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Spinocerebellar ataxia 6 (SCA6) is a hereditary disease characterized by a trinucleotide repeat expansion in the CACNA1A gene and late-onset bilateral cerebellar atrophy. It is unclear if there is significant pathology outside of the cerebellum. We used transcranial magnetic stimulation to assess sensorimotor cortical circuits and cortical plasticity in 8 SCA6 patients and 8 age-matched controls. Behavioral performance was assessed using a rhythmic tapping task. Neurophysiological measures of SCA6 patients showed a prolonged cortical silent period (CSP) but normal MEP recruitment curve, short-latency afferent inhibition, long-latency afferent inhibition and ipsilateral silent period. Paired-associative stimulation induction also increased motor-evoked potentials normally. SCA6 patients had greater variability with cued rhythmic tapping than normals and deteriorated when the cue was removed; in comparison, normal subjects had similar variability between cued and uncued rhythmic tapping. Analysis using a Wing-Kristofferson timing model indicated that both clock variance and motor delay variance were abnormal. Conclusion. In SCA6, the circuits for sensorimotor integration and the mechanisms for LTP-like plasticity in the sensorimotor cortex are unimpaired. A prolonged CSP in SCA6 just like in other cerebellar atrophies would suggest that this neurophysiological change typifies cerebellar dysfunction.

Original publication

DOI

10.1002/mds.21847

Type

Journal article

Journal

Mov Disord

Publication Date

15/02/2008

Volume

23

Pages

378 - 385

Keywords

Adult, Aged, Case-Control Studies, Electric Stimulation, Evoked Potentials, Motor, Evoked Potentials, Somatosensory, Female, Humans, Male, Middle Aged, Motor Cortex, Neural Inhibition, Neuronal Plasticity, Reaction Time, Somatosensory Cortex, Spinocerebellar Ataxias, Transcranial Magnetic Stimulation