|Year : 2019 | Volume
| Issue : 2 | Page : 113-115
Wernicke korsakoff syndrome as a rare complication of hyperemesis gravidarum: A case report
Khalil Malki, Mohammed Aabdi, Moussa Lezreg, Brahim Housni
Department of Critical Care, Mohammed 6 University Hospital of Oujda, Oujda, Morocco
|Date of Submission||19-Mar-2018|
|Date of Decision||27-Apr-2019|
|Date of Acceptance||06-Jun-2019|
|Date of Web Publication||06-Sep-2019|
Dr. Khalil Malki
Department of Critical Care, Mohammed 6 University Hospital of Oujda, Oujda
Source of Support: None, Conflict of Interest: None
Wernicke Korsakoff Syndrome is a rare brain disorder secondary to Thiamine deficiency. We report here a rare case 40-year-old Gravida3 Para 2, at 12 weeks of gestational age with severe hyperemesis gravidarum. The symptomatology was aggravated by acute installation of drowsiness, a diplopia, and a progressive change in the state of Consciousness; symptoms suggesting Wernicke Korakoff syndrome. The diagnosis was confirmed with MRI imaging and later by thiamine level dosage. The patient conditions improved after High dosage Thiamine supplementation therapy. Wernicke's encephalopathy is a rare complication of hyperemesis gravidarum. EARLY Diagnosis and thiamine supplementation are the main keys to prevent long-term neurological sequela or death. Prevention with Thiamine supplementation should be initiated in all pregnant women with prolonged vomiting.
Keywords: Hyperemesis gravidarum, thiamine deficiency, Wernicke encephalopathy, Wernicke Korakoff syndrome
|How to cite this article:|
Malki K, Aabdi M, Lezreg M, Housni B. Wernicke korsakoff syndrome as a rare complication of hyperemesis gravidarum: A case report. J Obstet Anaesth Crit Care 2019;9:113-5
|How to cite this URL:|
Malki K, Aabdi M, Lezreg M, Housni B. Wernicke korsakoff syndrome as a rare complication of hyperemesis gravidarum: A case report. J Obstet Anaesth Crit Care [serial online] 2019 [cited 2019 Sep 19];9:113-5. Available from: http://www.joacc.com/text.asp?2019/9/2/113/266138
| Introduction|| |
Wernicke Korsakoff syndrome (WKS) is the association of two different conditions that often occur together: Wernicke encephalopathy and Korsakoff syndrome. They are both brain disorders caused by thiamine deficiency (Vitamin B1). WKS first described By Dr. Carl Wernicke consists of paralysis of eye movements, ataxia, and mental confusion. On the other hand, Korsakoff syndrome is a chronic memory disorder which is a sequel of Wernicke's encephalopathy (WE). Lack of vitamin B1 is commonly seen in people who have alcohol use disorders. Thiamine deficiency was also linked to diet deficiencies or other medical conditions that impair the absorption of vitamin B-1. In the following case, we report an unusual etiology of WKS: hyperemesis gravidarum (HG).
| Case|| |
This case is about a 40-year-old woman, gravida 3 para 2 at 12 weeks of gestation, with unremarkable medical history, who presented during her 6th week of gestation severe nausea, and vomiting for which she was treated with antiemetics (metoclopramide 10 mg*3/day) and dietary treatment. Nevertheless, she continued to have persistent vomiting and complained of general weakness.
The patient was hospitalized for persistent vomiting with abdominal pain, which began a month earlier and resulted in a loss of 10 kg.
On admission, the Clinical examination revealed a temperature of 37.5°C, a pulse of 112/minute. Her blood pressure was 112/68 Hg mm, and her respiratory rate was 18 breaths per minute. The patient showed important signs of dehydration such as a skin trugor and ocular hypotonia. The rest of her physical examination was unremarkable.
Blood tests showed a hypokaliemia (3 mmol/L), hypothyroidism, low TSH (0.4 mlU/L) elevated liver enzymes (lipase at 774 IU/L, SGPT: 4 N, SGOT: 4.5 N) and altered renal function (creat 150 mmol/l).
The treatment at first consisted of intravenous glucose, saline solution with added potassium, and antiemetic therapy (metoclopramide, ondansetron hydrochloride).
The initial days of hospitalization showed that the patient had confusion (GCS 14/15), then began to lose all perception of time, people, and places, and eventually was unable to articulate and became bedridden. On the eighth day of hospitalization, she presented an intense headache, drowsiness, diplopia, and a progressive change in the state of Consciousness. Neurological examination found gait ataxia, a bilateral horizontal nystagmus (Horizontal + rotatory nystagmus in primary position and on lateral gaze bilaterally), and osteotendinous areflexia.
Cerebrospinal fluid (CSF) examination and cranial magnetic resonance imaging (MRI) were also undertaken to exclude other intracranial causes. The CSF findings were normal, but the cerebral MRI showed a bilateral thalamic hyper signal in FLAIR sequence and a Bilateral hypersignal of mammalian bodies in T2 [Figure 1], in favor of Gayet Wernicke's encephalopathy. Cerebral venous thrombosis was ruled out by angio-MRI, while, blood tests showed low levels of thiamine at 0.8 μg/dl which confirmed the diagnosis of WE.
|Figure 1: MRI changes related to Wernickeæs encephalopathy. The Tectum of the mid brain, the periaqueducal gray matter and the mamilary bodies (arrows) show signal intensity alternations|
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The vitamin B1 treatment was administered at a dose of 500 mg twice a day. The patient therefore received multivitamin infusion including thiamine, oral feeding was resumed 3 days later. We witnessed a complete standardization of all organic parameters. However, the neurological disorders disappeared only one month later, while memory disorders diminished less rapidly and persisted for at least 1 month postpartum.
