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The Cerebellum
Erschienen in: The Cerebellum 2/2011

01.06.2011

Loss of Intrinsic Organization of Cerebellar Networks in Spinocerebellar Ataxia Type 1: Correlates with Disease Severity and Duration

verfasst von: Ana Solodkin, Eitan Peri, E. Elinor Chen, Eshel Ben-Jacob, Christopher M. Gomez

Erschienen in: The Cerebellum | Ausgabe 2/2011

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Abstract

The spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of cerebellar degenerative disorders, characterized by progressive gait unsteadiness, hand incoordination, and dysarthria. The mutational mechanism in SCA1, a dominantly inherited form of SCA, consists of an expanded trinucleotide CAG repeat. In SCA1, there is loss of Purkinje cells, neuronal loss in dentate nucleus, olives, and pontine nuclei. In the present study, we sought to apply intrinsic functional connectivity analysis combined with diffusion tensor imaging to define the state of cerebellar connectivity in SCA1. Our results on the intrinsic functional connectivity in lateral cerebellum and thalamus showed progressive organizational changes in SCA1 noted as a progressive increase in the absolute value of the correlation coefficients. In the lateral cerebellum, the anatomical organization of functional clusters seen as parasagittal bands in controls is lost, changing to a patchy appearance in SCA1. Lastly, only fractional anisotropy in the superior peduncle and changes in functional organization in thalamus showed a linear dependence to duration and severity of disease. The present pilot work represents an initial effort describing connectivity biomarkers of disease progression in SCA1. The functional changes detected with intrinsic functional analysis and diffusion tensor imaging suggest that disease progression can be analyzed as a disconnection syndrome.
Fußnoten
1
Anatomically [21], the lateral cerebellum included the following lobules: V lat (culmen inferior), VI lat (simplex), VIIA_Crus I/1,2 (superior semilunar lobule), VIIA_Crus II/1,2 (inferior semilunar lobule), VIIB lat (paramedian/gracilis), VIIIA lat (biventer, pars copularis), and VIIIB lat (biventer, pars paraflocculus dorsalis).
 
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Metadaten
Titel
Loss of Intrinsic Organization of Cerebellar Networks in Spinocerebellar Ataxia Type 1: Correlates with Disease Severity and Duration
verfasst von
Ana Solodkin
Eitan Peri
E. Elinor Chen
Eshel Ben-Jacob
Christopher M. Gomez
Publikationsdatum
01.06.2011
Verlag
Springer-Verlag
Erschienen in
The Cerebellum / Ausgabe 2/2011
Print ISSN: 1473-4222
Elektronische ISSN: 1473-4230
DOI
https://doi.org/10.1007/s12311-010-0214-5

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