Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and its variable initial presentation make early diagnosis fairly challenging, especially considering it is a diagnosis of exclusion. We present a case of a patient initially admitted for sudden onset of confusion, memory loss, dysmetria, and ataxia after a recent diagnosis of Waldenstrom’s Macroglobulinemia. Within four weeks, after work-up for suspected Bing-Neel syndrome and failure to respond to appropriate symptomatic treatment, acute deterioration of mental status, akinetic mutism, and myoclonus were evident & Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the cortex and basal ganglia in diffusion-weighted imaging (DWI). The probable diagnosis of sCJD was reached based on the patient’s unresponsiveness to treatment, clinical features, characteristic findings on MRI, as well as positive 14-3-3 CSF assay.

Waldenstrom’s Macroglobulinemia, Bing-Neel syndrome, Creutzfeldt-Jakob disease, 14-3-3 CSF assay