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CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 46-49

Wernicke&'s encephalopathy in hyperemesis gravidorum


1 Department of Medicine, Sher-I-Kashmir Institute of Medical Sciences Medical College and Hospital, Srinagar, Jammu and Kashmir, India
2 Department of Radiodiagnosis, Sher-I-Kashmir Institute of Medical Sciences Medical College and Hospital, Srinagar, Jammu and Kashmir, India
3 Department of Radiodiagnosis, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India

Date of Submission09-Jul-2015
Date of Acceptance28-Sep-2015
Date of Web Publication12-Jan-2016

Correspondence Address:
Muzafar Naik
Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences Medical College and Hospital, Bemina, Srinagar, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2231-0738.173785

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   Abstract 

Wernicke&'s encephalopathy (WE) is potentially a fatal complication due to the deficiency of vitamin B1 and presents with a neurological triad of nystagmus, encephalopathy, and ataxia. It is most commonly seen in alcoholics and is a rare complication of pregnancy associated with hyperemesis gravidorum (HG). Since it is a rare condition, difficult to diagnose in pregnancy, and can prove to be fatal, a high index of suspicion should be made in case of pregnancy complicated by HG. We hereby report a case of WE caused by HG successfully treated with a high dose intravenous thiamine. This case is the first of its kind from our part of world.

Keywords: Hyperemesis gravidorum, thiamine, Wernicke&'s encephalopathy


How to cite this article:
Bhat T, Naik M, Mir MF, Robbani I, Mohiuddin M. Wernicke&'s encephalopathy in hyperemesis gravidorum. Int J Nutr Pharmacol Neurol Dis 2016;6:46-9

How to cite this URL:
Bhat T, Naik M, Mir MF, Robbani I, Mohiuddin M. Wernicke&'s encephalopathy in hyperemesis gravidorum. Int J Nutr Pharmacol Neurol Dis [serial online] 2016 [cited 2020 Jan 20];6:46-9. Available from: http://www.ijnpnd.com/text.asp?2016/6/1/46/173785


   Introduction Top


Long-lasting and uncontrollable nausea and vomiting due to hyperemesis gravidorum (HG) may lead to dehydration, ketosis, and hypochloremic metabolic alkalosis resulting in increased maternal morbidity and mortality. It may lead to Wernicke&'s encephalopathy (WE) in some pregnant women, manifesting with diplopia, acute confusional state, and gait ataxia. The usual risk factors for WE are chronic alcoholism, previous gastrectomy, malnutrition, etc. WE due to severe HG is uncommon (0.1–0.5%).[1] We report a case of WE induced by severe HG.


   Case Report Top


A 28-year-old woman gravida 2, para 1, with 16 weeks amenorrhea was on gynecological follow-up for severe nausea and vomiting of 2 months duration with diagnosis of HG and was managed with antiemetic therapy. She was referred to the the general medicine department of the hospital in view of her history of immediate memory loss of 3 weeks duration. The patient also complained of diplopia for the past 7 days and unsteadiness of gait for the last 3 days.

Physical examination revealed pulse rate of 100/min, afebrility, respiratory rate of 18 breaths/min, blood pressure (BP) of 90/60 mmHg, pallor with no jaundice, cyanosis, and edema. Systemic examination revealed a normal chest, cardiovascular system and abdominal examination. In central nervous system mental state examination: Mental state examination revealed that the patient was conscious but not oriented in time, place, and person. She had inattentiveness and her calculation was impaired. She also had immediate memory loss. Cranial nerves examination: There was bilateral horizontal GIII nystagmus; the rest of the cranial nerves were normal. The sensory and motor systems were normal and the patient had ataxic gait.

On admission, laboratory investigations revealed hemoglobin of 8.7 g/dL with mean corpuscular volume (MCV) of 17.5 ft with normal total leukocyte count and platelet count, normal kidney function tests, and liver function tests: Bilirubin 0.56 mg/dL, alanine aminotransferase 216 units/L total protein 8.14 g/dL, albumin 4.29 g/dL, globulin 3.85 g/dL, total cholesterol 239 mg/dL, triglycerides: 221 mg/dL, high-density lipoprotein (HDL) 45, low-density lipoprotein (LDL) 169, lactate dehydrogenase (LDH)385, and uric acid (UA) 3.3 mg. The urine examination was normal, electrocardiogram (ECG) was normal, electrolytes were normal, and thyroid-stimulating hormone (TSH) was 4.03 m u/L with normal T3 and T4. The abdominal ultrasound revealed a single intrauterine fetus with mean gestational age (MGA) 14 weeks ± 5 days. Magnetic resonance imaging (MRI) of the brain [Figure 1] and [Figure 2] performed on the 3rd day showed periacqueductal gray and medial thalamus hyperintensity suggestive of WE.
Figure 1: Magnetic resonance imaging of the brain showing periacqueductal gray hyperintensity

