SummaryThe authors reported the clinical picture of myoclonus epilepsy occurred in three siblings of one family. In Case 1 the illness began at 14 years, and she is now still alive. In Case 2 the disease began at 7 years and death occurred at the age of 14 after the clinical course of about 7 years. In Case 3 (autopsy case), the onset of the disease was 6 years of age, and death occurred at 11 after the clinical history of 5 years. Autopsy revealed the degenerative lesions of the dentate and the olivary nucleus associated with gliosis of the pallidum due to a perinatal anoxic damage.In two siblings (Cases 2, 3) the duration of the disease is shorter, and the age of death is less advanced as compared with the cases of degenerative type of myoclonus epilepsy reported in the literature, and the authors discussed its reason.
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