Abstract

<b>Objective: </b>To report two unusual cases of thiamine deficiency presenting as acute on chronic cognitive/psychiatric impairment. In both cases, brain MRI also suggested Creutzfeldt Jakob disease (CJD). <b>Background: </b>Thiamine is needed to sustain osmotic gradient and it is crucial for glucose metabolism and neurotransmitter synthesis. Daily thiamine requirement is about 2 mg; without thiamine intake, the reserves could be depleted in 4-6 weeks. Thiamine deficiency classically presents as acute Wernicke’s encephalopathy (WE) with the triad of altered mental status, ataxia, and ocular signs. CJD is a rapidly progressive dementia that can also present acutely with psychiatric prodrome and altered mental status. We present two WE cases which posed diagnostic challenge due to prior cognitive/psychiatric impairment, non-classic presentation, and challenging MRI findings. <b>Design/Methods:</b> Case series. <b>Results: </b>Our cases include two women, ages 58 and 62 years old with underlying schizophrenia and Alzheimer disease respectively. Both stopped eating due to their underlying conditions and presented with acute changes in mental status. Neither patient had ocular signs or ataxia. In both cases, brain MRIs showed diffusion restriction and FLAIR hyperintensity in bilateral thalami, which suggested CJD as the underlying cause. Both patients were later diagnosed with thiamine deficiency, and CJD was ruled out. <b>Conclusions:</b> Both of our patients presented as worsening of their underlying disease, in a setting of decreased nutritional intake. However, since both did not present with the full WE triad, and brain MRI highly suggested CJD, further work up was necessary to rule out the possibility of CJD. WE is likely underrecognized as the classic triad occurs in only 16-38[percnt] of patients. It should be in the differential of acute cognitive decline in patients with underlying conditions that can result in decreased nutritional intake. Early recognition is crucial as with early treatment prognosis is usually favorable. <b>Disclosure:</b> Dr. Mikhaeil-Demo has nothing to disclose. Dr. Schuele has received personal compensation for activities with Sunovion and Eisai as a speaker. Dr. Srivastava has nothing to disclose. Dr. Bonakdarpour has nothing to disclose.

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