PROGRESSIVE MYOCLONIC EPILEPSY WITH LAFORA''S BODIES

Two cases of Lafora's disease of rapid evolution with a typical clinical picture, and increased glutamic acid urine excretion are described. Partial critical control was obtained using clonazepam. The relation-ship between PME and heredodegenerative ataxia is discussed; the existence of subacute and precocious forms with long evolution in Lafora's disease is also considered. Cerebral biopsies showed multiple Lafora's bodies. Ultrastructurally, the smaller Lafora's bodies revealed a membrane that separated them from the neuronal cytoplasm; differences were found between the periphery and the central zone of Lafora's bodies, as well as between the different sizes of these inclusions. Their origin and the present concept of a possible biochemical composition are also discussed.