Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome.

ABSTRACT:

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 characterize and estimate the frequency of neuropathy in this condition, and determine its typical MRI features.

Methods

Retrospective review of 18 subjects (including 4 from the original description) who met the criteria for bilateral vestibulopathy with cerebellar ataxia.

Results

The reported age at onset range was 39-71 years, and symptom duration was 3-38 years. The syndrome was identified in one sibling pair, suggesting that this may be a late-onset recessive disorder, although the other 16 cases were apparently sporadic. All 18 had sensory neuropathy with absent sensory nerve action poten

SUBMITTER: Szmulewicz DJ 

PROVIDER: S-EPMC3115806 | biostudies-literature | 2011 May

REPOSITORIES: biostudies-literature