Neurologic disorders complicating pregnancy

INTRODUCTION — Pregnancy adds a unique and, at times, challenging facet to the management of neurologic disease. The pregnant state can affect many neurologic diseases, while certain neurologic diseases or their treatments may have a significant negative effect on pregnancy, labor, or delivery. This topic review will focus on the relationship between pregnancy and a wide spectrum of neurologic illnesses.

HEADACHE — Headaches may present during pregnancy. Most are migraine and tension-type headaches, but they may present some treatment challenges during this time. A minority of new headaches during pregnancy represent more serious, even life-threatening neurologic conditions. This topic is discussed separately. (See "Headache during pregnancy and postpartum".)

LOW BACK PAIN AND DISC DISEASE — One- to two-thirds of all pregnant women will experience back pain. Usually this reflects the musculoskeletal consequences that ensue as a result of normal hormonal changes and weight gain. This topic is discussed separately. (See "Maternal adaptations to pregnancy: Musculoskeletal changes and pain", section on 'Low back pain and disc disease'.)

CEREBROVASCULAR DISEASE — Cerebrovascular disease during pregnancy results from any of three major mechanisms: arterial infarction/ischemia, hemorrhage, or venous thrombosis. Pregnancy and the postpartum period are associated with a marked increase in the relative risk (RR) and a small increase in the absolute risk of stroke.

Risk factors for stroke related to pregnancy include cesarean delivery, pregnancy-induced hypertension, postpartum infection, and possibly multiple gestations. In addition, conditions unique to pregnancy can occur, which can present as either a stroke or a stroke-like event. These include eclampsia, peripartum cardiomyopathy, peripartum angiopathy, and gestational trophoblastic disease. Of these, preeclampsia/eclampsia is the most common cause of stroke in pregnancy. Other causes are listed in the table (table 1). The risk of stroke in women with preeclampsia/eclampsia and cerebrovascular disease associated with pregnancy are discussed in detail separately. (See "Cerebrovascular disorders complicating pregnancy".)

MULTIPLE SCLEROSIS — Multiple sclerosis (MS) is an immune-mediated demyelinating disease of the central nervous system typically characterized by relapses and remissions of neurologic deficits. MS affects women more than men by a ratio of approximately 2:1; the mean age of onset of MS is approximately 30 years. (See "Pathogenesis and epidemiology of multiple sclerosis" and "Manifestations of multiple sclerosis in adults".)

Issues related to pregnancy planning for patients with MS are reviewed separately. (See "Multiple sclerosis: Pregnancy planning".)

Care during pregnancy and the postpartum period, including breastfeeding, is also reviewed separately. (See "Multiple sclerosis: Pregnancy and postpartum care".)

NEUROPATHY — A variety of neuropathies can occur in pregnancy.

Carpal tunnel syndrome — Carpal tunnel syndrome (CTS) refers to paresthesias, hypesthesia, pain, or numbness of the thumb, index, and middle fingers as a result of compression of the median nerve in the carpal tunnel. Affected patients often awake with burning, numbness, and tingling in the median nerve distribution, which is bilateral in 75 percent of cases. Patients commonly report shaking the hand to relieve the discomfort. The diagnosis is based upon presence of characteristic symptoms and objective findings and is similar to that in nonpregnant individuals. (See "Carpal tunnel syndrome: Clinical manifestations and diagnosis".)

CTS is relatively common during pregnancy, with an incidence of 2 to 35 percent [1,2]. In one large series, pregnancy accounted for 7 percent of cases of CTS in women between the ages of 15 and 44 [3]. The increased prevalence in pregnant women is thought to be caused by pregnancy-related fluid retention leading to compression of the nerve in the carpal tunnel; hormonal changes affecting the musculoskeletal system may also play a role. This was demonstrated by a study of 76 pregnant women with symptoms suggestive of CTS: 43 percent had abnormal nerve conduction studies of their carpal tunnel, and these women were more likely to have edema than women with normal studies [4].

Symptoms tend to occur during the last trimester but can occur at any time [2,5]. In most cases, they gradually resolve over a period of weeks to months after delivery; however, symptoms can be prolonged for several months in women who are breastfeeding [6]. In one series of 37 women followed prospectively from near term to approximately 12 months after delivery, symptoms remained in 46 percent and were more common in women with early onset of disease [7]. Symptoms may recur in subsequent pregnancies.

Patients may receive benefit from splinting the wrist at night in a neutral position or slight extension. Wrist splints may need to be worn throughout the day in severe cases. (See "Carpal tunnel syndrome: Treatment and prognosis", section on 'Wrist splinting'.)

Corticosteroid injection or surgery to release the flexor retinaculum is rarely indicated during pregnancy since the disease has a better prognosis than idiopathic CTS and often resolves postpartum. In a prospective case-control study of 45 pregnant women with CTS and 90 controls (age-matched women with idiopathic CTS) who were followed for three years, the pregnancy cohort was significantly more likely to show improvement in severity and function scores (68 and 73 percent, respectively) than the control cohort (42 and 39 percent) [8].

