Williams Obstetrics, 24th Edition

CHAPTER 60. Neurological Disorders

CENTRAL NERVOUS SYSTEM IMAGING

HEADACHE

SEIZURE DISORDERS

CEREBROVASCULAR DISEASES

DEMYELINATING OR DEGENERATIVE DISEASES

NEUROPATHIES

SPINAL-CORD INJURY

IDIOPATHIC INTRACRANIAL HYPERTENSION

MATERNAL VENTRICULAR SHUNTS

MATERNAL BRAIN DEATH

A number of neurological diseases are relatively common in women of childbearing age. In the past, some of these may have precluded pregnancy, however, few do so now. Most of those encountered during pregnancy are the same as for nonpregnant women. That said, there are a few neurological disorders that may be seen more frequently in pregnant women. Some examples are Bell palsy, specific types of strokes, and benign intracranial hypertension or pseudotumor cerebri. Neurovascular disorders are an important cause of maternal mortality and accounted for 10 percent of maternal deaths in the United States from 1998 through 2005 (Berg, 2010b).

Because many neurological disorders are chronic, they frequently precede pregnancy. Although most women with chronic neurological disease who become pregnant will have successful outcomes, some of these disorders have specific risks with which clinicians should be familiar. Conversely, some women will have new-onset neurological symptoms during pregnancy, and these frequently must be distinguished from other pregnancy complications. Psychiatric disorders may also manifest with cognitive and neuromuscular abnormalities, and they should be considered in the evaluation.

CENTRAL NERVOUS SYSTEM IMAGING

Computed tomography (CT) and magnetic resonance (MR) imaging have opened new vistas for the diagnosis, classification, and management of many neurological and psychiatric disorders. As discussed in Chapter 46 (p. 933), these cranial imaging methods can be used safely during pregnancy. CT scanning is commonly used whenever rapid diagnosis is necessary and is excellent for detecting recent hemorrhage (Smith, 2012). Because it does not use radiation, MR imaging is often preferred. It is particularly helpful to diagnose demyelinating diseases, screen for arteriovenous malformations, evaluate congenital and developmental nervous system abnormalities, identify posterior fossa lesions, and diagnose spinal cord diseases (Gjelsteen, 2008). Whenever either test is done, the woman should be positioned in a left lateral tilt with a wedge under one hip to prevent hypotension as well as to diminish aortic pulsations, which may degrade the image.

Cerebral angiography with contrast injection, usually via the femoral artery, is a valuable adjunct to the diagnosis and treatment of some cerebrovascular diseases. Fluoroscopy delivers more radiation but can be performed with abdominal shielding. Positron emission tomography (PET) and functional MRI (fMRI) have not been evaluated for use in pregnant patients (Chiapparini, 2010).

HEADACHE

The National Health Interview Survey is provided by the Centers for Disease Control and Prevention (Pleis, 2010). In the 2009 survey, a fifth of all women aged 18 to 44 years reported a severe headache or migraine within the past 3 months, and headache was the most common neurological complaint during pregnancy. Smitherman and coworkers (2013) found that 26 percent of nonpregnant women in this age group were similarly affected. Interestingly, Aegidius and colleagues (2009) reported an overall decrease in prevalence of all headache types during pregnancy in nulliparas, especially during the third trimester. The classification of headaches by the International Headache Society (2004) is shown in Table 60-1. Primary headaches are more common in pregnant women than those with secondary causes (Digre, 2013). Migraine headaches are those most likely to be affected by the hormonal changes of pregnancy (Torelli, 2010).

TABLE 60-1. Classification of Headachea


Primary

Tension-type (69%)

Migraine (16%)

Cluster and other trigeminal autonomic cephalgias (< 1%)

Other

Secondary

Systemic infection (63%)

Head or neck trauma (4%)

Cranial or cervical vascular disorders

Intracranial nonvascular

Substance use or withdrawal

Disorders of homeostasis

Head and neck disorders

Psychiatric disorders

aPercentages in parentheses are estimated frequencies.

Data from Goadsby, 2012. Adapted from the International Headache Society, 2004.

image Tension-Type Headache

These are most common, and characteristic features include muscle tightness and mild to moderate pain that can persist for hours in the back of the neck and head. There are no associated neurological disturbances or nausea. The pain usually responds to rest, massage, application of heat or ice, antiinflammatory medications, or mild tranquilizers.

image Migraine Headache

These headaches have a 1-year prevalence in all women of approximately 15 percent, and thus they are frequently encountered during pregnancy. The term migraine describes a periodic, sometimes incapacitating neurological disorder characterized by episodic attacks of severe headache and autonomic nervous system dysfunction (Goadsby, 2012). The International Headache Society (2004) classifies three migraine types based on the presence or absence of an aura as well as chronicity:

1. Migraine without aura—was formerly termed common migraine—and is characterized by a unilateral throbbing headache, nausea and vomiting, or photophobia.

2. Migraine with aura—formerly termed classic migraine—has similar symptoms preceded by premonitory neurological phenomena such as visual scotoma or hallucinations. A third of patients have this type of migraine, which sometimes can be averted if medication is taken at the first premonitory sign.

3. Chronic migraine is defined by a migraine headache occurring at least 15 days each month for more than 3 months.

Migraines may begin in childhood, peak in adolescence, and tend to diminish in both frequency and severity with advancing years. According to Lipton and associates (2007), their annual prevalence is 17 percent in women and 6 percent in men. Another 5 percent of women have probable migraine, that is, they have all criteria but one (Silberstein, 2007). Migraines are especially common in young women and have been linked to hormone levels (Torelli, 2010).

The exact pathophysiology of migraines is uncertain, but they occur when neuronal dysfunction leads to decreased cortical blood flow, activation of vascular and meningeal nociceptors, and stimulation of trigeminal sensory neurons (Brandes, 2007; D’Andrea, 2010). A predilection for the posterior circulation has been described by Kruit and coworkers (2004). Migraines—especially those with aura in young women—are associated with increased risk for ischemic strokes as discussed on page 1191. The risk is greater in those who smoke or use combination oral contraceptives.

Migraine in Pregnancy

The prevalence of migraine headaches in the first trimester is 2 percent (Chen, 1994). Prospective as well as observational studies have shown that most migraineurs have improvement during pregnancy (Adeney, 2006; Menon, 2008; Torelli, 2010). Still, migraines—usually those with an aura—occasionally appear for the first time during pregnancy. Pregnant women with preexisting migraine symptoms may have other symptoms suggestive of a more serious disorder, and new neurological symptoms should prompt a complete evaluation (Detsky, 2006; Heaney, 2010).

Although conventional thinking has been that migraine headaches do not pose increased maternal or fetal risks, several recent studies have refuted this (Allais, 2010). For example, women with severe migraines in the first 8 weeks may be at slightly increased risk for a fetus with limb-reduction defects (Banhidy, 2006). Preeclampsia and other cardiovascular morbidities are also increased (Facchinetti, 2009; Sanchez, 2008; Schürks, 2009). In a case-control study of nearly 18.5 million pregnancy-related discharges from 2000 through 2003, Bushnell and coworkers (2009) identified an incidence of migraine discharge codes of 185 per 100,000. Associated diagnoses and increased significant risks were found for migraines and stroke—15.8-fold; myocardial infarction—4.9; heart disease—2.1; venous thromboembolism—2.4; and preeclampsia/gestational hypertension—2.3-fold.

