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Kurt Jellinger Prize 2010 for Outstanding Scientific Writing in NeuropathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - November 14, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNPEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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-Schei...
Source: Acta Neuropathologica - November 12, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Heterozygous germ-line mutations in the NBN gene predispose to medulloblastoma in pediatric patientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 pathway...
Source: Acta Neuropathologica - November 11, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Kurt Jellinger Prize 2010 for Outstanding Scientific Writing in NeuropathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - November 3, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Sporadic corticobasal syndrome due to FTLD-TDPEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 10.100...
Source: Acta Neuropathologica - October 29, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Deletion of 15q11.2–15q13.1 in isolated human hemimegalencephalyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - October 22, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Oligodendrocytes: biology and pathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 wer...
Source: Acta Neuropathologica - October 21, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

GFAP, Ki67 and IDH1: perhaps the golden triad of glioma immunohistochemistryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - October 21, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Frontotemporal lobar degeneration: toward the end of conFUSionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - October 21, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Absence of FUS-immunoreactive pathology in frontotemporal dementia linked to chromosome 3 (FTD-3) caused by mutation in the CHMP2B geneEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - October 21, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Novel neuropathologic findings in the Haddad syndromeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 ...
Source: Acta Neuropathologica - October 21, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

FUS pathology in basophilic inclusion body diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 ...
Source: Acta Neuropathologica - October 14, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Plasma phosphorylated-TDP-43 protein levels correlate with brain pathology in frontotemporal lobar degenerationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 a...
Source: Acta Neuropathologica - October 13, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

The morbid anatomy of dementia in Parkinson’s diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 ...
Source: Acta Neuropathologica - October 10, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Diminished tyrosine hydroxylase immunoreactivity in the cardiac conduction system and myocardium in Parkinson’s disease: an anatomical studyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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, an...
Source: Acta Neuropathologica - October 3, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Monoclonal antibody specific for IDH1 R132H mutationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 Cap...
Source: Acta Neuropathologica - October 1, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

High expression of cathepsin B and plasminogen activator inhibitor type-1 are strong predictors of survival in glioblastomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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....
Source: Acta Neuropathologica - September 23, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

The cuprizone animal model: new insights into an old storyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - September 17, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Prenatal stress alters microglial development and distribution in postnatal rat brainEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 corticos...
Source: Acta Neuropathologica - September 16, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Kurt Jellinger Prize 2010 for Outstanding Scientific Writing in NeuropathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - September 3, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

The dorsal root ganglion in Friedreich’s ataxiaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 polyethylen...
Source: Acta Neuropathologica - September 3, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Paclitaxel induces axonal microtubules polar reconfiguration and impaired organelle transport: implications for the pathogenesis of paclitaxel-induced polyneuropathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 reco...
Source: Acta Neuropathologica - September 3, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Vacuolar myopathy in a dog resembling human sporadic inclusion body myositisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 prec...
Source: Acta Neuropathologica - August 29, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classificationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 d...
Source: Acta Neuropathologica - August 29, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Evidence for antibody-mediated pathogenesis in anti-NMDAR encephalitis associated with ovarian teratomaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 obse...
Source: Acta Neuropathologica - August 19, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Extraosseous aneurysmal bone cyst of cerebello-pontine angle with USP6 rearrangementEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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. Ericks...
Source: Acta Neuropathologica - August 19, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Deficient high-affinity binding of Pittsburgh compound B in a case of Alzheimer’s diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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,...
Source: Acta Neuropathologica - August 19, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 t...
Source: Acta Neuropathologica - August 9, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Persistent cleavage and nuclear translocation of apoptosis-inducing factor in motor neurons in the spinal cord of sporadic amyotrophic lateral sclerosis patientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 ag...
Source: Acta Neuropathologica - August 9, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Loss of astrocyte polarity marks blood–brain barrier impairment during experimental autoimmune encephalomyelitisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - August 7, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Brain progranulin expression in GRN-associated frontotemporal lobar degenerationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 Floo...
Source: Acta Neuropathologica - August 2, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Reduced myelinated nerve fibre and endoneurial capillary densities in the forearm of diabetic and non-diabetic patients with carpal tunnel syndromeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - July 30, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

54. Annual Meeting of the German Society of Neuropathology and Neuroanatomy (DGNN)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - July 28, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Sulfation of heparan sulfate associated with amyloid-β plaques in patients with Alzheimer’s diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 epitope...
Source: Acta Neuropathologica - July 27, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

TARDBP 3′-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - July 18, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Announcing the winner of the 2009 Kurt Jellinger PrizeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - July 18, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

The ubiquitin proteasome system in neuropathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 develo...
Source: Acta Neuropathologica - July 14, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Oxidative stress damage and oxidative stress responses in the choroid plexus in Alzheimer’s diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - July 14, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Llama VHH antibody fragments against GFAP: better diffusion in fixed tissues than classical monoclonal antibodiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 imm...
Source: Acta Neuropathologica - July 14, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Inflammatory myopathy with abundant macrophages and dermatomyositis: two stages of one disorder or two distinct entities?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - July 10, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

The anterior cingulate cortex in autism: heterogeneity of qualitative and quantitative cytoarchitectonic features suggests possible subgroupsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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....
Source: Acta Neuropathologica - July 10, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Parvalbumin-positive GABAergic interneurons are increased in the dorsal hippocampus of the dystrophic mdx mouseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 topogra...
Source: Acta Neuropathologica - July 9, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular disseminationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 pr...
Source: Acta Neuropathologica - July 9, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Kurt Jellinger Prize 2010 for Outstanding Scientific Writing in NeuropathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - July 7, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Primary leptomeningeal oligodendroglioma with documented progression to anaplasia and t(1;19)(q10;p10) in a childEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - June 29, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Neuronal loss in Pelizaeus–Merzbacher disease differs in various mutations of the proteolipid protein 1Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 P...
Source: Acta Neuropathologica - June 29, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Our impact factor of 5.31: why it increased and what it means to readers and authorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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)
Source: Acta Neuropathologica - June 29, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

p62/SQSTM1 is overexpressed and prominently accumulated in inclusions of sporadic inclusion-body myositis muscle fibers, and can help differentiating it from polymyositis and dermatomyositisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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 brai...
Source: Acta Neuropathologica - June 26, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Type and frequency of IDH1 and IDH2 mutations are related to astrocytic and oligodendroglial differentiation and age: a study of 1,010 diffuse gliomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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, Insti...
Source: Acta Neuropathologica - June 25, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Intraneuronal pyroglutamate-Abeta 3–42 triggers neurodegeneration and lethal neurological deficits in a transgenic mouse modelEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
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...
Source: Acta Neuropathologica - June 23, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals