A rare case of rapidly progressive dementia- Creutfeldt Jakob disease: Case report

Abstract

Background: Creutzfeldt Jakob disease is a rare and usually fatal neurodegenerative disorder characterized by rapidly progressive dementia with other neuropsychiatric manifestations like pyramidal, extrapyramidal, cerebellar, visual and behavioral abnormalities. Here we report a case of clinically probable Creutzfeldt Jakob disease in a 55 years old service holder lady presented with rapidly progressive dementia.  

Objective: The aim was to report a rare case of rapidly progressive dementia caused by Creutzfeldt Jakob disease.

Methods: The case was thoroughly evaluated clinically then probable diagnosis was made by characteristic generalized periodic discharges on EEG and cortical and basal ganglia hyperintensity on FLAIR and DWI sequences of MRI of brain in addition to the clinical features of rapidly progressive dementia, rigidity, myoclonus, akinetic mutism and behavioral abnormalities. Other causes of dementia were excluded.

Result: Finally probable diagnosis of Creutzfeldt Jakob disease was done according to CDC diagnostic criteria. The case could not be confirmed due to lack of available newer investigations in our country and refusal of autopsy by the patient’s guardian.

Conclusion: Though rare, a suspicion of Creutzfeldt Jakob disease should be considered in patients with rapidly progressive dementia with complex neurological manifestations. Variable clinical presentation and rarity of the disease always delay the diagnosis. The disease is always fatal and no accepted treatment is available till date. So if early and accurate diagnosis is possible then the prognosis and plan of management could be explained to the caregiver properly.

Bangladesh Med Res Counc Bull 2023; 49: 190-194