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PROGRESSIVE MYOCLONIC EPILEPSY WITH LAFORA'S BODIES
Author(s) -
Lope Enrique Såenz,
Junquera Santiago Ramón y Cajal,
Berenguel Alfredo Blanes
Publication year - 1974
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1974.tb02800.x
Subject(s) - lafora disease , progressive myoclonus epilepsy , myoclonic epilepsy , clonazepam , epilepsy , pathology , medicine , neuroscience , disease , biology , anesthesia , biochemistry , phosphorylation , phosphatase
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.