Early identification and treatment of Wernicke encephalopathy in an adolescent patient

Thiamine (vitamin B1) deficiency has two forms, dry and wet beriberi. Wet beriberi involves the cardiovascular system. Dry beriberi involves the central nervous system and is associated with Wernicke encephalopathy (WE). The clinical triad of WE includes ophthalmoplegia, ataxia, and confusion. Although most common in older individuals, WE rarely occurs in the pediatric population, and many children have delayed diagnoses.1 The likelihood of thiamine deficiency is also increased after gastric surgery due to increased loss or malabsorption of thiamine, poor dietary intake, and/or increased metabolic requirement. We describe an adolescent with recent sleeve gastrectomy who presented with subacute encephalopathy, neuropathy, and ataxia. She was promptly treated with thiamine supplementation for suspected thiamine deficiency. This neurotypical adolescent girl presented to the emergency department (ED) after three days of encephalopathy, visual changes, dysarthria, ataxia, and paresthesias. She reported consuming an excessive amount of alcohol the night prior to the onset of her symptoms but denied other toxic ingestions. She had no fever or neck stiffness and denied bowel and bladder symptoms. In the ED she was confused, prompting computed tomography of the head without contrast. She was started on dextrose-containing maintenance intravenous fluids (IV) within the first six hours of arrival. Neurology was consulted and performed an evaluation at bedside the morning after her arrival and obtained further history. She had undergone a sleeve gastrectomy in a foreign country six months earlier and admitted noncompliance with vitamin supplementation and nutritional guidelines. Her vital signs were normal, and her general examination was notable only for an abdominal surgical scar. Her neurological examination was significant for fluctuating attentiveness requiring repetitive tactile stimulation, bilateral mydriasis, bilateral cranial nerve VI palsy, dysarthria, distal symmetric sensory deficits of her extremities, areflexia, and gait ataxia. The patient was evaluated for toxic, metabolic, infectious disease, vascular, and autoimmune disorders (Table 1) because of her initial findings. Due to the encephalopathy, visual changes, and ataxia, there was high suspicion for thiamine deficiency. Within 12 hours of presentation, she was empirically started on IV thiamine 500 mg every eight hours for two days. The dose was decreased to 250 mg, given intravenously, daily for five days. Her symptoms improved within two days of starting thiamine supplementation, and her thiamine level returned to the lower range of normal. Due to improvement in examination with thiamine, no further interventions were performed. She was discharged home on oral thiamine 100 mg daily. Although our patient's thiamine level was in the lower limit of normal, we do not have a baseline level prior to her sleeve gastrectomy for comparison. There is controversy related to the reference ranges reported for thiamine. Laboratories may extrapolate reference ranges from nonthiamine-deficient patients and are likely not specific to the pediatric population.2 Individuals undergoing a sleeve gastrectomy have a 94% chance of developing WE within six months after surgery.3 Our patient presented within the six-month mark due to recent excessive alcohol consumption and sleeve gastrectomy. Previous literature demonstrates the importance of follow-up and compliance with thiamine supplementation to prevent WE.4 Given her age, thiamine deficiency was not initially considered, and thiamine supplementation was delayed 12 hours after she arrived at the ED. Although the diagnosis of WE is rare in children and adolescents, it should be considered in high-risk situations.5 Intravenous thiamine should be administered promptly after obtaining pertinent surgical history in a patient who presents with encephalopathy associated with oculomotor dysfunction and ataxia, regardless of age, to prevent permanent neurological injury. Divya Gupta: Writing—original draft; writing—review and editing. Janetta L. Arellano: Writing—original draft; writing—review and editing. The authors have no funding to report. The authors declare no conflicts of interest.