Clinical findings and diagnostic tests in Creutzfeldt-Jakob disease and variant Creutzfeldt-Jakob disease.

Abstract:

:Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare transmissible disease caused by accumulation of pathological prion protein in the CNS. sCJD typically affects patients in their sixties. The median disease duration in sCJD (6 months) is shorter than in variant Creutzfeldt-Jakob disease (vCJD) (14 months). The clinical diagnosis in sCJD is supported by the detection of periodic sharp and slow wave complexes (PSWC) in the electroencephalogram, 14-3-3 proteins in the cerebrospinal fluid (CSF) and hyperintense basal ganglia on magnetic resonance imaging (MRI). In contrast to sCJD, hyperintensities in the posterior pulvinar (the "pulvinar sign") are seen in vCJD. Different sCJD subtypes characterised by distinct neuropathological lesion profiles, clinical features and codon 129 genotype of the prion protein gene (PRNP) are described, together with the type with a proteinase K-resistant core of the prion protein. The sensitivity of diagnostic tests varies considerably in different sCJD subtypes. Alzheimer 's disease and Lewy body dementia are the most frequent differential diagnoses in elderly patients, while chronic inflammatory CNS disorders have to be considered in younger patients.

journal_name

Folia Neuropathol

journal_title

Folia neuropathologica

authors

Krasnianski A,Meissner B,Heinemann U,Zerr I

subject

Has Abstract

pub_date

2004-01-01 00:00:00

pages

24-38

eissn

1641-4640

issn

1509-572X

journal_volume

42 Suppl B

pub_type

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