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Evoked potential abnormalities in the various inherited ataxias
Author(s) -
Nuwer Marc R.,
Perlman Susan L.,
Packwood James W.,
Kark R. A. Pieter
Publication year - 1983
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410130106
Subject(s) - olivopontocerebellar atrophy , somatosensory evoked potential , ataxia , brainstem , audiology , atrophy , evoked potential , psychology , neuroscience , medicine , degenerative disease , central nervous system disease , pathology
Visual (VEP), brainstem auditory (BAEP), and somatosensory (SEP) evoked potential tests were performed in 45 patients representing ten types of inherited disorders in which ataxia was the most prominent symptom. Comparable VEP abnormalities were present among all types of patients. Normal BAEP tests were recorded in most patients except those with olivopontocerebellar atrophy. SEP results were often more severely abnormal in patients with Friedreich's ataxia. The observations emphasize the similarity in expression of different metabolic‐degenerative disorders. When these tests are used clinically, certain features of evoked potentials (especially left‐right symmetry) are typical of the inherited ataxias as a group. Few distinguishing features differentiate the individual disorders.