Management

Data are limited regarding nonpharmacological management in pregnancy such as biofeedback techniques, acupuncture, and transcranial magnetic stimulation (Airola, 2010; Dodick, 2010). Effective pharmacological interventions include nonsteroidal antiinflammatory drugs, and most migraine headaches respond to simple analgesics such as ibuprofen or acetaminophen, especially if given early. Because of patient idiosyncrasies, multitarget drug therapy is necessary in most cases for migraine relief (Gonzalez-Hernandez, 2014). Severe headaches should be treated aggressively with intravenous hydration and parenteral antiemetics and opioids. Although intravenous magnesium sulfate has gained favor in the past few years, a recent metaanalysis reported no beneficial effects (Choi, 2014). Ergotamine derivatives are potent vasoconstrictors that should be avoided in pregnancy (Briggs, 2011).

Triptans are serotonin 5-HT1B/2D-receptor agonists that effectively relieve headaches by causing intracranial vasoconstriction (Contag, 2010). They also relieve nausea and vomiting and greatly reduce the need for analgesics. They can be given orally, by injection, as a rectal suppository, or as a nasal spray. The greatest experience is with sumatriptan (Imitrex), and although not studied extensively in pregnancy, it appears to be safe (Briggs, 2011; Nezvalová-Henriksen, 2010).

For women with frequent migraine headaches, oral prophylactic therapy is warranted. Amitriptyline (Elavil), 10 to 150 mg daily; propranolol (Inderal), 20 to 80 mg three times daily; or metoprolol (Lopressor, Toprol), 50 to 100 mg twice daily, is safe in pregnancy and has been used with success (Contag, 2010; Lucas, 2009; Marcus, 2007).

image Cluster Headaches

This rare primary headache disorder is characterized by severe unilateral lancinating pain radiating to the face and orbit, lasting 15 to 180 minutes, and occurring with autonomic symptoms and agitation. Pregnancy does not affect symptom severity. Affected women should avoid tobacco and alcohol. Acute management includes 100-percent oxygen therapy and sumatriptan given as a 6-mg dose subcutaneously (Calhoun, 2010; Francis, 2010). If recurrent, prophylaxis is administered using a calcium-channel blocking agent.

SEIZURE DISORDERS

The Centers for Disease Control and Prevention reported that the prevalence of epilepsy in adults in 2005 was 1.65 percent (Kobau, 2008). There are 1.1 million American women of childbearing age who are affected. After headaches, seizures are the next most prevalent neurological condition encountered in pregnant women, and they complicate 1 in 200 pregnancies (Brodie, 1996; Yerby, 1994). Importantly, epilepsy accounted for 13 percent of maternal deaths in the United Kingdom for the 2005 to 2007 triennium (Lewis, 2007). Seizure disorders are also associated with altered fetal development, and they can adversely affect other pregnancy outcomes. The teratogenic effects of several anticonvulsant medications are unquestioned. The American Academy of Neurology and the American Epilepsy Society have developed guidelines regarding treatment in pregnant women, which are discussed subsequently (Harden, 2009a–c).

image Pathophysiology

A seizure is defined as a paroxysmal disorder of the central nervous system characterized by an abnormal neuronal discharge with or without loss of consciousness. Epilepsy encompasses different syndromes whose cardinal feature is a predisposition to recurrent unprovoked seizures. The International League Against Epilepsy Commission on Classification and Terminology recently updated its terminologies for seizures (Berg, 2010a, 2011; Shorvon, 2011). This new classification schema is under development, and for now, most adults can be said to have either focal or generalized seizures.

Focal Seizures

These originate in one localized brain area and affect a correspondingly localized area of neurological function. They are believed to result from trauma, abscess, tumor, or perinatal factors, although a specific lesion is rarely demonstrated. Focal seizures without dyscognitive features start in one region of the body and progress toward other ipsilateral areas of the body, producing tonic and then clonic movements. Simple seizures can affect sensory function or produce autonomic dysfunction or psychological changes. Cognitive function is not impaired, and recovery is rapid. Focal seizures with dyscognitive features are often preceded by an aura and followed by impaired awareness manifested by sudden behavioral arrest or motionless stare. Involuntary movements such as picking motions or lip smacking are common.

Generalized Seizures

These involve both brain hemispheres and may be preceded by an aura before an abrupt loss of consciousness. There is a strong hereditary component. In generalized tonic-clonic seizures, loss of consciousness is followed by tonic contraction of the muscles and rigid posturing, and then by clonic contractions of all extremities while the muscles gradually relax. Return to consciousness is gradual, and the patient may remain confused and disoriented for several hours. Absence seizures—also called petit mal seizures—are a form of generalized epilepsy that involve a brief loss of consciousness without muscle activity and are characterized by immediate recovery of consciousness and orientation.

image Causes of Seizure

Some identifiable causes of convulsive disorders in young adults include head trauma, alcohol- and other drug-induced withdrawals, cerebral infections, brain tumors, biochemical abnormalities, and arteriovenous malformations. A search for these is prudent with a new-onset seizure disorder in a pregnant woman. The diagnosis of idiopathic epilepsy is one of exclusion.

image Preconceptional Counseling

Women with epilepsy should undergo education and counseling before pregnancy (Chap. 8p. 158). Folic acid supplementation with 0.4 mg per day is begun at least 1 month before conception. The dose is increased to 4 mg when the woman taking antiepileptic medication becomes pregnant. These medications are assessed and adjusted with a goal of monotherapy using the least teratogenic medication. If this is not feasible, then attempts are made to reduce the number of medications used and to use them at the lowest effective dose (Dunlop, 2008). Medication withdrawal should be considered if a woman is seizure free for 2 years or more.

image Epilepsy During Pregnancy

The major pregnancy-related risks to women with epilepsy are increased seizure rates with attendant mortality risks and fetal malformations. Seizure control is the main priority. Earlier studies described worsening seizure activity during pregnancy, however, this is not as common nowadays because of more effective drugs. Contemporary studies cite increased seizure activity in only 20 to 30 percent of pregnant women (Mawer, 2010; Vajda, 2008; Viinikainen, 2006). Women who are seizure free for at least 9 months before conception will likely remain so during pregnancy (Harden, 2009b).

Increased seizure frequency is often associated with decreased and thus subtherapeutic anticonvulsant serum levels, a lower seizure threshold, or both. An impressive number of pregnancy-associated alterations can result in subtherapeutic serum levels. These include nausea and vomiting, decreased gastrointestinal motility, antacid use that diminishes drug absorption, pregnancy hypervolemia offset by protein binding, induction of hepatic enzymes such as cytochrome oxidases, placental enzymes that metabolize drugs, and increased glomerular filtration that hastens drug clearance. Importantly, some women discontinue medication because of teratogenicity concerns. Finally, the seizure threshold can be affected by pregnancy-related sleep deprivation as well as hyperventilation and pain during labor.

Pregnancy Complications

Women with epilepsy have a small increased risk of some pregnancy complications (Borthen, 2011; Harden, 2009b). A population-based study from Iceland found that epileptic women had a twofold increased cesarean delivery rate (Olafsson, 1998). In a cohort study from Montreal, Richmond and coworkers (2004) reported an increased incidence of nonproteinuric hypertension and labor induction. From a Swedish study of 1207 epileptic women, Pilo and colleagues (2006) reported a 1.5-fold increased incidence of cesarean delivery, preeclampsia, and postpartum hemorrhage. Postpartum depression rates have also been reported to be increased in epileptic women (Turner, 2009). Finally, children of epileptic mothers have a 10-percent risk of developing a seizure disorder.

