Diagnosis of new variant Creutzfeldt‐Jakob disease

Abstract

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 probable 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 dinical 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 immunoassay, 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.

From mad cows to sensible blood transfusion: the risk of prion transmission by labile blood components in the United Kingdom and in France

“…The disease was found chiefly in adults under 40 years of age, contrasting with the mean age for sCJD. The disease is fatal in a mean of 14 months, which is slower than the sporadic form 10 . Nuclear magnetic resonance imaging scanning shows hypersignals situated in the posterior thalamus (“pulvinar sign”).…”

Review: Laboratory diagnosis and surveillance of Creutzfeldt-Jakob disease

“…The national centers or programs responsible for CJD surveillance are summarized in Table . National surveillance systems for CJD/vCJD are similar across different countries as a result of their following the 2003 WHO and United Kingdom criteria [Will et al, ; WHO, ; Gubbels et al, ] (Table ).…”

“…Changes in the nucleus caudatus are usually seen in sCJD, while most changes are observed in the posterior thalamus in vCJD [Zeidler et al, ]. While DWI has been increasingly favored over FLAIR in diagnosing vCJD, FLAIR can still be helpful in sCJD diagnosis [Will et al, ]. Both sensitivity and specificity based on objective criteria are at approximately 80% [Zerr et al, ].…”

Prion diseases: risks, characteristics, and infection control considerations in dentistry

“…Iatrogenic CJD (iCJD) has affected at least 400 people worldwide 7 . The prion is acquired via cadaver‐derived growth hormone, pituitary gonadotropins, dura mater homografting, corneal grafts, or inadequately sterilized intracerebral surgical equipment.…”

“…Oral manifestations of human prion disease comprise dysphagia and dysarthria due to pseudobulbar palsy. In patients with vCJD, there can be orofacial dysesthesia or paresthesia 7,8 . Recently, loss of taste and smell was reported as a feature of one patient with vCJD.…”