sergievsky Center, College of Physicians and Surgeons New York NY USAPhyllis L. Faust, Columbia University Department of Pathology and Cell Biology, College of Physicians and Surgeons New York NY USAJean-Paul G. Vonsattel, Columbia University Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, College of Physicians and Surgeons New York NY USACordelia Erickson-Davis, Columbia University GH Sergievsky Center, College of Physicians and Surgeons New York NY USA Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2335215 Fri, 10 Apr 2009 05:57:34 +0100 2335215 http://www.medworm.com/index.php?rid=2335216&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fbnwv5v03gv434t1w%2F The objective of the present study was to investigate a possible role for HIF-1α in the activation of JCV. Glial cell cultures infected with JCV demonstrated a significant increase in the levels of HIF-1α, in where it is located to the nucleus. Immunohistochemical studies corroborated upregulation of HIF-1α in JCV infected oligodendrocytes and astrocytes in clinical samples of PML compared with normal glial cells from the same samples in which HIF-1α expression is weak. CAT assays performed in co-transfected glial cells demonstrated activation of the JCV early promoter in the presence of HIF-1α. This activation was potentiated in the presence of Smad3 and Smad4. Finally, chromatin immunoprecipitation assays demonstrated the binding of HIF-1α to the JCV control region. These res... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2335216 Fri, 10 Apr 2009 05:57:32 +0100 2335216 http://www.medworm.com/index.php?rid=2335218&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fj1312881w7520376%2F Abstract  The term gastrointestinal neuromuscular disease describes a clinically heterogeneous group of disorders of children and adults in which symptoms are presumed or proven to arise as a result of neuromuscular, including interstitial cell of Cajal, dysfunction. Such disorders commonly have impaired motor activity, i.e. slowed or obstructed transit with radiological evidence of transient or persistent visceral dilatation. Whilst sensorimotor abnormalities have been demonstrated by a variety of methods in these conditions, standards for histopathological reporting remain relatively neglected. Significant differences in methodologies and expertise continue to confound the reliable delineation of normality and specificity of particular pathological changes for disease. Su... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2335218 Fri, 10 Apr 2009 05:57:31 +0100 2335218 http://www.medworm.com/index.php?rid=2335217&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fjuh0563h129v7302%2F We report a 78-year-old woman who presented with depression, in whom postmortem examination revealed almost complete loss of neurons with gliosis in the subiculum and CA1-3 regions of the hippocampus and abundant neuronal cytoplasmic inclusions in the dentate gyrus. The inclusions were round, slightly basophilic and argyrophilic, resembling Pick bodies. However, they were Gallyas- and 4-repeat tau-positive, and 3-repeat tau- and ubiquitin-negative. To our knowledge, the histopathological features in this case were different from those in hippocampal sclerosis or 4-repeat tauopathies reported previously. It is likely that this case is a new variant of 4-repeat tauopathy presenting with hippocampal sclerosis. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00401-009-0531... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2335217 Fri, 10 Apr 2009 05:57:31 +0100 2335217 http://www.medworm.com/index.php?rid=2311881&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fw5h763q82368072w%2F Abstract  MicroRNAs (miRNAs) are short non-protein-coding RNAs that function as key regulators of diverse biological processes through negative control on gene expression at the post-transcriptional level. Emerging evidence indicates that miRNAs play an important role in the development of human cancers, with their deregulation resulting in altered activity of downstream tumor suppressors, oncogenes and other signaling molecules. Recent years have seen considerable progress in miRNA research in brain tumors, particularly in glioblastomas and medulloblastomas, providing novel insights into the pathogenesis of these malignant lesions. Expression profiling has unveiled miRNA signatures that not only distinguish brain tumors from normal tissues, but can also differentiate histo... