Assuntos
Canais de Cloreto/genética , Doenças Genéticas Ligadas ao Cromossomo X/genética , Nefropatias/genética , Fenótipo , Regiões 5' não Traduzidas/genética , Adolescente , Adulto , Criança , Pré-Escolar , Doenças Genéticas Ligadas ao Cromossomo X/patologia , Humanos , Lactente , Nefropatias/patologia , Mutação/genética , Polimorfismo Conformacional de Fita Simples , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Análise de Sequência de DNARESUMO
Herpes Simplex Encephalitis (HSE) is an uncommon but severe disease with high mortality and morbidity. The major clinical manifestations are deteriorating consciousness with confusion, drowsiness or coma, altered behaviour, convulsions and a variety of neurological signs (hemiplegia, aphasia, ataxia, etc.). An uncommon complication of HSE is Kluver Bucy syndrome (KBS), characterized by hyperorality, bulimia and changes in emotional behaviour. Neuroimaging studies frequently show an involvement of the temporal lobes and limbic areas. Another uncommon complication of HSE is central diabetes insipidus as a result of herpes simplex infection of the hypothalamus. We report two pediatric cases of HSE complicated with Kluver Bucy syndrome and central diabetes insipidus.
