First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features

Abstract

Objective To describe the early psychiatric and neurological features of variant Creutzfeldt-Jakob disease.

Prions

“…More than 200 teenagers and young adults have died of vCJD, mostly in Britain (Spencer et al 2002;Will 2003). Both epidemiologic and experimental studies have built a convincing case that vCJD resulted from prions being transmitted from cattle with bovine spongiform encephalopathy (BSE, or "mad cow" disease) to humans through consumption of contaminated beef products (Chazot et al 1996;Will et al 1996;Cousens et al 1997).…”

Presentation of a case of variant CJD in general dental practice

“…Typical presentations are depression, anxiety and behavioural change. 1 Neurological symptoms such as pain, paraesthesia and numbness precede psychiatric symptoms in 15% of cases and are present in combination with psychiatric symptoms in 22% of cases from the onset of disease. The most common early neurological (< 4 months onset) features are persistent pain affecting the limbs, trunk and face but not associated with sensory symptoms.…”

Mouse Behavioural Studies and What They Can Teach Us about Prion Diseases

“…The balance is more clearly weighted towards psychiatric symptoms in the new variant form of disease. In the study of Spencer et al, 2 on the first 100 patients diagnosed with vCJD, psychiatric symptoms preceded neurological ones in 63 cases while the opposite was true in only 15 cases. The most common psychiatric symptoms included dysphoria, withdrawal, anxiety, irritability, insomnia and loss of interest.…”

“…Thus, although comparisons between impaired nesting in mice and altered behaviours in demented patients are not obvious, it is of interest that apathy or loss of interest is a consistent early observation in new variant CJD 2 . A decrease in performance of a "domestic" task like nesting may have parallels in the impairments in "activities of daily living" observed in Alzheimer's disease 29 .…”

“…This is due to the gradual appearance in the population of young adults suffering from new variant CJD, the disease entity apparently caused by the ingestion of BSE-infected meat 1 . These patients are affected at a much younger age than is seen with other prion diseases and show a pattern of behavioural abnormalities that are less often seen in familial or sporadic forms of prion disease 2 . These behavioural abnormalities include a number of features of depression and precede the appearance of dementia and typical clinical signs.…”