Electron Microscopic Observations in Creutzfeldt-Jakob Disease

Abstract

This discussion of spongiosis in Creutzfeldt-Jakob disease is based on two cases. The first has been published previously (1). The patient was a 59-year-old woman whose previous history remains unknown. She showed a progressive dementia and prominent Korsakov-like amnesic deficiency. Neurological findings were not conclusive; vision appeared grossly normal; muscular wasting was not present and rhythmic clonus appeared only two days before death, which occured approximately four months after onset. An EEG was recorded on six instances but not after the onset of myoclonus and was non-characteristic. Right frontal biopsy was performed for diagnostic purpose after 2 1/2 months. An abnormality was recognized, but no specific diagnosis was made until electron microscopic examination drew attention to the spongiosis which was clearly to be seen on H-E routine preparations in retrospect. Autopsy confirmed the diagnosis with microspongiosis, neuronal rarefaction and heavy gliosis being characteristic. The cerebral cortex was prominently affected, with the occipital regions comparatively spared. An unusual feature was the gross brain atrophy (890 g). The second case is reported here for the first time. A 42-year-old woman without significant previous history developed progressive amnesic deficiency and deteriorated during eleven months. A frontal tumor was suspected, and the patient was referred by Professor Garcin to the neurosurgeon. Ventriculography which was normal and frontal cortical biopsy were performed. Histological features were microspongiosis, neuronal retraction and rarefaction with no significant gliosis. Creutzfeldt-Jakob disease was diagnosed. The subsequent course was not typical for this condition.