[Subacute spongiform encephalopathy and Creutzfeldt-Jakob disease. Apropos of 1 case].
The authors describe a case of sub-acute spongiform encephalopathy with an ultrastructural study in a man of 65 with associated dementia, extra-pyramidal syndrome, myoclonia, optical disorders, pyramidal syndrome and speech disorders, rapidly leading to death one and a half months later. Electroencephalograms were at first disorganized with persistent delta and theta activity and only in the last record did short runs of repetitive activity appear. Histologically, there was severe status spongiosus, a moderate degree of neuronal depopulation with a noticeable astrocytary gliosis. Under the electron microscope, the neuropile in the astrocytes was found to be pitted with numerous cavities and so were, to a lesser extent, the axonal projections. The vessels were normal. Nothing was found to indicate viral structures. Attention is drawn to the nosological connection between this type of observation and Creutzfeldt-Jakob disease.
D000368:Aged; D003072:Cognition Disorders / Q000209:etiology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D011596:Psychomotor Disorders / Q000209:etiology; D012897:Slow Virus Diseases / Q000175:diagnosis / Q000473:pathology; D013064:Speech Disorders / Q000209:etiology; D014786:Vision Disorders / Q000209:etiology
Encéphalopathie spongiforme subaiguë et maladie de Creutzfeldt-Jakob. A propos d'un cas