Obstetric management consisted of fetal growth scans every 4 weeks and monthly assessment of uterine fundal height and urinalysis.
Delivery occurred at 38 weeks and was incident-free. Satisfactory epidural analgesia was provided, and labor went with normal evolution. A 3130-g male infant was born via vaginal delivery and had 3-minute and 5-minute Apgar scores of 8 and 10, respectively; Neurologic examination and cerebral ultrasound were unremarkable at1 month of age.
| Discussion|| |
In this particular case, the patient presented with altered sensorium during pregnancy, many diagnoses were suggested in the beginning; the patient could've had disseminated encephalomyelitis, pituitary apoplexy, deep cerebral vein thrombosis or neuroinfection. The presence of confusion diplopia an ataxia were in favor of Wernicke encephalopathy but It was the neuro MRI and the biological findings that confirmed the diagnosis.
In the last decade some similar cases of WE secondary to Hyperemsis gravidarum have been reported; we tried to compare our case with 5 others cases described in the last decade.,, All patients in this series have in common altered consciousness with dysarthria, nystagmus and ataxia. Two patients had ophthalmoplegia. MRI was the main paraclinical exam to confirm the diagnosis. The areas that were involved are: the medial thalami, tectum, mammillary bodies, hypothalamus, dorsal pons, and medulla. All patients were treated with high thiamine therapy (400 mg to 1.5 g a day) and showed significant improvement. However, only two of the six patients had successful childbirth (outcome to their pregnancy. Residual deficits were seen in one patient who presented depression, and only Yoon CK's patient and ours presented an associated korsakoff syndrome. The latter had a Korsakoff syndrome that persisted until one month after childbirth.
In all these cases, it was the thiamine deficiency that was responsible of the illness.
THIAMINE Also Known as vitaminB1 is a water-soluble, essential B-complex vitamin obtained from the diet. A daily intake of 1.1 mg is recommended for Adult Women. During Pregnancy, the needs in thiamine are higher than normal as a result of the demands of rapidly growing fetal tissues.
In fact, the recommended dietary allowance (RDA) during pregnancy is 1.5 mg. The body stores approximately 25 to 30 mg of vitamin B1; such stores can be depleted in 2 to 3 weeks with inadequate intake.
Carl Wernicke first described in 1881, an illness that consisted of paralysis of eye movements, ataxia, and mental confusion. These three symptoms can appear simultaneously, but one may precede the others by several days or several weeks. This triad is only part of a wide variety of features that can present in Wernicke encephalopathy.
Sergei Korsakov, a Russian psychiatrist, described the disturbance of memory in the course of long-term alcoholism in a series of articles from 1887-1891. Korsakov's syndrome is a chronic neuropsychiatric condition characterized by behavioral abnormalities and memory impairment that consists of defective learning (anterograde amnesia) associated with loss of memory (retrograde amnesia).
Wernicke-Korsakoff Syndrome More Details arises due to thiamine deficiency and is common among alcohol abusers, but it also appears in cases of malnutrition, malignant tumor, gastrointestinal dysfunction, pregnancy, and hyperthyroidism, and in patients who breast-feed and undergo hemodialysis or peritoneal dialysis. Thiamine deficiency is known to cause Wernicke's syndrome, but its pathophysiology is still uncertain.
The diagnosis of WE is based on the clinical manifestations and rapid reversal of symptoms with thiamine. Determination of blood transketolase activity and thiamine pyrophosphate reflects the thiamine status in the body. MRI is the imaging modality of choice because it is highly specific (93%) and comparatively safer than computed tomography (CT) scan. Radiological diagnosis can be made for Wernicke's encephalopathy when MRI shows high signal changes in the periaqueductal area and medial aspects of both thalami, and in the case of atrophy of papilla.
Wernicke's encephalopathy is currently recognized as a potentially fatal but reversible medical emergency. Mortality ranges from10% to 20%, and many survivors are left with persistent neurologic deficits despite appropriate treatment.
When Wernicke-Korsakoff syndrome due to pregnancy-related hyperemesis is suspected, thiamine should be administered immediately. Guidelines by the European Federation of Neurological Societies (EFNS) recommend that thiamine should be given 200 mg thrice daily via intravenous route, started before any carbohydrate, and continued until there is no further improvement in signs and symptoms. Due to clinical severity of our case, we opted for a high thiamine dose of 500 mg twice daily.
| Conclusion|| |
A rare case of WKS associated with Korsakoff syndrome during pregnancy is described. The diagnosis is based on clinical and MRI findings. Treatment is based on thiamine supplementation with successful Outcome. This suggests that thiamine supplementation in pregnant women with Hypermesis gravidarum is the key to prevent Wernicke Korsakoff syndrome.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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