Summary
Two cases of hereditary motor sensory neuropathy type III (Déjérine-Sottas disease) examined by audiological, vestibular and electrophysiological methods are reported. In both cases there were signs of vestibular and acoustic central pathway involvement, shown by vestibular examination and by the study of auditory evoked potentials. The presence of central involvement in this hereditary neuropathy suggests central as well as peripheral myelin alteration.
Zusammenfassung
Es wird über zwei Fälle von hereditärer motorischer und sensorischer Neuropathie vom Typ-III (Déjérine-Sottas-Krankheit) berichtet, welche otoneurologisch und elektrophysiologische eingehend untersucht wurden. In beiden Fällen fand man Hinweise dafür, daß die vestibulären und akkustischen zentralen Bahnen mitbetroffen sind. Dies läßt darauf schließen, daß bei dieser Erkrankung die Myelinstrukturen nicht nur in den peripheren Nerven sondern auch im Zentralorgan betroffen sein können.
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Baiocco, F., Testa, D., d'Angelo, A. et al. Abnormal auditory evoked potentials in Déjérine-Sottas disease. J Neurol 231, 46–49 (1984). https://doi.org/10.1007/BF00313652
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DOI: https://doi.org/10.1007/BF00313652