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 'Cerebellum' source. Sun, 21 Mar 2010 17:31:11 +0100 http://www.medworm.com/index.php?rid=3374534&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20232190%26dopt%3DAbstract Authors: Ruigrok TJ The modular concept of cerebellar connections has been advocated in the lifetime work of Jan Voogd. In this concept, a cerebellar module is defined as the conglomerate of one or multiple and non-adjacent, parasagittally arranged zones of Purkinje cells, their specific projection to a well-defined region of the cerebellar nuclei, and the climbing fiber input to these zones by a well-defined region of the inferior olivary complex. The modular organization of these olivo-cortico-nuclear connections is further exemplified by matching reciprocal connections between inferior olive and cerebellar nuclei. Because the different regions of the cerebellar nuclei show highly specific output patterns, cerebellar modules have been suggested to constitute functional entities. This... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3374534 Tue, 16 Mar 2010 00:00:00 +0100 3374534 http://www.medworm.com/index.php?rid=3322764&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20191336%26dopt%3DAbstract Authors: Herweh C, Akbar M, Wengenroth M, Heiland S, Bendszus M, Stippich C Besides supratentorial abnormalities, spina bifida menigomyelocele (SBM) is typically associated with Chiari-II malformation comprising a small cerebellum, which herniates downward due to a shallow posterior fossa. We used diffusion tensor imaging to probe additional microstructural alterations of the major cerebellar white matter tracts, the cerebellar peduncles. A region-of-interest approach was employed in six SBM patients and six matched controls to compare the fractional anisotropy (FA) within the superior, middle, and inferior cerebellar peduncle (SCP, MCP, and ICP, respectively). The FA in the MCP was significantly reduced in the SBM patients (0.44 vs. 0.65, p = 0.002), while there was no significant dif... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3322764 Sat, 27 Feb 2010 00:00:00 +0100 3322764 http://www.medworm.com/index.php?rid=3303341&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20178014%26dopt%3DAbstract Authors: Sawada Y, Kajiwara G, Iizuka A, Takayama K, Shuvaev AN, Koyama C, Hirai H Lentiviral vectors are promising as gene-transfer vehicles for gene therapy targeted to intractable brain diseases. Although lentiviral vectors are thought to exert little toxicity on infected cells, the adverse influence of viral infection on vulnerable developing neurons has not been well studied. Here, we examined whether lentiviral vector infection and subsequent transgene expression affected the morphological and functional maturation of vigorously developing cerebellar Purkinje cells in vivo. Lentiviral vectors expressing GFP under the control of the murine stem cell virus (MSCV) promoter were injected into the cerebellar cortex of neonatal rat pups. Three weeks after treatment, GFP-expressing Purk... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3303341 Tue, 23 Feb 2010 00:00:00 +0100 3303341 http://www.medworm.com/index.php?rid=3291505&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20169431%26dopt%3DAbstract Authors: Valle MS, Bosco G, Casabona A, Garifoli A, Perciavalle V, Coco M, Perciavalle V The interpositus nucleus (IN) receives a large amount of sensory information from the limbs and, in turn, elaborates signals for movement control. In this paper, we tried to gather evidence on the possibility that neurons in the IN may elaborate sensory representations of the forelimb kinematics and, particularly, of the movement velocity vector. For this purpose, the forepaw of anesthetized rats was attached to a computer-controlled robot arm displaced passively along two types of trajectories (circular and figure eight), with the limb joints unconstrained. The firing activity of single cells was recorded and related to limb position and the two components of the movement velocity vector, namely, ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3291505 Sat, 20 Feb 2010 00:00:00 +0100 3291505 http://www.medworm.com/index.php?rid=3280240&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20157791%26dopt%3DAbstract Authors: Fonteyn EM, Schmitz-Hübsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, Bunn L, Charles P, Dürr A, Filla A, Giunti P, Globas C, Klockgether T, Melegh B, Pandolfo M, De Rosa A, Schöls L, Timmann D, Munneke M, Kremer BP, van de Warrenburg BP To investigate the frequency, details, and consequences of falls in patients with autosomal dominant spinocerebellar ataxias (SCAs) and to derive specific disease-related risk factors that are associated with an increased fall frequency. Two hundred twenty-eight patients with SCA1, SCA2, SCA3, or SCA6, recruited from the EuroSCA natural history study, completed a fall questionnaire that assessed the frequency, consequences, and several details of falls in the previous 12 months. Relevant disease characteristics were retriev... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3280240 Tue, 16 Feb 2010 00:00:00 +0100 3280240 http://www.medworm.com/index.php?rid=3280241&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20155408%26dopt%3DAbstract Authors: Umahara T, Uchihara T This mini-review focuses on the possible relevance of 14-3-3 proteins in spinocerebellar ataxia type 1 (SCA1). 14-3-3 proteins are mainly localized in the synapses and neuronal cytoplasm, and seven isoforms have been identified in mammals. This family of proteins was initially identified as adaptor proteins which bind to phosphoserine-containing motifs. Binding motifs and potential functions of 14-3-3 proteins are now recognized to have a wide range of functional relevance. SCA1 is an autosomal-dominant neurodegenerative disorder and is linked to polyglutamine expansion (ataxin-1 protein). The Zoghbi and Orr group showed direct interaction of 14-3-3 proteins with ataxin-1 where nuclear recruitment of the ataxin-1 protein is dependent on its phosphorylatio... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3280241 Sat, 13 Feb 2010 00:00:00 +0100 3280241 http://www.medworm.com/index.php?rid=3273183&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20148319%26dopt%3DAbstract Authors: Pedroarena CM Cerebellar cortical signals are carried to their principal target, the deep cerebellar nuclear neurons (DCNs), via the inhibitory pathway formed by Purkinje cell (PC) axons. Two different intrinsic properties of DCNs, rebound excitation and automatic firing, have been proposed to support ensuing mechanisms for information transfer via inhibitory synapses. The efficacy of these mechanisms was investigated using whole-cell recordings of spontaneously firing DCNs in cerebellar slices. Results using current injection revealed that both mechanisms are effective in spontaneously firing DCNs but operate at different ranges of membrane potential. Rebound frequency was well correlated to the duration and amplitude of the preceding hyperpolarization. Activation of PC synap... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3273183 Fri, 12 Feb 2010 00:00:00 +0100 3273183 http://www.medworm.com/index.php?rid=3263307&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20146039%26dopt%3DAbstract Authors: Manto M, Oulad Ben Taib N PMID: 20146039 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=3263307 Wed, 10 Feb 2010 00:00:00 +0100 3263307 http://www.medworm.com/index.php?rid=3263308&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20143197%26dopt%3DAbstract Authors: Juranek J, Dennis M, Cirino PT, El-Messidi L, Fletcher JM Few volumetric MRI studies of the entire cerebellum have been published; even less quantitative information is available in patients with hindbrain malformations, including the Chiari II malformation which is ubiquitous in patients with spina bifida meningomyelocele (SBM). In the present study, regional volumetric analyses of the cerebellum were conducted in children with SBM/Chiari II and typically developing (TD) children. Total cerebellar volume was significantly reduced in the SBM group relative to the TD group. After correcting for total cerebellum volume, and relative to the TD group, the posterior lobe was significantly reduced in SBM, the corpus medullare was not different, and the anterior lobe was significantl... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3263308 Tue, 09 Feb 2010 00:00:00 +0100 3263308 http://www.medworm.com/index.php?rid=3238978&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20127431%26dopt%3DAbstract Authors: Demilly A, Reeber SL, Gebre SA, Sillitoe RV Despite the general uniformity in cellular composition of the adult cerebellum (Cb), the expression of proteins such as ZebrinII/AldolaseC and the small heat shock protein HSP25 reveal striking patterns of parasagittal Purkinje cell (PC) stripes. Based on differences in the stripe configuration within subsets of lobules, the Cb can be further divided into four anterior-posterior transverse zones: anterior zone (AZ) = lobules I-V, central zone (CZ) = lobules VI-VII, posterior zone (PZ) = lobules VIII and anterior IX, and the nodular zone (NZ) = lobules posterior IX-X. Here we used whole-mount and tissue section immunohistochemistry to show that neurofilament heavy chain (NFH) expression alone divides all lobules of the mouse Cb into a... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3238978 Wed, 03 Feb 2010 00:00:00 +0100 3238978 http://www.medworm.com/index.php?rid=3189470&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20082166%26dopt%3DAbstract We examined a four-generation Italian family segregating an autosomal dominant cerebellar ataxia, in which linkage analysis was positive for the SCA15 locus. We performed a genomic real-time polymerase chain reaction to search for ITPR1 gene deletions in this family and in 60 SCA index cases negative for mutations in the SCA1-3, 6-8, 10, 12, and dentatorubral-pallidoluysian atrophy genes. The deleted segments were characterized using a custom array comparative genomic hybridization analysis. We have identified two families with an ITPR1 gene deletion: in one, the deletion involved ITPR1 only, while in the other both sulfatase-modifying factor 1 and ITPR1. Clinical data of ten patients and brain MRI (available for six) showed that the phenotype substantially overlapped known SCA15 cases, bu... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3189470 Sat, 16 Jan 2010 00:00:00 +0100 3189470 http://www.medworm.com/index.php?rid=3149398&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20049574%26dopt%3DAbstract Authors: Zhang W, Linden DJ Action potential-evoked calcium influx into presynaptic boutons is a key determinant of synaptic strength and function. Here, we have examined the calcium dynamics at individual presynaptic boutons of the cerebellar granule cells in the molecular layer of cerebellar slices and investigated whether different subpopulations of granule cell boutons exhibit different calcium dynamics. We found that a population of boutons with low basal calcium clearance rates may activate a second clearance mechanism and exhibit biphasic calcium decay on high calcium influx induced by bursts of action potentials. We also found that boutons on ascending axons and parallel fibers show similar calcium influx amplitudes and calcium clearance rates in response to action potentials. ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3149398 Tue, 05 Jan 2010 00:00:00 +0100 3149398 http://www.medworm.com/index.php?rid=3120655&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20033360%26dopt%3DAbstract Authors: Cerminara NL, Rawson JA, Apps R In the present study, a non-pulsatile arterially perfused hindbrain and upper body rat preparation is described which is an extension of the brainstem preparation reported by Potts et al., (Brain Res Bull 53(1):59-67), 1. The modified in situ preparation allows study of cerebellar function whilst preserving the integrity of many of its interconnections with the brainstem, upper spinal cord and the peripheral nervous system of the head and forelimbs. Evoked mossy fibre, climbing fibre and parallel fibre field potentials and EMG activity elicited in forelimb biceps muscle by interpositus stimulation provided evidence that both cerebellar inputs and outputs remain operational in this preparation. Similarly, the spontaneous and evoked single unit ac... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3120655 Tue, 22 Dec 2009 00:00:00 +0100 3120655 http://www.medworm.com/index.php?rid=3105669&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20016963%26dopt%3DAbstract Authors: Reetz K, Lencer R, Hagenah JM, Gaser C, Tadic V, Walter U, Wolters A, Steinlechner S, Zühlke C, Brockmann K, Klein C, Rolfs A, Binkofski F Spinocerebellar ataxia (SCA17) is a rare genetic disorder characterized by a variety of neuropsychiatric symptoms. Recently, using magnetic resonance imaging (MRI) voxel-based morphometry (VBM), several specific functional-structural correlations comprising differential degeneration related to motor and psychiatric symptoms were reported in patients with SCA17. To investigate gray matter volume (GMV) changes over time and its association to clinical neuropsychiatric symptomatology, nine SCA17 mutation carriers and nine matched healthy individuals underwent a detailed neuropsychiatric clinical examination and a high-resolution T1-weight... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3105669 Fri, 18 Dec 2009 00:00:00 +0100 3105669 http://www.medworm.com/index.php?rid=3105668&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D20016964%26dopt%3DAbstract Authors: Garcia-Martinez R, Miquel M, Garcia LI, Coria-Avila GA, Perez CA, Aranda-Abreu GE, Toledo R, Hernandez ME, Manzo J The sexual behavior of male rats constitutes a natural model to study learning of motor skills at the level of the central nervous system. We previously showed that sexual behavior increases Fos expression in granule cells at lobules 6 to 9 of the vermis cerebellum. Herein, we obtained multiunit recordings of lobules 6a and 7 during the training period of naive subjects, and during consecutive ejaculations of expert males. Recordings from both lobules and the inferior olive showed that the maximum amplitude of mount, intromission, and ejaculation signals were similar, but sexual behavior during training tests produced a decrease in the amplitude for mount and intr... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3105668 Fri, 18 Dec 2009 00:00:00 +0100 3105668 http://www.medworm.com/index.php?rid=3009316&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19921532%26dopt%3DAbstract In this study, perfusing slices with orexin A (100 nM-1 muM) or orexin B (100 nM-1 muM) both produced neurons in the rat cerebellar interpositus nucleus (IN) a concentration-dependent excitatory response (96/143, 67.1%). Furthermore, both of the excitations induced by orexin A and B were not blocked by the low-Ca(2+)/high-Mg(2+) medium (n = 8), supporting a direct postsynaptic action of the peptides. Highly selective orexin 1 receptor antagonist SB-334867 did not block the excitatory response of cerebellar IN neurons to orexins (n = 22), but [Ala(11), D-Leu(15)] orexin B, a highly selective orexin 2 receptor (OX(2)R) agonist, mimicked the excitatory effect of orexins on the cerebellar neurons (n = 18). These results demonstrate that orexins excite the cerebellar IN neurons through OX(2)R a... Cerebellum journals http://www.medworm.com/rss/comments.php?id=3009316 Thu, 19 Nov 2009 00:00:00 +0100 3009316 http://www.medworm.com/index.php?rid=2985575&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19904577%26dopt%3DAbstract Authors: Hoogland TM, Kuhn B Several studies have contributed to our understanding of astrocytes, especially Bergmann glia, in the cerebellum; but, until recently, none has looked at their function in vivo. Multicell bolus loading of fluorescent calcium indicators in combination with the astrocytic marker SR101 has allowed imaging of up to hundreds of astrocytes at once in the intact cerebellum. In addition, the selective targeting of astrocytes with fluorescent calcium indicator proteins has enabled the study of their function in vivo without the confounding effects of other neuropil signals and with a resolution that surpasses multicell bolus loading and SR101 staining. The two astrocyte types of the cerebellar cortex, Bergmann glia, and velate protoplasmic astrocytes display a diver... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2985575 Wed, 11 Nov 2009 00:00:00 +0100 2985575 http://www.medworm.com/index.php?rid=2985577&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19902318%26dopt%3DAbstract Authors: Schwarz C How does the cerebellum participate in neocortical rhythms? Neocortical signals destined for the cerebellum are integrated in the pontine nuclei (PN) with cerebellar output signals via a direct, reciprocal feedback loop with the cerebellar nuclei (CN). The present study investigated the fate of two spontaneously occurring rhythms in rat neocortex under ketamine anesthesia-slow wave activity at around 1 Hz and gamma oscillations-within this pontonuclear feedback loop. Coordinated oscillatory neuronal activity was studied using simultaneous multineuron recordings in primary motor cortex (M1), PN, and lateral CN. It was revealed that slow burst firing-known in neocortex as "up and down states"-is readily conveyed within the pontonuclear feedback loop and thus engages th... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2985577 Tue, 10 Nov 2009 00:00:00 +0100 2985577 http://www.medworm.com/index.php?rid=2985581&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19898914%26dopt%3DAbstract Authors: Habas C, Guillevin R, Abanou A Few imaging studies have been devoted to the structural and functional connectivity of the red and inferior olivary nuclei although these two nuclei represent two main targets of the cerebellum within the brainstem. However, the RN is anatomically and functionally related to a widespread sensorimotor, limbic, and executive brain network. It projects massively onto the principal olive with which it contributes to a cerebello-rubro-olivo-cerebellar loop modulated by cortical and subcortical afferents. Despite a minor role in planning and execution of rhythmic movements, the red nucleus in conjunction with the inferior olive, more specifically involved in the detection of "unexpected" events, contributes to sensorimotor, sensory and, likely, cogniti... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2985581 Sat, 07 Nov 2009 00:00:00 +0100 2985581 http://www.medworm.com/index.php?rid=2985579&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19898915%26dopt%3DAbstract Authors: Kieslich M, Hoche F, Reichenbach J, Weidauer S, Porto L, Vlaho S, Schubert R, Zielen S Ataxia telangiectasia (AT) is a rare autosomal recessive disorder characterized by progressive ataxia, neurodegeneration, immunodeficiency, and cancer predisposition. Pathoanatomical studies reported a degeneration of cerebellar Purkinje cells as the striking feature of the disease. Although recent studies suggested the involvement of extracerebellar structures such as the brainstem and basal ganglia, this has rarely been studied in human AT. Thus, we performed a detailed cliniconeuroradiological investigation of 11 AT patients, aged 8 to 26 years by collecting clinical neurological data, ataxia scores, growth status, body mass index (BMI), growth hormone (GH), and insulin-like-growth factor... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2985579 Sat, 07 Nov 2009 00:00:00 +0100 2985579 http://www.medworm.com/index.php?rid=2968570&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19890685%26dopt%3DAbstract Authors: Matilla-Dueñas A, Sánchez I, Corral-Juan M, Dávalos A, Alvarez R, Latorre P The autosomal dominant spinocerebellar ataxias (SCAs) are a group of progressive neurodegenerative diseases characterised by loss of balance and motor coordination due to the primary dysfunction of the cerebellum. To date, more than 30 genes have been identified triggering the well-described clinical and pathological phenotype, but the underlying cellular and molecular events are still poorly understood. Studies of the functions of the proteins implicated in SCAs and the corresponding altered cellular pathways point to major aetiological roles for defects in transcriptional regulation, protein aggregation and clearance, alterations of calcium homeostasis, and activation of pro-apoptotic ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2968570 Thu, 05 Nov 2009 00:00:00 +0100 2968570 http://www.medworm.com/index.php?rid=2935708&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19859774%26dopt%3DAbstract In conclusion, physical maltreatment was observed to interact with cerebellar morphology resulting in a strong association with alcohol and substance use. Lingula thickness may represent a novel, experientially sensitive, phenotypic risk factor for enhanced alcohol and drug use that perhaps modulates sensitivity to these agents. PMID: 19859774 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2935708 Wed, 28 Oct 2009 00:00:00 +0100 2935708 http://www.medworm.com/index.php?rid=2935709&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19859773%26dopt%3DAbstract Authors: Manto M, Marmolino D Our understanding of the pathogenesis of cerebellar ataxias has started several decades ago and is continuously growing. The numerous mechanisms of cerebellar dysfunction are being discovered by numerous groups of researchers worldwide. Neuronal damage results from a complex interaction of metabolic pathways, which leads to symptoms observed in cerebellar disorders. The main mechanisms at the molecular level are the following: impairment of DNA repair and replication, deregulation of transcription/deficits of processing/transport of RNA, abnormal protein transport and misfolding, aggregates both at the nuclear and cytosolic level, activation of caspases, apoptosis, involvement of autophagic mechanisms, oxidative stress and mitochondrial dysfunction, excito... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2935709 Tue, 27 Oct 2009 00:00:00 +0100 2935709 http://www.medworm.com/index.php?rid=2924412&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19851820%26dopt%3DAbstract Authors: Bareš M, Lungu OV, Husárová I, Gescheidt T There is evidence that both the basal ganglia and the cerebellum play a role in the neural representation of time in a variety of behaviours, but whether one of them is more important is not yet clear. To address this question in the context of predictive motor timing, we tested patients with various movement disorders implicating these two structures in a motor-timing task. Specifically, we investigated four different groups: (1) patients with early Parkinson's disease (PD); (2) patients with sporadic spinocerebellar ataxia (SCA); (3) patients with familial essential tremor (ET); and (4) matched healthy controls. We used a predictive motor-timing task that involved mediated interception of a moving target, and we asse... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2924412 Fri, 23 Oct 2009 00:00:00 +0100 2924412 http://www.medworm.com/index.php?rid=2920629&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19847585%26dopt%3DAbstract Authors: Zheng N, Raman IM Neurons of the cerebellar nuclei generate the non-vestibular output of the cerebellum. Like other neurons, they integrate excitatory and inhibitory synaptic inputs and filter them through their intrinsic properties to produce patterns of action potential output. The synaptic and intrinsic features of cerebellar nuclear cells are unusual in several respects, however: these neurons receive an overwhelming amount of basal and driven inhibition from Purkinje neurons, but are also spontaneously active, producing action potentials even without excitation. Moreover, not only is spiking by nuclear cells sensitive to the amount of inhibition, but the strength of inhibition is also sensitive to the amount of spiking, through multiple forms of long-term plasticity. Here... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2920629 Wed, 21 Oct 2009 23:00:00 +0100 2920629 http://www.medworm.com/index.php?rid=2912911&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19841996%26dopt%3DAbstract Authors: Haines DE, Manto MU, Brown BL, Graybiel A PMID: 19841996 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2912911 Mon, 19 Oct 2009 23:00:00 +0100 2912911 http://www.medworm.com/index.php?rid=2912912&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19838760%26dopt%3DAbstract Authors: Lyons JA, Prohaska JR Copper is required for activity of several key enzymes and for optimal mammalian development, especially within the central nervous system. Copper-deficient (CuD) animals are visibly ataxic, and previous studies in rats have demonstrated impaired motor function through behavioral experiments consistent with altered cerebellar development. Perinatal copper deficiency was produced in Holtzman rat dams by restricting dietary copper during the last two thirds of gestation and lactation. Male offspring were evaluated at postnatal day 25. Compared to cerebella from copper-adequate pups, the CuD pups had larger Purkinje cell (PC) size and irregularities in the Purkinje cell monolayer. These results suggest that the ataxic behavioral phenotype of CuD rats may res... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2912912 Fri, 16 Oct 2009 23:00:00 +0100 2912912 http://www.medworm.com/index.php?rid=2895504&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19826891%26dopt%3DAbstract Authors: Uusisaari M, Knöpfel T The deep cerebellar nuclei (DCN) are a major hub in the cerebellar circuitry but the functional classification of their neurons is incomplete. We have previously characterized three cell groups in the lateral cerebellar nucleus: large non-GABAergic neurons and two groups of smaller neurons, one of which express green fluorescence protein (GFP) in a GAD67/GFP mouse line and is therefore GABAergic. However, as a substantial number of glycinergic and glycine/GABA co-expressing neurons have been described in the DCN, this classification needed to be refined by considering glycinergic neurons. To this end we took advantage of a glycine transporter isoform 2 (GlyT2)-eGFP mouse line that allows identification of GlyT2-expressing, presumably glycinergic neu... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2895504 Tue, 13 Oct 2009 23:00:00 +0100 2895504 http://www.medworm.com/index.php?rid=2887966&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19821003%26dopt%3DAbstract We present the case of a patient with Chiari II malformation mimicking partial rhombencephalosynapsis. PMID: 19821003 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2887966 Fri, 09 Oct 2009 23:00:00 +0100 2887966 http://www.medworm.com/index.php?rid=2859307&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19798544%26dopt%3DAbstract Authors: Foti F, Mandolesi L, Cutuli D, Laricchiuta D, De Bartolo P, Gelfo F, Petrosini L The influence of a hemicerebellar lesion on the exploration of environments with different spatial distributions of multiple rewards was analyzed. Hemicerebellectomized (HCbed) and intact rats were submitted to a search task in which they had to explore nine food trays in an open field, avoiding repeated visits. Trays were spatially arranged in four configurations: cross, 3 x 3 matrix, circle, and three clusters of three trays each. Lesioned and intact rats' performances improved in all configurations used. However, the explorative activity of the HCbed animals differed from that of intact rats. Lesioned animals spent more time, made more errors, displayed lower search efficiency, exhibited shorte... