[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.