Embryo-Fetal Malformations

For years, it was difficult to separate effects of epilepsy versus its therapy as the primary cause of fetal malformations. As discussed in Chapter 8 (p. 158), it is now believed that untreated epilepsy is not associated with an increased fetal malformation rate (Thomas, 2008; Viinikainen, 2006). That said, the fetus of an epileptic mother who takes certain anticonvulsant medications has an indisputably increased risk for congenital malformations. Moreover, monotherapy is associated with a lower birth defect rate compared with multiagent therapy. Thus, if necessary, increasing monotherapy dosage is at least initially preferable to adding another agent (Buhimschi, 2009).

Specific drugs, when given alone, increase the malformation rate (Chap. 12p. 246). Some of these are listed in Table 60-2. Phenytoin and phenobarbital increase the major malformation rate two- to threefold above baseline (Perucca, 2005; Thomas, 2008). A particularly potent teratogen is valproate, which has a dose-dependent effect and increases the malformation risk four- to eightfold (Eadie, 2008; Klein, 2014; Wyszynski, 2005). In general, with polytherapy, the risk increases with each drug added. At least at this time, the newer antiepileptic medications are reported to have no associations with a markedly increased risk of major birth defects (Molgaard-Nielson, 2011).

TABLE 60-2. Teratogenic Effects of Common Anticonvulsant Medications

image

Management in Pregnancy

The major goal is seizure prevention. To accomplish this, treatment for nausea and vomiting is provided, seizure-provoking stimuli are avoided, and medication compliance is emphasized. The fewest necessary anticonvulsants are given at the lowest dosage effective for seizure control. Although some providers routinely monitor serum drug levels during pregnancy, these concentrations may be unreliable because of altered protein binding. Free or unbound drug levels, although perhaps more accurate, are not widely available. Importantly, there is no evidence that such monitoring improves seizure control (Adab, 2006). For these reasons, drug levels may be informative if measured following seizures or if noncompliance is suspected. Some of the newer agents such as lamotrigine and oxcarbazepine may be more amenable to serum drug level monitoring (Harden, 2009a; Pennell, 2008).

For women taking anticonvulsant drugs, a targeted sonographic examination at midpregnancy is recommended by some to search for anomalies (Chap. 10p. 197). Testing to assess fetal well-being is generally not indicated for women with uncomplicated epilepsy.

Breast Feeding and Contraception

There are limited available data regarding the safety of breast feeding with the various anticonvulsant medications. That said, no obvious deleterious effects, such as long-term cognitive issues, have been reported (Briggs, 2011; Harden, 2009c). Some of the anticonvulsant agents are associated with increased oral contraceptive failures. Thus, other more reliable methods should be considered (Chap 38p. 696).

CEREBROVASCULAR DISEASES

Abnormalities of the cerebrovascular circulation include strokes—both ischemic and hemorrhagic, as well as anatomical anomalies, such as arteriovenous malformations and aneurysms. The current endemic of obesity in this country, along with concomitant increases in heart disease, hypertension, and diabetes, has also resulted in increased stroke prevalence (Centers for Disease Control and Prevention, 2012). Women have a higher lifetime risk of stroke than men as well as higher associated mortality rates (Martínez-Sánchez, 2011; Roger, 2012). Moreover, pregnancy increases the immediate and lifetime risk of both ischemic and hemorrhagic stroke (Jamieson, 2010; Jung, 2010).

Stroke is relatively uncommon in pregnant women, but it contributes disparately to maternal mortality rates. Reported incidences of strokes in pregnancy range from 1.5 to 71 per 100,000 pregnancies (James, 2005; Kuklina, 2011; Scott, 2012). The incidence is increasing as measured by pregnancy-related hospitalizations for stroke (Callaghan, 2008; Kuklina, 2011). Importantly, most are associated with hypertensive disorders or heart disease. Almost 9 percent of the pregnancy-related mortality rate in the United States is due to cerebrovascular accidents, with a third being associated with preeclampsia (Berg, 2010b).

image Risk Factors

Most strokes in pregnancy manifest either during labor and delivery or in the puerperium. In a study of 2850 pregnancy-related strokes, approximately 10 percent developed antepartum, 40 percent intrapartum, and almost 50 percent postpartum (James, 2005). Several risk factors—unrelated and related to pregnancy—have been reported from studies that included more than 10 million pregnancies. These include older age; migraines, hypertension, obesity, and diabetes; cardiac disorders such as endocarditis, valvular prostheses, and patent foramen ovale; and smoking. Those related to pregnancy include hypertensive disorders, gestational diabetes, obstetrical hemorrhage, and cesarean delivery. By far, the most common risk factors are pregnancy-associated hypertensive disorders. As noted, one third of strokes are associated with gestational hypertension, and there is a three- to eightfold increased risk of stroke in hypertensive compared with normotensive women (Scott, 2012; Wang, 2011). Women with preeclampsia undergoing general anesthesia may be at higher risk of stroke compared with those given neuraxial anesthesia (Huang, 2010). Another risk factor for peripartum stroke is cesarean delivery, which increases the risk 1.5-fold compared with vaginal delivery (Lin, 2008).

Pregnancy-induced effects on cerebrovascular hemodynamics are unclear as related to risk for stroke. Although cerebral blood flow decreased by 20 percent from midpregnancy until term, importantly, it increased significantly with gestational hypertension (Zeeman, 2003, 2004b). Such hyperperfusion would at least intuitively be dangerous in women with certain vascular anomalies.

image Ischemic Stroke

Acute occlusion or embolization of an intracranial blood vessel causes cerebral ischemia, which may result in death of brain tissue (Fig. 60-1). The more common associated conditions and etiologies of ischemic stroke are shown in Table 60-3. A transient ischemic attack (TIA) is caused by reversible ischemia, and symptoms usually last less than 24 hours. Patients with a stroke usually have a sudden onset of severe headache, hemiplegia or other neurological deficits, or occasionally, seizures. Focal neurological symptoms accompanied by an aura usually signify a first-episode migraine (Liberman, 2008).

image

FIGURE 60-1 Illustrations of a brain showing various types of strokes seen in pregnancy: (1) subcortical infarction (preeclampsia), (2) hypertensive hemorrhage, (3) aneurysm, (4) embolism or thrombosis in middle cerebral artery, (5) arteriovenous malformation, and (6) cortical vein thrombosis.

TABLE 60-3. Some Associated Disorders or Causes of Ischemic and Hemorrhagic Strokes During Pregnancy or the Puerperium

image

Evaluation of an ischemic stroke includes echocardiography and cranial imaging with CT, MR, or angiography. Serum lipids are measured with the caveat that their values are distorted by normal pregnancy (Appendixp. 1291). Antiphospholipid antibodies and lupus anticoagulant are sought—these cause up to a third of ischemic strokes in otherwise healthy young women (Chap. 59p. 1173). Also, tests for sickle-cell syndromes are completed when indicated. With a thorough evaluation, most causes can be identified, although treatment is not always available. Some of these include cardiac-associated embolism, vasculitis, or vasculopathy such as Moyamoya disease (Ishimori, 2006; Miyakoshi, 2009; Simolke, 1991).

Preeclampsia Syndrome

In reproductive-age women, a significant proportion of pregnancy-related ischemic strokes are caused by gestational hypertension and preeclampsia syndrome (Jeng, 2004). As shown in Figure 60-1, areas of subcortical perivascular edema and petechial hemorrhage may progress to cerebral infarction (Aukes, 2007, 2009; Zeeman, 2004a). Although these are usually clinically manifest by an eclamptic convulsion, a few women will suffer a symptomatic stroke from a larger cortical infarction (Chap. 40p. 742).