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2859307 Thu, 01 Oct 2009 23:00:00 +0100 2859307 http://www.medworm.com/index.php?rid=2768020&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19727998%26dopt%3DAbstract Authors: Fogel BL, Lee JY, Perlman S Ataxia with oculomotor apraxia type 2 (AOA2) is caused by a diversity of mutations within the coding region of the senataxin gene. Recently, rare noncoding senataxin mutations affecting RNA processing have been identified in AOA2. Here, we report the case of an 18-year-old woman, with classic clinical features of AOA2, who was found to harbor a mutation within senataxin intron 16. This mutation disrupts the local 5' splice site architecture via a novel intronic frameshift mechanism, causing skipping of exon 16 with predicted disruption of the conserved DNA/RNA helicase domain. RNA processing mutations expand the growing complexity of pathogenic senataxin mutations. PMID: 19727998 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2768020 Tue, 01 Sep 2009 23:00:00 +0100 2768020 http://www.medworm.com/index.php?rid=2768019&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19727999%26dopt%3DAbstract Authors: Karmon Y, Inbar E, Cordoba M, Gadoth N Acute cerebellitis is a monophasic non-progressive encephalitis restricted to the cerebellum, while paraneoplastic cerebellar degeneration is a subacute progressive disorder, which may either accompany or herald Hodgkin's disease. In the present report, we describe a young man with clinical and laboratory features of acute post-infectious cerebellitis in whom the progressive relapsing course subsequently led to the diagnosis of Hodgkin's disease. Dynamic changes observed on repeated MRIs during the protracted clinical course imply the presence of early active inflammation and subsequent neuronal atrophy. PMID: 19727999 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2768019 Tue, 01 Sep 2009 23:00:00 +0100 2768019 http://www.medworm.com/index.php?rid=2713468&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19688409%26dopt%3DAbstract Authors: Angelucci F, De Bartolo P, Gelfo F, Foti F, Cutuli D, Bossù P, Caltagirone C, Petrosini L The molecular mechanism of environmental enrichment (EE) on brain function and anatomy has been partially attributed to the up-regulation of proteins involved in neuronal survival and activity-dependent plasticity, such as the neurotrophins nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF), in the cerebral cortex and hippocampus of animal models. Nevertheless, at present, little indication is available on the influence of EE on neurotrophin levels in the cerebellum. Thus, in this study, we exposed male Wistar rats to EE from weaning to 5 months of age and evaluated the production of NGF and BDNF in the cerebellum and compared the neurotrophin changes in this regi... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2713468 Tue, 18 Aug 2009 23:00:00 +0100 2713468 http://www.medworm.com/index.php?rid=2692243&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19669387%26dopt%3DAbstract Authors: Manto M, Marmolino D Cerebellar ataxias represent a group of disabling neurological disorders. Our understanding of the pathogenesis of cerebellar ataxias is continuously expanding. A considerable number of laboratory animals with neurological mutations have been reported and numerous relevant animal models mimicking the phenotype of cerebellar ataxias are becoming available. These models greatly help dissecting the numerous mechanisms of cerebellar dysfunction, a major step for the assessment of therapeutics targeting a given deleterious pathway and for the screening of old or newly synthesized chemical compounds. Nevertheless, differences between animal models and human disorders should not be overlooked and difficulties in terms of characterization should not be occulted. T... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2692243 Fri, 07 Aug 2009 23:00:00 +0100 2692243 http://www.medworm.com/index.php?rid=2603165&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19593677%26dopt%3DAbstract We examined the expression levels (by Western blotting) of microtubule-associated protein light chain 3 (LC3)-I and LC3-II, and the degradation levels of p62 (a LC3 partner) in the cerebellar fractions prepared from pre-symptomatic SCA1 and age-matched wild-type mice. No p62 degradation was observed; however, LC3-II/(LC3-I + LC3-II) ratios were significantly altered in SCA1 mice indicating changes in the autophagic flux. In addition, LC3 localized to PC vacuoles. Further, we observed a co-localization of myo-inositol monophosphatase 1 (IMPA1) with S100B in PC vacuoles. IMPA1 is present in PC spines and has been implicated in autophagy. In vitro studies using purified IMPA1 and S100B demonstrated that S100B interacted with and activated IMPA1. Both apo and Ca(2+)-bound S100B were found to a... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2603165 Fri, 10 Jul 2009 23:00:00 +0100 2603165 http://www.medworm.com/index.php?rid=2588591&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19585179%26dopt%3DAbstract Authors: Grant JK, Yin NC, Zaytoun AM, Waseem H, Hobbs JA We previously reported in a large cohort (N = 104) of post-mortem tissues the detection of both the non-pathogenic adeno-associated virus (AAV2) in approximately 13% and the pathogenic human parvovirus B19 (B19) in approximately 42% of human brains, particularly the dorsolateral prefrontal cortex. Multiple animal parvoviruses target the developing cerebellum (CBLM) resulting in hypoplasia and ataxia, but very little is known about the human parvoviruses and their ability to infect or cause disease in the CBLM. We have now confirmed in the above cohort the presence of AAV2 and B19 sequences in the CBLM. Our results show that approximately 27% and approximately 70% of human CBLM are positive by nested polymerase chain reaction for... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2588591 Tue, 07 Jul 2009 23:00:00 +0100 2588591 http://www.medworm.com/index.php?rid=2573543&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19575274%26dopt%3DAbstract Authors: Sajdel-Sulkowska EM, Xu M, Koibuchi N We previously reported that the effects of perinatal exposure to hypergravity on cerebellum and motor functions in rat neonates are strongly dependent on the specific developmental period of exposure. In the present study, we explored the hypothesis that neurodevelopmental changes are associated with altered expression of brain neurotrophins critical for normal brain growth and differentiation. We compared the effects of hypergravity exposure during four developmental periods: period I extended from gestational day (G) 8 through G15; period II from G15 to birth, period III from birth to postnatal day (P) 6; and period IV extended from G8-P12. For comparison we used stationary control (SC) neonates not exposed to hypergravity. Neurotrophins... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2573543 Thu, 02 Jul 2009 23:00:00 +0100 2573543 http://www.medworm.com/index.php?rid=2525532&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19554387%26dopt%3DAbstract Authors: Schilling K, Oberdick J We mined the Allen Mouse Brain Atlas for genes expressed in cerebellar cortical inhibitory interneurons that would allow identification and possibly distinction of these cells. We identified some 90 genes that are highly expressed in specific subsets of cerebellar cortical inhibitory interneurons or various combinations thereof. Four genes are exclusively expressed, within the cerebellar cortex, in molecular layer interneurons, and ten genes label exclusively inhibitory interneurons in the granule cell layer or subsets thereof. Differential expression of many of these genes in cells residing in the lower versus the upper molecular layer provides evidence that these cells, traditionally referred to as basket and stellate cells, are indeed molecularly dis... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525532 Wed, 24 Jun 2009 23:00:00 +0100 2525532 http://www.medworm.com/index.php?rid=2525534&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19548045%26dopt%3DAbstract Authors: Ariel M, Ward KC, Tolbert DL The turtle's cerebellum (Cb) is an unfoliated sheet, so the topography of its entire cortex can be easily studied physiologically by optical recordings. However, unlike the mammalian Cb, little is known about the topography of turtle Purkinje cells (PCs). Here, topography was examined using calbindin-D(28K) immunohistochemistry of adult and hatchling turtles (Trachemys scripta elegans, 2.5-15 cm carapace length). Each Cb was flattened between two Sylgard sheets and fixed in paraformaldehyde. Sections (52 microm thick) were cut parallel to the flattened cortex (tangential), resulting in calbindin-immunolabeled PCs being localized to three to six sections for each turtle. PC position and size were quantified using Neurolucida Image Analysis system. A... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525534 Mon, 22 Jun 2009 23:00:00 +0100 2525534 http://www.medworm.com/index.php?rid=2525533&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19548046%26dopt%3DAbstract Authors: Cameron DB, Raoult E, Galas L, Jiang Y, Lee K, Hu T, Vaudry D, Komuro H Pituitary adenylate cyclase-activating polypeptide (PACAP), a neuropeptide, regulates a wide variety of cellular functions, but little is known about its role in neuronal cell migration. Recent studies revealed that PACAP has short-term, cortical layer-specific effects on neuronal cell migration. In this article, we focus on the role of PACAP in controlling the migration of cerebellar granule cells. PMID: 19548046 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525533 Mon, 22 Jun 2009 23:00:00 +0100 2525533 http://www.medworm.com/index.php?rid=2525535&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19517204%26dopt%3DAbstract We report a very rare anomalous anatomic variant of the cavernous internal carotid artery supplying directly the posterior inferior cerebellar artery, with no basilar artery opacification. A systematic review as well as a description of other variants of trigeminal-cerebellar anastomosis is given. PMID: 19517204 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525535 Wed, 10 Jun 2009 23:00:00 +0100 2525535 http://www.medworm.com/index.php?rid=2525536&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19513801%26dopt%3DAbstract Authors: Habas C The present mini-review focused on functional imaging of human deep cerebellar nuclei, mainly the dentate nucleus. Although these nuclei represent the unique output channel of the cerebellum, few data are available concerning their functional role. However, the dentate nucleus has been shown to participate in a widespread functional network including sensorimotor and associative cortices, striatum, hypothalamus, and thalamus, and plays a minor role in motor execution and a major role in sensorimotor coordination and learning, and cognition. The dentate nucleus appears to be predominantly involved in conjunction with the neocerebellum in executive and affective networks devoted, at least, to attention, working memory, procedural reasoning, and salience detection. PM... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525536 Tue, 09 Jun 2009 23:00:00 +0100 2525536 http://www.medworm.com/index.php?rid=2525538&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19495900%26dopt%3DAbstract This article will review these computational models and discuss the possible computational power of the cerebellum. PMID: 19495900 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525538 Thu, 04 Jun 2009 23:00:00 +0100 2525538 http://www.medworm.com/index.php?rid=2525537&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19495901%26dopt%3DAbstract Authors: Potts MB, Adwanikar H, Noble-Haeusslein LJ Traumatic brain injury (TBI) is a major cause of morbidity and mortality worldwide. Studies of human TBI demonstrate that the cerebellum is sometimes affected even when the initial mechanical insult is directed to the cerebral cortex. Some of the components of TBI, including ataxia, postural instability, tremor, impairments in balance and fine motor skills, and even cognitive deficits, may be attributed in part to cerebellar damage. Animal models of TBI have begun to explore the vulnerability of the cerebellum. In this paper, we review the clinical presentation, pathogenesis, and putative mechanisms underlying cerebellar damage with an emphasis on experimental models that have been used to further elucidate this poorly understood but ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525537 Thu, 04 Jun 2009 23:00:00 +0100 2525537 http://www.medworm.com/index.php?rid=2525539&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19488825%26dopt%3DAbstract Authors: Schmitt I, Bitoun E, Manto M Tyrosine phosphorylation is a powerful mechanism of modulation for proliferation, differentiation, and functioning of neurons. The protein products of the neuronal mouse gene PTPRR are physiological regulators of mitogen-activated protein kinase (MAPK) activities. PTPRR(-/-) mice display deficits of motor coordination and balance skills. PTPRR gene orthologues are found in many vertebrates. Recent observations suggest that the human episodic ataxia 2 (EA2) and spinocerebellar ataxia types 6 (SCA6), 12 (SCA12), and 14 (SCA14) might be associated with impaired phosphorylation levels of cerebellum calcium channels and receptors. The concept that MAPK signaling is a key process in tuning synaptic plasticity in cerebellar circuits is now emerging, with ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525539 Sun, 31 May 2009 23:00:00 +0100 2525539 http://www.medworm.com/index.php?rid=2525540&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19484318%26dopt%3DAbstract Authors: Plomp JJ, van den Maagdenberg AM, Kaja S Homozygous rolling Nagoya natural mutant mice display a severe ataxic gait and frequently roll over to their side or back. The causative mutation resides in the Cacna1a gene, encoding the pore-forming alpha(1) subunit of Ca(v)2.1 type voltage-gated Ca(2+) channels. These channels are crucially involved in neuronal Ca(2+) signaling and in neurotransmitter release at many central synapses and, in the periphery, at the neuromuscular junction. We here review the behavioral, histological, biochemical, and neurophysiological studies on this mouse mutant and discuss its usefulness as a model of human neurological diseases associated with Ca(v)2.1 dysfunction. PMID: 19484318 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525540 Fri, 29 May 2009 23:00:00 +0100 2525540 http://www.medworm.com/index.php?rid=2525541&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19472026%26dopt%3DAbstract Authors: van Broekhoven PC, Schraa-Tam CK, van der Lugt A, Smits M, Frens MA, van der Geest JN Saccades are fast eye movements that direct the point of regard to a target in the visual field. Repeated post-saccadic visual errors can induce modifications of the amplitude of these saccades, a process known as saccadic adaptation. Two experiments using the same paradigm were performed to study the involvement of the cerebrum and the cerebellum in the processing of saccadic errors using functional magnetic resonance imaging and in-scanner eye movement recordings. In the first active condition, saccadic adaptation was prevented using a condition in which the saccadic target was shifted to a variable position during the saccade towards it. This condition induced random saccadic errors as opp... