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Visual Evoked Potentials, Brainstem Auditory Evoked Potentials, and Quantitative EEG in Baltic Progressive Myoclonus Epilepsy

1986; Wiley; Volume: 27; Issue: 5 Linguagem: Inglês

10.1111/j.1528-1157.1986.tb03581.x

1528-1167

Esa Mervaala, Tapani Keränen, Ari Pääkkönen, Juhani Partanen, P. Riekkinen,

Glycogen Storage Diseases and Myoclonus

Visual and brainstem auditory evoked potentials (VEP and BAEP, respectively) and quantitative EEG were studied in 16 patients with Baltic progressive myoclonus epilepsy (PME). The study demonstrated significantly delayed VEP latencies but normal amplitudes in Baltic PME. BAEPs showed slight but significant prolongation in central conduction time. Quantitative EEG revealed diminution of beta and alpha activity and accentuation of theta and delta activity. The slowing in VEP latencies is suggested to be due to impaired synaptic transmission and to reflect dopaminergic dysfunction in Baltic PME. We conclude that there is a multimodal disturbance in sensory projections to cortical areas in Baltic PME. The results give further evidence that nondemyelinating disorders--but with synaptic transmission defects--can produce changes in evoked potentials. The changes in epileptic brain are not confined to hyperexcitable epileptic neurons, but more widespread electrophysiological phenomena are produced.