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Rare Complication of Hyperemesis Gravidarum: Wernicke-Korsakoff Syndrome
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Rifaat Abo-Leyah, Sara Al-Shubat, Loula Salih, Mariam Khaldi and Mariam Dossary
King Fahd Military Medical Complex, Dhahran, Saudi Arabia
Introduction
Case Report
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.
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.
Discussion
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.
Conclusions
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.
References
1. Henderson D. Korsaow’s psychosis occuring during pregnancy.
Bulletin of the Johns Hopkins Hospital 1914;25:261-70
2. 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 Syndrome
5. Davis , Icke G. Clinical chemisty of Thiamine. Adv Clin Chem
1983;23:93-140
6. Blass J, Gibson G. Abnormality of a thiamine requiring enzyme in
patents with Wernicke-korsakoff Syndrome. N Engl J Med 1977;297:136770
7. Committee on Dietary Allowances. Recommended dietary allowances.
9th ed. Washington: National Academy of Sciences 1980
8. Zimitat C, Nixon P (2000). “ Glucose loading precipitates
encephalopathy in thiamine –deficient rats”. Metabolic Brain Disease
14(1): 1-10
9. 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
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