Teaching Neuro Images : Extensive cortical involvement in Creutzfeldt-Jakob disease

Abstract

A 53-year-old alcoholic man presented with a 2-day history of worsening confusion. Initial examination showed appendicular hypertonia with multifocal arrhythmic asynchronous myoclonic jerks suggestive of cortical myoclonus occurring spontaneously intermittently and stimulus sensitive, with exaggerated deep tendon reflexes and extensor plantars. MRI brain demonstrated lowgrade restricted diffusion affecting the entire cerebral cortex (figure 1), sparing the subcortical gray matter and cerebellum.1 T1-weighted imaging and fluid-attenuated inversion recovery sequences showed mild cerebral atrophy. 14-3-3 protein testing was positive and generalized slowing was demonstrated on EEG.2 Changes consistent with Creutzfeldt-Jakob disease (CJD) were found on histopathology (figure 2). The patient had no risk factors for familial, new variant, or iatrogenic CJD. Sporadic CJD was the final diagnosis.

Rapidly progressive dementia with psychosis caused by CJD

“…1,16 Furthermore, PET can show a generalised hypometabolism due to diffuse atrophy of the brain. 17 The diagnosis of sCJD is based on the World Health Organization (WHO) clinical diagnostic criteria, which have been updated in 2009 to include the aforementioned typical MRI findings. 18 However, a definitive diagnosis of sCJD can be made only after the demonstration of prions in the brain.…”