New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests

Zeidler, Martin; Stewart, G.; Barraclough, CR; Bateman, David; Bates, David; Burn, David J.; Colchester, AC; Durward, W. F.; Fletcher, NA; Hawkins, S. A.; Mackenzie, J.; Will, R. G.

doi:10.1016/s0140-6736(97)07472-2

Cited by 249 publications

(152 citation statements)

References 27 publications

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“…The clinical features are remarkably stereotyped. There is an initial phase of around 6 mo dominated by psychiatric symptoms, including depression, delusions, and anxiety 89 followed by the rapid development of neurological features, 90 typically confusion, ataxia, and involuntary movements, which may be choreiform, dystonic, or myoclonic. The duration of illness from onset to death averages 14 mo 91 in contrast to sCJD in which the mean survival is 4 mo.…”

Section: Diagnostics and Treatmentmentioning

confidence: 99%

Variant CJD

Diack

Head

McCutcheon

et al. 2014

Prion

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Section: Diagnostics and Treatmentmentioning

confidence: 99%

Variant CJD

Diack

Head

McCutcheon

et al. 2014

Prion

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“…This transmissible prion agent has different clinical features compared to the more frequent sporadic variant, including a younger median age at onset of 26 years (range, 12-74 years) (60). The disease may present with early psychiatric symptoms and sensory symptoms, which are uncommon in sCJD.…”

Section: Variant Cjdmentioning

confidence: 99%

“…Later, overt neurological symptoms develop. The disease has a longer course than sCJD with an average median duration of 14 months (range, 9-35 months) (60). The typical PSWCs seen on EEG in sporadic CJD are typically absent.…”

Section: Variant Cjdmentioning

confidence: 99%

Imaging of prion diseases

Létourneau-Guillon

Wada²,

Kucharczyk

2012

Magnetic Resonance Imaging

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Prion diseases are caused by self‐replicating proteins that induce lethal neurodegenerative disorders. In the last decade, the understanding of the different clinical, pathological, and neuroimaging phenotypes of this group of disorders has evolved paralleling the advances in prion molecular biology. From an imaging standpoint, the implementation of diffusion‐weighted imaging in routine practice has markedly facilitated the detection of prion diseases, especially Creutzfeldt‐Jakob. Less frequent prion‐related disorders, including genetic diseases, may also benefit from progresses in the field of quantitative diffusion‐weighted imaging, MR spectroscopy or molecular imaging. Herein, we present a review of the neuroimaging features of the prion disorders known to affect humans emphasizing the important contribution of MRI in the diagnosis of this group of disorders. J. Magn. Reson. Imaging 2012;35:998‐1012. © 2012 Wiley Periodicals, Inc.

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“…These symptoms last several months and are followed by dementia, cerebellar and other neurological signs, myoclonus or other involuntary movements, and finally akinetic mutism. 38,39 The clinical course of the variant disease is much longer than that of sporadic CJD and affected patients do not have the typi-PRACTICE cross infection control undertaken by the minimum number of healthcare staff needed to ensure effective treatment. All treatment should be carefully planned in advance.…”

Section: Variant Creutzfeldt-jakob Diseasementioning

confidence: 99%