Other conditions with findings similar to preeclampsia include thrombotic microangiopathies (Chap. 56p. 1116) and the reversible cerebral vasoconstriction syndrome (Chap. 40p. 743). The latter, which is also termed postpartum angiopathy, can cause extensive cerebral edema with necrosis as well as widespread infarction with areas of hemorrhage (Katz, 2014; Ramnarayan, 2009; Singhal, 2009).

Cerebral Embolism

These strokes usually involve the middle cerebral artery (see Fig. 60-1). They are more common during the latter half of pregnancy or early puerperium (Lynch, 2001). The diagnosis can be made with confidence only after thrombosis and hemorrhage have been excluded. The diagnosis is more certain if an embolic source is identified. Hemorrhage may be more difficult to exclude because embolization and thrombosis are both followed by hemorrhagic infarction. Paradoxical embolism is an uncommon cause, even considering that more than a fourth of adults have a patent foramen ovale through which right-sided venous thromboemboli are deported (Kizer, 2005; Scott, 2012). Foraminal closure may not improve outcomes in these patients, however, this procedure has been performed during pregnancy (Dark, 2011; Furlan, 2012). Assorted cardioembolic causes of stroke include arrhythmias—especially atrial fibrillation, valvular lesions, mitral valve prolapse, mural thrombus, infective endocarditis, and peripartum cardiomyopathy.

Management of embolic stroke in pregnancy consists of supportive measures and antiplatelet therapy. Thrombolytic therapy and anticoagulation are controversial issues at this time (Kizer, 2005; Li, 2012).

Cerebral Artery Thrombosis

Most thrombotic strokes affect older individuals and are caused by atherosclerosis, especially of the internal carotid artery. Many are preceded by one or more transient ischemic attacks. Thrombolytic therapy with recombinant tissue plasminogen activator—rt-PA or alteplase is recommended within the first 3-hour window if there is measurable neurological deficit and if neuroimaging has excluded hemorrhage. This can be used in pregnancy. A principal risk is hemorrhagic transformation of an ischemic stroke in approximately 5 percent of treated patients (van der Worp, 2007).

Cerebral Venous Thrombosis

In a 10-center study in the United States, 7 percent of cerebral venous thromboses were associated with pregnancy (Wasay, 2008). Even so, pregnancy-associated cerebral venous thrombosis is rare in developed countries, and reported incidences range from 1 in 11,000 to 1 in 45,000 pregnancies (Lanska, 1997; Simolke, 1991). In the Nationwide Inpatient Sample of more than 8 million deliveries, James and associates (2005) observed that venous thrombosis caused only 2 percent of pregnancy-related strokes (Saposnik, 2011). There are numerous predisposing causes, and the greatest risk is in late pregnancy and the puerperium.

Thrombosis of the lateral or superior sagittal venous sinus usually occurs in the puerperium and often in association with preeclampsia, sepsis, or thrombophilia (see Fig. 60-1). It is more common in patients with inherited thrombophilias, lupus anticoagulant, or antiphospholipid antibodies (Chaps. 52p. 1029 and 59p. 1173). Headache is the most common presenting symptom, neurological deficits are common, and up to a third of patients have convulsions (Wasay, 2008). Diagnosis is with MR venography (Saposnik, 2011).

Management includes anticonvulsants for seizures, and although heparinization is recommended by most, its efficacy is controversial (de Freitas, 2008; Saposnik, 2011; Smith, 2012). Antimicrobials are given if there is septic thrombophlebitis, and fibrinolytic therapy is reserved for those women failing systemic anticoagulation. The prognosis for venous thrombosis in pregnancy is better than in nonpregnant subjects, and mortality rates are less than 10 percent (McCaulley, 2011). The recurrence rate is 1 to 2 percent during a subsequent pregnancy (Mehraein, 2003).

Recurrence Risk of Ischemic Stroke

Women with previous ischemic stroke have a low risk for recurrence during a subsequent pregnancy unless a specific, persistent cause is identified. Lamy and colleagues (2000) followed 37 women who had an ischemic stroke during pregnancy or the puerperium, and none of their 24 subsequent pregnancies was complicated by another stroke. In another study of 23 women who had prepregnancy strokes from a variety of causes, there were 35 subsequent pregnancies without a stroke recurrence (Coppage, 2004). Finally, in a follow-up study of 1770 nonpregnant women with antiphospholipid-related ischemic stroke, investigators reported no difference in the recurrence risk as long as preventative treatment was given with warfarin or aspirin (Levine, 2004). In another study of nonpregnant subjects, low-dose aspirin following venous thromboembolism decreased the risk of a subsequent stroke (Brighton, 2012).

Currently, there are no firm guidelines regarding prophylaxis in pregnant women with a stroke history (Helms, 2009). The American Heart Association stresses the importance of controlling risk factors such as hypertension and diabetes (Furie, 2011). Women with antiphospholipid antibody syndrome or certain cardiac conditions should be considered for prophylactic anticoagulation as discussed in Chapters 49 (p. 979) and 59 (p. 1175).

image Hemorrhagic Stroke

The two distinct categories of spontaneous intracranial bleeding are intracerebral and subarachnoid hemorrhage. The symptoms of a hemorrhagic stroke are similar to those of an ischemic stroke. Their differentiation is only possible with CT or MR imaging (Morgenstern, 2010).

Intracerebral Hemorrhage

Bleeding into the brain parenchyma most commonly is caused by spontaneous rupture of small vessels previously damaged by chronic hypertension as depicted in Figure 60-1 (Qureshi, 2001; Takebayashi, 1983). Thus, pregnancy-associated hemorrhagic strokes such as the one shown in Figure 60-2 are often associated with chronic hypertension and superimposed preeclampsia (Cunningham, 2005; Martin, 2005). Because of its location, this type of hemorrhage has much higher morbidity and mortality rates than does subarachnoid hemorrhage (Morgenstern, 2010). Chronic hypertension is uniquely associated with Charcot-Bouchard microaneurysms of the penetrating branches of the middle cerebral artery. Pressure-induced rupture causes bleeding in the putamen, thalamus, adjacent white matter, pons, and cerebellum. In the 28 women described by Martin and associates (2005), half died and most survivors had permanent disabilities. This cautions for the importance of proper management for gestational hypertension—especially systolic hypertension—to prevent cerebrovascular pathology (Chap. 40p. 761).

image

FIGURE 60-2 arge intracerebral hemorrhage caused by hypertensive stroke.

Subarachnoid Hemorrhage

In a study of 639 cases of pregnancy-related subarachnoid hemorrhage from the Nationwide Inpatient Sample, the incidence was 5.8 per 100,000 pregnancies, with half being postpartum (Bateman, 2012). These bleeds are more likely caused by an underlying cerebrovascular malformation in an otherwise normal patient (see Fig. 60-1). Ruptured saccular or “berry” aneurysms cause 80 percent of all subarachnoid hemorrhages. The remaining cases are caused by a ruptured arteriovenous malformation, coagulopathy, angiopathy, venous thrombosis, infection, drug abuse, tumors, or trauma. Such cases are uncommon, and a ruptured aneurysm or angioma or bleeding from a vascular malformation has an incidence of 1 in 75,000 pregnancies. Although this frequency is not different from that in the general population, the mortality rate during pregnancy is reported to be as high as 35 percent.

