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Rare Complication of Hyperemesis Gravidarum: Wernicke-Korsakoff Syndrome Rifaat Abo-Leyah, Sara Al-Shubat, Loula Salih, Mariam Khaldi and Mariam Dossary

King Fahd Military Medical Complex, Dhahran, Saudi Arabia

Introduction Discussion Conclusions

References

1. Henderson D. Korsaow’s psychosis occuring during pregnancy. Bulletin of the Johns Hopkins Hospital 1914;25:261-702. Lavin P, Smith D, Kori S, Ellenberger C. Wernicke’s encephalopathy: a predictable complication of hyperemesis gravidarum. Obstet Gynecol 1983;62:13-55 3. Wood P, Murry A, Sinha B, Godley M, Goldsmith H. Wernicke’s encephalopathy induced by hyperemesis gravidarum. J Obstet Gynecol 1983;90:583-6 4. Medline Plus – Wernicke-Korsakoff Syndrome5. Davis , Icke G. Clinical chemisty of Thiamine. Adv Clin Chem 1983;23:93-1406. Blass J, Gibson G. Abnormality of a thiamine requiring enzyme in patents with Wernicke-korsakoff Syndrome. N Engl J Med 1977;297:1367-70 7. Committee on Dietary Allowances. Recommended dietary allowances. 9th ed. Washington: National Academy of Sciences 19808. Zimitat C, Nixon P (2000). “ Glucose loading precipitates encephalopathy in thiamine –deficient rats”. Metabolic Brain Disease 14(1): 1-109. Watson A J, Walker J F, Tomkin G H, Finn M M, Keogh J A. (1981). Acute Wernickes encephalopathy pecipitated by glucose loading. Irish Journal of Medical science; 150(10):301-303

Hyperemesis Gravidarum (HG), affects a small percentage of pregnancies but can be life threatening. Wernicke-Korsakoff Syndrome is usually expected when a chronic alcoholic develops nystagmus, ataxia, confusion and amnesia. However, when these signs develop in other circumstances, the clinician may not suspect this diagnosis. Wernicke-Korsakoff Syndrome was reported as a complication of HG in 1914 [1] and cases continue to occur [2,3]. Objectives: We report this case to raise awareness of such serious, yet treatable condition.

Wernicke-Korsakoff Syndrome may result from neglected or inappropriately managed HG. Being aware of this possibility is the key point in managing this serious condition as treatment is usually available and successful. Thiamine supplements should be given for any pregnant woman who develops hyperemesis.

Case Report

A 27 year old Saudi woman who was 16 weeks pregnant, admitted to the OB/GYN ward through the hospital Emergency Room (ER) as a case of HG. She presented with intractable vomiting and nausea of about 8 weeks duration, during which she was seen at the ER few times but refused admission. She was dehydrated, rather confused, with unstable gait. This was her fourth pregnancy. She had had two normal full term deliveries and one early miscarriage. She had milder grades of nausea and vomiting in all previous pregnancies, but her diet before the current pregnancy was normal. Hydration with intravenous fluid (Normal Saline and Ringer Lactate) and antiemetic medications was initiated on admission, but she became more confused and ataxic. Family members reported that she no longer recognized them. She was drowsy and unable to follow commands. Coarse nystagmus was present in all directions of gaze.A clinical diagnosis of Wernicke-Korsakoff Syndrome complicating HG was made. Investigations suggested initial unbalanced electrolytes which has been corrected. As expected, Thiamine level was very low but MRI and EEG were normal.The patient showed a dramatic improvement soon after adding the Thiamine therapy. She was allowed home 3 weeks later in a satisfactory general conditions with mild nystagmus and loss of short memory. She continue to improve and became fully recovered by 36 weeks. At 39 weeks gestation, she delivered by caesarean section for breech presentation to a healthy baby boy.

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Wernicke-Korsakoff syndrome (WKS) is a combined manifestation of two eponymous disorders, Korsakoff’s Psychosis (KP) and Wernicke’s Encephalopathy (WE) . KP is characterized by: anterograde amnesia, retrograde amnesia, confabulation and hallucinations. WE is characterized by: confusion, nystagmus, ophthalmoplegia, anisocoria (unequal size of pupils), ataxia, sluggish pupillary reflexes in addition to coma and death if untreated [4]. WKS is due to a deficiency of thiamine (vitamin B-1), which plays a key role in various stages of carbohydrate metabolism [5]. HG is fairly common, but WKS is an uncommon complication. This may reflect a genetic background for this syndrome. People who develop WKS have a form of transketolase enzyme which binds thiamine less avidly than controls [6]. Daily requirement of thiamine increases during pregnancy to 1-5 mg [7]. Ingestion of carbohydrate rich food in presence of thiamine deficiency may worsen the neurological disorders and precipitate WKS [8, 9]. Glucose administration may promote decarboxylation of pyruvate, a biochemical reaction which requires thiamine. Thiamine supplements should be given to any pregnant women who develops hyperemesis. If confusion, nystagmus, ataxia, amnesia or hallucination develop, parenteral thiamine should be given until a normal diet is resumed. Clinician should not wait for a confirming investigation before commencing thiamine administration.