Title
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Author
Faculty/Department
Faculty of Medicine and Health Sciences
Publication type
article
Publication
London ,
Subject
Human medicine
Source (journal)
Brain. - London
Volume/pages
132(2009) :10 , p. 2659-2668
ISSN
0006-8950
ISI
000270685600014
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
Several molecular subtypes of sporadic CreutzfeldtJakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic CreutzfeldtJakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic CreutzfeldtJakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic CreutzfeldtJakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic CreutzfeldtJakob disease. Patients with sporadic CreutzfeldtJakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as suspected sporadic CreutzfeldtJakob disease but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic CreutzfeldtJakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic CreutzfeldtJakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic CreutzfeldtJakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic CreutzfeldtJakob disease to include findings from magnetic resonance imaging scans.
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