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Common dopaminergic mechanism for epileptic photosensitivity in progressive myoclonus epilepsies

Montreal Neurological Institute and Hospital and Department of Neurology and Neurosurgery, McGill University, Montreal, Québéc, Canada.

We studied the effect of apomorphine, a dopamine receptor agonist, on epileptic photosensitivity in 7 patients with progressive myoclonus epilepsy (PME). Specific diagnoses included Baltic PME (Unverricht-Lundborg disease), Lafora disease, Kufs' disease, juvenile neuroaxonal dystrophy, and action myoclonus-renal failure syndrome; 2 patients had PME of uncertain etiology. Apomorphine blocked the epileptic photosensitivity in all patients and also reduced intention myoclonus in a patient with Baltic PME. There is a common deficit of dopaminergic inhibitory neurotransmission at the level of the striate cortex in patients with PME, regardless of the nature of the specific underlying neuropathologic process.

Address correspondence and reprint requests to Dr. Frederick Andermann, Montreal Neurological Hospital, 3801 University Street, Montreal, PQ H3A 2B4, Canada.

Received May 5, 1989. Accepted for publication in final form June 23, 1989.