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|Year : 2017 | Volume
| Issue : 5 | Page : 1190-1191
Gestational Wernicke's encephalopathy
Vidhi S Shah1, Maheshkumar Shanmugam1, Akkayasamy Kowsalya1, KG Srinivasan2, Prathibha Siddappa1, Vellaiappan Neethiarasu3, Anand K Annamalai4
1 Department of Neuro-Ophthalmology, Aravind Eye Care Hospitals, Madurai, Tamil Nadu, India
2 KGS Scan Centre, Madurai, Tamil Nadu, India
3 Department of Neurology, Neethiarasu Neurology Hospital, Madurai, Tamil Nadu, India
4 Department of Endocrinology, Ashwin Speciality Hospital, Madurai, Tamil Nadu, India
|Date of Web Publication||6-Sep-2017|
Anand K Annamalai
Consultant Endocrinologist, Ashwin Speciality Hospital, Madurai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Shah VS, Shanmugam M, Kowsalya A, Srinivasan K G, Siddappa P, Neethiarasu V, Annamalai AK. Gestational Wernicke's encephalopathy. Neurol India 2017;65:1190-1
Hyperemesis gravidarum leading to thiamine deficiency and Wernicke's encephalopathy (WE) is under recognized., Although a life threatening medical emergency, gestational WE could be completely reversed by prompt identification and administration of high dose thiamine injections.
Here, we report the case of a 25-year old primigravida with anorexia and severe hyperemesis gravidarum from 10 weeks of gestation. Her primary care physician initiated multiple dextrose infusions and antiemetics. She presented to us at 16 weeks of gestation with disorientation, acute visual impairment, horizontal nystagmus, ataxia, and areflexia. She had tachycardia and her blood pressure was 100/60 mmHg. Her optic fundus revealed bilateral disc edema with retinal haemorrhages [Figure 1]a. Ultrasonography of the abdomen showed a singleton pregnancy with appropriate beta-human chorionic gonadotrophin (HCG) and normal thyroid hormone levels. A clinical diagnosis of WE was made.
|Figure 1: (a) Retinal images showing retinal hemorrhages along the vascular arcades and complete resolution after 6 weeks (e); MRI appearances at presentation (b-d) and at 6 weeks post-treatment (f-h). T2 weighted axial image demonstrating hyperintense signals involving the paramedian thalami [arrows; also confirmed on T2 weighted coronal image (c), sagittal image (d)]; (b) Flair axial image showing similar changes in the periaqueductal gray matter (arrows). (f-h) Follow-up MRI demonstrating complete regression of the previous hyperintense signal changes|
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Magnetic resonance imaging (MRI) revealed bilateral symmetric bright signals involving the thalami, subthalamic region, periaqueductal gray matter, and mammillary bodies confirming the diagnosis of WE [Figure 1]b,[Figure 1]c,[Figure 1]d.
She was treated with intravenous thiamine 900 mg/24 h in divided doses for 5 days and had a dramatic improvement in her mental status and nystagmus within 48 h. After 6 weeks, the visual signs [Figure 1]e, gait, reflexes, and obstetric progress regained their normal status. A repeat MRI of the brain [Figure 1]f,[Figure 1]g,[Figure 1]h revealed a complete resolution of the lesions in comparison with the previous images.
WE is most commonly encountered with excessive alcohol intake; however, hyperemesis gravidarum-induced WE is under-recognized. In our patient, there was no history of alcohol consumption, which precluded this as a contributory factor. The classic triad of mental confusion, ataxia, and ophthalmoplegia to diagnose gestational WE is seen in approximately 47% of the cases; this condition, therefore, remains underdiagnosed. Of the trinity of characteristic manifestations, ocular signs are the most commonly encountered in 96% of gestational WE. Our patient presented with less common ocular manifestations of retinal hemorrhages and bilateral disc edema. Thiamine is hypothesized to have a possible role in maintaining vascular integrity, and a deficiency causes a decrease in the transketolase activity, which results in vascular damage and retinal hemorrhages. Retinal hemorrhages have also been attributed to ischemic changes secondary to decreased cerebral blood flow, cerebral oxygen, and glucose consumption during the acute stages of the illness. Papilloedema in WE is rare and occurs in <5% of the patients with WE; it is postulated to be secondary to necrosis and edema of myelin and optic nerve cells.
The daily thiamine requirement is 1–2 mg per day with the body reserves being approximately 30–50 mg. Within 3 weeks, the body's thiamine stores are depleted when the oral intake is deficient. This affects the enzymes requiring thiamine pyrophosphate as a coenzyme. Thus, the enzymes, alpha-ketoglutarate-dehydrogenase and transketolase, show a reduced activity and are critically affected in the presence of a high carbohydrate intake and dextrose loading because these enzymes are involved in Krebs cycle and carbohydrate metabolism. Thiamine deficiency and WE in our patient was probably precipitated by anorexia and a poor nutritional state that was compounded by hyperemesis gravidarum along with the increased metabolic demands of pregnancy, which was augmented by the dextrose infusions.
Measuring serum thiamine or red blood cell transketolase levels lack specificity in diagnosis. Magnetic resonance imaging (MRI) is a valuable diagnostic tool in WE.,, In our patient, the MRI showed the typical hyperintense signals in the thalami, mammillary bodies, and periaqueductal regions. These signal alterations have been attributed to cytotoxic edema because these regions have high rates of thiamine-related glucose and oxidative metabolism., High doses of parenteral thiamine are both therapeutic and diagnostic and significant symptomatic improvement occurs within 48 h. Inadequate replenishment leads to irreversible neurological deficits. Our patient showed a remarkable clinical and radiological recovery within 6 weeks.
Although not frequently encountered during gestation, WE due to severe hyperemesis gravidarum could have disastrous consequences for the mother and fetus. In a woman with hyperemesis gravidarum, avoidance of dextrose loading is vital because it accelerates thiamine deficiency. This case highlights the need for an increased vigilance towards early identification and treatment of WE to facilitate complete recovery from this life-threatening emergency.
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