BERİL DİLBER, Tülay KAMAŞAK, İLKER EYÜBOĞLU, Mehmet KOLA, Ahmet Taner UYSAL, Haluk SARIHAN, Hatice Sonay YALÇIN CÖMERT, Elif SAĞ, Ali CANSU

Wernicke’s encephalopathy manifesting with diplopia after ileojejunostomy: report of a pediatric case with Hirschsprung disease

Background. Wernicke’s encephalopathy (WE) is a coenzyme-induced disease with acute neuropsychiatric symptoms leading to high mortality and morbidity due to thiamine deficiency. WE is mostly caused by alcoholism in adult populations; however, it is often associated with gastrointestinal surgical procedures, recurrent vomiting, chronic diarrhea, cancer and chemotherapy treatment, systemic diseases, drugs, magnesium deficiency, and malnutrition in children. Although these predisposing factors are considered to be uncommon in children, they are actually highly frequent and can be fatal if not treated promptly. Case. In this report, we present a patient who developed diplopia during total parenteral nutrition following surgical resection and was diagnosed with WE. The findings of the patient's cranial magnetic resonance imaging (MRI) findings were consistent with those of WE and the ocular findings of the patient resolved completely after thiamine treatment. Conclusion. Although WE is rare in children it can be prevented by early diagnosis and treatment and oculomotor findings such as diplopia can be a warning sign.

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1. Wernicke C. Die akute haemorrhagische polioencephalitis superior. In: Lehrbuch der Gehirnkrankheiten fur Aerzte und Studirende, Bd II, Kassel: Fisher Verlag, 1881: 229-242.

2. Sechi G, Serra A. Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet Neurol 2007; 6: 442-455.

3. Kamaşak T, Kul S, Tuşat M, Ozgun N, Cansu A. A case of wernicke encephalopathy developing after ileal bypass surgery. Pediatr Emerg Care 2018; 34: e223-e225.

4. Acar Arslan E, Ekinci S, Zengin Akkuş P, Göçmen R, Haliloğlu G. Wernicke encephalopathy due to thiamine deficiency after surgery on a child with duodenal stenosis. Pediatr Neurol 2014; 51: 840-842.

5. Park SW, Yi YY, Han JW, Kim HD, Lee JS, Kang HC. Wernicke's encephalopathy in a child with high dose thiamine therapy. Korean J Pediatr 2014; 57: 496-499.

6. Cerase A, Rubenni E, Rufa A, et al. CT and MRI of Wernicke's encephalopathy. Radiol Med 2011; 116: 319-333.

7. Manzo G, Gennaro AD, Cozzolino A, Serino A, Fenza G, Manto A. MR imaging findings in alcoholic and nonalcoholic acute wernicke's encephalopathy: a review. Biomed Res Int 2014; 2014: 503596

8. Victor M, Adams RD, Collins GH. The WernickeKorsakoff syndrome: a clinical and pathological study of 245 patients, 82 with post-mortem examinations. Contemp Neurol Ser 1971; 7: 201-206.

9. Manzo L, Locatelli C, Candura SM, Costa LG. Nutrition and alcohol neurotoxicity. Neurotoxicology 1994; 15: 555-565.

10. Harper CG, Giles M, Finlay-Jones R. Clinical signs in the Wernicke-Korsakoff complex: a retrospective analysis of 131 cases diagnosed at necropsy. J Neurol Neurosurg Psychiatry 1986; 49: 341-345.

11. Vasconcelos MM, Silva KP, Vidal G, Silva AF, Domingues RC, Berditchevsky CR. Early diagnosis of pediatric Wernicke’s encephalopathy. Pediatr Neurol 1999; 20: 289-294.

12. Lockman PR, McAfee JH, Geldenhuys WJ, Allen DD. Cation transport specificity at the blood-brain barrier. Neurochem Res 2004; 29: 2245-2250.

13. Chan F, Butterworth RF, Hazell AS. Primary cultures of rat astrocytes respond to thiamine deficiencyinduced swelling by downregulating aquaporin-4 levels. Neurosci Lett 2004; 366: 231-234.

14. Desjardins P, Butterworth RF. Role of mitochondrial dysfunction and oxidative stress in the pathogenesis of selective neuronal loss in Wernicke’s encephalopathy. Mol Neurobiol 2005; 31: 17-25.

15. Mumford CJ. Papilloedema delaying diagnosis of Wernicke's encephalopathy in a comatose patient. Postgrad Med J 1989; 65: 371-373.

16. Manzanares W, Hardy G. Thiamine supplementation in the critically ill. Curr Opin Clin Nutr Metab Care 2011; 14: 610-617.

17. Zuccoli G, Pipitone N. MR Imaging: an increasingly important tool in the early diagnosis of Wernicke encephalopathy. AJNR Am J Neuroradiol 2012; 33: E92.

18. Galvin R, Brathen G, Ivashynka A, Hillbom M, Tanasescu R, Leone MA. EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy. Eur J Neurol 2010; 17: 1408-1418.