Creutzfeldt-Jakob Disease With Amyotrophy and Demyelinating Polyneuropathy

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Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy.

OBJECTIVE To report the clinical and neuropathological features in a patient with Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy. DESIGN Case report. PATIENT AND RESULTS A 62-year-old man had progressive numbness of the left foot, unsteady gait, diminished deep reflexes, fasciculations, and tactile hypesthesia on the feet. Cerebrospinal fluid, electroneurography,...

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Genetic Creutzfeldt-Jakob disease mimicking chronic inflammatory demyelinating polyneuropathy

Creutzfeldt-Jakob disease (CJD) is a relentlessly progressive neurodegenerative disorder, belonging to the transmissible spongiform encephalopathies. It is characterized by the deposition of the abnormal isoform of the prion protein (PrP), causing spongiform neurodegeneration. The disease is classically known to mainly affect the gray matter of the CNS. However, involvement of the peripheral ne...

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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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A patient with Creutzfeldt-Jakob disease presenting with amyotrophy: a case report

INTRODUCTION Creutzfeldt-Jakob disease (CJD) is an ultimately fatal, neurodegenerative disease caused by misfolded prion protein aggregation and accumulation. The development of amyotrophic features has been described in CJD, though rarely as an early or prominent feature. Consequently, the significance of amyotrophy in prion disease etiology remains unclear. CASE PRESENTATION Our patient, a ...

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ژورنال

عنوان ژورنال: Archives of Neurology

سال: 2002

ISSN: 0003-9942

DOI: 10.1001/archneur.59.11.1811