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Creutzfeldt-Jakob disease: A great masquerade in neurology, a rare case report from South India
Journal article - Case report   Open access   Peer reviewed

Creutzfeldt-Jakob disease: A great masquerade in neurology, a rare case report from South India

Sivaprakash Varadan, Sudagar Singh, Deepak Rajkumar Vangipuram and Damodharan Jayachandran
Journal of the scientific society (Belgaum), Vol.42(3), pp.201-203
09/01/2015
DOI: 10.4103/0974-5009.165581
url
https://doi.org/10.4103/0974-5009.165581View
Published (Version of record) Open Access

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disease caused by an infectious protein called prion and is characterized by spongiform changes, neuronal loss, reactive astrocytic proliferation, and accumulation of pathologic cellular protein. Clinical presentation of CJD is characterized by rapidly progressive dementia, neurologic symptoms and visual impairment, and the development of akinetic mutism, which can mimic many neurological conditions. The diagnosis is based on clinical presentation, electroencephalogram, and typical cerebrospinal fluid and magnetic resonance imaging (MRI) findings. Literature on the incidence and prevalence of CJD is lacking in South India. We report the case of a 57-year-old woman with progressive dementia and typical neurologic symptoms, myoclonic jerks, and MRI findings of CJD. This case highlights the need for a high index of suspicion to diagnose CJD.
Dementia Electroencephalography Bovine spongiform encephalopathy Creutzfeldt-Jakob disease Dementia disorders Proteins

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