New Variant Creutzfeldtjakob Disease nvCJD

Epidemiology. In 1996, a report by Will and colleagues described a distinctive cliniconeuropathological variant of CJD in 10 patients living in the United Kingdom. It was suggested that this variant syndrome might be due to the consumption of beef contaminated by the agent of bovine spongiform encephalopathy (BSE, or "mad cow disease") that had been killing cattle in epidemic fashion for the previous decade. Several additional cases have occurred each year since then, bringing the total to 24, and it is hoped that this continuing accumulation does not presage an epidemic "echo" in the human United Kingdom population, but it is still too early to know.

Clinical Features. The distinctive features of nvCJD are the young age at onset of illness (the average is 27 years, with no patient older than 50 years of age), a psychiatric or sensory disturbance on clinical presentation in the majority of patients, and a long duration of illness (median, 14 months). In its evolution, the disease shares with typical sporadic CJD the features of progressive dementia, myoclonus, and multisystem neurological deficits.

Laboratory Evaluation. None of the nvCJD patients has shown periodic EEG activity, nor has the CSF "14-3- 3" protein test proved to be as helpful as in sporadic cases of CJD. MRI studies in several cases suggest that an abnormally high symmetrical signal in the posterior thalamus (pulvinar) may be a strong diagnostic clue, but additional cases need to be studied before this can be stated with any assurance. Neuropathological examination (without which a definitive diagnosis cannot be made) reveals diffuse spongiform changes that are especially severe in the basal ganglia, posterior thalamus, and cerebellum, with the presence of myriad amyloid plaques surrounded by halos of vacuolation--the so-called "florid" or "daisy" plaques.

Management, Treatment, and Prevention. The management, treatment, and prevention of nvCJD are similar to those other varieties of TSE, with the understanding that measures taken by the United Kingdom to prevent BSE-contaminated tissue from reaching the human food

chain should make even British beef safe to eat at the present time. Neither BSE nor nvCJD has been found in the United States.

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