Resumen

POZO ALONSO, Albia J.; POZO LAUZAN, Desiderio  y  POZO ALONSO, Desi. A propósito del síndrome de Ohtahara. Rev Cubana Pediatr [online]. 2003, vol.75, n.4, pp. 0-0. ISSN 1561-3119.

Ohtahara's syndrome is an epyleptic encephalopathy depending on age that present a varied origin. The clinical manifestations appear mostly during the neonatal period. The tonic spasms (up to 10 sec of duration), which are grouped in series or sporadic are the most common form of presentation. Partial crises are observed in approximately the third part of the patients. There is a prevalence of males. The characteristic of the interictal electroencephalogram is the presence of generalized waves of great amplitude alternating with phases of suppression of the brain electrical activity. The main objective of this paper is to call the attention about the existance of this entity in the neonatal and early breast-feeding stage. A male patient of 16 days of age that began to have tonic spasms associated with severe hypotonia of the neck and trunk, with hypertonia of the limbs and EEG compatible with this entity, is presented. The cranial CAT showed signs of brain atrophy of cortical predominance. The response to the treatment was unfavorable. This syndrome has a very severe prognosis and crises are highly resistant to treatment.

Palabras clave : SPASMS; INFANTILE [etiology]; INFANT, NEWBORN; SEX DISTRIBUTION.