Intracranial Aneurysm. Approximately 2 to 5 percent of adults have this lesion (see Fig. 60-1). Fortunately, only a small percentage rupture—approximately 0.1 percent for aneurysms < 10 mm and 1 percent for those > 10 mm (Smith, 2008). Most aneurysms identified during pregnancy arise from the circle of Willis, and 20 percent are multiple (Stoodley, 1998). Pregnancy does not increase the risk for aneurysmal rupture. However, because of their high prevalence, they are more likely to cause subarachnoid bleeding than other causes (Hirsch, 2009; Tiel Groenestege, 2009). Aneurysms are more likely to bleed during the second half of pregnancy—only approximately 20 percent bleed during the first half (Dias, 1990).

The cardinal symptom of a subarachnoid hemorrhage from an aneurysm rupture is sudden severe headache, accompanied by visual changes, cranial nerve abnormalities, focal neurological deficits, and altered consciousness. Patients typically have signs of meningeal irritation, nausea and vomiting, tachycardia, transient hypertension, low-grade fever, leukocytosis, and proteinuria. Prompt diagnosis and treatment may prevent potentially lethal complications. The American Heart Association recommends noncontrast cranial CT imaging as the first diagnostic test, although MR imaging may be superior (Chalela, 2007; Connolly, 2012).

Treatment includes bed rest, analgesia, and sedation, with neurological monitoring and strict blood pressure control. Repair of a potentially accessible aneurysm during pregnancy depends in part on the recurrent hemorrhage risk versus surgical risks. At least in nonpregnant patients, the risk of subsequent bleeding with conservative treatment is 20 to 30 percent for the first month and then 3 percent per year. The risk of rebleeding is highest within the first 24 hours, and recurrent hemorrhage leads to death in 70 percent.

Early repair is done by surgical clipping of the aneurysm or by endovascular coil placement completed using fluoroscopic angiography yet attempting to limit radiation exposure. For women remote from term, repair without hypotensive anesthesia seems optimal. For women near term, cesarean delivery followed by aneurysm repair is a consideration, and we have successfully done this in several cases. For aneurysms repaired either before or during pregnancy, most allow vaginal delivery if remote from aneurysmal repair. A problem is what defines “remote,” and although some recommend 2 months, the time for complete healing is unknown. For women who survive subarachnoid hemorrhage, but in whom surgical repair is not done, we agree with Cartlidge (2000) and recommend against bearing down—put another way, we favor cesarean delivery.

Arteriovenous Malformations. These are congenital focal abnormal conglomerations of dilated arteries and veins with subarteriolar disorganization (see Fig. 60-1). They lack capillaries and have resultant arteriovenous shunting. Although unclear, the risk of bleeding may increase with gestational age. When arteriovenous malformations (AVMs) bleed, half do so into the subarachnoid space, whereas half are intraparenchymal with subarachnoid extension (Smith, 2008). They are uncommon and are estimated to occur in 0.01 percent of the general population. Bleeding does not appear to be more likely during pregnancy (Finnerty, 1999; Horton, 1990). AVMs are correspondingly rare during pregnancy, and in the study from Parkland Hospital, there was only one AVM in nearly 90,000 deliveries (Simolke, 1991).

Treatment of AVMs in nonpregnant patients is largely individualized. There is no consensus whether all those that are accessible should be resected. It also depends on whether the lesion is symptomatic or an incidental finding; its anatomy and size; presence of associated aneurysm, which is found in up to 60 percent; and especially, whether or not the lesion has bled. After hemorrhage, the risk of recurrent bleeding in unrepaired lesions is 6 to 20 percent within the first year, and 2 to 4 percent per year thereafter (Friedlander, 2007; Smith, 2008). The mortality rate with a bleeding AVM is 10 to 20 percent. In pregnancy, the decision to operate is usually based on neurosurgical considerations, and Friedlander (2007) recommends strong consideration for treatment if bleeding occurs. Because of the high risk of recurrent hemorrhage from an unresected or inoperable lesion, we favor cesarean delivery. An unusual case of spontaneous regression of a cerebral AVM has been described (Couldwell, 2011).

DEMYELINATING OR DEGENERATIVE DISEASES

The demyelinating diseases are neurological disorders characterized by immune-mediated focal or patchy destruction of myelin sheaths accompanied by an inflammatory response. The degenerative diseasesare multifactorial and are characterized by progressive neuronal death.

image Multiple Sclerosis

In the United States, multiple sclerosis (MS) is second only to trauma as a cause of neurological disability in middle adulthood (Hauser, 2012b). Because the disease affects women twice as often as men and usually begins in the 20s and 30s, women of reproductive age are most susceptible. The familial recurrence rate of MS is 15 percent, and the incidence in offspring is increased 15-fold.

The demyelinating characteristic of this disorder results from predominately T cell-mediated autoimmune destruction of oligodendrocytes that synthesize myelin. There is a genetic susceptibility and likely an environmental trigger such as exposure to certain bacteria and viruses, for example, Chlamydophila pneumoniae, human herpesvirus 6, or Epstein-Barr virus (Frohman, 2006; Goodin, 2009).

There are four clinical types of multiple sclerosis (Hauser, 2012b):

1. Relapsing-remitting MS accounts for initial presentation in 85 percent of affected individuals. It is characterized by unpredictable recurrent episodes of focal or multifocal neurological dysfunction usually followed by full recovery. Over time, however, relapses lead to persistent deficits.

2. Secondary progressive MS disease is relapsing-remitting disease that begins to pursue a progressive downhill course after each relapse. It is likely that all patients eventually develop this type.

3. Primary progressive MS accounts for 15 percent of cases. It is characterized by gradual progression of disability from the time of initial diagnosis.

4. Progressive-relapsing MS refers to primary progressive MS with apparent relapses.

Classic findings of MS include sensory loss, visual symptoms from optic neuritis, weakness, paresthesias, and a host of other neurological symptoms. Almost 75 percent of women with isolated optic neuritis develop multiple sclerosis within 15 years. Clinical diagnosis is confirmed by MR imaging and cerebrospinal fluid analysis. In greater than 95 percent of cases, MR imaging shows characteristic multifocal white matter plaques that represent discrete areas of demyelination such as shown in Figure 60-3. Their appearance and extent are less helpful for treatment response. Similarly, Kuhle and colleagues (2007) reported that identification of serum antibodies against myelin oligodendrocyte glycoprotein and myelin basic protein are not predictive of recurrent disease activity.

image

FIGURE 60-3 Magnetic resonance cranial images from a woman with multiple sclerosis. A. T2-weighted axial image shows bright signal abnormalities in white matter, typical for multiple sclerosis. B.Sagittal T2-FLAIR image shows hyperintense areas within the corpus callosum that are representative of demyelination in multiple sclerosis. (From Hauser, 2012b, with permission.)

Effects of Pregnancy on MS

The PRegnancy IMultiple Sclerosis—PRIMS—study is a European prospective multicenter study in which 254 pregnancies were described (Vukusic, 2006). A principal finding was a confirmed 70-percent reduction in relapse risk during pregnancy, but with a significantly increased relapse rate postpartum. This may be related to increased pregnancy-induced numbers of T-helper lymphocytes with an increased T2/T1 ratio (Airas, 2008). In a metaanalysis of women with more than 1200 pregnancies complicated by multiple sclerosis, their relapse rate was 0.4 per year before pregnancy; 0.26 per year during pregnancy; and this increased to 0.7 per year after delivery (Finkelsztejn, 2011). Factors associated with postpartum relapse include a high relapse rate before pregnancy, relapses during pregnancy, and a high MS disability score (Portaccio, 2014; Vukusic, 2006). Breast feeding has no apparent effect on postpartum relapses (Airas, 2010; Portaccio, 2011).

