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As of December 31, 1998, 35 deaths had been attributed to new variant Creutzfeldt-Jakob disease (nvCJD) in the United Kingdom, of which 33 cases had been neuropathologically confirmed and 2 classified as probably nvCJD. Fifteen cases were male and 20 female. The median illness duration was 14 months (range 8-38 months) and the median age at death was 29 years (range, 18-53 years). The clinical features were consistent with previous descriptions. In nearly all cases, there were early psychiatric symptoms after a median period of 6 months ataxia developed, followed by involuntary movements and cognitive impairment. Electroencephalograms did not show the "typical" appearances found in sporadic CJD, about half the cases tested had a positive 14-3-3 imunoassay, and over 70% of cases had bilateral pulvinar high signal on magnetic resonance brain scanning. Prion protein gene analysis showed that all cases were homozygous for methionine at codon 129. Diagnostic criteria for nvCJD have been formulated, which have a high sensitivity and specificity. |
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brain biopsy
Jakob-Creutzfeldt disease
Jakob-Creutzfeldt disease,variant
MRI
MRI,abnormal
neurologic disease,diagnoses of
neurologic signs
neuropathology
neuropathology,brain
protein 14-3-3,cerebrospinal fluid
pulvinar sign
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