A new variant of Creutzfeldt-Jakob Disease in the United Kingdom

Abstract

This thesis details the first 14 cases of variant Creutzfeldt-Jakob disease (CJD) identified in the UK and reports the findings of a study to assess magnetic resonance imaging (MRI) as a diagnostic tool for variant CJD (vCJD).

MRI shows striatal high signal on dual echo sequences in the majority of sporadic CJD cases. In contrast two early cases of vCJD were reported to have bilateral pulvinar high signal. This prompted a study of MRI in vCJD.  MRI from patients 36 patients with vCJD and 57 controls (patients with suspected CJD) were analysed. Scans were reviewed blind on two separate occasions by two neuroradiologists and scored for the distribution of changes and likely final diagnosis. Images showing bilateral pulvinar high signal were classified as diagnostic for vCJD and the degree of such changes were graded on a three point scale: minimal/equivocal, moderate and marked. Scans were later openly reassessed to reach a consensus on all abnormalities.

A radiological diagnosis of vCJD was made in 86% of case assessments and 3% of control assessments. The seven false-positive assessments in the controls were graded as minimal/equivocal in six and moderate in one (<0.5% of all marked control assessments). However, 80% of the assessments in the vCJD cases were graded as moderate or marked. On consensus review, 28 of 36 cases and none of 57 controls were considered to have prominent bilateral pulvinar signal - sensitivity 78% (95% CI 60-90%) and specificity 100% (95% CI 94-100%).  Other common MRI features of vCJD were medial thalamic and periaqueductal grey matter high signal, and the notable absence of cerebral atrophy. Pulvinar high signal correlated with histological gliosis.  The study concluded that in the appropriate clinical context the identification of bilaterally increased pulvinar signal on MRI is a useful non-invasive test for the diagnosis of vCJD.

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