Elsevier

The Lancet Neurology

Volume 12, Issue 2, February 2013, Pages 175-185
The Lancet Neurology

Review
Diagnosis of acute neurological emergencies in pregnant and post-partum women

https://doi.org/10.1016/S1474-4422(12)70306-XGet rights and content

Summary

Acute neurological symptoms in pregnant and post-partum women could be caused by exacerbation of a pre-existing neurological condition, the initial presentation of a non-pregnancy-related problem, or a new acute-onset neurological problem that is either unique to or occurs with increased frequency during or just after pregnancy. Pregnant and postpartum patients with headache and neurological symptoms are often diagnosed with pre-eclampsia; however, a range of other causes must also be considered, such as cerebral venous sinus thrombosis and reversible cerebral vasoconstriction syndrome. Precise diagnosis is essential to guide subsequent management. Our ability to differentiate between the specific causes of acute neurological symptoms in pregnant and post-partum patients is likely to improve as we learn more about the pathogenesis of these disorders.

Introduction

Acute neurological symptoms in pregnant and postpartum women could be caused by exacerbation of a pre-existing neurological condition (eg, multiple sclerosis or a seizure disorder) or by initial presentation of a non-pregnancy-related problem (eg, brain neoplasm). Alternatively, patients can present with new acute-onset neurological conditions that are either unique to or occur with increased frequency during and just after pregnancy; here we focus on diagnosis of this patient group. If specific treatments are not started promptly, many of these conditions can result in morbidity or mortality in these young, usually previously healthy individuals. The approach most commonly used to assess many of these symptoms—non-contrast brain CT—is often non-diagnostic. If a patient has a poor outcome, the medical, social, and medico-legal impact is often high. For all these reasons, prompt diagnosis is imperative.

In the past 5 years, the unique pathophysiological states of pregnancy and puerperium have been reviewed:1, 2 raised oestrogen concentrations stimulate the production of clotting factors, which increases the risk of thromboembolism; increased plasma and total blood volumes increase the risk of hypertension; and raised progesterone concentrations towards the end of pregnancy enhance venous distensibility, and potential leakage from small blood vessels. The high oestrogen levels fall in the postpartum period. Combined, these hormonal changes can result in leaky capillaries and vasogenic oedema. Pre-eclampsia is defined as the new onset of hypertension and proteinuria after 20 weeks in a previously normotensive woman. Diagnostic criteria for mild pre-eclampsia are blood pressure greater than or equal to 140/90 mm Hg and proteinuria greater than or equal to 0·3 g in a 24-h urine specimen. Criteria for severe pre-eclampsia are two occasions of hypertension (ie, blood pressure greater than or equal to 160/110 mm Hg) at least 6 h apart, proteinuria greater than 5 g per 24 h, and other signs of end-organ injury. Pre-eclampsia occurs in 2–8% of all pregnancies.3 Eclampsia is defined as pre-eclampsia and a grand mal seizure in the absence of other conditions that could account for the seizure. Up to 0·6% of mildly pre-eclamptic women and 2–3% of severely pre-eclamptic women have eclamptic seizures.4 Maternal mortality rates for eclamptic women have been reported to be 0–14% during the past few decades, and are higher in poor countries than in high-income countries.5 The most common cause of death in eclamptic women is brain ischaemia or haemorrhage; however, the neurological events of eclampsia are usually acute and transient, and long-term deficit is rare in properly managed patients.6

Because pre-eclampsia and eclampsia are common, they are often the default diagnoses in pregnant and postpartum women who present with acute neurological symptoms. However, there are other conditions that overlap with eclampsia and with each other in terms of their presentations, including acute ischaemic stroke (AIS), intracerebral and subarachnoid haemorrhage (ICH and SAH), and cerebral venous sinus thrombosis (CVT). Severe vasoconstriction often develops in women with pre-eclampsia—especially when blood pressure is poorly controlled—and can cause brain infarction and haemorrhage. A reversible cerebral vasoconstriction syndrome (RCVS; also referred to as postpartum angiopathy and Call-Fleming syndrome) can develop during puerperium in the absence of hypertension or other features of pre-eclampsia. Pre-eclampsia, eclampsia, and RCVS can all be complicated by posterior reversible encephalopathy syndrome (PRES). In fact, 8–39% of patients with RCVS also have PRES.7, 8 PRES is not a primary diagnosis, but a clinical and imaging syndrome caused by vascular abnormalities that are present in pre-eclampsia and eclampsia, RCVS, and other conditions. The continuum between potential causes of some neurological problems that can arise in pregnancy must be recognised, and we need to understand that various diagnoses can arise independently or simultaneously, and are not mutually exclusive. Additionally, whereas eclampsia is specific to pregnancy, PRES, RCVS, and CVT occur in non-pregnant individuals too.