Effects of MS on Pregnancy

There are usually no adverse effects on pregnancy outcome with uncomplicated disease. Some women may become fatigued more easily, those with bladder dysfunction are predisposed to urinary infection, and women with spinal lesions at or above T6 are at risk for autonomic dysreflexia. Dahl and coworkers (2006) described 449 such pregnancies, and they reported a higher labor induction rate and longer second-stage labor. The increased induction rate as well as elective operations contributed to the overall increased cesarean delivery rate. They also reported outcomes in 649 affected women and described a lower mean birthweight but similar perinatal mortality rate compared with that of controls (Dahl, 2005). Other studies have corroborated that MS does not significantly affect obstetrical and neonatal outcomes (Finkelsztejn, 2011).

Management During Pregnancy and the Puerperium

Goals are to arrest acute or initial attacks, employ disease-modifying agents, and provide symptomatic relief. Some treatments may need to be modified during pregnancy. Acute or initial attacks are treated with high-dose intravenous methylprednisolone—500 to 1000 mg daily for 3 to 5 days, followed by oral prednisone for 2 weeks. Plasma exchange may be considered. Symptomatic relief can be provided by analgesics; carbamazepine, phenytoin, or amitriptyline for neurogenic pain; baclofen (Lioresal, Kemstro) for spasticity; α2-adrenergic blockade for bladder neck relaxation; and cholinergic and anticholinergic drugs to stimulate or inhibit bladder contractions.

Several disease-modifying therapies can be used for relapsing multiple sclerosis or exacerbations. Examples include interferons β1a (Rebif) and β1b (Betaseron) and glatiramer acetate (Copaxone), which have been shown to decrease relapse rates by a third (Rudick, 2011). Data concerning safety in pregnancy are limited but overall reassuring (Amato, 2010; Salminen, 2010). Clinical trials with natalizumab (Tysabri), an alpha 4 integrin antagonist—especially when combined with interferon β1a—significantly reduced MS clinical relapse rates (Polman, 2006; Rudick, 2006). In a review of 35 pregnancies, early pregnancy drug exposure did not worsen outcomes (Hellwig, 2011). Because of limited data, however, women are currently advised to stop the drug 3 months before conception. Fingolimad (Gilenya) is a new oral medication, and pregnancy safety data are unavailable (Briggs, 2011).

Prevention of relapses postpartum is afforded by treatment with intravenous immune globulin (IVIG) given in a dose of 0.4 g/kg daily for 5 days during weeks 1, 6, and 12 (Argyriou, 2008). The Prevention of Postpartum Relapses with Progestin and Estradiol in Multiple Sclerosis (POPART’MUS) trial is a multicenter randomized controlled trial currently enrolling patients (Vukusic, 2009).

image Huntington Disease

This adult-onset neurodegenerative disease results from an autosomal dominant expanded CAG trinucleotide repeat within the Huntington gene on chromosome 4. It is characterized by a combination of choreoathetotic movements, progressive dementia, and psychiatric manifestations. Because the mean age of onset is 40 years, Huntington disease rarely complicates pregnancy. Prenatal diagnosis is discussed in Chapter 14 (p. 297). However, prenatal screening is controversial because this usually is a late-onset adult disease. Thus, extensive pretest counseling is imperative (Novak, 2010).

image Myasthenia Gravis

This autoimmune-mediated neuromuscular disorder affects approximately 1 in 7500 persons. It is more common in women, and its incidence peaks in their 20s and 30s. The etiology is unknown, but genetic factors likely play a role. Most patients demonstrate antibodies to the acetylcholine receptor, although 10 to 20 percent are seronegative. The latter often have antibodies to muscle-specific tyrosine kinase (MuSK) that regulates assembly of the acetylcholine receptor subunits at the neuromuscular junction (Cavalcante, 2011; Pal, 2011).

Cardinal features of MG are weakness and easy fatigability of facial, oropharyngeal, extraocular, and limb muscles. Deep tendon reflexes are preserved. Cranial muscles are involved early and disparately, and diplopia and ptosis are common. Facial muscle weakness causes difficulty in smiling, chewing, and speaking. In 85 percent of patients, the weakness becomes generalized. Other autoimmune diseases may coexist, and hypothyroidism should be excluded. The clinical course is marked by exacerbations and remissions, especially when it first becomes clinically apparent. Remissions are not always complete and are seldom permanent. Systemic diseases, concurrent infections, and even emotional upset may precipitate exacerbations, of which there are three types:

1. Myasthenic crises—characterized by severe muscle weakness, inability to swallow, and respiratory muscle paralysis.

2. Refractory crises—characterized by the same symptoms but unresponsive to the usual therapy.

3. Cholinergic crises—excessive cholinergic medication leads to nausea, vomiting, muscle weakness, abdominal pain, and diarrhea.

All three of these can be life threatening, but a refractory crisis is a medical emergency. Those with bulbar myasthenia are at particular risk because they may be unable to swallow or even ask for help.

Management

Myasthenia is manageable but not curable. Thymectomy is generally recommended for long-term benefits in the 75 percent of patients who have thymic hyperplasia or a thymoma seen with CT or MR imaging (Drachman, 2012; Nam, 2011). Anticholinesterase medications such as pyridostigmine (Mestinon), an analogue of neostigmine, improve symptoms by impeding acetylcholine degradation. They seldom produce normal muscle function. Ironically, overdose is manifest by increased weakness—the cholinergic crisis—that may be difficult to differentiate from myasthenic symptoms. Most of those refractory to anticholinesterase therapy respond to immunosuppressive therapy with glucocorticoids, azathioprine, methotrexate, cyclosporine, and mycophenolate mofetil. Tacrolimus, an immunosuppressant used in organ transplantation, and rituximab, a chimeric monoclonal antibody, are currently being evaluated (Ibrahim, 2010; Maddison, 2011; Yoshikawa, 2011). Cyclophosphamide is reserved for severe, generalized refractory cases. When short-term, rapid clinical improvement is needed—such as for a surgical procedure or a myasthenic crisis—high-dose immunoglobulin G or plasma exchange is usually effective (Barth, 2011; Cortese, 2011; Mandawat, 2010).

Myasthenia and Pregnancy

Because the greatest period of risk is within the first year following diagnosis, it seems reasonable to postpone pregnancy until there is sustained improvement. Antepartum management of myasthenia includes close observation with liberal rest and prompt treatment of infections (Heaney, 2010; Kalidindi, 2007). Women in remission who become pregnant while taking corticosteroids or azathioprine should continue these. Thymectomy has been successfully performed during pregnancy in refractory cases (Ip, 1986). Acute onset of myasthenia or its exacerbation demands prompt hospitalization and supportive care. Plasmapheresis and high-dose immunoglobulin therapy should be used for emergency situations (Drachman, 2012).

Although pregnancy does not appear to affect the overall course of MG, fatigue common to most pregnancies may be exacerbated, and the expanding uterus may compromise respiration. During pregnancy, maternal hypotension or hypovolemia are avoided. The clinical course of MG during pregnancy is unpredictable, and frequent hospitalizations are the norm. Up to a third of women have worsening MG during pregnancy, with exacerbations occurring equally in all three trimesters (Djelmis, 2002; Podciechowski, 2005).

