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Background/objectives: Thiamine deficiency is a treatable disease with an excellent prognosis. However, it is often unrecognized because of the diversity of its clinical presentations., Subjects/methods: Herein, we report two atypical cases of nonalcoholic thiamine deficiency that presented with refractory hypotension in the absence of lactic acidosis., Results: Case 1 developed recurrent hypotension, right-sided heart failure, and a classic triad of Wernicke's encephalopathy (WE) after gastrointestinal surgery. Case 2 had decreased dietary intake and diuretic abuse, and had multiple episodes of syncope prior to present admission with refractory hypotension and mental status changes. The diagnosis of both cases was confirmed by undetectable pretreatment serum thiamine and dramatic improvement with thiamine replacement., Conclusions: In this report, we highlight refractory hypotension as a complication of, not only cardiovascular, but also neurologic beriberi. Moreover, thiamine replacement should be considered without delay in hypotensive patients with signs of WE and/or risk factors for beriberi.

Background and Purpose: Brain imaging is central to the diagnosis of infantile encephalitic beriberi. Because cranial sonography findings have not been described in infantile encephalitic beriberi, our aim was to investigate its role in the diagnosis of this condition., Materials and Methods: We performed a retrospective review of head sonography of infants (admitted between November 1, 2014, and March 31, 2015) who presented with encephalopathy. Cranial ultrasonography scans were studied for the alteration of echogenicity of the basal ganglia., Results: Of the 145 consecutive infants who presented with encephalopathy, 58 had thiamine-responsive encephalopathy (infantile encephalitic beriberi) and 87 had encephalopathy due to other causes. Forty-eight of 145 infants with encephalopathy showed hyperechoic basal ganglia. A hyperechoic appearance of the basal ganglia on cranial ultrasonography was found to have a sensitivity of 71% (41/58) and a specificity of 92% (80/87) in diagnosing infantile encephalitic beriberi. The sensitivity of cranial sonography increased with age. It was a maximum of 93% (14/15) in the 5 months and older age group. Specificity was a maximum of 100% (18/18) in infants older than 5 months of age. Sensitivity was maximum in Wernicke encephalopathy at 90% (18/20) and least in the acidotic form at 43% (10/23). Follow-up showed gradual normalization of the hyperechoic appearance of the basal ganglia during 8 weeks in 26/41 (63%), with mild atrophy of the basal ganglia in 6/41 (15%), Conclusions: Hyperechogenicity of the basal ganglia on cranial ultrasonography is a sensitive finding for the diagnosis of infantile encephalitic beriberi in infants who present with Wernicke encephalopathy., (© 2016 by American Journal of Neuroradiology.)