Stem Cell Transplant-Associated Wernicke Encephalopathy in a Patient with High-Risk Neuroblastoma

Abstract

Children undergoing intense cancer treatment frequently require total parenteral nutrition (TPN). Rarely, vitamins are removed due to hypersensitivity to the carrier vehicle in the formulation. We present the case of a 5-year-old patient with stage 4, high-risk neuroblastoma who developed altered mental status, ataxia, and tachycardia during consolidative autologous stem cell transplantation. Skin findings and brain MRI were consistent with thiamine (vitamin B1) deficiency and Wernicke encephalopathy. Vitamin B1 administration rapidly reversed all skin and neurologic symptoms. This case highlights the importance of close monitoring of micronutrients in pediatric patients receiving prolonged courses of chemotherapy and stem cell transplantation.

Thiamine Deficiency in a Child with Short Bowel Syndrome and Review

“…There are no guidelines for treatment of thiamine deficiency-caused Wernicke encephalopathy in children in terms of dosing, preferred route of administration, duration of therapy, or maintenance therapy. The most frequent therapy includes administration of 100 mg of thiamine intravenously daily (Table 1) [56789101112]. However, we achieved similar results—that is, improvement within 48 hours and complete reversion of clinical and imaging findings—with a lower dose of 25 mg of thiamine administered intramuscularly.…”

“…Finally, the caregivers were asked if, during home administration of the PN, vitamin supplementation had been omitted. They denied such a possibility, but it remains a likely cause, since our patient was greatly improved clinically, and considering that even medical staff can underevaluate the significance of vitamin supplementation in these settings, as shown in our case series (Table 1) [56789101112], a caregiver is even more likely to underappreciate their importance.…”

“…We included all cases after 1999 of children below 18 years of age who developed Wernicke encephalopathy while receiving TPN. We found eight cases that met our criteria (Table 1) [56789101112].…”

“…Our review of pediatric patients after 1999 showed that all patients who were receiving TPN and ultimately developed Wernicke encephalopathy were not routinely receiving vitamin supplementation. Out of the eight cases, one was due to caregivers' non-compliance [9] and one to discontinuation after a presumed hypersensitivity reaction [12]. Therefore, despite no shortage in parenteral vitamin supplementation, iatrogenic non-alcoholic Wernicke encephalopathy remains a problem, which must be addressed with increasing awareness.…”

Pediatric thiamine deficiency disorders in high‐income countries between 2000 and 2020: a clinical reappraisal

“…This encompasses a wide panel of clinical conditions from intestinal resection (short bowel syndrome) to inflammatory bowel disease in children 85–91 C8 : PTD related to pediatric malignancies (such as leukemia and neuroblastoma) constantly associated with hyperlactatemia and patients undergoing bone marrow transplant (7%) 92–107 . Of note, three cases of drug‐induced TD (namely, methotrexate and metronidazole) have been included in this review 87,93,101 …”