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Figure 2: MRI of the brain showing medial thalamus hyperintensity

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As the clinical signs were consistent with the diagnosis of HG with WE, which was later confirmed by MRI. Tests to assess blood thiamine levels or erythrocyte transketolase activity (ETKA) were not performed because of the nonavailability of such tests in our hospital. The patient was started on immediate intravenous thiamine replacement at doses of 500 mg 8 hourly over 30 min for 2 days followed by 500 mg/day for another 7 days. Her condition improved gradually over the next few days and the neurological examination conducted over the day revealed improvement in diplopia/confusional state and unsteadiness of gate; however, the patient persisted with minimal memory impairment. Vomiting also improved on Ondem 8 mg intravenously 8 hourly.

The patient was discharged with the advice to take oral thiamine 100 mg/day for the rest of the gestation with proton pump inhibitor (PPI) and antiemetics intermittently.


   Discussion Top


WE is most often associated with chronic alcoholism, malabsorption, poor dietary intake, increased metabolic requirement (e.g., during systemic illnesses), anorexia nervosa or dieting,[2] hyperemesis of pregnancy,[3],[4] prolonged intravenous feeding without proper supplementation,[5] prolonged fasting or starvation, or unbalanced nutrition, especially with refeeding,[6] gastrointestinal surgery (including bariatric surgery),[7] systemic malignancy,[8] transplantation,[9] hemodialysis or peritoneal dialysis,[10] acquired immunodeficiency syndrome,[11] and genetic disorder of thiamine metabolism.[12] Thiamine is a water soluble vitamin. The recommended daily requirement is 1.1 mg and the body stores are around are 25–30 mg. During pregnancy, the requirement for thiamine increases parallel to the fetal development and the requirement increases to 1.5 mg/day.[13] The body stores of thiamine usually last for around 18 days and inadequate oral intake and the recurrent vomiting increase the potential risk for thiamine deficiency.

HG as a predisposing condition of WE was first reported in 1914. In patients with HG, uncontrolled vomiting, prolonged intravenous feeding, and thiamine free glucose infusion may lead to the development to WE.[14] The longest and shortest duration of vomiting until the development of WE have been reported as 17 weeks and 4 weeks, respectively.[15] In our patient, the vomiting had a total duration of 8 weeks.

Although HG is a common complication of pregnancy, WE is uncommon complication of HG. WE is usually associated with chronic alcoholism; however, due to religious and cultural factors in our valley there is lower prevalence of chronic alcoholism. Therefore, the index of suspicion of WE in our setting remains very low. On the other hand, thiamine deficiency occurs where the diet consists mainly of milled white cereals, including polished rice, and wheat flour, all of which are very poor sources of thiamine. In Kashmir, India polished rice is the staple diet and tendency to develop thiamine deficiency remains high in general population. In a study on 650 heart failure patients in Kashmir valley, India 27 (4.15%) patients had beriberi as the cause of high output failure.[16]

WE is primarily a clinical diagnosis. Institution of treatment takes priority over diagnosis, and response to treatment may be diagnostic. Imaging studies are not necessary in all patients with suspected WE and should not delay treatment. However, diagnostic imaging can be helpful by providing evidence of WE in many patients and may rule out alternative diagnoses.

MRI is more sensitive than computed tomography (CT) in detecting acute diencephalic and periventricular lesions.[17] The typical MRI brain findings of areas of increased T2 and decreased T1 signal surrounding the aqueduct and third ventricle and within the medial thalamus and mamillary bodies [17],[18] were consistent with our patient and supported the diagnosis of WE.

Patients with suspected WE require immediate parenteral administration of thiamine. Our patient received the recommended regimen of 500 mg of thiamine intravenously, infused over 30 min, three times daily for 2 consecutive days and 500 mg intravenously or intramuscularly once daily for an additional five days, in combination with other B vitamins.[19] The ocular symptoms, signs, and gait instability improved immediately and patient was able to walk without support on the next day. There was also improvement in the impaired mental status; however, the patient persisted with mild impairment of memory.


