A 10-year-old boy was referred for inpatient psychiatric evaluation at the Children’s National Medical Center, Washington, DC, because of a 2-month history of food refusal and a 9 kg weight loss after choking on a raisin. He ate only carbonated, sweetened beverages and occasional soft food. Complaining of diplopia, he was initially diagnosed with conversion disorder, and was treated for dehydration with 10% dextrose and water IV. He developed ptosis, vertical and horizontal nystagmus, ophthalmoplegia, ataxia and mild encephalopathy. Thiamine concentration was 0.12 mcmol/L (N. 0.16-0.23). Treatment with thiamine 100 mg IV was followed by neurologic improvement within 12 hours. Attention, short-term memory, and orientation to place and time were slower to resolve than eye muscle paresis and ataxia. He accepted a full diet after 1 week, and recovery was almost complete in 1 month. [1]
COMMENT. Wernicke’s encephalopathy is rare in children in the US. Reduction of whole blood transketolase enzyme is a diagnostic test for thiamine deficiency [2]. Infants fed thiamine-deficient formula, and children with emesis and weight loss during chemotherapy are at risk of Wernicke’s disease. The diagnosis should be suspected in malnourished infants and children, especially those with persistent vomiting. Reports of 9 cases and pathological findings at autopsy are included in Progress in Pediatric Neurology I, PNB Publ, 1991;pp544-5). The diagnosis is easily missed during life, especially in acute cases. IV glucose may precipitate symptoms of WE by depleting thiamine levels.