Here we review clinical presentations and diagnostic evaluation of common and serious neurological emergencies that present in pregnant and post-partum women, with the aim to help clinicians to avoid misdiagnosis in these high-risk patients. We restrict our discussion to clinical manifestations and diagnoses, because after a diagnosis is established, specific treatments should naturally follow. We have organised data by both presenting symptoms and specific diagnosis, and we have created clinical algorithms on the basis of our interpretation of available literature and our own practice.

Section snippets

Headache

Primary headache disorders, tension-type and migraine, are the most common causes of headache in both pregnant and post-partum women;9, 10, 11 paradoxically, this can make diagnosis more difficult unless physicians pay careful attention to the so-called red flags that are suggestive of a secondary cause (figure 1). An estimated 40% of post-partum women have headaches, often within the first week after delivery.9 Migraine usually improves during pregnancy, but often returns postpartum when

Acute neurological deficit

Pregnant or post-partum patients who present with persistent acute motor, sensory, or visual findings—with or without headache—might have more serious causes and usually need urgent, thorough investigations (figure 1). Pregnant patients with transient motor, sensory, or visual symptoms commonly have migraine with aura, even if they have no headache. Using different methods, two studies found that in pregnant patients referred for transient neurological motor, sensory, or visual symptoms, most

Seizures

Pregnant or post-partum women with seizures can be grouped into three categories: first, and most common, are patients with an established seizure disorder before pregnancy;48 second are patients with a new non-pregnancy-related seizure disorder, such as a new seizure from an undiagnosed brain tumour or hypoglycaemia; third are patients with new seizures that are pregnancy related (caused by eclampsia, ICH, CVT, RCVS, PRES, or thrombotic thrombocytopenic purpura). Whereas in patients with PRES,

Clinical features

The clinical presentations of acute neurological symptoms in pregnant and post-partum women have substantial overlap between them, and several disorders can even coexist. However, the details—eg, headache characteristics, evolution of symptoms over time, and frequency of some symptoms such as seizures or visual problems—can often help to distinguish between them (table 2).

Cerebral venous sinus thrombosis

A rare cause of stroke overall, CVT is an important consideration in pregnant and post-partum women.50, 51, 52, 53 A spike

Rare conditions that cause acute neurological symptoms

Amniotic fluid embolism and metastatic choriocarcinoma are two pregnancy specific conditions that can present with neurological symptoms. Amniotic fluid embolism causes agitation, confusion, seizures, and encephalopathy in the context of cardiovascular and respiratory collapse during or immediately after labour.95, 96 Choriocarcinoma, a rare cancer of trophoblastic tissue, metastasises to the brain in 20% of patients.97, 98 Because the tumour can cause mass effect, bleed, and invade cerebral

Neuroimaging and multidisciplinary coordination of care

When brain imaging is used to make a specific diagnosis, several basic principles should be kept in mind. First, the clinician and the radiologist should discuss the case and differential diagnosis before imaging to minimise ionising radiation and intravenous contrast exposure, and to ensure that, when MRI is done, the correct sequences are obtained the first time to maximise the accuracy of the interpretation of the images. Second, the fetal radiation exposure from a non-contrast brain CT is

Conclusions

Pregnant and post-partum patients who present with new acute neurological symptoms need a thorough diagnostic evaluation that targets a range of pathological conditions that are either unique to or arise more frequently in this population. After an accurate diagnosis is made, specific treatment will follow. Because most of these conditions are multidisciplinary and infrequent, clinicians should consider early transfer of these patients to a centre that can deliver full diagnostic testing and

Search strategy and selection criteria

We searched for literature published from Jan 1, 1980 to Sep 20, 2012. Because we were focusing on more than one condition, we searched PubMed for English language articles containing the terms “pregnancy” or “postpartum” in the abstract in conjunction with other key terms, including “pre-eclampsia”, “eclampsia”, “headache”, “seizures”, “stroke”, “visual symptoms”, “PRES”, “reversible cerebral vasoconstriction”, and “cerebral vein thrombosis”, in the title. We manually searched the article

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