Hyperekplexia as the presenting symptom of Creutzfeldt-Jakob disease

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Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia.

OBJECTIVE To report the clinical, neuropsychological, linguistic, imaging, and neuropathological features of a unique case of sporadic Jakob-Creutzfeldt disease in which the patient presented with a logopenic variant of primary progressive aphasia. DESIGN Case report. SETTING Large referral center for atypical memory and aging disorders, particularly Jakob-Creutzfeldt disease. PATIENT Pat...

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Familial Creutzfeldt-Jakob disease presenting as epilepsia partialis continua.

Creutzfeldt-Jakob disease (CJD) is a rare disorder caused by prions that can affect any part of the central nervous system. It is characterized by a long incubation period, but once symptoms start there is a progressive neurological decline. Clinical features include dementia, ataxia and myoclonus (startle), among others. We report a biopsy-proven case of familial CJD (fCJD) presenting with con...

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Unilateral Creutzfeldt-Jakob disease presenting as rapidly progressive aphasia.

A 64-year-old man presented with a three day history of progressive Broca's aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasic. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed ...

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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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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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ژورنال

عنوان ژورنال: Neurology: Clinical Practice

سال: 2015

ISSN: 2163-0402,2163-0933

DOI: 10.1212/cpj.0000000000000150