Epilepsy and disorders of cortical development in children with congenital cytomegalo-virus infection
Introduction. Neuroimaging and experimental studies have related cytomegalovirus (CMV) to certain neuronal migration disorders. Material and methods. To define the electroclinical picture of children with epilepsy associated with disorders of cortical development (DCD) and congenital CMV infection, we conducted a clinical, electroencephalographic and neuroradiological study of 10 children with this condition. Results. Eighty per cent of them had dismorphic traits, or malformations outside CNS. All showed other neuroradiological signs (cerebral calcification, white matter damage, porencephaly). Six patients with bihemispheric DCD (agyria-pachigyria, 2; ‘poligyria’, 1; schizencephaly, 1; bilateral opercular DCD, 2) showed: Tetraparesis, severe or profound mental deficiency, early onset epilepsy (mean age at onset: 11 months) with spasms, tonic seizures, partial seizures, and multifocal paroxysms or unusual diffuse sharp Alfa-Beta EEG activity. One child developed Epilepsia Partialis Continua. Children with bilateral opercular DCD evolved to a continuous spike and wave (SW) electrical status during wakefulness and sleep, linked to a worsening of psychomotor derangement. Four patients with unilateral hemispheric DCD (pachigyric or ‘poligyric’) showed: Congenital hemiparesis, mild intellectual deficiency, motor seizures (orofacial, hemiclonic, generalized) beginning in the third year of live, atypical absences with focal atonic phenomena, frequent focal rhythmic SW discharges during wakefulness, and continuous SW status during sleep (CSWS). Conclusions. A wide spectrum of DCD due to congenital CMV infection is documented. Characteristic electroclinical pictures related to the extent and topographical distribution of the DCD are recognized, which may lead to an appropriate diagnosis and prognosis
Resultados El 80% presentaban dismorfias y/o malformaciones fuera del SNC. En todos se evidenciaban otros signos neurorradiológicos (calcificaciones intracerebrales, hipomielinización, porencefalia). Seis pacientes con ADC bihemisféricas (agiria-paquigiria, 2; ‘poligiria’, 1; esquisencefalia, 1; ADC biopercular, 2) presentaban: tetraparesia, déficit intelectual grave o profundo, epilepsia precoz (edad media de inicio, 11 meses), con espasmos, crisis tónicas, crisis parciales, y EEG con actividad paroxística multifocal o actividad inusual alfa-beta difusa. Un niño desarrolló una epilepsia parcial continua. Los niños con ADC biopercular desarrollaron un estado de punta-onda (PO) continua en vigilia y sueño, asociado a deterioro psicomotor. Cuatro pacientes con ADC de distribución unilateral hemisférica (paquigiria y/o ‘poligiria’) presentaban: hemiparesia congénita, déficit intelectual leve, inicio en el tercer año de vida de crisis motoras (orofaciales, hemiclónicas, generalizadas) y ausencias atípicas con fenómenos de atonía focal intermitente, EEG con PO rítmica focal persistente en vigilia, y estado de PO continua durante el sueño.
Conclusiones El espectro de ADC asociadas a infección CMV congénita es amplio. Se reconocen cuadros electroclínicos en relación con la extensión y distribución topográfica de las ADC, de valor diagnóstico y pronóstico