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Creutzfeldt-Jakob disease: clinical analysis of eight cases
M J Hsieh1, P Y Wang, L G Chia
1Department of Internal Medicine, Taichung Veterans General Hospital, Taiwan, R.O.C.
Background:
Creutzfeldt-Jakob disease (CJD) or spongiform encephalopathy in humans, is a rare, rapidly progressive and fatal disorder of the central nervous system. Since clinical diagnosis is challenging, this retrospective investigation was performed.
Methods:
Eight cases who had been diagnosed as CJD from 1987 to 1995 were reviewed thoroughly, with a resulting clinical diagnosis of CJD. Two cases were proved by brain biopsy.
Results:
Four males and four females were included; their ages were 53 to 81 years with a mean of 66 years. The mean illness duration of the six patients who died was six and a half months. Clinical findings were variable and included dementia, aphasia, ataxic gait, pyramidal, extrapyramidal, myoclonus and visual problems.
Conclusions:
For diagnosis of CJD, clinical suspicion is very important. Electroencephalogram (EEG) remains the most helpful laboratory diagnostic tool; serial recordings are necessary if the initial EEG finding is nonspecific. Single photon emission computed tomography (SPECT) may be helpful for premorten diagnosis of CJD, but sensitivity and specificity need further investigation. Brain biopsy seemed to be unnecessary if the clinical, EEG and SPECT findings are typical.