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525541 Tue, 26 May 2009 23:00:00 +0100 2525541 http://www.medworm.com/index.php?rid=2525542&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19468795%26dopt%3DAbstract Authors: Velasco-Sánchez D, Gómez-Lopez L, Vilaseca MA, Serrano M, Massaguer S, Campistol J, García-Cazorla A Cerebellar hemorrhage (CH) is a well-known complication in newborns. Among metabolic patients, it has been classically described but rarely reported. This is the first description of a patient with propionic acidemia in whom magnetic resonance imaging (MRI) allowed diagnosis of asymptomatic CH. Due to the usual silent presentation of CH at early ages, we suggest the possibility of including a brain MRI study as part of the routine neurological evaluation in metabolic patients, especially when neurological signs appear. PMID: 19468795 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525542 Mon, 25 May 2009 23:00:00 +0100 2525542 http://www.medworm.com/index.php?rid=2525543&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19462216%26dopt%3DAbstract Authors: Akita K, Arai S A spontaneous model of cerebellar ataxia in the Syrian hamster is described. Breeding data indicate that the condition is hereditary and that the mode of inheritance is autosomal recessive. Homozygotes are smaller in size than the wild-type but have a normal appearance. Mutants show a moderate ataxia beginning at 7 weeks of age. Although affected adults exhibit significant atrophy in the cerebellum, other parts of the brain appear relatively normal by light microscopy. Mutants lose almost all Purkinje cells by 18 months of age and exhibit a moderate reduction in granule cell density, probably as a consequence of the primary loss of Purkinje cells. In the homozygous hamster brain, Nna1 expression is suppressed, similar to that previously observed in Purkinje cel... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525543 Thu, 21 May 2009 23:00:00 +0100 2525543 http://www.medworm.com/index.php?rid=2525544&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19455379%26dopt%3DAbstract Authors: Molinari M, Restuccia D, Leggio MG State estimation has emerged as the keystone in determining the cerebellar contribution to motor control. Clinical, experimental, and neuroimaging data converge to focus on cerebellar multisensory information processing as the mechanism allowing to correctly establish the body state needed for movement. Further, recent data indicate the cerebellum specifically involved in predicting body state changes. The present editorial discusses these evidences and questions cerebellar state estimation in the cognitive domain. PMID: 19455379 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525544 Tue, 19 May 2009 23:00:00 +0100 2525544 http://www.medworm.com/index.php?rid=2525545&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19452239%26dopt%3DAbstract Authors: Kronenbuerger M, Konczak J, Ziegler W, Buderath P, Frank B, Coenen VA, Kiening K, Reinacher P, Noth J, Timmann D The pathogenesis of essential tremor (ET) is still under debate. Several lines of evidence indicate that ET is associated with cerebellar dysfunction. The aim of the present study was to find corroborating evidence for this claim by investigating balance and speech impairments in patients with ET. In addition, the effect of deep brain stimulation (DBS) on balance and speech function was studied. A group of 25 ET patients including 18 with postural and/or simple kinetic tremor (ETpt) and seven ET patients with additional clinical signs of cerebellar dysfunction (ETc) was compared to 25 healthy controls. In addition, 12 ET patients with thalamic DBS participated in th... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525545 Mon, 18 May 2009 23:00:00 +0100 2525545 http://www.medworm.com/index.php?rid=2525546&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19424768%26dopt%3DAbstract Authors: Chen K, Godfrey DA, Ilyas O, Xu J, Preston TW Among ten sodium channel alpha-subunit genes mapped in human and mouse genomes, the SCN8A gene is primarily expressed in neurons and glia. Mice with two types of Scn8a null mutations-Scn8a ( med ) and Scn8a ( medTg )-live for only 21-24 days, but those with incomplete mutations-Scn8a ( medJ ) and Scn8a ( medJo )-and those with knockout of Scn8a only in cerebellar Purkinje cells live to adult age. We review here previous work on cerebellum and related regions of Scn8a mutant mice and include some newer immunohistochemical and microchemical results. The resurgent sodium current that underlies the repeated firing of Purkinje cells is reduced in Scn8a mutant and knockout mice. Purkinje cells of mutant mice have greatly reduced spontane... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525546 Thu, 07 May 2009 23:00:00 +0100 2525546 http://www.medworm.com/index.php?rid=2525548&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19415407%26dopt%3DAbstract Authors: Glasauer S, Stephan T, Kalla R, Marti S, Straumann D Animal experiments have demonstrated that the vast majority of vertical gaze-velocity Purkinje cells in the cerebellar floccular lobe, whose firing rate is modulated during vertical smooth pursuit eye movements, show a preference for downward pursuit. Here we validate the functional vertical asymmetry of the cerebellar flocculus in humans using functional magnetic resonance imaging by demonstrating a significantly higher activation of the floccular lobe for downward than for upward pursuit. The findings corroborate our recent hypothesis on the pathogenesis of cerebellar downbeat nystagmus. PMID: 19415407 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525548 Mon, 04 May 2009 23:00:00 +0100 2525548 http://www.medworm.com/index.php?rid=2525550&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19408063%26dopt%3DAbstract We present the first reported case of fenestration of the double origin of the PICA associated with a dissecting aneurysm of the contralateral vertebral artery (VA) that healed spontaneously after medical observation. PMID: 19408063 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525550 Thu, 30 Apr 2009 23:00:00 +0100 2525550 http://www.medworm.com/index.php?rid=2525549&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19408064%26dopt%3DAbstract This study was undertaken to explain this apparent paradox. Eleven CJD patients with definite cerebellar or brain stem symptoms were selected from a large prospective study, as well as 11 healthy controls matched for age and gender. All subjects participated in a standardized MRI protocol, including SPGR, fluid-attenuated inversion recovery (FLAIR), DWI and diffusion tensor imaging (DTI). All subjects underwent detailed examination by a neurologist blinded to the radiological findings, who predicted the expected site of cerebral abnormalities. MRI showed good sensitivity for the abnormalities predicted in the cortex (80-90%) and basal ganglia (100%). None of the standard MRI sequences, including DWI, DTI, and FLAIR, revealed any tissue abnormalities in cerebellum or brain stem. Apparent di... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525549 Thu, 30 Apr 2009 23:00:00 +0100 2525549 http://www.medworm.com/index.php?rid=2525551&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19387761%26dopt%3DAbstract Authors: Viscomi MT, Florenzano F, Latini L, Molinari M Functional impairment after focal CNS lesion is highly dependent on damage that occurs in regions that are remote but functionally connected to the primary lesion site. This pattern is particularly evident in the cerebellar system, in which functional interactions between the cerebellar cortex, deep cerebellar nuclei, and precerebellar stations are of paramount importance. Diffuse degeneration after development of a focal CNS lesion has been associated with poor outcomes in several pathologies, such as stroke, multiple sclerosis, and brain trauma. A greater understanding of the mechanisms that underlie the spread of death signals from focal lesions, however, can aid in identifying a neuroprotective approach for CNS pathologies. To... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525551 Tue, 21 Apr 2009 23:00:00 +0100 2525551 http://www.medworm.com/index.php?rid=2525552&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19357934%26dopt%3DAbstract Authors: Sajdel-Sulkowska EM, Xu M, Koibuchi N Autism is a neurodevelopmental disorder characterized by social and language deficits, ritualistic-repetitive behaviors and disturbance in motor functions. Data of imaging, head circumference studies, and Purkinje cell analysis suggest impaired brain growth and development. Both genetic predisposition and environmental triggers have been implicated in the etiology of autism, but the underlying cause remains unknown. Recently, we have reported an increase in 3-nitrotyrosine (3-NT), a marker of oxidative stress damage to proteins in autistic cerebella. In the present study, we further explored oxidative damage in the autistic cerebellum by measuring 8-hydroxydeoxyguanosine (8-OH-dG), a marker of DNA modification, in a subset of cases analyze... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525552 Wed, 08 Apr 2009 23:00:00 +0100 2525552 http://www.medworm.com/index.php?rid=2525553&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19340490%26dopt%3DAbstract Authors: Bitoun E, Davies KE Neurological disorders represent a large share of the disease burden worldwide, and the incidence of age-related forms will continue to rise with life expectancy. Gene targeting has been and will remain a valuable approach to the generation of clinically relevant mouse models from which to elucidate the underlying molecular basis. However, as the aetiology of the majority of these conditions is still unknown, a reverse approach based on large-scale random chemical mutagenesis is now being used in an attempt to identify new genes and associated signalling pathways that control neuronal cell death and survival. Here, we review the characterisation of a novel model of autosomal dominant cerebellar ataxia which shows general growth retardation and develops adul... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2525553 Wed, 01 Apr 2009 23:00:00 +0100 2525553 http://www.medworm.com/index.php?rid=2300768&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19337779%26dopt%3DAbstract We report the results of a retrospective analysis of radiological and clinical findings in 45 cases of midbrain-hindbrain anomalies and review recent advances in embryology and molecular neurogenetics. Among 45 patients with midbrain-hindbrain malformations, 16 cases of molar tooth malformation, 12 of cerebellar hypoplasia, ten of posterior fossa cyst and cerebellar vermian hypoplasia, three of rhombencephalosynapsis, two of Fukuyama congenital muscular dystrophy and two cases of isolated cerebellar dysplasia were identified. Twenty-six patients presented with motor-mental retardation, which was the most common clinical finding. Eleven patients were born to consanguineous parents. The correct diagnosis of cerebellar malformation is important for determining prognosis, the risk of recurrenc... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2300768 Wed, 01 Apr 2009 04:00:00 +0100 2300768 http://www.medworm.com/index.php?rid=2300769&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19308649%26dopt%3DAbstract Authors: Popescu BF, Robinson CA, Chapman LD, Nichol H For the first time, synchrotron rapid-scanning X-ray fluorescence (RS-XRF) was used to simultaneously localize and quantify iron, copper, and zinc in spinal cord and brain in a case of spinocerebellar ataxia (SCA). In the normal medulla, a previously undescribed copper enrichment was seen associated with spinocerebellar fibers and amiculum olivae. This region was virtually devoid of all metals in the SCA case. Regions with neuronal loss and gliosis in the cerebellar cortex, inferior olivary, and dentate nuclei and areas showing loss of myelinated fibers were also low in all metals in SCA compared to control. In contrast, the ventral columns of the spinal cord that exhibited only moderate myelin pallor had increased metal levels. Ir... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2300769 Tue, 24 Mar 2009 04:00:00 +0100 2300769 http://www.medworm.com/index.php?rid=2242339&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19259754%26dopt%3DAbstract In this study, we used the cuprizone mouse model to investigate cerebellar demyelination in young adult male mice. The myelin status was analyzed by immunohistochemistry for proteolipoprotein and electron microscopy. The expression and presence of oligodendrocyte, astroglial, and microglia markers were supplementary studied. Cuprizone intoxication induced an almost complete demyelination of cerebellar nuclei. Cerebellar cortex regions were not (cortical gray matter) or only marginally (cortical white matter) affected. In addition, the affected areas displayed hypertrophic and hyperplastic astrocytosis accompanied by microglia or macrophage invasion. We conclude that cuprizone-induced demyelination pictures cerebellar deep gray matter involvement but not cerebellar cortex pathology as descr... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2242339 Wed, 04 Mar 2009 05:00:00 +0100 2242339 http://www.medworm.com/index.php?rid=2242338&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19259755%26dopt%3DAbstract Authors: Park IS, Lee KJ, Han JW, Lee NJ, Lee WT, Park KA, Rhyu IJ The cerebellum is involved in the learning and retention of motor skills. Using animal and human models, a number of studies have shown that long-term motor skill training induces structural and functional plasticity in the cerebellum. The aim of this study was to investigate whether macroscopic alteration in the volume of cerebellum occurs in basketball players who had learned complex motor skills and practiced them intensively for a long time. Three-dimensional magnetic resonance imaging volumetry was performed in basketball players (n = 19) and healthy controls (n = 20), and the volumes of cerebellum and vermian lobules were compared between two groups. Although there was no macroscopic plasticity detected in the cer... