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An 86-year-old female was admitted to our hospital with acute progressive gait disturbance and cognitive impairment. Brain MR diffusion weighted imaging revealed abnormal high signal intensities in the bilateral hemispheres, dominantly in the frontal lobe. We first suspected acute encephalopathy due to Creutzfeldt-Jakob disease (CJD) from her clinical information. At the same time, we could not negate the possibility of Sjögren's syndrome (SjS) -related encephalopathy based on the abnormal findings on brain MRI and positive anti-SS-A antibody in the serum. After consulting with an otorhinolaryngologist and a pathologist, biopsy of the salivary gland was performed with a strict precaution against infection of prion virus. Pathological examination of the biopsy specimen showed accumulation of many lymphocytes around the gland, which satisfied grade 4 in the Greenspan classification. A definite diagnosis of SjS was made based on the pathological findings, and intravenous high dose methylprednisolone followed by oral prednisolone were administered for suspected SjS-related encephalopathy. However, the neurological symptoms did not improve and we judged that SjS-related encephalopathy was unlikely. The poor response to steroid therapy and the presence of tau protein, strongly positive 14-3-3 protein and a codon 180: Val/Ile mutation in the cerebrospinal fluid finally led to a clinical diagnosis of genetic CJD. In-hospital cooperation in terms of infection prevention is important when performing invasive procedure in the case of suspected CJD to distinguish treatable encephalopathy.

A case of genetic Creutzfeldt–Jakob disease that required differential diagnosis of acute encephalopathy associated with Sjogren’s syndrome