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Progressive hereditary myoclonus epilepsy is a relentless disease which responds poorly to conventional drug therapy. Clonazepam (Ro 5-4023) a new benzodiazepine derivative, was given to 3 patients with progressive myoclonus epilepsy (Unverricht-Lundborg), and to 1 with focal myoclonus epilepsy (Kojewnikow). All 4 were females. Their ages ranged from 19 to 21 years. The duration of symptoms varied from 5 to 12 years. The duration of the trial was 30, 19, 3, and 4 months, respectively. With a daily dose of 6–8 mg, clonazepam effectively reduced the number of jerks in all patients; this effect appeared after 1 month of treatment. The duration of the effect looks promising. Intratrial EEG showed a clear decrease in spike-and-wave paroxysms. Photic stimulation provoked less jerks than before the trial; the amplitude of evoked activity was also reduced. There was some drowsiness when clonazepam was first given, but it disappeared after some weeks of treatment.
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Laitinen et al. (2009) studied this question.
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