De ziekte van Creutzfeldt-Jakob: diagnostiek bij tien patiënten

OBJECTIVE: To describe experiences with the diagnosis of Creutzfeldt-Jakob disease (CJD). SETTING: Department of Neurology, Academic Medical Center, Amsterdam, the Netherlands. DESIGN: Descriptive. METHODS: By retrospective analysis of patient files and follow-up data case histories were studied of ten patients diagnosed since 1980 as having CJD. RESULTS: Follow-up of two patients (who recovered) led to rejection of the diagnosis of CJD. Symptoms and results of ancillary investigations of the remaining eight patients were similar to those in large patient series from the literature. CONCLUSIONS: On the basis of our experience and data from the literature we conclude that a classification can be made of three degrees of probability of the diagnosis CJD: possible (rapidly progressive dementia with or without myoclonus); probable (if the clinical syndrome is accompanied by triphasic complexes in the EEG and/or hyperintense basal ganglia or (parts of) the cerebral cortex on T2-weighted MRI images); and certain (vacuolisation, neuronal loss and gliosis on neuropathological examination)