Epileptic negative myoclonus in a newborn with hemimegalencephaly.
OBJECTIVE
We report the case of a male newborn with Ohtahara syndrome and right hemimegalencephaly who presented epileptic negative myoclonus in the first days of life.


METHODS
Prolonged polygraphic studies were performed, as well as MRI and a full clinical examination.


RESULTS
EEG showed a constant and nonreactive pattern of burst suppression. There were several kinds of electro-clinical seizures (generalized myoclonia, short atonias, typical spasm and tonic spasms) at the beginning of the EEG's burst. The periods of EMG silence, lasting less than 300 ms, were associated with stereotyped EEG transients.


CONCLUSIONS
Epileptic negative myoclonus can be observed also in neonatal age. The short transient impairment of motor function observed in the newborn seems linked to the slow component of spike-wave discharge, but its mechanism is still not clear.
Neuropsichiatria Infantile, Catholic University of the Sacred Heart, Roma, Italy.
Francesco
F
Guzzetta
Domenica
D
Battaglia
Donatella
D
Lettori
Federica
F
Deodato
Emanuela
E
Sani
Teresa
T
Randò
Daniela
D
Ricci
Celeste
C
Acquafondata
Carmine
C
Faienza
D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D015897:Comorbidity; D004569:Electroencephalography / Q000379:methods / Q000706:statistics & numerical data; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D007839:Functional Laterality; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D009421:Nervous System Malformations / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D013577:Syndrome