[An autopsy case of panencephalopathic type of Creutzfeldt-Jakob disease: an early clinical sign documented by magnetic resonance imaging].

We reported an autopsy case of panencephalopathic type of Creutzfeldt-Jakob disease (CJD), one of whose early clinical signs, visual loss, had been documented by magnetic resonance imaging (MRI). The patient was a 59-year-old woman, who showed disturbance of visual acuity, memory and calculation at the early stage. About 2 months later she developed apallic syndrome and revealed myoclonic jerks in her eye balls, face, neck and extremities. Electroencephalography showed periodic synchronous discharge. Three months later after her first symptoms MR showed bilateral occipital atrophy, although we could find no brain atrophy on CT scan. She died of the suffocation caused by bronchopneumonia at the age of 60 years. The total duration of her illness was about 20 months. Neuropathological examination revealed a pronounced diffuse atrophy of the cerebral cortex and white matter. In addition to severe cortical neuronal loss and astrocytosis, spongiform changes were evident. The hippocampus was relatively well preserved. In the cerebral white matter both myelin sheaths and axons disappeared almost completely. The cerebellum showed the severe degeneration of granule cells. These neuropathological findings are consistent with those of panencephalopathic type of CJD. It is important to clarify the abnormalities of MR findings in relation to such early clinical signs of CJD. To the best of our knowledge such reports as ours have not been published previously.