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2242338 Wed, 04 Mar 2009 05:00:00 +0100 2242338 http://www.medworm.com/index.php?rid=2224032&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19247732%26dopt%3DAbstract Authors: Joho RH, Hurlock EC Different subunits of the Kv3 subfamily of voltage-gated potassium (Kv) channels (Kv3.1-Kv3.4) are expressed in distinct neuronal subpopulations in the cerebellum. Behavioral phenotypes in Kv3-null mutant mice such as ataxia with prominent hypermetria and heightened alcohol sensitivity are characteristic of cerebellar dysfunction. Here, we review how the unique biophysical properties of Kv3-type potassium channels, fast activation and fast deactivation that enable cerebellar neurons to generate brief action potentials at high frequencies, affect firing patterns and influence cerebellum-mediated behavior. PMID: 19247732 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2224032 Fri, 27 Feb 2009 05:00:00 +0100 2224032 http://www.medworm.com/index.php?rid=2218125&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19238500%26dopt%3DAbstract Authors: Sadakata T, Furuichi T The postnatal development of the cerebellum is accomplished via a series of cytogenetic and morphogenetic events encoded in the genome. To decipher the underlying genetic basis of these events we have systematized the spatio-temporal gene expression profiles during mouse cerebellar development in the Cerebellar Development Transcriptome Database (CDT-DB). Using the CDT-DB, Ca(2+)-dependent activator protein for secretion 2 (CAPS2 or CADPS2) was identified as a developmentally regulated gene that is predominantly expressed in cerebellar granule cells (GCs) with an expression peak around the first or second postnatal week. CAPS2 protein is concentrated in parallel fiber (PF) terminals and is associated with secretory vesicles containing brain-derived neuro... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2218125 Tue, 24 Feb 2009 05:00:00 +0100 2218125 http://www.medworm.com/index.php?rid=2218124&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19238501%26dopt%3DAbstract Authors: Jankowski J, Miething A, Schilling K, Baader SL Physiological cell death is crucial for matching defined cellular populations within the central nervous system. Whereas the time course of developmental cell death in the central nervous system is well analyzed, information about its precise spatial patterning is scarce. Yet, the latter one is needed to appraise its contribution to circuit formation and refinement. Here, we document that during normal cerebellar development, dying Purkinje cells were highly localized within the vermal midline and in a lobule specific, parasagittal pattern along the whole mediolateral axis. In addition, single hot spots of cell death localized to the caudal declive and ventral lobule IX within the posterolateral fissure. These hot spots of dying ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2218124 Tue, 24 Feb 2009 05:00:00 +0100 2218124 http://www.medworm.com/index.php?rid=2202004&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19224308%26dopt%3DAbstract Authors: Lee NS, Jeong YG The Pogo (pogo/pogo) mouse is a naturally occurring neurological mutant from a Korean wild-type mouse characterized by loss of balance and motor coordination due to dysfunction of the cerebellum. The Pogo mutation is believed to be an allele of P/Q-type calcium channel mutants such as tottering, leaner, and rolling mouse Nagoya. These mutants have been served as mouse models for a group of neurodegenerative diseases. The overall aim of this minireview is to summarize our current understanding of the ataxic Pogo mouse. To address this issue, we first describe the discovery of Pogo mouse and its morphological and behavioral defects. Then, we focus on the abnormal expression of several molecules in the Pogo cerebellum, including tyrosine hydroxylase, glutamate, c... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2202004 Wed, 18 Feb 2009 05:00:00 +0100 2202004 http://www.medworm.com/index.php?rid=2202003&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19224309%26dopt%3DAbstract Authors: Vaillant C, Monard D The morphogenetic factor Sonic hedgehog (SHH) has been discovered as one of the masterplayers in cerebellar patterning and was subjected to intensive investigation during the last decade. During early postnatal development, this continuously secreted cholesterol-modified protein drives the expansion of the largest neuronal population of the brain, the granular cells. Moreover, it acts on Bergmann glia differentiation and would potentially affect Purkinje cells homeostasis at adult age. The cerebellar cortex constituted an ideal developmental model to dissect out the upstream mechanisms and downstream targets of this complex pathway. Its deep understanding discloses some of the mechanistic disorders underlying pediatric tumorigenesis, congenital ataxia, and... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2202003 Wed, 18 Feb 2009 05:00:00 +0100 2202003 http://www.medworm.com/index.php?rid=2184401&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19212797%26dopt%3DAbstract Authors: Lo YL, Fook-Chong S, Chan LL, Ong WY Execution of rapid ballistic movement is characterized by triphasic, alternating electromyographic bursts in agonist (AG) and antagonist (ANT) muscles. The ability to rapidly initiate movement and cancel ongoing action is a basic requirement for efficient control of motor function. Normal functioning of the cerebellum is necessary for the generation of AG and ANT muscle activity that should be both of appropriate magnitude and timing to control the dynamic phase of arm movements. We studied AG, ANT reaction time (RT), and RT differences in both motor activation (MA) and motor cancellation (MC) tasks, in response to an auditory stimulus. The results showed that right cerebellar transcranial magnetic stimulation (TMS) with a horizontally appl... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2184401 Wed, 11 Feb 2009 05:00:00 +0100 2184401 http://www.medworm.com/index.php?rid=2131935&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19165551%26dopt%3DAbstract In this study, we identify phospholipase C (PLC) beta4 as an antigenic marker of a novel subset of UBCs. Double immunolabeling reveals that none of the CR+ subset expresses PLCbeta4. In contrast, most members of the mGluR1alpha subset also express PLCbeta4. In addition, 65% of the PLCbeta4+ subset does not express mGluR1alpha. Thus, there are three distinct UBC subsets in the mouse cerebellum: CR+/PLCbeta4-/mGluR1alpha-, PLCbeta4+/mGluR1alpha-/CR-, and mGluR1alpha+/PLCbeta4+/CR-. Each has a different topographical distribution, both between lobules and mediolaterally within the vermis. The development of PLCbeta4 expression in UBCs is exclusively postnatal-first seen only at P12 and mature at about 3 weeks. A distinct subset of PLCbeta4+ UBCs is also present in primary cerebellar cultures.... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2131935 Fri, 23 Jan 2009 05:00:00 +0100 2131935 http://www.medworm.com/index.php?rid=2131934&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19165552%26dopt%3DAbstract Authors: Marmolino D, Acquaviva F Friedreich's ataxia (FRDA) is a neurodegenerative disease due to a pathological expansion of a GAA triplet repeat in the first intron of the FXN gene encoding for the mitochondrial protein frataxin. The expansion is responsible for most cases of FRDA through the formation of a nonusual B-DNA structure and heterochromatin conformation that determine a direct transcriptional silencing and the subsequent reduction in frataxin expression. Among other functions, frataxin is an iron chaperone central for the assembly of iron-sulfur clusters in mitochondria; its reduction is associated with iron accumulation in mitochondria, increased cellular sensitivity to oxidative stress and cell damage. There is, nowadays, no effective therapy for FRDA and current therap... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2131934 Fri, 23 Jan 2009 05:00:00 +0100 2131934 http://www.medworm.com/index.php?rid=2122641&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19156474%26dopt%3DAbstract Authors: Brighina F, Palermo A, Panetta ML, Daniele O, Aloisio A, Cosentino G, Fierro B Subtle clinical cerebellar alterations have been found in migraine. Moreover, abnormalities in visual and motor cortex excitability consistent with a lack of inhibitory efficiency have been described in migraine, and it is known that cerebellum exerts an inhibitory control on cerebral cortex. Here, we investigated if impairment of cerebellar activity on motor cortex, i.e. reduced inhibitory control, can be found in migraine. Ten migraineurs with aura and seven healthy controls underwent a transcranial magnetic stimulation (TMS) protocol to investigate the cerebellar inhibitory drive on motor cortex: a conditioning pulse on right cerebellar cortex was delivered 5, 7, 10, 15 ms before a test stimulus ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2122641 Wed, 21 Jan 2009 05:00:00 +0100 2122641 http://www.medworm.com/index.php?rid=2105173&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19139969%26dopt%3DAbstract Authors: Popescu BF, Robinson CA, Rajput A, Rajput AH, Harder SL, Nichol H Synchrotron rapid-scanning X-ray fluorescence (RS-XRF) is employed for the first time to simultaneously map iron, copper, and zinc in the normal cerebellum. The cerebellum is a major repository of metals that are essential to normal function. Therefore, mapping the normal metal distribution is an important first step towards understanding how multiple metals may induce oxidative damage, protein aggregation, and neurotoxicity leading to cerebellar degeneration in a wide range of diseases. We found that cerebellar white and grey matter could be sharply defined based upon the unique metal content of each region. The dentate nucleus was particularly metal-rich with copper localized to the periphery and iron and zinc... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2105173 Tue, 13 Jan 2009 05:00:00 +0100 2105173 http://www.medworm.com/index.php?rid=2102134&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19137382%26dopt%3DAbstract Authors: Hendriks WJ, Dilaver G, Noordman YE, Kremer B, Fransen JA Protein tyrosine phosphatases (PTPs) are central players in many different cellular processes and their aberrant activity is associated with multiple human pathologies. In this review, we present current knowledge on the PTPRR subfamily of classical PTPs that is expressed in neuronal cells and comprises receptor-type (PTPBR7, PTP-SL) as well as cytosolic (PTPPBSgamma-37, PTPPBSgamma-42) isoforms. The two receptor-type isoforms PTPBR7 and PTP-SL both localize in late endosomes and the Golgi area. PTPBR7, however, is additionally localized at the cell surface and on early endosomes. During cerebellar maturation, PTPBR7 expression in developing Purkinje cells ceases and is replaced by PTP-SL expression in the mature Purkin... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2102134 Sat, 10 Jan 2009 05:00:00 +0100 2102134 http://www.medworm.com/index.php?rid=2093441&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19130164%26dopt%3DAbstract This article introduces representative animal models currently used to study various aspects of thyroid hormone, particularly to study the involvement of the thyroid hormone system on the development and functional maintenance of the cerebellum. PMID: 19130164 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2093441 Wed, 07 Jan 2009 05:00:00 +0100 2093441 http://www.medworm.com/index.php?rid=2062673&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19104905%26dopt%3DAbstract Authors: Marmolino D, Acquaviva F, Pinelli M, Monticelli A, Castaldo I, Filla A, Cocozza S Friedreich's ataxia is a neurodegenerative disease due to frataxin deficiency, and thus, drugs increasing the frataxin amount are excellent candidates for therapy. By screening Gene Expression Omnibus profiles, we identified records showing a frataxin response to the peroxisome proliferator-activated receptors gamma (PPAR-gamma) agonist rosiglitazone. We decided to investigate the effect of the PPAR-gamma agonist Azelaoyl PAF on the frataxin protein and mRNA expression profile. We treated human neuroblastoma cells SKNBE and primary fibroblasts from skin biopsies from Friedreich's ataxia (FRDA) patients and healthy controls with the PPAR-gamma agonist Azelaoyl PAF. We show in this paper for the fi... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2062673 Tue, 23 Dec 2008 05:00:00 +0100 2062673 http://www.medworm.com/index.php?rid=2054182&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19089525%26dopt%3DAbstract Authors: Verbeek DS The spinocerebellar ataxia type 23 locus was identified in 2004 based on linkage analysis in a large, two-generation Dutch family. The age of onset ranged 43-56 years and the phenotype was characterized by a slowly progressive, isolated ataxia. Neuropathological examination revealed neuronal loss in the Purkinje cell layer, dentate nuclei, and inferior olives. Ubiquitin-positive intranuclear inclusions were found in nigral neurons, but were considered to be Marinesco bodies. The disease locus on chromosome 20p13-12.3 was found to span a region of approximately 6 Mb of genomic DNA, containing 97 known or predicted genes. To date, no other families have been described that also map to this SCA locus. Direct sequencing of the coding regions of 21 prioritized candidate ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2054182 Wed, 17 Dec 2008 05:00:00 +0100 2054182 http://www.medworm.com/index.php?rid=2041720&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19082682%26dopt%3DAbstract Authors: Hoebeek FE, Khosrovani S, Witter L, De Zeeuw CI Homozygous tottering mice are spontaneous ataxic mutants, which carry a mutation in the gene encoding the ion pore of the P/Q-type voltage-gated calcium channels. P/Q-type calcium channels are prominently expressed in Purkinje cell terminals, but it is unknown to what extent these inhibitory terminals in tottering mice are affected at the morphological and electrophysiological level. Here, we investigated the distribution and ultrastructure of their Purkinje cell terminals in the cerebellar nuclei as well as the activities of their target neurons. The densities of Purkinje cell terminals and their synapses were not significantly affected in the mutants. However, the Purkinje cell terminals were enlarged and had an increased numbe... