Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

The association of bilateral vestibulopathy with cerebellar ataxia was first reported in 1991
and delineated as a distinct syndrome with a characteristic and measurable clinical sign …

BACKGROUND AND OBJECTIVE: Biomarkers to monitor neurological dysfunction in
autosomal dominant inherited spinocerebellar ataxias (SCA) are lacking. We therefore …

Spinocerebellar ataxias are dominantly inherited disorders that are associated with
progressive brain degeneration, mainly affecting the cerebellum and brainstem. As part of …

Objective: The syndrome of cerebellar ataxia with bilateral vestibulopathy was delineated in
2004. Sensory neuropathy was mentioned in 3 of the 4 patients described. We aimed to …

Spinocerebellar ataxia type 3, spinocerebellar ataxia type 6 and Friedreich's ataxia are
common hereditary ataxias. Different patterns of atrophy of the cerebellar cortex are well …

Background Given that new therapeutic options for spinocerebellar ataxias are on the
horizon, there is a need for markers that reflect disease‐related alterations, in particular, in …

Spinocerebellar Ataxia Type 3 (SCA3) is a neurodegenerative disease caused by a CAG
repeat expansion in the ATXN3 gene. Though the ATXN3 protein is expressed ubiquitously …

Spinocerebellar ataxia Type 3 (SCA3), the most common dominantly inherited ataxia, is a
polyglutamine neurodegenerative disease for which there is no disease-modifying therapy …

Objective Patients who have had vestibular neuritis (VN) show a remarkable clinical
improvement especially in gait and posture> 6 months after disease onset. Methods Voxel …