O-3-21. Electrophysiological evaluation of peripheral neuropathy in hereditary spinocerebellar ataxia

Peripheral neuropathy often occurs in hereditary spinocerebellar ataxia (hSCA), but the features of nerve involvement have not been fully investigated. We evaluated the prevalence of peripheral neuropathy in hSCAs and classified the underlying pathologies into length-dependent axonopathy and neuronopathy based on sural/radial amplitude ratio (SRAR). SRAR has been reported as a sensitive indicator to differentiate the pathology of peripheral neuropathy. In this study, we defined SRAR less than 0.3 as length-dependent axonopathy, and SRAR more than 0.3 as neuronopathy.

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