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2311881 Fri, 03 Apr 2009 07:02:12 +0100 2311881 http://www.medworm.com/index.php?rid=2311883&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F5g30535t4pt1015j%2F Abstract  Mutations of the IDH1 gene are frequent in gliomas, with R132H (CGT → CAT) being the most common (>85%). In astrocytomas, IDH1 mutations are typically co-present with, or precede, TP53 mutations. We assessed IDH1 mutations in brain tumors diagnosed in patients from three families with Li-Fraumeni syndrome. We identified IDH1 mutations in five astrocytomas that developed in carriers of a TP53 germline mutation. Without exception, all were R132C (CGT → TGT), which in sporadic astrocytomas accounts for <5% of IDH1 mutations. This remarkably selective occurrence of R132C mutations may reflect differences in the sequence of genetic events, with a preference for R132C mutations in astrocytes or precursor cells that already carry a germline ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2311883 Thu, 02 Apr 2009 05:50:25 +0100 2311883 http://www.medworm.com/index.php?rid=2311885&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Flu28732138n8p727%2F Abstract  Ubiquitin immunoreactive (UBQ-ir) inclusions were present to variable extents in the inferior olivary nucleus (ION) in 37/48 (77%) patients with frontotemporal lobar degeneration (FTLD), in 10/11 (91%) patients with motor neurone disease (MND), in 5/5 (100%) patients with Alzheimer’s disease (AD), 5/7 (71%) patients with dementia with Lewy bodies, 13/19 (68%) patients with Parkinson’s disease, 11/11(100%) patients with Progressive Supranuclear Palsy, 2/6 (33%) patients with Multisystem Atrophy, 1/3 (33%) patients with Huntington’s disease and in 14/14 (100%) normal elderly control subjects. In FTLD, UBQ-ir inclusions were present in 26/32 (81%) patients with FTLD-U, in 10/15 (67%) patients with tauopathy, and in the single patient with Dementia Lacking Disti... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2311885 Sat, 28 Mar 2009 08:55:25 +0100 2311885 http://www.medworm.com/index.php?rid=2311887&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F4670860065508g56%2F In conclusion, here we report a protocol for assessing αS pathology that can achieve a high inter-observer agreement for both the assignment to brainstem, limbic, neocortical and amygdala-predominant categories of synucleinopathy and the Braak stages. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00401-009-0523-2Authors Irina Alafuzoff, Kuopio University Department of Neuroscience and Neurology Kuopio FinlandPaul G. Ince, University of Sheffield Department of Neurosciences Sheffield UKThomas Arzberger, Ludwig-Maximilians-University Centre for Neuropathology and Prion Research Munich GermanySafa Al-Sarraj, London Institute of Psychiatry Department of Clinical Neuropathology London UKJeanne Bell, University of Edinburgh, Western General Hospital Department of Pathol... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2311887 Sat, 28 Mar 2009 08:55:24 +0100 2311887 http://www.medworm.com/index.php?rid=2311889&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F97543u9863215522%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0524-1Authors Pushpa Raj Joshi, Neurologische Universitätsklinik Halle GermanyManuela Knape, Neurologische Universitätsklinik Halle GermanyStephan Zierz, Neurologische Universitätsklinik Halle GermanyMarcus Deschauer, Neurologische Universitätsklinik Halle Germany Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2311889 Thu, 26 Mar 2009 08:04:22 +0100 2311889 http://www.medworm.com/index.php?rid=2311891&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F7506983221t7702r%2F Abstract  Vascular dysfunction has a critical role in Alzheimer’s disease (AD). Recent data from brain imaging studies in humans and animal models suggest that cerebrovascular dysfunction may precede cognitive decline and onset of neurodegenerative changes in AD and AD models. Cerebral hypoperfusion and impaired amyloid β-peptide (Aβ) clearance across the blood–brain barrier (BBB) may contribute to the onset and progression of dementia AD type. Decreased cerebral blood flow (CBF) negatively affects the synthesis of proteins required for memory and learning, and may eventually lead to neuritic injury and neuronal death. Impaired clearance of Aβ from the brain by the cells of the neurovascular unit may lead to its accumulation on blood vessels and in brain parenchyma. ... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2311891 Wed, 25 Mar 2009 06:59:35 +0100 2311891 http://www.medworm.com/index.php?rid=2289731&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F9398183411852132%2F Abstract  Mutations in the amyloid precursor protein (APP), presenilin 1 (PSEN1) and presenilin 2 (PSEN2) genes cause autosomal dominant familial Alzheimer’s disease (AD). PSEN1 and PSEN2 are essential components of the γ-secretase complex, which cleaves APP to affect Aβ processing. Disruptions in Aβ processing have been hypothesised to be the major cause of AD (the amyloid cascade hypothesis). These genetic cases exhibit all the classic hallmark pathologies of AD including neuritic plaques, neurofibrillary tangles (NFT), tissue atrophy, neuronal loss and inflammation, often in significantly enhanced quantities. In particular, these cases have average greater hippocampal atrophy and NFT, more significant cortical Aβ42 plaque deposition and more substantial inflammatio... