Sporadic Creutzfeldt-Jakob Disease Mimicking Variant Creutzfeldt-Jakob Disease

Martindale, Jennifer L.; Geschwind, Michael D.; Armond, Stephen De; Young, Geoffrey S.; Dillon, William P.; Henry, Roland G.; Uyehara-Lock, Jane; Gaskin, David A.; Miller, Bruce L.

doi:10.1001/archneur.60.5.767

Cited by 34 publications

(15 citation statements)

References 15 publications

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“…17 Young sCJD patients are being continually reported in many European countries, Australia, North America, Japan and others, based on the national surveillance data. [18][19][20][21][22][23] The identifying rate of young sCJD patients below 40 year-old in our surveillance system is comparable with that of other countries.…”

Section: Commentsmentioning

confidence: 52%

Clinical and laboratory features of 14 young Chinese probable sCJD patients

Shi

Xiao

Cao

et al. 2017

Prion

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ABSTRACT. Sporadic Creutzfeldt-Jakob disease (sCJD) occurs frequently in the relatively older population, mainly in the groups of 60-69 and 70-79 year-old. Since 2006 when China performed national CJD surveillance, 14 young probable sCJD patients below 40 year-old were identified, counting for 1.93% of all probable sCJD cases. The clinical features of young probable sCJD cases, including the onset feature, the presence of sCJD-associated signs and the clinical duration, are indistinguishable from those of older patients. Special sCJD-associated abnormalities on EEG and MRI were noticed in 7 and 10 cases. CSF 14-3-3 was positive in 7 cases. CSF RT-QuIC showed positive reactive curves in 9 cases, with short lag phases. PRNP sequencing did not find any mutation. Due to low rate of brain autopsy in China, performances of other CJD-associated examinations as much as possible are extremely important for the distinguish diagnosis of young probable sCJD patients.

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Section: Commentsmentioning

confidence: 52%

Clinical and laboratory features of 14 young Chinese probable sCJD patients

Shi

Xiao

Cao

et al. 2017

Prion

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“…Signal intensity abnormality in the thalamus was seen in 40% of fCJD cases in our study and is also seen in sCJD. 37,42,43 In vCJD, the thalamic signal intensity on FLAIR is generally believed to be brighter or of greater magnitude compared with the signal intensity of the caudate and putamen. 44 In sCJD, the thalamic signal intensity is felt to be equal or lower in magnitude compared with the caudate and putamen.…”

Section: Discussionmentioning

confidence: 99%

MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study

Fulbright

Hoffmann²,

Lee³

et al. 2008

AJNR Am J Neuroradiol

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“…Although high signal changes in the pulvinar thalami have also been reported in sporadic CJD [42,55], they are generally less pronounced than in the caudate head or putamen, whereas in variant CJD the highest signal is always in the pulvinar. The radiological differential diagnosis for the pulvinar sign includes rare cases of post-infectious encephalitis, cat-scratch disease, intracranial hypertension and Alpers syndrome (progressive infantile poliodystrophy, a rare genetically determined mitochondrial encephalomyelopathy) [54].…”

Section: Mri In Variant Cjdmentioning

confidence: 99%

Radiological assessment of Creutzfeldt-Jakob disease

2006

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Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The "pulvinar sign", defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD.

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Sporadic Creutzfeldt-Jakob Disease Mimicking Variant Creutzfeldt-Jakob Disease

Abstract: This case cautions against relying solely on T2- and diffusion-weighted pulvinar hyperintensity and clinical features to differentiate between vCJD and sCJD, and further supports established diagnostic criteria for vCJD.

Cited by 34 publications

References 15 publications

Clinical and laboratory features of 14 young Chinese probable sCJD patients

Clinical and laboratory features of 14 young Chinese probable sCJD patients

MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study

Radiological assessment of Creutzfeldt-Jakob disease

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