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Case Reports
. 2001 May 22;56(10):1412-4.
doi: 10.1212/wnl.56.10.1412.

Adult-onset familial laryngeal abductor paralysis, cerebellar ataxia, and pure motor neuropathy

F Barbieri  1 , M T Pellecchia, E Esposito, E Di Stasio, I Castaldo, F Santorelli, A Perretti, L Santoro, G De Michele
Affiliations
Case Reports

Adult-onset familial laryngeal abductor paralysis, cerebellar ataxia, and pure motor neuropathy

F Barbieri et al. Neurology. .

Abstract

Two brothers presented with late-onset cerebellar ataxia and severe dysphonia. Brain MRI showed vermian and hemispheric cerebellar atrophy. Laringofiberscopy revealed laryngeal abductor paralysis in both patients. Neurophysiologic studies showed a pure motor neuropathy. The combined findings and the molecular analysis suggest a new familial disorder. Inheritance is most likely autosomal recessive, but X-linked transmission is also possible.

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