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| Research article summary (published 29 Sep 2002): |
The vestibulo-ocular reflex and velocity storage in spinocerebellar ataxia 8.
Full Abstract
The autosomal dominant spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases characterized by progressive instability of posture and gait, incoordination, ocular motor dysfunction, and dysarthria due to degeneration of cerebellar and brainstem neurons. Among the more than 20 genetically distinct subtypes, SCA8 is one of several wherein clinical observations indicate that cerebellar dysfunction is primary, and there is little evidence for other CNS involvement. The aim of the present work was to study the decay of the horizontal vestibulo-ocular reflex (VOR) after a short period of constant acceleration to understand the pathophysiology of the VOR due to cerebellar Purkinje cell degeneration in SCA8. The VOR was recorded in patients with genetically defined SCA8 during rotation in the dark. Moderate to severely affected patients had a qualitatively intact VOR, but there were quantitative differences in the gain and dynamics compared to normal controls. During angular velocity ramp rotations, there was a reversal in the direction of the VOR that was more pronounced in SCA8 compared to controls. Modeling studies indicate that there are significant changes in the velocity storage network, including abnormal feedback of an eye position signal into the network that contributes to this reversal. These and other results will help to identify features that are diagnostic for SCA subtypes and provide new information about selective vulnerability of neurons controlling vestibular reflexes.
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Author information
Author/s: Anderson, J H (JH); Yavuz, M C (MC); Kazar, B M (BM); Christova, P (P); Gomez, C M (CM);
Affiliation: Department of Otolaryngology, University of Minnesota, Mayo Mail Code 396, 420 Delaware St. S.E., Minneapolis, MN 55455, USA.
Grants: NS37211 (Agency:United States NINDS)
Journal and publication information
Publication Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
Journal: Archives italiennes de biologie (Arch Ital Biol), published in Italy. (Language: eng)
Reference: 2002-Oct; vol 140 (issue 4) : pp 323-9
Dates: Created 2002/09/16; Completed 2003/01/22; Revised 2007/11/14;
PMID: 12228985, status: MEDLINE (last retrieval date: 11/6/2008)
Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.
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