Hyperemesis gravidarum presenting to the emergency department with Wernicke encephalopathy: a case report

Introduction

Wernicke encephalopathy (WE) is an under-diagnosed condition of thiamine deficiency (1) with estimates that as many as 85% of total cases are diagnosed only after death (2). Commonly attributed to chronic alcohol abuse, the condition has also been associated with other patient groups such as bariatric surgery patients (3), patients with anorexia nervosa (4,5) or pancreatitis (6), chronic diuretic use (7,8), and pregnant patients with hyperemesis gravidarum (HG) (9).

Between 1920 and 2019, only 177 cases of WE in HG were reported in almost exclusively obstetrics/gynecology and neurology journals (9). Emergency medicine literature documenting WE in pregnancy is limited though HG patients are thought to make up as many as 1 in 600 emergency department (ED) visits (10).

The variety of symptoms from a wide spectrum of organ systems contributes to the diagnostic challenge of WE. Mental status changes, ocular dysfunction and gait ataxia are considered the classic triad of symptoms and yet are only present in about 10% of cases (11). Other potential symptoms include peripheral neuropathies, hypothermia, and cardiovascular instability. Mental status changes can range from apathy, or mild neurocognitive symptoms such as confusion, all the way to coma (11). Laboratory results are generally nonspecific and thiamine levels are generally not available in the acute setting.

With a specificity of >90%, magnetic resonance imaging (MRI) can help confirm the diagnosis of WE but the sensitivity is thought to be low and thus is not helpful at ruling out disease (12). MRI findings consistent with WE have been reported extensively elsewhere (13). Reversal of the MRI findings in WE can be seen in conjunction with clinical improvement (14).

This case report of WE in a pregnant patient presenting to the ED seeks to highlight the varied, often undifferentiated presentation of WE in an ED population and document WE from HG, as well as its resolution, in emergency medicine literature. We present this case in accordance with the CARE reporting checklist (available at https://jeccm.amegroups.com/article/view/10.21037/jeccm-24-26/rc).

Case presentation

A 25-year-old G1, P0 at 18 weeks, 6 days gestational age presented to the ED by ambulance for altered mental status. Emergency medical services (EMS) had notified the ED prior to arrival due to the discovered pre-hospital hypotension, with a manual blood pressure of 58/36, and significant tachycardia. On arrival at the ED, the patient had a normal mental status with vital signs demonstrating a blood pressure of 129/100, a pulse of 125, with room air oxygen saturation of 100% and a respiratory rate of 20 breaths per minute. The patient had been seen and discharged from the same ED 6 days earlier after being treated for hypokalemia and vomiting.

The patient reported she had been receiving outpatient infusions of 10% dextrose (D10) intravenous fluids due to HG. Family reported confusion and short-term memory problems with repetitive questions over the past few weeks. On the day of presentation, the patient was noted to be off balance and EMS reported that the patient was unable to ambulate to their stretcher.

On exam, the patient had conjugate gaze with equal and reactive pupils described as “roving”. Patient had slow, repetitive but appropriate speech and slight dyscoordination on finger-to-nose and heel-to-shin testing bilaterally. The patient demonstrated some past-pointing on finger-to-nose and had difficulty with smooth movement of her heel up and down the opposite shin. Though the patient expressed appropriate concern regarding her ongoing nausea and vomiting during her pregnancy, she notably did not seem concerned about her inability to walk on day of presentation and was unaware of the repetitive questions she had been asking. The patient was otherwise cooperative, alert and behaving appropriately.

Laboratory work revealed a mildly low potassium of 3.2 (improved from previous visit), a low magnesium of 1.3 mg/dL, low albumin of 2.5 g/dL and a mild transaminitis with an aspartate aminotransferase (AST) of 132 U/L and an alanine aminotransferase (ALT) of 231 U/L, both of which had been seen previously. The lactic acid was notably 5 mmol/L and the patient was mildly anemic with a hemoglobin of 9.7 g/dL, though this was consistent with previous levels.

The patient was immediately treated with thiamine 500 mg intravenous (IV) and admitted with a presumptive diagnosis of WE. MRI without contrast of the brain performed the following day demonstrated subtle fluid attenuated inversion recovery (FLAIR) hyperintensity and restricted diffusion along the cortex of the posterior frontal lobes consistent with acute WE (see Figure 1). Patient continued treatment and was discharged on hospital day 4.

