Wernicke encephalopathy in a child with acute lymphoblastic
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Wernicke encephalopathy (WE) is characterized by a clinical triad of ataxia, ophthalmoplegia, and altered mental state. It is said to be a rare entity in infants and children. The case of a 2-year-old child with acute leukemia on chemotherapy who presented with acute encephalopathy is described. MRI of the brain revealed isolated cranial nerve nuclei involvement. There was remarkable improvement in sensorium following thiamine therapy.

Wernicke encephalopathy (WE) is a medical emergency caused by thiamine deficiency. It is an acute neurological condition characterized by a clinical triad of ophthalmoparesis with nystagmus, ataxia, and confusion. Pediatric WE has been described in severe malnutrition, starvation, prolonged parenteral nutrition, and malignancies, following gastrointestinal surgeries and stem cell transplantation.

A previously healthy 2-year-old female child who had been diagnosed with acute lymphoblastic leukemia 5 months ago presented with excessive sleepiness, irritability, and incessant cry of 3-day duration. She is on maintenance phase chemotherapy according to the Indian childhood collaborative leukemia protocol and a case of standard risk under risk stratification. On examination, the child was drowsy, arousable but irritable, was not interacting, and not communicating. Extraocular movements were full and pupils equal and reacting to light. She was moving all her limbs, however was not sitting or standing on her own. There was generalized hypotonia; deep tendon reflexes were normal with flexor plantars.

MRI of the brain showed symmetric high signal alterations in facial nerve nuclei with diffusion restriction. There was no involvement of the thalamus, mammillary bodies, periaqueductal gray matter, or tectum in the midbrain Based on the clinical and imaging findings, WE was considered, and injection thiamine 100 mg intravenously twice daily was given for 3 days and once daily for the next 4 days. A rapid improvement in the neurological status was noticed within 24 h of thiamine treatment. She became active, alert, communicating, and started walking and playing on the 4th day.

Source: https://www.ijmpo.org/article.asp?issn=0971-5851;year=2020;volume=41;issue=5;spage=767;epage=769;aulast=Cornelius