Pulvinar Sign in Sporadic Creutzfeldt-Jakob Disease: Exploring the Diagnostic Modalities of Prion Diseases

Abstract

Creutzfeldt-Jakob disease ( CJD ) refers to the most common t ransmissible s pongiform e ncephalopathies. The distinction between a diagnosis of sporadically acquired CJD and the exogenously acquired CJD is important, and has previously been assisted by imaging modalities revealing the pulvinar sign. This case report describes a 71-year-old n ative-American female with rapidly deteriorating mental status, progressive personality changes, ataxia, and 50-pound weight loss. Non-contrast MRI of the brain was significant for symmetrical high diffusion signal in the bilateral pulvinar thalami. CSF studies performed by the National Prion Disease Surveillance Center showed a positive 14-3-3 level and an elevated tau conveying a positive predictive value of 72.6% for CJD. The case exemplifies commonality between sporadic CJD and variant CJD and challenges the utility of radiological findings differentiating the diseases. Our case emphasizes that the pulvinar sign may not be as specific for a diagnosing variant CJD as we once thought. doi: http://dx.doi.org/10.4021/jmc1278w