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Unusual Phenotype of the Brownell-Oppenheimer Variant of Sporadic Creutzfeldt-Jakob Disease

Authors: Dronacharya Lamichhane

DOI : 10.18639/RABM.2016.02.222222

Section : Case Report

Published Date : Mar 23, 2016

Abstract

Creutzfeldt-Jakob disease is a rare, transmissible, neurodegenerative disease caused by conformationally changed abnormal prion protein. Most patients present with cognitive impairment, myoclonus, ataxia, visual impairment alone or in combination. Patients who present with ataxia only at the onset are said to have Brownell-Oppenheimer variant of the disease. However, here we present a case where visual symptoms preceded the clinical presentation and hallucinations accompanied the ataxia at the onset of the disease.


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