RESUMO
Les auteurs rapportent 29 observations de phacomatoses dont 18 cas de sclerose tubereuse de Bourneville et 11 cas de neurofibromatose de Recklinghausen; observees dans le service de neuro l ogie du centre hospitalo-universitaire de Conakry; sur une periode de 10 ans. Dans cette peri o d e;les donnees classiques de la litterature sont retrouvees : frequence elevee des lesions dermatologiques evoluees atteignant de volumineuses tumeurs royales; des crises epileptiques sous toutes ses formes et les complica-tions diverses en raison du retard du diagnostic. Nos resultats soulignent la necessite de suivi et de surveillance de ces patients par un examen somatique (neurologique; ophtal- mologique) et un examen scannographique dicte par les donnees cliniques
Assuntos
Esclerose TuberosaRESUMO
This rep o rt describes 6 cases of Wi l s o n 's disease observed at the Unive rsity Hospital Center in Conakry; Guinea. P resenting symptoms invo l ved ex t rapy ramidal syndrome in 4 cases; p s y chomotor ep i l epsy in 1; and confusional syndrome in 1 c a s e. Diagnosis was based on neuro p s y chic manife s t ations; Kayser- F l e i cher corneal ring; liver failure; and impaired copper metabolism. Brain CT-scan depicted normal fe at u res in 3 cases; s y m m e t ric hypodensity of the lenticular nu cleus in one; discrete ve nt ricular dilat ation of in one a and cortico-subcoetical atro p hy in one
Assuntos
Cobre , Degeneração HepatolenticularRESUMO
Late occurrence of epilepsy is common in tropical areas. This report describes a series of 42 cases of late epilepsy collected over a three-year period. Findings were consistent with the literature: highest incidence in young age groups; predominance of partial symptomatic epilepsy; and wide spectrum of etiologies. The most frequent etiology was tumoral disease followed by vascular and infectious disease. Brain CT scan and electro-encephalography were highly useful for identification of clinical and etiological entities