Creutzfeldt-Jakob disease experience in patients presenting to a tertiary care hospital: A case series

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare cause of rapidly progressive dementia, it is a neurodegenerative disorder caused by the misfolding of prion proteins in the brain, and misfolded proteins further propagate by causing misfolding of other proteins. It presents with insidious onset of neurobehavioral symptoms that rapidly develop into cognitive and motor decline and uncontrolled seizures.  The diagnosis is established with help of clinical signs and symptoms, and using imaging and lab investigations to rule out other treatable causes. Some imaging findings point to the diagnosis of CJD that will be viewed in the cases below.  Diagnoses can be augmented by CSF studies but due to the risk of biohazard and spread of CJD, special care needs to be taken. Confirmation is only via brain biopsy.  In this article we share our experience with four cases of CJD that presented to our hospital.