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2289731 Sun, 22 Mar 2009 06:11:34 +0100 2289731 http://www.medworm.com/index.php?rid=2279062&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2F033gp452g210nt6l%2F Content Type Journal ArticleCategory CorrespondenceDOI 10.1007/s00401-009-0520-5Authors Federica Cerri, San Raffaele Scientific Institute Department of Neurology and INSPE Via Olgettina 60 20132 Milan ItalyArmando Gavazzi, Santa Maria Hospital Department of Neurology Castellanza ItalyStefano C. Previtali, San Raffaele Scientific Institute Division of Neuroscience, Department of Neurology and INSPE Milan ItalyMassimo Franceschi, Santa Maria Hospital Department of Neurology Castellanza ItalyIgnazio D. Lopez, San Raffaele Scientific Institute Division of Neuroscience, Department of Neurology and INSPE Milan ItalyMarina Scarlato, San Raffaele Scientific Institute Division of Neuroscience, Department of Neurology and INSPE Milan ItalyPaola Podini, San Raffaele Scientific Institute Milan Ita... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2279062 Tue, 17 Mar 2009 07:04:46 +0100 2279062 http://www.medworm.com/index.php?rid=2279064&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fx42m12v125144248%2F Content Type Journal ArticleCategory AnnouncementsDOI 10.1007/s00401-009-0507-2 Journal Acta NeuropathologicaOnline ISSN 1432-0533Print ISSN 0001-6322 Journal Volume Volume 117 Journal Issue Volume 117, Number 4 / April, 2009 (Source: Acta Neuropathologica) Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2279064 Sat, 14 Mar 2009 13:15:59 +0100 2279064 http://www.medworm.com/index.php?rid=2279065&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fe2v823x335178p56%2F We report tau hyperphosphorylation occurring in multiple cell types, with biochemical analysis confirming restriction to the soluble fraction. The absence of sarcosyl-insoluble tau fraction in early disease and its presence in secondary progression raises the possibility that insoluble tau accumulates with disease progression. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00401-009-0515-2Authors Jane Marian Anderson, University of Cambridge Cambridge Centre for Brain Repair, Department of Clinical Neurosciences Forvie Site, Robinson Way Cambridge UKRickie Patani, University of Cambridge Cambridge Centre for Brain Repair, Department of Clinical Neurosciences Forvie Site, Robinson Way Cambridge UKRichard Reynolds, Imperial College Faculty of Medicine, Charing Cross Hosp... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2279065 Sat, 14 Mar 2009 13:15:50 +0100 2279065 http://www.medworm.com/index.php?rid=2279066&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fd37502755j76l407%2F Abstract  Oxidative stress has been implicated in the pathogenesis of a number of diseases including Alzheimer’s disease (AD). The oxidative stress hypothesis of AD pathogenesis, in part, is based on β-amyloid peptide (Aβ)-induced oxidative stress in both in vitro and in vivo studies. Oxidative modification of the protein may induce structural changes in a protein that might lead to its functional impairment. A number of oxidatively modified brain proteins were identified using redox proteomics in AD, mild cognitive impairment (MCI) and Aβ models of AD, which support a role of Aβ in the alteration of a number of biochemical and cellular processes such as energy metabolism, protein degradation, synaptic function, neuritic growth, neurotransmission, cellular defense sys... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2279066 Sat, 14 Mar 2009 13:15:44 +0100 2279066 http://www.medworm.com/index.php?rid=2265097&cid=s_33262_25_f&fid=33262&url=http%3A%2F%2Fwww.springerlink.com%2Fcontent%2Fa38k4h2067k07311%2F This article reviewed the current literature of these two issues. Content Type Journal ArticleCategory ReviewDOI 10.1007/s00401-009-0516-1Authors Yi Ping Liu, University of Wisconsin Department of Neurological Surgery K4/8 Mail code CSC 8660, 600 Highland Ave Madison WI 53792 USABradley T. Lang, University of Wisconsin Department of Neurological Surgery K4/8 Mail code CSC 8660, 600 Highland Ave Madison WI 53792 USAMustafa K. Baskaya, University of Wisconsin Department of Neurological Surgery K4/8 Mail code CSC 8660, 600 Highland Ave Madison WI 53792 USARobert J. Dempsey, University of Wisconsin Department of Neurological Surgery K4/8 Mail code CSC 8660, 600 Highland Ave Madison WI 53792 USARaghu Vemuganti, University of Wisconsin Department of Neurological Surgery K4/8 Mail code CSC 8... Acta Neuropathologica journals http://www.medworm.com/rss/comments.php?id=2265097 Fri, 13 Mar 2009 10:09:01 +0100 2265097