Abstract Details

Title Shoshin Beriberi Heralding Thiamine Deficiency as a Mimic of Guillain-Barre Syndrome

Topic Critical Care/Emergency Neurology/Trauma

Presentation(s) P01 - (-)

Poster/Presentation
Number 031

Objective

Background Thiamine deficiency can rarely mimic acquired neuropathies such as Guillain-Barre Syndrome(GBS). Thiamine deficiency may have variable presentation due to multisystem dysfunction including dry beriberi affecting peripheral nerves, Shoshin/wet beriberi affecting cardiovascular system or Wernicke/Korsakoff with CNS involvement.

Design/Methods Chart review of two cases of thiamine deficiency with features of dry beriberi, wet beriberi and Wernicke's encephalopathy.

Results Case 1: 72 year-old male with a history of diabetes and remote Whipple procedure who presented with four weeks of weakness and sensory loss. Exam showed distal sensory loss, mild proximal and distal lower limb weakness, and reduced reflexes. Axonal neuropathy was noted on EMG. CSF showed cytoalbuminologic dissociation. IVIG was initiated for a presumptive diagnosis of GBS. He subsequently developed hypotension requiring pressors, lactic acidosis, encephalopathy, ocular muscle weakness and respiratory failure. Case 2: 38 year-old male with a history of alcoholism and Crohn's was transferred to our facility with 3-4 weeks of progressive sensory and motor deficits, hypotension, tachycardia, and lactic acidosis attributed to GBS and superimposed sepsis. He subsequently developed severe generalized weakness, respiratory failure, encephalopathy and extraocular muscle paresis. Workup revealed axonal neuropathy, no infection and normal CSF findings. Diagnosis/Treatment: In both cases, features of Wernicke's and wet beriberi prompted the clinical suspicion supported by brain MRI studies and reduced thiamine levels (19&23 nmol/liter; normal 80-150nmol/liter). Hypotension and cardiac failure rapidly resolved with thiamine repletion. Ocular muscle weakness and encephalopathy resolved within days, but limb weakness only partially improved.

Conclusions A high index of suspicion for thiamine deficiency in patients presenting with progressive neuropathy is important as there is significant overlap with GBS. Development of hypotension, tachycardia, lactic acidosis, mental status changes, or extraocular muscle paresis in cases of possible GBS should prompt consideration of thiamine deficiency.

Authors/Disclosures
Ramit Panara, MD (Florida Neurology PA) PRESENTER	No disclosure on file
William D. Arnold, MD	Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for La Hoffmann Roche. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cadent Therapeutics . Dr. Arnold has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. The institution of Dr. Arnold has received research support from NIH. The institution of Dr. Arnold has received research support from NMD Pharma. The institution of Dr. Arnold has received research support from Gilead Sciences. The institution of Dr. Arnold has received research support from CureSMA. Dr. Arnold has received intellectual property interests from a discovery or technology relating to health care.
Leslie A. Meltzer, PhD (Biogen Idec)	No disclosure on file

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