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2041720 Thu, 11 Dec 2008 05:00:00 +0100 2041720 http://www.medworm.com/index.php?rid=2018788&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19057977%26dopt%3DAbstract In conclusion, the cerebellum seems to play an important role in many higher cognitive functions, especially in learning. There is a suggestion that the earlier the incorrect influence, the more pronounced the problems. PMID: 19057977 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=2018788 Fri, 05 Dec 2008 05:00:00 +0100 2018788 http://www.medworm.com/index.php?rid=2018789&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19052829%26dopt%3DAbstract Authors: Nowak DA, Hufnagel A, Ameli M, Timmann D, Hermsdörfer J When an object is repeatedly grasped and lifted, the balance between grip force (normal to the object's surface) and lift force (tangential to the object's surface) is programmed according to the mechanical object features within a few lifts. Information related to the mechanical object properties is easily transferred in between both hands, and the cerebellum may play an essential role for the integration and generalization of this information. Recently, we have shown that the transfer of weight-related information in between both hands is impaired in cerebellar degeneration (Nowak et al., Neuropsychologia, 43:20-27, 2005). Here, we investigated the role of the cerebellum for the inter-manual transfer of friction-re... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2018789 Thu, 04 Dec 2008 05:00:00 +0100 2018789 http://www.medworm.com/index.php?rid=2007505&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19048357%26dopt%3DAbstract Authors: Shih LY, Chen LF, Kuo WJ, Yeh TC, Wu YT, Tzeng OJ, Hsieh JC It has been suggested that the cerebellum participates in diverse neuropsychological functions by adjusting the sensory information acquired for the connected brain regions to support its processing capabilities. Nevertheless, the knowledge of how the cerebellum is modulated by the sensory information is far from clear. Function magnetic resonance imaging was exploited to investigate how the cerebellum activity and cerebrocerebellum interaction can be affected by the interaction between visual size and duration information during visual duration discrimination. The present findings support the sensory acquisition hypothesis that the cerebellum, together with extensive cortical networks, yields higher activation with i... Cerebellum journals http://www.medworm.com/rss/comments.php?id=2007505 Tue, 02 Dec 2008 05:00:00 +0100 2007505 http://www.medworm.com/index.php?rid=1981869&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19023636%26dopt%3DAbstract Authors: Yiş U, Dirik E, Kurul SH, Eken AG, Başak AN Spinocerebellar ataxia type 2 is a neurodegenerative disease caused by a CAG repeat expansion in the ataxin-2 gene. Gain-of-toxic effects caused by expanded polyglutamine tracts are important for the disease pathogenesis and there is an inverse relationship between the number of CAG repeats and the age of onset and clinical severity. Previously, we reported an extended Turkish family with spinocerebellar ataxia type 2 with several affected members in three generations. Two sisters in this generation showed an earlier age of onset (5 and 7years, respectively) than their father (30years). In this paper, we present a further interesting finding regarding the disease onset and manifestation in the two sisters. Interestingly, ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1981869 Fri, 21 Nov 2008 05:00:00 +0100 1981869 http://www.medworm.com/index.php?rid=1962024&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19009326%26dopt%3DAbstract Authors: Koyama Y, Asahina M, Honma K, Arai K, Hattori T To assess heart rate (HR) regulation in Machado-Joseph disease (MJD), we evaluated HR variability at rest and the initial HR response to standing suddenly in 13 MJD patients and 26 normal control subjects. A head-up tilt (HUT) test involving the monitoring of blood pressure, HR, and cerebral oxy/deoxyhemoglobin concentration was also performed in each participant. There was no significant difference in HR variability at rest between the two groups, but the transient HR rise just after standing suddenly in the MJD group was significantly less than that in the control group (p < 0.01). The HUT test, where each participant was gradually tilted upward, induced a significantly greater HR increase in the MJD group compared with the ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1962024 Fri, 14 Nov 2008 05:00:00 +0100 1962024 http://www.medworm.com/index.php?rid=1962023&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19009327%26dopt%3DAbstract Authors: Alahyane N, Fonteille V, Urquizar C, Salemme R, Nighoghossian N, Pelisson D, Tilikete C Sensory-motor adaptation processes are critically involved in maintaining accurate motor behavior throughout life. Yet their underlying neural substrates and task-dependency bases are still poorly understood. We address these issues here by studying adaptation of saccadic eye movements, a well-established model of sensory-motor plasticity. The cerebellum plays a major role in saccadic adaptation but it has not yet been investigated whether this role can account for the known specificity of adaptation to the saccade type (e.g., reactive versus voluntary). Two patients with focal lesions in different parts of the cerebellum were tested using the double-step target paradigm. Each patient was s... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1962023 Fri, 14 Nov 2008 05:00:00 +0100 1962023 http://www.medworm.com/index.php?rid=1962022&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19009328%26dopt%3DAbstract Authors: Ekerot CF, Jörntell H To understand the function of cerebellar granule cells, we need detailed knowledge about the information carried by their afferent mossy fibers and how this information is integrated by the granule cells. Recently, we made whole cell recordings from granule cells in the non-anesthetized, decerebrate cats. All recordings were made in the forelimb area of the C3 zone for which the afferent and efferent connections and functional organization have been investigated in detail. Major findings of the study were that the mossy fiber input to single granule cells was modality- and receptive field-specific and that simultaneous activity in two and usually more of the afferent mossy fibers were required to activate the granule cell spike. The high threshold fo... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1962022 Fri, 14 Nov 2008 05:00:00 +0100 1962022 http://www.medworm.com/index.php?rid=1956095&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19002744%26dopt%3DAbstract Authors: Leto K, Bartolini A, Rossi F The cerebellar circuits comprise a limited number of neuronal phenotypes embedded in a defined cytoarchitecture and generated according to specific spatio-temporal patterns. The local GABAergic network is composed of several interneuron phenotypes that play essential roles in information processing by modulating the activity of cerebellar cortical inputs and outputs. A major issue in the study of cerebellar development is to understand the mechanisms that underlie the generation of different interneuron classes and regulate their placement in the cerebellar architecture and integration in the cortico-nuclear network. Recent findings indicate that the variety of cerebellar interneurons derives from a single population of multipotent progenitors whos... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1956095 Wed, 12 Nov 2008 05:00:00 +0100 1956095 http://www.medworm.com/index.php?rid=1956094&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19002745%26dopt%3DAbstract Authors: Fatemi SH, Folsom TD, Reutiman TJ, Thuras PD Autism is a neurodevelopmental disorder that is often comorbid with seizures. Gamma-aminobutyric acid (GABA) is the main inhibitory neurotransmitter in brain. GABA(B) receptors play an important role in maintaining excitatory-inhibitory balance in brain and alterations may lead to seizures. We compared levels of GABA(B) receptor subunits GABA(B) receptor 1 (GABBR1) and GABA(B) receptor 2 (GABBR2) in cerebellum, Brodmann's area 9 (BA9), and BA40 of subjects with autism and matched controls. Levels of GABBR1 were significantly decreased in BA9, BA40, and cerebellum, while GABBR2 was significantly reduced in the cerebellum. The presence of seizure disorder did not have a significant impact on the observed reductions in GABA(B) receptor... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1956094 Wed, 12 Nov 2008 05:00:00 +0100 1956094 http://www.medworm.com/index.php?rid=1956096&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19002543%26dopt%3DAbstract Authors: Glickstein M, Doron K In addition to its role in motor control, reflex adaptation, and motor learning, three sorts of evidence have been put forward to support the idea that the cerebellum may also be involved in cognition. Patients with cerebellar lesions are reported to have deficits in performing one or another cognitive task. The cerebellum is often seen to be activated when normal subjects perform such tasks. There are connections to and from areas of the prefrontal cortex that may be involved in cognition. In this paper, we review the anatomical evidence to support the claim. We suggest that there are only minor connections with cognitive areas of the cerebral cortex and that some of the imaging evidence may reflect the cerebellum's role in the control of eye movements r... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1956096 Tue, 11 Nov 2008 05:00:00 +0100 1956096 http://www.medworm.com/index.php?rid=1940521&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18985424%26dopt%3DAbstract Authors: Feng SS, Jaeger D We used the method of dynamic current clamping to determine the properties and function of the SK calcium-dependent K(+) current in neurons of the deep cerebellar nuclei (DCN). As previously reported, block of SK current with apamin leads to bursting of DCN neurons and a steepening of the f-I curve. We show here that the properties of the slow spike afterhyperpolarization are fully controlled by SK current and we derive kinetic properties of this current that explain its action on DCN neurons. Overall, the SK current provides an effective mechanism to tune the regularity of spiking and the f-I curve of DCN neurons. PMID: 18985424 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=1940521 Wed, 05 Nov 2008 05:00:00 +0100 1940521 http://www.medworm.com/index.php?rid=1921810&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18972180%26dopt%3DAbstract Viewing the Cerebellum through the Eyes of Ramón Y Cajal. Cerebellum. 2008 Oct 30; Authors: Sotelo C The modern age in the study of the cerebellum started 120 years ago when Cajal published his first paper with Golgi-impregnated material. In this publication, he selected the cerebellum to initiate his gigantic work aimed at unraveling the complexity of the CNS organization. It was not by chance that he selected the cerebellum but because of the occurrence of specific types of fibers, particularly climbing and mossy afferents and basket fibers. The peculiarity of these fibers offered Cajal one of the clearest situations to envision his "neuron doctrine", which proposes that between the nerve cell processes there is no continuity, only contiguity. In 4 years of intense inves... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1921810 Thu, 30 Oct 2008 04:00:00 +0100 1921810 http://www.medworm.com/index.php?rid=1921809&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18972181%26dopt%3DAbstract Authors: Lawyer G, Nesvåg R, Varnäs K, Okugawa G, Agartz I Magnetic resonance imaging studies frequently report abnormalities of the cerebellar vermis in schizophrenia, though with some discrepancies as to the nature and location of such abnormalities. Imaging studies typically investigate volumetric differences between groups. Yet substantial evidence supports the hypothesis that grey and white matter proportions in the mammalian brain are controlled by scaling relationships. If strong proportional relationships between grey and white matter tissue volumes are observed in the healthy vermis, then disturbances to these proportions might characterize vermian dysmorphology in schizophrenia. Measures of grey and white matter tissue volumes from three anatomical divisions of the ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1921809 Thu, 30 Oct 2008 04:00:00 +0100 1921809 http://www.medworm.com/index.php?rid=1921808&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18972182%26dopt%3DAbstract Authors: Dean P, Porrill J Many current models of the cerebellar cortical microcircuit are equivalent to an adaptive filter using the covariance learning rule. The adaptive filter is a development of the original Marr-Albus framework that deals naturally with continuous time-varying signals, thus addressing the issue of 'timing' in cerebellar function, and it can be connected in a variety of ways to other parts of the system, consistent with the microzonal organization of cerebellar cortex. However, its computational capacities are not well understood. Here we summarise the results of recent work that has focused on two of its intrinsic properties. First, an adaptive filter seeks to decorrelate its (mossy fibre) inputs from a (climbing fibre) teaching signal. This procedure can be used... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1921808 Thu, 30 Oct 2008 04:00:00 +0100 1921808 http://www.medworm.com/index.php?rid=1906681&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18949529%26dopt%3DAbstract Authors: Repici M, Zanjani HS, Gautheron V, Borsello T, Dusart I, Mariani J In the Lurcher mutant mouse (+/Lc), Purkinje cells (PCs) selectively die due to the mutation that converts alanine to threonine in the glutamate ionotropic receptor GRID 2, thus resulting in a constitutively leaky cation channel. This intrinsic cell death determines a target-dependent cell death of granule cells and olivary neurons and cerebellum cytoarchitecture is severely disrupted in the adult Lurcher mutant. Although the +/Lc mutant has been widely characterized, less is known about the molecules involved in +/Lc PC death. We, here, used organotypic cerebellar slice cultures from P0 mice to investigate the role of c-jun N-terminal kinase (JNK) in +/Lc PC death by using D-JNKI1 as very specific tool to inhi... