ESPE2024 Poster Category 1 Fat, Metabolism and Obesity 3 (10 abstracts)
1Marmara University Pendik Training and Research Hospital, Department of Pediatric Neurology, Istanbul, Turkey. 2Marmara University, Pendik Training and Research Hospital, Department of Pediatric Endocrinology, Istanbul, Turkey
Introduction and Aim: Historically known as Beriberi, Wernicke's encephalopathy (WE), is a neurological emergency caused by thiamine (vitamin B1) deficiency. It's characterized by ophthalmoparesis and ataxia and carries a risk of permanent sequelae if not treated immediately. Diagnosis and treatment are often delayed because most cases don't present with typical history and neurological picture. With the advent of nutritional conditions worldwide, WE is almost eradicated, however, there is a resurgence of this old disease now particularly due to bariatric surgery in patients with obesity. In this presentation, we aim to draw attention of Pediatric Endocrinologists to this re-emerging entity by discussing four patients aged 11-18 years diagnosed recently with Wernicke encephalopathy in our Pediatric Neurology clinic.
Cases: Case 1. 17 9/12 y/o girl presented with gradually worsening muscle weakness. History revealed bariatric surgery for obesity 3 months ago and cholescytectomy two weeks ago. She has lost 50 kg in that time. Case 2. 11 y/o girl was consulted from Pediatric Surgery clinic presented with acute ataxia, nystagmus and headache. History revealed that she had been on total parenteral nutrition (for 30 days) due to short bowel syndrome following an intussusception operation 40 days ago. Case 3. 17 y/o girl presented with gradually worsening loss of vision, vomiting/nausea. History revealed (initially undisclosed) bariatric surgery for obesity 6 months ago and cholecystectomy 3 weeks ago. Case 4. 15 y/o boy with recent onset nystagmus, ataxic gait and decreased affection. History revealed a rapid weight loss (85 to 54 kg) in three months due to Anorexia Nervosa following diagnosis of her mother with cancer. Diagnosis of WE was promptly made in patients 1 and 2 based on their history and neurological findings, allowing timely initiation of high-dose thiamine treatment followed by rapid recovery. However, in Case 3 (bariatric surgery history was initially hidden by the parents) and Case 4, atypical neurological findings, led to delayed diagnosis resulting an extended ICU stayin Case3, and a prolonged recovery with some neurological sequelae in Case 4. The progression and findings of these cases will be discussed in detail at the congress.
Conclusion: As the age at which bariatric surgery is performed is decreasing, pediatric endocrinologists need to be aware ofthe complications related to these procedures. Bariatric surgeries must be performed only in competent centers with strict adherence to pre-and post-operative preparation and nutritional care guidelines to avoid such complications. Additionally, the increasing incidence of anorexia nervosa in pediatric endocrinology practice further underscores the importance of thiamine deficiency.