Figure 1 Patients MRI (axial FLAIR sequent) demonstrating subtle FLAIR hyperintensity and restricted diffusion along the cortext of the posterior frontal lobes (perisylvian), right worse than left. MRI, magnetic resonance imaging; FLAIR, fluid attenuated inversion recovery.

The patient ultimately delivered a healthy term infant at 40 weeks, 4 days via spontaneous vaginal delivery with nuchal cord approximately 5 months later. Apgars scores were 6 and 9. Follow up MRI performed after treatment for WE and delivery of baby no longer demonstrated the previously seen findings of WE.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent for publication of this case report and accompanying image was not obtained from the patient or the relatives after all possible attempts were made.

Discussion

The patient in this case report of WE in HG demonstrated classic and lesser-known symptoms of WE. Though the patient did not demonstrate a clear or complete ophthalmoplegia with paralysis of the extraocular muscles, the description of “roving” conjugate gaze could indicate muscle weakening that precedes muscle paralysis. It is significant to note that in this patient’s case, mental status changes were subtle and involved poor insight and short term memory problems but not a change in level of consciousness. The patient was alert and oriented to person, place and time. The patient was reported by EMS to be initially hypotensive though she maintained normal blood pressures through her admission and demonstrated notable tachycardia during her hospital stay with signs of orthostasis. This likely relates to the cardiovascular instability that can be seen in WE as well as dehydration. As emergency physicians often hone in on vital signs as a marker of critical illness, WE should not be overlooked in patients with such cardiovascular instability.

This patient also demonstrated a lactic acidosis which often implies sepsis or other emergent condition leading to anaerobic metabolism. However, lactic acidosis can occur with thiamine deficiency (15) and WE should be considered in any situation of unexplained lactic acidosis.

The patient had reported receiving IV dextrose infusions prior to ED presentation with WE. Current guidelines do not recommend the use of dextrose containing solutions when providing IV hydration to HEG patients (16) and a randomized control trial evaluating rehydration of pregnant patients with dextrose containing solutions versus electrolyte-only solutions demonstrated no benefit of dextrose (17). Additionally, the association of WE and dextrose administration has been documented (9). Current guidelines recommend the administration of thiamine supplementation in all pregnant women with vomiting or reduced dietary intake (18).

A previous case series reported 50% of women with WE and HG had fetal loss or miscarriage (9) and MRI changes seen in patients with WE have been reported in the fetuses of pregnant patients (19). Therefore, the timely diagnosis of WE in HG is important, particularly given the fact that the condition is reversible and preventable with adequate thiamine repletion. This case documents patient follow up through delivery of a healthy term infant.

HG is the third most common reason for early pregnancy related ED visits and accounts for approximately 165 per 100,000 ED visits (10). The percentage of pregnant patients who visit the ED for nausea and vomiting is as high as 5.7% (10). Though documented extensively in obstetrics/gynecology, neurology, radiology and pathology literature, only one other case report of WE in HG could be found in emergency medicine literature (20). Due to the prevalence of pregnancy related vomiting presenting to the ED and the overall under-diagnosis of WE, it is imperative for emergency physicians to maintain a high index of suspicion for WE in any pregnant patient with vomiting.

Conclusions

Though commonly attributed to chronic alcoholism, WE should be on the differential diagnosis of any condition associated with malnutrition or malabsorption. As pregnant patients with vomiting frequently present to the ED, emergency physicians should maintain a high index of suspicion for WE and should maintain a low threshold to initiate treatment with IV thiamine in addition to traditional antiemetic measures. Administration of intravenous thiamine has little downside and offers prevention of and treatment for WE.

Acknowledgments

Funding: None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://jeccm.amegroups.com/article/view/10.21037/jeccm-24-26/rc

Peer Review File: Available at https://jeccm.amegroups.com/article/view/10.21037/jeccm-24-26/prf

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://jeccm.amegroups.com/article/view/10.21037/jeccm-24-26/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent for publication of this case report and accompanying image was not obtained from the patient or the relatives after all possible attempts were made.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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