Neurocutaneous melanosis: hydrocephalus and intracranialhypertension - rare case

Objective: report a case of neurocutaneousmelanosis in Belem, Para. Case report: a 13-yearsoldboy, with history 8 months later presented paroxistic absence recurrent seizures controlledwith carbamazepine. At three years old, the seizures became generalized type, associated tonistagmus and incipient apendicular ataxia. Until 11 years old, he had neurologic exam stable,without diagnostic, when presented persistent holocranial headache, nausea, morning vomit,oftalmoparesis type III pair lesion on the right and somnolence. At general exam, it was founduncountable melanocytic nevi in his dorsum, buttocks, left arm, thorax and right shoulder. MRIstudy was performed and suggested CNS melanicytic infiltration. The patient underwentventriculoperitoneal derivation and presented good evaluation, asymptomatic.Conclusion: theearly diagnostic is very important, considering the possibility to precocious neurosurgicalintervention, allowing the increase of survival in these patients. Objetivo: relatar um caso de melanoseneurocutanea em Belem, Para. Relato de caso:adolescente, 13 anos de idade, genero masculino, com historia de crises convulsivasparoxisticas, tipo ausencia, recorrentes, controladas com uso de carbamazepina. Aos tres anosde idade, as crises tornaram-se do tipo generalizada, associada com nistagmo e ataxiaapendicular incipiente. Ate 11 anos de idade, o paciente teve quadro neurologico estavel, semdiagnostico, quando apresentou cefaleia persistente holocraniana, nausea, vomitos pela manha,sonolencia e oftalmoparesia por lesao do III par craniano a direita. No exame geral, foramencontrados incontaveis nevos melanociticos no dorso, nadegas, braco esquerdo, torax e ombrodireito. A RNM sugeriu infiltracao melanocitica do SNC. O paciente foi submetido a derivacaoventriculo-peritoneal e apresentou boa evolucao, ficando assintomatico apos o procedimento.Conclusao: o diagnostico precoce e muito importante, considerando a possibilidade deintervencao neurocirurgica precoce, permitindo o aumento da sobrevida nesses pacientes