[Creutzfeldt-Jakob disease: diagnosis in 10 patients].

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).