Myasthenia gravis has no significant adverse effects on pregnancy outcomes (Wen, 2009). Preeclampsia is a concern because magnesium sulfate may precipitate a severe myasthenic crisis (Hamaoui, 2009; Heaney, 2010). Although phenytoin use is also problematic in this regard, its adverse effects are less troublesome, and thus many choose it for neuroprophylaxis in MG patients with severe preeclampsia. Because smooth muscle is unaffected, most women have normal labor. Oxytocin is given for the usual indications, and cesarean delivery reserved for obstetrical indications. Because narcotics may cause respiratory depression, close observation and respiratory support are essential during labor and delivery. Curariform drugs should be avoided—examples include magnesium sulfate discussed above, muscle relaxants used with general anesthesia, and aminoglycosides. Neuraxial analgesia is accomplished with amide-type local agents. Regional analgesia is preferred unless there is significant bulbar involvement or respiratory compromise (Almeida, 2010; Blichfeldt-Lauridsen, 2012). During second-stage labor, some women may have impaired voluntary expulsive efforts that may warrant operative vaginal delivery.

Neonatal Effects

As discussed above, 80 percent of mothers with myasthenia gravis have anti-acetylcholine-receptor IgG antibodies. These and anti-MuSK antibodies are transported transplacentally, and the fetus can be affected to cause hydramnios (Heaney, 2010). Similarly, 10 to 20 percent of neonates manifest MG symptoms (Murray, 2010; Niks, 2008). Transient symptoms usually include a feeble cry, poor suckling, and respiratory distress. Symptoms usually respond to cholinesterase inhibitors and resolve within a few weeks.

NEUROPATHIES

Peripheral neuropathy is a general term used to describe disorders of peripheral nerve(s) of any cause. Because neuropathy can result from a variety of sources, its discovery should prompt a search for an etiology. Polyneuropathiescan be either axonal or demyelinating as well as acute, subacute, or chronic (Chaudhry, 2008). They are often associated with systemic diseases such as diabetes, with drug or environmental toxin exposure, or with genetic diseases.

Mononeuropathies are relatively common in pregnancy, and they signify focal involvement of a single nerve trunk and imply local causation such as trauma, compression, or entrapment. Traumatic pudendal, obturator, femoral, and common fibular mononeuropathies are usually caused by childbirth and are discussed in Chapter 36 (p. 676).

image Guillain-Barré Syndrome

In 75 percent of cases, this acute demyelinating polyradiculoneuropathy has clinical or serological evidence for an acute infection. Commonly associated are infection with Campylobacter jejuni, cytomegalovirus, and Epstein-Barr virus; surgical procedures; and immunizations (Haber, 2009; Hauser, 2012a). Guillain-Barré syndrome (GBS) is thought to be immune-mediated from antibodies formed against nonself antigens. Demyelination causes sensory and motor conduction blockade, and recovery occurs with remyelination in most cases.

Clinical features include areflexic paralysis—usually ascending—with or without sensory disturbances. Autonomic dysfunction is common. The full syndrome develops over 1 to 3 weeks. Management is supportive, but in the worsening phase, patients should be hospitalized because at least a third will need ventilatory assistance. Intravenous high-dose immunoglobulin (IVIG) or plasmapheresis is beneficial if begun within 1 to 2 weeks of motor symptoms, however, neither decreases mortality rates (Cortese, 2011; Gwathmey, 2011; Hughes, 2011). Although most patients recover fully within several months to a year, up to 20 percent are severely disabled, and 5 percent die, despite treatment (Yuki, 2012). Some manifest as chronic inflammatory demyelinating polyneuropathy, and our experiences indicate that it may be relatively common in these young women.

Pregnancy

Guillain-Barré syndrome is not more common in pregnancy, although inconclusive data suggest rates are increased in the puerperium (Cheng, 1998). Its clinical course in pregnancy is the same as for nonpregnant individuals, and after an insidious onset, paresis and paralysis most often continue to ascend to cause ventilatory weakness. Hurley and colleagues (1991) reported that a third of pregnant women required ventilatory support, with a mortality rate of 13 percent. The acute syndrome is treated with either high-dose IVIG or plasmapheresis (Chan, 2004; Yuki, 2012).

image Bell Palsy

This disfiguring palsy is usually a mononeuropathic acute facial paralysis that is relatively common in reproductive-aged women (Fig. 60-4). It has a female predominance, and pregnant women are at a fourfold risk compared with nonpregnant women (Cohen, 2000; Heaney, 2010). The disease is characterized by facial nerve inflammation and often is associated with reactivation of herpes virus or herpes zoster virus.

image

FIGURE 60-4 Bell facial nerve palsy developing on the day of delivery after a cesarean for dichorionic twins. This woman was treated with prednisone and antiviral medication, and the palsy had almost resolved 3 weeks postpartum.

Bell palsy usually has an abrupt and painful onset with maximum weakness by 48 hours. In some cases, hyperacusis and loss of taste accompany paralysis (Beal, 2012). Management includes supportive care with facial muscle massage and eye protection against corneal lacerations from drying. There is general consensus that prednisone, 1 mg per kg given orally daily for 5 days, will result in improved outcomes and a shortened recovery period (Salinas, 2010; Sullivan, 2007). It is controversial if addition of an antiviral medication will improve these outcomes (de Almeida, 2009; Lockhart, 2009; Quant, 2009).

Pregnancy

It is unclear if pregnancy alters the prognosis for spontaneous facial palsy recovery. Gillman and colleagues (2002) found that only half of pregnant women recovered to a satisfactory level after 1 year—this compared with approximately 80 percent of nonpregnant women and men. Some prognostic markers for incomplete recovery are bilateral palsy, recurrence in a subsequent pregnancy, greater percentage of nerve function loss, and a faster rate of loss (Cohen, 2000; Gilden, 2004). Corticosteroid therapy given early in the course of the disease significantly improves outcomes. Other than a fivefold increased rate for gestational hypertension or preeclampsia, women with Bell palsy do not have increased adverse pregnancy outcomes (Katz, 2011; Shmorgun, 2002).

image Carpal Tunnel Syndrome

This syndrome results from compression of the median nerve and is the most frequent mononeuropathy in pregnancy (Padua, 2010). Symptoms include burning, numbness, or tingling along the inner half of one or both hands. Also, wrist pain and numbness extend into the forearm and sometimes into the shoulder (Katz, 2002). Symptoms are bilateral in 80 percent of pregnant women, and 10 percent have evidence for severe denervation (Seror, 1998). Differential diagnosis includes cervical radiculopathy of C6–C7 and de Quervain tendonitis, which is caused by swelling of the conjoined tendons and their sheaths near the distal radius. Nerve conduction studies may be helpful (Alfonso, 2010).

Pregnancy

The reported incidence is 7 to 43 percent and varies greatly because the range of symptoms is marked (Finsen, 2006; Padua, 2010). Symptomatic treatment with a splint applied to the slightly flexed wrist during sleep relieves pressure and usually provides relief. Although symptoms typically are self-limited, occasionally surgical decompression and corticosteroid injections are necessary (Keith, 2009; Shi, 2011). Symptoms may persist in more than half of patients at one year and in a third at 3 years (Padua, 2010).

SPINAL-CORD INJURY

According to the National Spinal Cord Injury Statistical Center (2012), there are approximately 12,000 spinal cord injuries each year. At least half affect reproductive-age individuals, with a male-to-female predominance of 4 to 1. The severity of cord injury determines the short- and long-term prognosis as well as that for pregnancy. Incomplete neurological lesions are associated with at least some sensory and/or motor function below the level of injury, whereas there is none with complete cord transection. Many women develop sexual function alteration and transient hypothalamic pituitary hypogonadism, however, pregnancy is not uncommon if menstruation resumes (Bughi, 2008).

