[Neonatal myoclonic encephalopathy (author's transl)].
The authors studied 5 patients with neonatal myoclonic encephalopathy commencing in the hours immediately after birth and consisting of erratic, asynchronous, non-periodic myoclonus associated with generalized jerks in 4. The EEG showed bursts of pseudo-periodic activity on a low voltage background rhythm. Neuropathological study showed poliodystrophy in one case and a disturbance of cortical lamination in another. The relation of these cases with non-ketotic hyperglycinaemia is discussed.
D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications* / Q000503:physiopathology; D001928:Brain Diseases, Metabolic / Q000503:physiopathology; D004569:Electroencephalography; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000175:diagnosis / Q000503:physiopathology; D009207:Myoclonus / Q000209:etiology* / Q000503:physiopathology
Encéphalopathie myoclonique néonatale.