Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder characterized by rapidly progressive dementia and myoclonus. Additional clinical features include visual disturbances, cerebellar, and pyramidal/extrapyramidal signs. The rarity of this disease and the wide variety of initial symptoms make the early diagnosis quite challenging. Nevertheless, it can also be diagnosed in a centre with limited resources through good clinical analysis. Here a case of a 53-year-old man is presented who had a 6 weeks history of cognitive deterioration and myoclonus. Brain magnetic resonance imaging (MRI) showed ribbon-like areas of hyperintensity in the bilateral cortex on diffusion-weighted imaging (DWI); bilateral hyperintense caudate nucleus and putamen on T2-weighted and Fluid-attenuated inversion recovery (FLAIR) images. Electroencephalogram (EEG) showed intermittent generalized periodic sharp waves of triphasic morphology. The diagnosis of probable sCJD was reached based on the clinical features and characteristic findings in his MRI and EEG according to WHO criteria. Several other works of the literature were also reviewed for early diagnosis of sCJD and for the exclusion of other differential diagnoses which may mimic sCJD.