Pathoanatomy of Cerebellar Degeneration in Spinocerebellar Ataxia Type 2 (SCA2) and Type 3 (SCA3) View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2011-12-24

AUTHORS

W. Scherzed, E. R. Brunt, H. Heinsen, R. A. de Vos, K. Seidel, K. Bürk, L. Schöls, G. Auburger, D. Del Turco, T. Deller, H. W. Korf, W. F. den Dunnen, U. Rüb

ABSTRACT

The cerebellum is one of the well-known targets of the pathological processes underlying spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3). Despite its pivotal role for the clinical pictures of these polyglutamine ataxias, no pathoanatomical studies of serial tissue sections through the cerebellum have been performed in SCA2 and SCA3 so far. Detailed pathoanatomical data are an important prerequisite for the identification of the initial events of the underlying disease processes of SCA2 and SCA3 and the reconstruction of its spread through the brain. In the present study, we performed a pathoanatomical investigation of serial thick tissue sections through the cerebellum of clinically diagnosed and genetically confirmed SCA2 and SCA3 patients. This study demonstrates that the cerebellar Purkinje cell layer and all four deep cerebellar nuclei consistently undergo considerable neuronal loss in SCA2 and SCA3. These cerebellar findings contribute substantially to the pathogenesis of clinical symptoms (i.e., dysarthria, intention tremor, oculomotor dysfunctions) of SCA2 and SCA3 patients and may facilitate the identification of the initial pathological alterations of the pathological processes of SCA2 and SCA3 and reconstruction of its spread through the brain. More... »

PAGES

749-760

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s12311-011-0340-8

DOI

http://dx.doi.org/10.1007/s12311-011-0340-8

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1049428761

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/22198871


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35 schema:description The cerebellum is one of the well-known targets of the pathological processes underlying spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3). Despite its pivotal role for the clinical pictures of these polyglutamine ataxias, no pathoanatomical studies of serial tissue sections through the cerebellum have been performed in SCA2 and SCA3 so far. Detailed pathoanatomical data are an important prerequisite for the identification of the initial events of the underlying disease processes of SCA2 and SCA3 and the reconstruction of its spread through the brain. In the present study, we performed a pathoanatomical investigation of serial thick tissue sections through the cerebellum of clinically diagnosed and genetically confirmed SCA2 and SCA3 patients. This study demonstrates that the cerebellar Purkinje cell layer and all four deep cerebellar nuclei consistently undergo considerable neuronal loss in SCA2 and SCA3. These cerebellar findings contribute substantially to the pathogenesis of clinical symptoms (i.e., dysarthria, intention tremor, oculomotor dysfunctions) of SCA2 and SCA3 patients and may facilitate the identification of the initial pathological alterations of the pathological processes of SCA2 and SCA3 and reconstruction of its spread through the brain.
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43 SCA3
44 SCA3 patients
45 alterations
46 ataxia
47 brain
48 cell layer
49 cerebellar Purkinje cell layer
50 cerebellar degeneration
51 cerebellar findings
52 cerebellar nuclei
53 cerebellum
54 clinical picture
55 clinical symptoms
56 data
57 deep cerebellar nuclei
58 degeneration
59 disease process
60 events
61 findings
62 identification
63 important prerequisite
64 initial event
65 investigation
66 layer
67 loss
68 neuronal loss
69 nucleus
70 pathoanatomical data
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73 pathoanatomy
74 pathogenesis
75 pathological alterations
76 pathological processes
77 patients
78 picture
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83 process
84 reconstruction
85 role
86 sections
87 serial tissue sections
88 spinocerebellar ataxia type 2
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91 symptoms
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