Abstract
Relatamos o caso de uma paciente com sintomas de cegueira noturna congenita estacionaria (CNCE) no qual constatamos alteracoes oftalmoscopicas peculiares da doenca de Oguchi, inedita, nos limites de nosso conhecimento, a consideracao da literatura medica brasileira publicada. Observamos e documentamos as alteracoes classicas desta doenca e do seu importante comemorativo, o tambem infrequente, fenomeno de Mizuo-Nakamura. O exame eletrorretinografico (ERG) tambem mostrou sinais tipicos. Acrescentamos, brevemente, revisao bibliografica da doenca.
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