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1906681 Fri, 24 Oct 2008 04:00:00 +0100 1906681 http://www.medworm.com/index.php?rid=1906680&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18949530%26dopt%3DAbstract Authors: Timmann D, Brandauer B, Hermsdörfer J, Ilg W, Konczak J, Gerwig M, Gizewski ER, Schoch B High-resolution structural magnetic resonance imaging (MRI) has become a powerful tool in human cerebellar lesion studies. Structural MRI is helpful to analyse the localisation and extent of cerebellar lesions and to determine possible extracerebellar involvement. Functionally meaningful correlations between a cerebellar lesion site and behavioural data can be obtained both in subjects with degenerative as well as focal cerebellar disorders. In this review, examples are presented which demonstrate that MRI-based lesion-symptom mapping is helpful to study the function of cerebellar cortex and cerebellar nuclei. Behavioural measures were used which represent two main areas of cerebellar... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1906680 Thu, 23 Oct 2008 04:00:00 +0100 1906680 http://www.medworm.com/index.php?rid=1902991&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18941861%26dopt%3DAbstract Authors: Molinari M, Chiricozzi FR, Clausi S, Tedesco AM, De Lisa M, Leggio MG The idea that cerebellar processing is required in a variety of cognitive functions is well accepted in the neuroscience community. Nevertheless, the definition of its role in the different cognitive domains remains rather elusive. Current data on perceptual and cognitive processing are reviewed with special emphasis on cerebellar sequencing properties. Evidences, obtained by neurophysiological and neuropsychological lesion studies, converge in highlighting comparison of temporal and spatial information for sequence detection as the key stone of cerebellar functioning across modalities. The hypothesis that sequence detection might represent the main contribution of cerebellar physiology to brain functioning ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1902991 Wed, 22 Oct 2008 04:00:00 +0100 1902991 http://www.medworm.com/index.php?rid=1896512&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18937022%26dopt%3DAbstract Authors: Uttner I, Tumani H, Arnim CV, Brettschneider J Superficial siderosis is a rare disease characterized by cerebellar ataxia and sensorineural deafness. So far, there are only few reports on cognitive dysfunctions associated with superficial siderosis. Using a comprehensive psychometric test battery, we describe the cognitive impairments in a 65-year-old woman fulfilling the clinical and magnetic resonance imaging criteria of superficial siderosis. The neuropsychological findings included deterioration of primary and episodic memory, behavioral and linguistic changes characterized by social disinhibition, and decreased verbal fluency. These findings may correspond to the "cerebellar cognitive affective syndrome" which was suggested to occur in patients with selective cerebellar l... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1896512 Tue, 21 Oct 2008 04:00:00 +0100 1896512 http://www.medworm.com/index.php?rid=1896514&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18931885%26dopt%3DAbstract Authors: Rasmussen A, Jirenhed DA, Hesslow G Classical blink conditioning is known to depend critically on the cerebellum and the relevant circuitry is gradually being unravelled. Several lines of evidence support the theory that the conditioned stimulus is transmitted by mossy fibers to the cerebellar cortex whereas the unconditioned stimulus is transmitted by climbing fibers. This view has been dramatically confirmed by recent Purkinje cell recordings during training with a classical conditioning paradigm. We have tracked the activity of single Purkinje cells with microelectrodes for several hours in decerebrate ferrets during learning, extinction, and relearning. Paired peripheral forelimb and periocular stimulation, as well as paired direct stimulation of cerebellar afferent pathwa... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1896514 Sat, 18 Oct 2008 04:00:00 +0100 1896514 http://www.medworm.com/index.php?rid=1896513&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18931886%26dopt%3DAbstract Authors: Tal Z, Chorev E, Yarom Y The cerebellum has been the focus of extensive research for more than a century. However, its functional role is still under debate. The comprehensive description of its anatomy and physiology seem to deepen rather than resolve the controversy about its function. Recently, it was shown that Purkinje cells' (PC) membrane potential is bistable and can be found in one of two states: periods of simple spike bursting ("up state"), followed by periods of electrical quiescence and hyperpolarized membrane potential ("down state"). This bistability, which challenges the current dogma regarding the functional organization of the cerebellum, has immediate implications on the mode by which the cerebellar cortex reads incoming input. The well-documented, all-or-non... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1896513 Sat, 18 Oct 2008 04:00:00 +0100 1896513 http://www.medworm.com/index.php?rid=1884173&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18923818%26dopt%3DAbstract Authors: Koibuchi N Thyroid hormone plays a crucial role in cerebellar development. Deficiency of thyroid hormone results in abnormal cerebellar growth and differentiation. In rodent, thyroid hormone mainly affects cerebellar development during the first 2 weeks of postnatal life. Thyroid hormone replacement after such critical period cannot fully rescue abnormal cerebellar development induced by perinatal hypothyroidism. Thyroid hormone receptor (TR) is a ligand-regulated transcription factor that binds to a specific DNA sequence called thyroid-hormone-responsive element. TR recruits various coregulators such as coactivator and corepressor in a ligand-dependent manner to regulate transcription of target genes. In cerebellum, at least three different TRs are expressed in a cell-specifi... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1884173 Thu, 16 Oct 2008 04:00:00 +0100 1884173 http://www.medworm.com/index.php?rid=1880993&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18855092%26dopt%3DAbstract Authors: Miall RC, King D An exciting hypothesis about the cerebellum is that its role is one of state estimation-a process that combines efferent copies of motor commands with afferent sensory signals to produce a representation of the current status of the peripheral motor system. Sensory inputs alone cannot provide a perfect state signal because of inevitable delays in their afferent pathways. We have recently reported the effects of transcranial magnetic stimulation (TMS) over the ipsilateral cerebellum as healthy subjects made rapid reaching movements towards visually defined targets (Miall et al. in PLoS Biology 5:2733-2744, 2007). Errors in the initial direction and in the final finger position of this reach-to-target movement were consistent with the reaching movements being pl... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1880993 Wed, 15 Oct 2008 04:00:00 +0100 1880993 http://www.medworm.com/index.php?rid=1880992&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18855093%26dopt%3DAbstract Authors: Manto M Research on the cerebellum is evolving rapidly. The exquisiteness of the cerebellar circuitry with a unique geometric arrangement has fascinated researchers from numerous disciplines. The painstaking works of pioneers of these last two centuries, such as Rolando, Flourens, Luciani, Babinski, Holmes, Cajal, Larsell, or Eccles, still exert a strong influence in the way we approach cerebellar functions. Advances in genetic studies, detailed molecular and cellular analyses, profusion of brain imaging techniques, emergence of behavioral assessments, and reshaping of models of cerebellar function are generating an immense amount of knowledge. Simultaneously, a better definition of cerebellar disorders encountered in the clinic is emerging. The essentials of a trans-disciplin... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1880992 Wed, 15 Oct 2008 04:00:00 +0100 1880992 http://www.medworm.com/index.php?rid=1880991&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18855094%26dopt%3DAbstract Authors: Yoshida K, Shimizu Y, Morita H, Okano T, Sakai H, Ohata T, Matsumoto N, Nakamura K, Tazawa KI, Ohara S, Tabata K, Inoue A, Sato S, Shimojima Y, Hattori T, Ushiyama M, Ikeda SI 16q22.1-linked autosomal dominant cerebellar ataxia (16q-ADCA) is a recently defined subtype of ADCA identified by a disease-specific C/T substitution in the 5' untranslated region of the puratrophin-1 gene. In Nagano, the central mountainous district of the main island of Japan, 16q-ADCA and spinocerebellar ataxia type 6 (SCA6) are the most and second most prevalent subtypes of ADCA, respectively. Both subtypes are classified into Harding's ADCA III, but little attention has been given to the differences in the severity and progression rate of cerebellar ataxia between 16q-ADCA and SCA6. We investigated... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1880991 Wed, 15 Oct 2008 04:00:00 +0100 1880991 http://www.medworm.com/index.php?rid=1880990&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18855095%26dopt%3DAbstract Authors: Liu SJ, Lachamp P, Liu Y, Savtchouk I, Sun L Inhibitory transmission controls the action potential firing rate and pattern of Purkinje cell activity in the cerebellum. A long-term change in inhibitory transmission is likely to have a profound effect on the activity of cerebellar neuronal circuits. However, little is known about how neuronal activity regulates synaptic transmission in GABAergic inhibitory interneurons (stellate/basket cells) in the cerebellar cortex. We have examined how glutamate released from parallel fibers (the axons of granule cells) influences postsynaptic alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionate (AMPA) receptors in stellate cells and modulates gamma-aminobutyric acid (GABA) release from these neurons. First, we found that burst stimulation of... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1880990 Wed, 15 Oct 2008 04:00:00 +0100 1880990 http://www.medworm.com/index.php?rid=1880989&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18855096%26dopt%3DAbstract Authors: Schutter DJ, van Honk J Several lines of evidence suggest that the cerebellum may play a role in the regulation of emotion. The aim of this study was to investigate the hypothesis that inhibition of cerebellar function using slow repetitive transcranial magnetic stimulation (rTMS) would lead to increased negative mood as a result of impaired emotion regulation. In a randomized counterbalanced within-subjects design, 12 healthy young right-handed volunteers received 20 min of cerebellar, occipital, or sham 1 Hz rTMS on three separate days. Mood state inventories were acquired prior to and immediately after rTMS and after an emotion regulation task (ERT). In the ERT, participants were instructed to either look at aversive and neutral scenes, or to suppress the negative feelings ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1880989 Wed, 15 Oct 2008 04:00:00 +0100 1880989 http://www.medworm.com/index.php?rid=1873613&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18850257%26dopt%3DAbstract Authors: Cendelín J, Korelusová I, Vožeh F Lurcher mutant mice represent a model of olivocerebellar degeneration. They are used to investigate cerebellar functions, consequences of cerebellar degeneration and methods of therapy influencing them. The aim of the work was to assess the effect of foetal cerebellar graft transplantation, repeated enforced physical activity and the combination of both these types of treatment on motor skills, spontaneous motor activity and spatial learning ability in adult B6CBA Lurcher mice. Foetal cerebellar grafts were applied into the cerebellum of Lurchers in the form of solid tissue pieces. Enforced motor activity was realised through rotarod training. Motor functions were examined using bar, ladder and rotarod tests. Spatial learning w... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1873613 Sat, 11 Oct 2008 04:00:00 +0100 1873613 http://www.medworm.com/index.php?rid=1873612&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18850258%26dopt%3DAbstract Authors: Ebner TJ, Pasalar S Feed forward control and estimates of the future state of the motor system are critical for fast and coordinated movements. One framework for generating these predictive signals is based on the central nervous system implementing internal models. Internal models provide for representations of the input-output properties of the motor apparatus or their inverses. It has been widely hypothesized that the cerebellum acquires and stores internal models of the motor apparatus. The results of psychophysical, functional imaging and transcranial magnetic stimulation studies in normal subjects support this hypothesis. Also, the deficits in patients with cerebellar dysfunction can be attributed to a failure of predictive feed forward control and/or to accurately estim... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1873612 Sat, 11 Oct 2008 04:00:00 +0100 1873612 http://www.medworm.com/index.php?rid=1869025&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18846412%26dopt%3DAbstract Authors: Carrillo F, Schneider SA, Taylor AM, Srinivasan V, Kapoor R, Bhatia KP Ataxia telangiectasia (A-T) typically presents with early-onset progressive cerebellar ataxia, oculomotor apraxia and later, oculo-cutaneous telangiectasia. Extrapyramidal symptoms, apart from chorea, are rare. In this paper, we report a case of A-T with an atypically mild and slowly progressive disease course. Although by history there was mild gait clumsiness in early childhood, the leading problem was that of dystonia with onset at age 15, in the absence of gross gait imbalance or ocular motor apraxia. Dystonia was generalized and with prominent oromandibular involvement. Unusually, a leash of telangiectasia was present on the posterior pharyngeal wall, while other features frequently associated with A-T... