Brainstem auditory evoked responses in Lafora disease

Brainstem auditory-evoked responses (BAERs) have been studied in five patients suffering from Lafora-type of progressive myoclonus epilepsy proven by skin biopsy and in ten healthy volunteers. At the time of examination the patients were not taking benzodiazepines and showed myoclonic jerks. In all patients the central conduction time (interpeak latencies I-V, I-III, III-V) and the amplitude (amplitude ratio I/V) of BAERs were within the +/- 2 S.D. limits of the normal values. Since Lafora disease is a neuropathologically prevalent grey-matter illness (typical inclusion bodies are stored intraneuronally) these data indicate that diseases primarily affecting the brainstem grey-matter are usually associated with normal BAERs.