Sporadic CJD clinically mimicking variant CJD with bilateral increased signal in the pulvinar

The premortem diagnosis of probable new-variant Creutzfeldt–Jakob disease (vCJD) may have important implications in terms of public health. Recently, a bilaterally increased pulvinar signal on T2-weighted MRI has been described in patients with vCJD.1,2⇓ The presence of a pulvinar high signal is necessary to classify a suspect case as probable vCJD according to the recently proposed diagnostic criteria, the estimated specificity of which is 100%.3 We report routine MRI and diffusion-weighted imaging (DWI) features in a neuropathologically confirmed case of sporadic CJD, which exhibits a bilateral increased signal in the pulvinar and a clinical phenotype of vCJD. A 55-year-old woman with no history of iatrogenic exposure to CJD was referred to a neurologic center for behavioral disorders. She acted aggressively toward her coworkers and family and quickly developed jealous delusions, dyscalculia, and memory impairments. Over the next year, her psychiatric signs worsened, and visual hallucinations and frontal lobe symptoms presented. Apraxia, spatial orientation anomalies, and parkinsonism occurred and the patient progressively developed cerebellar ataxia and myoclonus. Over …