Familial Creutzfeldt-Jakob Disease with M232R Mutation Progressed Slowly like Alzheimer's Disease

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Familial Creutzfeldt-Jakob Disease with V180I Mutation

Creutzfeldt-Jakob disease (CJD) is an uncommon neurodegenerative disorder with an incidence of 1 per 1000,000 per year typically characterized by rapidly progressive dementia, ataxia, myoclonus and behavioral changes. Genetic prion diseases, which develop due to a mutations in the prion protein gene (PRNP), account for an estimated 10 to 15% of all CJD cases. We report a 75-yr-old woman with fa...

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Creutzfeldt-Jakob disease: A case report

Background: Creutzfeldt-Jakob disease (CJD) as a life-threatening neurodegenerative disorder is not usually diagnosed in early stages of the disease because of a variety in its clinical manifestations. This study aimed to present a middle-aged woman with psychiatric symptoms who ultimately was diagnosed as a CJD case. Case presentation: This 48-year-old woman had progressive symptoms of depres...

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Familial Creutzfeldt-Jakob Disease without Periodic

Four members of a kindred wi th Creutzfeldt-Jakob disease are reported, i n whom myoclonus d id not develop and in whom serial electroencephalograms performed late in their illness failed to show periodic sharp wave complexes. Otherwise, the patients’ disease duration, clinical features, and neuropathological findings were similar to those described in sporadic cases of Creutzfeldt-Jakob diseas...

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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: Dementia and Neurocognitive Disorders

سال: 2017

ISSN: 1738-1495,2384-0757

DOI: 10.12779/dnd.2017.16.3.91