“Hot Cross Bun” Sign in Variant Creutzfeldt-Jakob Disease: Fig 1.

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"Hot cross bun" sign in variant Creutzfeldt-Jakob disease.

Disease The “hot cross bun” sign refers to pontine cruciform hyperintensity on long TR sequences, which can be observed in multiple-system atrophy, spinocerebellar atrophy types 2 and 3, and in parkinsonism secondary to vasculitis. It has not been previously demonstrated in variant Creutzfeldt-Jakob disease (vCJD), to our knowledge. A 16-year-old girl, diagnosed with vCJD at age 14 years and de...

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The hot cross bun sign.

Hot cross bun sign refers to the cruciform-shaped hyperintensity on T2W axial magnetic resonance images (MRI) in multisystem atrophy due to the selective loss of myelinated transverse pontocerebellar fibers and neurons in the pontine raphe and sparing of the pontine tegmentum and corticospinal tracts [Figure 1, Figure 2].[1] The name derives from a sweet spiced bun baked by the Christian church...

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Variant Creutzfeldt-Jakob disease.

Current evidence indicates that variant Creutzfeldt-Jakob disease is caused by the transmission of bovine spongiform encephalopathy to humans. The clinical and investigative features of variant CJD are relatively distinct from sporadic CJD and the neuropathological appearances are novel. The number of cases of vCJD in the UK may have peaked, but the total future number of cases of vCJD is uncer...

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Genetic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease.

The ‘‘pulvinar sign’’ on MRI brain scan is defined as hyperintensity of the posterior thalamus relative to the signal intensity of the anterior putamen. In the appropriate clinical context, the pulvinar sign and hyperintensity in the dorsomedial thalamic nuclei (the hockey stick sign) are sensitive and specific features of variant Creutzfeldt– Jakob disease (vCJD). Variant CJD is an acquired fo...

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Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease.

BACKGROUND The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons. OBJECTIVE To describe a patient with sporadic Creutzfeldt-Jakob disease (sCJD) who had a clinical history and initial electroencephalogram and magnetic resonance imaging findings consistent with variant CJD (vCJD). RESULTS Results of a repeate...

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ژورنال

عنوان ژورنال: American Journal of Neuroradiology

سال: 2009

ISSN: 0195-6108,1936-959X

DOI: 10.3174/ajnr.a1335