Women with spinal cord injury have an increased frequency of pregnancy complications that include preterm and low-birthweight infants. Most have asymptomatic bacteriuria with sporadic symptomatic urinary infections. Bowel dysfunction causes constipation in more than half, and anemia and pressure-necrosis skin lesions are also common. There are two serious and life-threatening complications with spinal cord injuries:

1. If the cord is transected above T10, the cough reflex is impaired, respiratory function may be compromised, and pneumonitis from covert aspiration can be serious. Pulmonary function tests are considered, and some women may need ventilatory support in late pregnancy or in labor.

2. Women with lesions above T5–T6 are at risk for autonomic dysreflexia. With this, stimuli from structures innervated below the level of the spinal lesion results in massive disordered sympathetic stimulation. Abrupt catecholamine release can cause vasoconstriction with severe hypertension and symptoms that include throbbing headaches, facial flushing, sweating, bradycardia, tachycardia, arrhythmias, and respiratory distress. Dysreflexia can be precipitated by a variety of stimuli, such as urethral catheterization; bladder distention from retention; rectal or cervical distention with digital examinations; uterine contractions and cervical dilatation; or any other pelvic structure manipulation (American College of Obstetricians and Gynecologists, 2005; Krassioukov, 2009). In one report, 12 of 15 women at risk for autonomic dysreflexia suffered at least one episode during pregnancy (Westgren, 1993).

Because uterine contractions are not affected by spinal cord lesions, labor is usually easy—even precipitous, and comparatively painless. If the lesion is below T12, uterine contractions are felt normally. For lesions above T12, the risk of out-of-hospital delivery is substantial and can be minimized by teaching women to palpate for uterine contractions. This is especially important because up to 20 percent of women deliver preterm (Westgren, 1993). Some recommend tocodynamometry and weekly cervical examinations beginning at 28 to 30 weeks. Another reasonable option that we frequently employ at Parkland Hospital is elective hospitalization after 36 to 37 weeks (Hughes, 1991).

Spinal or epidural analgesia extending to T10 prevents autonomic dysreflexia and should be instituted at the start of labor. If there are severe symptoms before epidural placement, steps are taken to abolish the provoking stimulus. A parenteral antihypertensive agent such as hydralazine or labetalol is given. Labor and vaginal delivery with epidural or spinal analgesia are preferable and will minimize autonomic dysreflexia (Kuczkowski, 2006). Operative vaginal delivery is frequently necessary.

IDIOPATHIC INTRACRANIAL HYPERTENSION

Also known as pseudotumor cerebri or benign intracranial hypertension, this disorder is characterized by increased intracranial pressure without hydrocephalus. The cause is unknown, but it may be the result of either overproduction or underabsorption of cerebrospinal fluid. Symptoms include headache in at least 90 percent of cases, visual disturbances such as loss of a visual field or central visual acuity in 70 percent, and commonly occurring papilledema that may be sight-threatening (Evans, 2000; Heaney, 2010). Other complaints are stiff neck, back pain, pulsatile tinnitus, cranial nerve palsies such as facial palsy, ataxia, or paresthesias. The syndrome is commonly found in young women and is prevalent in those who are obese, who recently gained weight, or both (Fraser, 2011). Along with symptoms, other criteria for diagnosis include elevated intracranial pressure > 250 mm H2O, normal cerebrospinal fluid (CSF) composition, normal cranial CT or MR imaging findings, and no evidence for systemic disease (International Headache Society, 2005).

Idiopathic intracranial hypertension is usually self-limited. Visual defects can be prevented by lowering the cerebrospinal fluid pressure. Drugs given to lower pressure include acetazolamide (Diamox) to reduce fluid production, furosemide (Lasix), or topiramate (Topamax). Corticosteroids are now rarely used. Surgical intervention is occasionally necessary and is accomplished by either lumboperitoneal shunting of spinal fluid or optic nerve sheath fenestration.

image Effects of Pregnancy

It is controversial if pregnancy is a risk factor for idiopathic intracranial hypertension. Certainly, symptoms may first appear in pregnancy, and women previously diagnosed may become symptomatic. These usually develop by midpregnancy, tend to be self-limited, and usually resolve postpartum.

There is general agreement that pregnancy does not alter management. Some recommend serial visual field testing to prevent permanent vision loss. In a report of 16 pregnant women, visual field loss developed in four, and it became permanent in one (Huna-Baron, 2002). Visual field loss is often coincident with the development of papilledema, for which acetazolamide is given. Lee and associates (2005) reported successful treatment of 12 pregnant women. Although outmoded for treatment of nonpregnant individuals, repeated lumbar punctures are generally successful in providing temporary relief throughout pregnancy. In some pregnant women, surgical therapy becomes necessary, and we and others have had promising results with optic nerve sheath fenestration (Thambisetty, 2007).

Pregnancy complications are likely due to associated obesity and not to intracranial hypertension. In a review of 54 pregnancies, there were no excessive adverse perinatal outcomes (Katz, 1989). The route of delivery depends on obstetrical indications, and conduction analgesia is safe (Aly, 2007; Karmaniolou, 2011).

MATERNAL VENTRICULAR SHUNTS

Pregnancies in women with previously placed ventricular shunts for obstructive hydrocephalus usually have satisfactory outcomes (Landwehr, 1994). Shunts may be ventriculoperitoneal, ventriculoatrial, or ventriculopleural. Partial obstruction of a shunt is common, especially late in pregnancy (Schiza, 2012). In one report of 17 such pregnancies, neurological complications were reported in 13 (Wisoff, 1991). Findings included headaches in 60 percent, nausea and vomiting in 35 percent, lethargy in 30 percent, and ataxia or gaze paresis, each in 20 percent. Most symptoms respond to conservative management. However, if CT scanning during symptom evaluation discloses acute hydrocephaly, then the shunt is tapped or pumped several times daily. In some cases, surgical revision is necessary and may be emergently required (Murakami, 2010).

Another shunting procedure involves placement of an endoscopic third ventriculostomy for hydrocephalus in children or adults (de Ribaupierre, 2007). One report described successful results in five pregnant women who underwent successful ventriculostomy placement (Riffaud, 2006). In a review, reproductive function and miscarriage rates were found to significantly worsen in these women (Bedaiwy, 2008).

Vaginal delivery is preferred in women with shunts, and unless there is a meningomyelocele, conduction analgesia is permitted. Antimicrobial prophylaxis is indicated if the peritoneal cavity is entered for cesarean delivery or tubal sterilization.

MATERNAL BRAIN DEATH

Brain death is rare in obstetrics. Life-support systems and parenteral alimentation for up to 15 weeks have been described while awaiting delivery (Hussein, 2006; Powner, 2003; Souza, 2006). Some women were treated with aggressive tocolysis and antimicrobial therapy. Chiossi and coworkers (2006) reviewed outcomes in 17 women with persistent vegetative state who were given various levels of support. At least five died after delivery, and most of the remainder continued to be in the vegetative state. There are no published reports of neurological recovery with a diagnosis of brain death using the uniform Determination of Death Act definition (Wijdicks, 2010). The ethical, financial, and legal implications, both civil and criminal, that arise from attempting or not attempting such care are profound (Farragher, 2005; Feldman, 2000). In some women, perimortem cesarean delivery is performed as discussed in Chapter 47 (p. 956).

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