Summary
Electromyographic examination and studies of motor and sensory conduction velocities were performed in 11 patients with a presumptive diagnosis of olivopontocerebellar atrophy with autosomal dominant transmission. Peripheral nervous system involvement was shown in eight. In two patients with early onset of disease, electrophysiological alterations clearly pointed to severe axonal degeneration, whereas in six they were compatible with slight demyelination.
Zusammenfassung
Bei elf Patienten, bei welchen eine olivopontocerebelläre Atrophie mit autosomal dominanter Übertragung angenommen wurde, führten wir eine elektromyographische Untersuchung und eine Bestimmung der motorischen und sensiblen Erregungsleitungsgeschwindigkeit durch. Bei acht dieser Patienten wurde eine Mitbeteiligung des peripheren Nervensystems nachgewiesen. In zwei Fällen mit frühem Krankheitsbeginn wiesen die elektrophysiologischen Veränderungen eindeutig auf eine schwere axonale Degeneration hin, während bei sechs die Befunde mit einer leichten Demyelinisation vereinbar waren.
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Carenini, L., Finocchiaro, G., Di Donato, S. et al. Electromyography and nerve conduction study in autosomal dominant olivopontocerebellar atrophy. J Neurol 231, 34–37 (1984). https://doi.org/10.1007/BF00313649
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DOI: https://doi.org/10.1007/BF00313649