Case Presentation: A 39-year old female with a history of alcohol abuse presented with an eight-day history of numbness involving her bilateral lower extremities and hands which had progressed proximally to involve her chest wall. She reported perioral and perianal numbness, lethargy and difficulty ambulating. Examination was significant for decreased sensation in a glove-stocking distribution, areflexia and ataxia. Initial laboratory data was significant for folate and magnesium deficiencies which were treated. MRI of the brain, cervical and thoracic spine were normal. A clinical diagnosis of GBS was made and she received IVIG and subsequently plasmapheresis with no symptomatic improvement. Serial negative inspiratory force and vital capacity measurements were normal. CSF analysis, viral, Lyme and rheumatologic serologies were unremarkable. EMG was significant for axonal sensory neuropathy. Thiamine levels returned low at 60nmol/L (normal 78-148). These findings were consistent with a diagnosis of dry beriberi and treatment with IV thiamine was started with mild improvement in her symptoms. She was eventually discharged on oral thiamine. At 4-month follow-up, symptoms had slowly improved but she remained dependent on a walker for mobility.
Discussion: Dry Beriberi remains an under-recognized cause of peripheral neuropathy despite reports in the literature, and may be misdiagnosed as Guillain-Barré Syndrome (GBS). We report a case of thiamine deficiency causing GBS-like neuropathy in the setting of remote alcohol abuse. Thiamine deficiency causes clinical phenotypes of Beriberi and Wernicke-Korsakoff syndrome. Beriberi can be classified as “dry” or “wet”. Dry beriberi presents as weakness, paresthesia and neuropathic-type pain. The evolution of symptoms can occur very rapidly over the course of a few days (1), as seen in our patient. Limb involvement may be the only presentation in most cases of nutritional neuropathy compared with GBS where there is often involvement of the respiratory and bulbar muscles necessitating ventilation, which is extremely rare in beriberi.(2) Treatment is by thiamine supplementation via parenteral or oral route, and typically starts with parental administration if the patient is critically ill. Recovery from nutritional neuropathy is a slow process. There is often considerable improvement in mild cases but in severe forms, recovery may not be complete.(3)
Conclusions: It is important that thiamine deficiency be part of the differential diagnosis in any patient presenting with an acute neuropathy which may mimic GBS especially in the setting of alcohol abuse whether remote or recent.
References:
1. Windebank AJ. Polyneuropathy due to nutritional deficiency and alcoholism. In: Dyck PJ, Thomas PK, Griffin JW, et al, eds. Peripheral neuropathy, 3rd Edn. Philadelphia: WB Saunders 1993:1310–21.
2. Ropper AH, Brown RH. Diseases of the nervous system due to nutritional deficiency. In: Ropper AH, Brown RH, eds. Adams and Victor’s principles of neurology, 8th Edn. New York: McGraw-Hill, 2005:983–90
3. Murphy C, Bangash IH, Varma A Dry beriberi mimicking the Guillain–Barré syndrome Practical Neurology 2009;9:221-224.