Evolution of Clinical, Electroencephalographic, and Radiological Changes in Sporadic Creutzfeldt–Jakob Disease

Abstract

Creutzfeldt–Jakob disease (CJD) is a rare, progressive, and fatal disease. Sporadic CJD (sCJD) accounts for 85% of the CJD cases. It is characterized by rapidly progressive dementia, myoclonic jerks, and pyramidal, extrapyramidal, visual, and psychiatric symptoms. Electroencephalography (EEG) and magnetic resonance imaging (MRI) of the brain are integral to the diagnosis of the disease and show serial changes as the disease progresses. MRI brain in sCJD may be initially normal and later evolve to high-intensity signal changes in cortical, striatal, and thalamic regions on diffusion-weighted sequences and fluid-attenuated inversion recovery sequences. EEG shows evolution from the early, nonspecific slowing to the characteristic periodic sharp wave complexes (PSWC) in middle and late stages. We, hereby, report the case of a 68-year-old male who presented with rapidly progressive dementia, psychiatric symptoms, and myoclonus, whose MRI brain was initially normal and later showed diffusion restriction involving bilateral caudate, putamen, and bilateral cingulate gyrus and EEG showed evolution from diffuse slowing to typical PSWC.

Keywords

• electroencephalography
• magnetic resonance imaging
• periodic sharp wave complexes
• sporadic creutzfeldt–jakob disease