Os autores relatam caso de paciente com 27 anos de idade com oftalmoplegia externa, anisocoria, ataxia, arreflexia profunda, disfagia, disfonia, paralisia facial periférica bilateral, hipopalestesia e discreta hipoestesia táctil palmo-plantar. O quadro teve início 10 dias após virose de vias aéreas superiores e ocorreu rápida melhora após terapêutica com prednisona. Os achados do exame neurológico e dos subsidiários são compatíveis a alterações centrais e periféricas.

The authors report a case of a 27 year old male patient who has exhibited external ophthalmoplegia, anisocoria, ataxia, absent deep reflexes, dysphagia, dysphonia, bilateral peripheral facial paralysis, hypopallesthesia and a mild sensory impairment of the four extremities. In addition, his cerebrospinal fluid showed albuminocytologic dissociation. The otoneurologic examination showed important findings. These symptoms began ten days after a "cold", and quickly subsided after prednisone therapy. Two months after the onset of clinical features, just a deep apendicular areflexia was detected on his neurological examination. The neurological signs and complementary laboratory findings suggest that both central and peripheral alterations are responsible for the clinical manifestations.

É apresentado o caso de paciente de 53 anos de idade com quadro de rebaixamento mental e comprometimento piramidal, com evolução progressiva até mutismo acinético em 6 meses de evolução. Um pneumencefalograma mostrou aumento simétrico do sistema ventricular. Uma biópsia cerebral permitiu encontrar alterações neuronais, gliose e estado espongioso que permitiram classificar o caso como de moléstia de Creutzfeldt-Jakob. São discutidos os achados eletromicroscópicos.

The case of a patient 53 years old that had a picture of cortical impairment and pyramidal disfunction, leading in 6 months to acinetic mutism is reported. The histopathology lead to the diagnostic of Creutzeldt-Jakob disease, with neuronal loss, gliosis and status spongiosus. The electron-microscopic examination showed that the status spongiosus was due to cellular processes, some having vesicular bodies sugestive of pre-synaptic vesicles.