   Conclusion Top


In conclusion, despite the low prevalence of chronic alcoholism in our patient population WE should be suspected in nutritionally compromised patients with impaired mental status, gait instability, or visual disturbances, and especially in patients of HG. More importantly, in patients of HG suspected of WE high dose thiamine therapy should be given priority over intravenous fluid therapy and confirmation of diagnosis of WE as, if left untreated, it could lead to irreversible brain damage.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Nelson-Piercy C. Treatment of nausea and vomiting in pregnancy when should it be treated and what can be safely taken? Drug Saf 1998;19:155-64.  Back to cited text no. 1
    
2.
Sharma S, Sumich PM, Francis IC, Kiernan MC, Spira PJ. Wernicke&'s encephalopathy presenting with upbeating nystagmus. J Clin Neurosci 2002;9:476-8.  Back to cited text no. 2
    
3.
Gárdián G, Vörös E, Járdánházy T, Ungureán A, Vécsei L. Wernicke&'s encephalopathy induced by hyperemesis gravidorum. Acta Neurol Scand 1999;99:196-8.  Back to cited text no. 3
    
4.
Spruill SC, Kuller JA. Hyperemesis gravidorum complicated by Wernicke&'s encephalopathy. Obstet Gynecol 2002;99:875-7.  Back to cited text no. 4
    
5.
Parkin AJ, Blunden J, Rees JE, Hunkin NM. Wernicke-Korsakoff syndrome of nonalcoholic origin. Brain Cogn 1991;15:69-82.  Back to cited text no. 5
    
6.
Suzuki Y, Matsuda T, Washio N, Ohtsuka K. Transition from upbeat to downbeat nystagmus observed in a patient with Wernicke&'s encephalopathy. Jpn J Ophthalmol 2005;49:220-2.  Back to cited text no. 6
    
7.
Chaves LC, Faintuch J, Kahwage S, Alencar Fde A. A cluster of polyneuropathy and Wernicke&'s -Korsakoff syndrome in a bariatric unit. Obes Surg 2002;12:328-34.  Back to cited text no. 7
    
8.
Macleod AD. Wernicke&'s encephalopathy and tyerminal cancer: Case report. Palliat Med 2000;14:217-8.  Back to cited text no. 8
    
9.
Bleggi-Torres LF, de Medeiros BC, Werner B, Neto JZ, Loddo G, Pasquini R, et al. Neuropathological findings after bone marrow transplantation: An autopsy study of 180 cases. Bone Marrow Transplant 2000;25:301-7.  Back to cited text no. 9
    
10.
Jagadha V, Deck JH, Halliday WC, Smyth HS. Wernicke&'s encephalopathy in patients on peritoneal dialysis or hemodialysis. Ann Neurol 1987;21:78-84.  Back to cited text no. 10
    
11.
Soffer D, Zirkin H, Alkan M, Berginer VM. Wernicke&'s encephalopathy in Acquired immune deficiency Syndrome (AIDS): A case report. Clin Neuropathol 1989;8:192-4.  Back to cited text no. 11
    
12.
Kono S, Miyajima H, Yoshida K, Togawa A, Shirakawa K, Suzuki H. Mutations in a thiamine-transporter gene and Wernicke&'s-like encephalopathy. N Engl J Med 2009;360:1792-4.  Back to cited text no. 12
    
13.
Subcommittee on nutritional status and weight gain during pregnancy; subcommittee on nutritional status during pregnancy and lactation; Food and nutrition board; Institute of Medicine (US). Nutrition during pregnancy: Summary. Washington, DC: National Academy Press; 1990. p. 1-23.  Back to cited text no. 13
    
14.
Selitsky T, Chandra P, Schiavello HJ. Wernicke&'s encephalopathy with hyperemesis and ketoacidosis. Obstet Gynecol 2006;107:486-90.  Back to cited text no. 14
    
15.
Togay-Işikay C, Yiğit A, Mutluer N. Wernicke&'s encephalopathy due to hyperemesis gravidorum: An under-recognised condition. Aust N Z J Obstet Gynaecol 2001;41:453-6.  Back to cited text no. 15
    
16.
Iqbal K, Ali M, Tramboo N, Lone A, Mohiuddin K, Naikoo B, et al. Cardiac beriberi as a cause of high output failure in a developing country: Resurgence or overlooked? J Mol Cell Cardiol 2008;44:786-7.  Back to cited text no. 16
    
17.
Gallucci M, Bozzao A, Splendiani A, Masciocchi C, Passariello R. Wernicke&'s encephalopathy: MR findings in five patients. AJNR Am J Neuroradiol 1990;11:887-92.  Back to cited text no. 17
    
18.
Zuccoli G, Pipitone N. Neuroimaging findings in acute Wernicke&'s encephalopathy: Review of the literature. AJR Am J Roentgenol 2009;192:501-8.  Back to cited text no. 18
    
19.
Cook CC, Hollywood PM, Thomson AD. B vitamin deficiency and neuropsychiatric syndromes in alcohol misuse. Alcohol Alcohol 1998;33:317-36.  Back to cited text no. 19
    


    Figures

  [Figure 1], [Figure 2]


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