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1869025 Fri, 10 Oct 2008 04:00:00 +0100 1869025 http://www.medworm.com/index.php?rid=1857063&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18836790%26dopt%3DAbstract Authors: PMID: 18836790 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=1857063 Fri, 03 Oct 2008 04:00:00 +0100 1857063 http://www.medworm.com/index.php?rid=1789292&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18787912%26dopt%3DAbstract Authors: Hadjivassiliou M, Sanders DS, Woodroofe N, Williamson C, Grünewald RA Gluten ataxia is an immune-mediated disease triggered by the ingestion of gluten in genetically susceptible individuals. It should be considered in the differential diagnosis of all patients with idiopathic sporadic ataxia. Early diagnosis and treatment with a gluten free diet can improve ataxia and prevent its progression. Readily available and sensitive markers of gluten ataxia include antigliadin antibodies. IgA deposits against TG2 in the small bowel and at extraintestinal sites are proving to be additional reliable and perhaps more specific markers of the whole spectrum of gluten sensitivity. They may also hold the key to its pathogenesis. PMID: 18787912 [PubMed - as supplied by publisher] (Sou... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1789292 Fri, 12 Sep 2008 04:00:00 +0100 1789292 http://www.medworm.com/index.php?rid=1786585&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18784973%26dopt%3DAbstract Authors: Singaravelu K, Lohr C, Deitmer JW Store-operated Ca(2+) entry (SOCE) has been extensively studied in non-neuronal cells, such as glial cells and smooth muscle cells, in which Ca(2+)-independent phospholipase A(2) (iPLA(2)) has been shown to play a key role in the regulation of SOCE channels. In the present study, we have investigated the role of iPLA(2) for store-operated Ca(2+) entry in rat cerebellar granule neurons in acute brain slices using confocal Ca(2+) imaging. Depletion of Ca(2+) stores by cyclopiazonic acid (CPA) induced a Ca(2+) influx, which could be inhibited by SOCE channel blockers 2-aminoethoxy-diphenylborate (2-APB) and 3,5-bistrifluoromethyl pyrazole derivative (BTP2), but not by the voltage-operated Ca(2+) channel blocker diltiazem and by the Na+ channel bl... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1786585 Thu, 11 Sep 2008 04:00:00 +0100 1786585 http://www.medworm.com/index.php?rid=1776279&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18777197%26dopt%3DAbstract Authors: Shen Y, Mishra R, Mani S, Meiri KF Growth-associated protein 43 (GAP-43) is required for development of a functional cerebral cortex in vertebrates; however, its role in cerebellar development is not well understood. Recently, we showed that absence of GAP-43 caused defects in proliferation, differentiation, and polarization of cerebellar granule cells. In this paper, we show that absence of GAP-43 causes defects in cerebellar patterning that reflect both cell-autonomous and non-autonomous functions. Cell-autonomous effects of GAP-43 impact precursor proliferation and axon targeting: In its absence, (1) proliferation of granule cell precursors in response to sonic hedgehog and fibroblast growth factor is inhibited, (2) proliferation of neuroepithelial precursors is inhibited, ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1776279 Sat, 06 Sep 2008 04:00:00 +0100 1776279 http://www.medworm.com/index.php?rid=1765372&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18769991%26dopt%3DAbstract Authors: Mariotti C, Brusco A, Di Bella D, Cagnoli C, Seri M, Gellera C, Di Donato S, Taroni F We have recently mapped the spinocerebellar ataxia type 28 (SCA28) locus on chromosome 18p11.22 in a four-generation Italian family. The clinical phenotype in affected individuals of this family was characterized by juvenile onset, slowly progressive gait and limb ataxia, dysarthria, hyperreflexia at lower limbs, nystagmus, and ophthalmoparesis. The mean age at onset was 19.5 years, and no evidence of anticipation between generations was observed. The disease locus on chromosome 18p11.22-q11.2 was found to span a region of 7.9 Mb of genomic DNA. Direct sequencing of candidate genes within the critical interval led to the identification of a heterozygous point mutation in one of them. The muta... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1765372 Thu, 04 Sep 2008 04:00:00 +0100 1765372 http://www.medworm.com/index.php?rid=1765371&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18769992%26dopt%3DAbstract Authors: Zhang R, Zhang X, Bian F, Pu XA, Schilling K, Oberdick J Pcp2(L7) is a Purkinje cell-specific GoLoco domain protein that modulates activation of Galphai/o proteins by G protein-coupled receptors. A likely downstream effector of this pathway is the P-type Ca(2+) channel, and thereby, the intrinsic electrophysiology of Purkinje cells could be modulated by Pcp2(L7). It has long been known that the Pcp2(L7) mRNA is abundantly localized in dendrites, suggesting the possibility of distal synthesis and local changes in levels of the protein. As a first step to uncover the trafficking and translational mechanisms for this mRNA, we have begun identifying the cis-acting sequences important for its localization in dendrites. Using expression of modified transgenes in vivo, we show that t... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1765371 Thu, 04 Sep 2008 04:00:00 +0100 1765371 http://www.medworm.com/index.php?rid=1635784&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18633686%26dopt%3DAbstract Authors: Delgado L, Schmachtenberg O Gamma-aminobutyric acid (GABA) is the principal inhibitory neurotransmitter in the central nervous system. Its role is especially prominent in the cerebellum, where most neuron types are GABAergic. However, little is known about its function in the cerebellum of teleost fish, which is only partly homologous to its mammalian counterpart. Here, we investigated the expression and distribution of GABA, the GABA-synthesizing enzyme glutamic acid decarboxylase 65 (GAD65), and the receptor subunits GABA(Aalpha1) and GABA(B1) in the cerebellum of adult zebrafish. GABA and GAD65 presented a similar expression pattern that comprised the molecular layer, Purkinje cells and groups of presumed Golgi cells in the granular layer, both in the cerebellar corpus and ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1635784 Thu, 17 Jul 2008 04:00:00 +0100 1635784 http://www.medworm.com/index.php?rid=1603600&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18612713%26dopt%3DAbstract Authors: Koibuchi N Cerebellar development and plasticity is involved in various epigenetic processes that activate specific genes at different time point. The epigenetic influences include humoral influences from endocrine cells of peripheral organs. A number of hormone receptors are expressed in cerebellum, and cerebellar function is greatly influenced by hormonal status. Furthermore, recent studies have shown that some of such substances are produced locally and affect through their specific hormone receptors. The aim of this special issue was to introduce several key features of hormones and their receptors to regulate cerebellar development and plasticity. The contribution covers thyroid/steroid hormone systems including orphan receptors and co-regulators, neurosteroids, and trans... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1603600 Wed, 09 Jul 2008 04:00:00 +0100 1603600 http://www.medworm.com/index.php?rid=1597578&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18607668%26dopt%3DAbstract Authors: Kakizawa S, Moriguchi S, Ikeda A, Iino M, Takeshima H Junctophilins (JPs) contribute to the formation of junctional membrane complexes between the plasma membrane and the endoplasmic/sarcoplasmic reticulum, and provide a structural platform for channel communication during excitation-contraction coupling in muscle cells. In the brain, two neuronal JP subtypes are widely expressed in neurons. Recent studies have defined the essential role of neural JPs in the communication between cell-surface and intracellular channels, which modulates the excitability and synaptic plasticity of neurons in the cerebellum and hippocampus. PMID: 18607668 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=1597578 Tue, 08 Jul 2008 04:00:00 +0100 1597578 http://www.medworm.com/index.php?rid=1577910&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18597149%26dopt%3DAbstract Authors: Timmann D, Lee P, Watts S, Hore J Cerebellar subjects and unskilled throwers cannot produce fast arm movements when throwing. We investigated the arm movement kinematics associated with this lack of skill. Cerebellar subjects and matched controls, and skilled throwers throwing with their skilled (dominant) and unskilled (nondominant) arms, were instructed to make slow, medium, and fast 3-D overarm throws from a sitting position. Only the fast throws were analyzed in detail. Joint motions were computed from angular positions of arm segments recorded with search coils. When throwing, both the cerebellar group and the unskilled-arm group had slower arm movements, and slower elbow extension and wrist flexion velocities than their reference groups. They also had similar magnitudes ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1577910 Thu, 03 Jul 2008 04:00:00 +0100 1577910 http://www.medworm.com/index.php?rid=1569417&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18594939%26dopt%3DAbstract Authors: Yiş U, Kurul SH, Cakmakçi H, Dirik E Although cerebellitis is common in childhood but cerebellitis with cerebellar swelling is rarely reported. Pulsed high dose methylprednisolone treatment is the choice of treatment for cases who have non-progressive symptoms. An 8-year-old girl presented acutely with vertigo, headache, and vomiting. Brain magnetic resonance imaging showed marked bilateral cerebellar swelling with increased signal on T2-weighted imaging. Following treatment with standard dexamethasone dose, the clinical and radiological signs resolved in 1 week. We conclude that standard dexamethasone treatment should be used in mild cases of acute cerebellitis in order to avoid adverse reactions of pulsed high dose methylprednisolone treatment. PMID: 18594939 ... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1569417 Wed, 02 Jul 2008 04:00:00 +0100 1569417 http://www.medworm.com/index.php?rid=1569422&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18592331%26dopt%3DAbstract Authors: Thoma P, Bellebaum C, Koch B, Schwarz M, Daum I The cerebellum has recently been discussed in terms of a possible involvement in reward-based associative learning. To clarify the cerebellar contribution, eight patients with focal vascular lesions of the cerebellum and a group of 24 healthy subjects matched for age and IQ were compared on a range of different probabilistic outcome-based associative learning tasks, assessing acquisition, reversal, cognitive transfer, and generalization as well as the effect of reward magnitude. Cerebellar patients showed intact acquisition of stimulus contingencies, while reward-based reversal learning was significantly impaired. In addition, the patients showed slower acquisition of new stimulus contingencies in a second reward-based learning t... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1569422 Tue, 01 Jul 2008 04:00:00 +0100 1569422 http://www.medworm.com/index.php?rid=1569421&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18592332%26dopt%3DAbstract Authors: Alvarez MI, Lacruz C, Toledano-Díaz A, Monleón E, Monzón M, Badiola JJ, Toledano A Calretinin (CR)-immunopositive cells and fibers in the cerebellar cortex (vermal archicerebellum-lobules X and IX-and neocerebellum-lobules VIIb and VIII) of two and 4-year-old Manchega and Rasa Aragonesa sheep were studied. CR-immunoreactivity was seen in subsets of all neurons and afferent fibers described in the cerebellar cortex. Generally, immunopositive cells were seen in very high densities in lobules X and IX, and in low density in lobule VIIb. Apparently, all unipolar brush cells were CR-immunopositive and showed a greater variety of shape than had been reported in other species. CR-immunoreactivity of Purkinje cells was either absent or varied from low to medium intensit... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1569421 Tue, 01 Jul 2008 04:00:00 +0100 1569421 http://www.medworm.com/index.php?rid=1569420&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18592333%26dopt%3DAbstract In this study, we investigated the effectiveness of minocycline treatment in reducing remote cell death. Glial activation and neuronal loss in precerebellar stations following cerebellar lesion were investigated using immunohistochemistry and Western blot techniques. Our results show that minocycline was effective in reducing microglial activations in axotomized precerebellar nuclei, but failed to mitigate either astrocytic response or neuronal loss. This finding supports the role of minocycline in modulating inflammatory response after CNS lesion and suggests its ineffectiveness in influencing degenerative phenomena in areas remote from the primary lesion site. PMID: 18592333 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=1569420 Tue, 01 Jul 2008 04:00:00 +0100 1569420 http://www.medworm.com/index.php?rid=1569419&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18592334%26dopt%3DAbstract SCA13. Cerebellum. 2008 Jul 1; Authors: Waters MF, Pulst SM Spinocerebellar ataxia 13 (SCA13), initially described in a four-generation French family, has now also been characterized in a large Filipino pedigree. Ongoing investigations continue to identify additional SCA13 families and individuals. Recently, studies have shown that mutations in the voltage-gated potassium channel KCNC3 are causative for SCA13. Sequence analysis of KCNC3 revealed mutations 1554G-->A (R420H) in the Filipino and 1639C-->A (F448L) in the French pedigrees. Both mutations alter KCNC3 function in a Xenopus laevis oocyte expression system. KCNC3 (R420H), located in the voltage sensor of the channel, has no detectable channel activity when expressed alone, and strong dominant negative effects when... Cerebellum journals http://www.medworm.com/rss/comments.php?id=1569419 Tue, 01 Jul 2008 04:00:00 +0100 1569419 http://www.medworm.com/index.php?rid=1569418&cid=s_37290_168_f&fid=37290&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18592335%26dopt%3DAbstract This study provides compelling anatomical evidence for a common link between the cerebellum and the hypothalamo-pituitary axis. Dysfunction of this link occurs in the rare genetic ataxia disorders-Gordon Holmes syndrome and Boucher-Neuhauser syndrome. PMID: 18592335 [PubMed - as supplied by publisher] (Source: Cerebellum) Cerebellum journals http://www.medworm.com/rss/comments.php?id=1569418 Tue, 01 Jul 2008 04:00:00 +0100 1569418