Bell's palsy — Bell's palsy is defined as a paralysis of the facial nerve, typically involving all three peripheral branches, resulting in asymmetric facial expression and unilateral weakness of eye closure. There is a two- to fourfold increase in prevalence during pregnancy, especially in the third trimester or in the first postpartum week [9-11]. The cause for this increased risk of Bell's palsy in pregnancy is unclear, in part because the mechanism underlying Bell's palsy is uncertain. Perineural edema, hypercoagulability causing thrombosis of the vasa nervosum, and relative immunosuppression in pregnancy have been proposed as potential etiologic factors [12]. One study found an association of Bell's palsy in pregnancy with preeclampsia and gestational hypertension [13].

The clinical manifestations and diagnosis of Bell’s palsy are discussed in detail separately. (See "Bell's palsy: Pathogenesis, clinical features, and diagnosis in adults".)

Recovery of facial paresis appears slightly worse in women who develop the disease during pregnancy. A retrospective study of 77 women with pregnancy-related Bell's palsy compared recovery outcome in pregnant women with that of nonpregnant women and men [14]. All patients in all groups with incomplete paralysis ultimately recovered satisfactory function. However, more pregnant woman developed complete paralysis (65 versus 50 percent for nonpregnant and male patients). Pregnant women (as well as nonpregnant patients) with complete paralysis are less likely to recover satisfactory function than the other groups (52 versus 77 to 88 percent). The fact that pregnant women are less likely to receive treatment may also contribute to an apparent poorer prognosis [11,15]. It is not clear whether the risk of recurrence is higher in pregnant versus nonpregnant women. (See "Bell's palsy: Treatment and prognosis in adults".)

Treatment recommendations for Bell's palsy during pregnancy are the same as for nonpregnant patients, and are discussed separately (see "Bell's palsy: Treatment and prognosis in adults"). However, glucocorticoid administration during embryogenesis may be associated with an increased risk of cleft palate, although data are conflicting. (See "Safety of rheumatic disease medication use during pregnancy and lactation", section on 'Glucocorticoids'.)

Meralgia paresthetica — Meralgia paresthetica is a sensory neuropathy that occurs when the lateral femoral cutaneous nerve is compressed as it penetrates the tensor fascia lata at the inguinal ligament. Symptoms include dysesthesias in the upper and middle part of the lateral thigh and are probably caused by the expanding abdominal wall and increased lumbar lordosis. Symptoms occur late in pregnancy, typically resolve within three months postpartum, and rarely require treatment. (See "Meralgia paresthetica (lateral femoral cutaneous nerve entrapment)".)

Guillain-Barré syndrome — The Guillain-Barré syndrome (GBS) is an acute monophasic illness causing a rapidly progressive polyneuropathy with weakness or paralysis. GBS is thought to result from an immune response to a preceding infection that cross-reacts with peripheral nerve components because of molecular mimicry. The immune response can be directed towards the myelin or the axon of peripheral nerve, resulting in demyelinating and axonal forms of GBS. It typically begins within 30 days of an infection. A small percentage of patients develop GBS after another triggering event such as immunization, surgery, or trauma. (See "Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and diagnosis".)

The cardinal clinical features of GBS are progressive, mostly symmetric muscle weakness and absent or depressed deep tendon reflexes. The weakness can vary from mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory, and bulbar muscles. Severe respiratory muscle weakness necessitating ventilatory support develops in approximately 30 percent, and dysautonomia occurs in 70 percent of patients. GBS usually progresses over a period of approximately two weeks, followed by a plateau phase of two to four weeks, and then gradual recovery of function. (See "Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and diagnosis" and "Guillain-Barré syndrome in adults: Treatment and prognosis", section on 'Prognosis'.)

GBS does not appear to occur with increased frequency during pregnancy nor has uncomplicated disease been shown to affect pregnancy, labor, or delivery [16,17]. However, the incidence of GBS may be increased in the postpartum period [16]. There is one case report of congenital GBS in the neonate of an affected mother [18].

The main modalities of therapy for GBS are plasma exchange and administration of intravenous immune globulin (IVIG). These treatments hasten recovery from GBS, as shown in randomized controlled trials. However, neither treatment has been studied specifically in pregnant women with GBS. Aggressive fluid loading prior to plasma exchange may help to avoid hypotension. (See "Guillain-Barré syndrome in adults: Treatment and prognosis", section on 'Immunomodulatory therapy'.)

Maternal GBS is not an indication for cesarean delivery, which should be reserved for the usual obstetric indications only. Consultation with the anesthesia service is important, as these patients have some additional anesthetic issues (eg, need to avoid succinylcholine, concerns about exacerbation of neurologic deficit, autonomic instability) [16].

Supportive care is extremely important since up to 30 percent of patients with GBS develop neuromuscular respiratory failure requiring mechanical ventilation. In addition, autonomic dysfunction may be severe enough to require intensive care unit monitoring. Thus, many patients with GBS are initially admitted to the ICU for close monitoring of respiratory, cardiac, and hemodynamic function. Less severely affected patients can be managed in intermediate care units. Prophylaxis for deep vein thrombosis, bladder and bowel care, physical and occupational therapy, and psychological support are essential. Adequate pain control is necessary. (See "Guillain-Barré syndrome in adults: Treatment and prognosis", section on 'Supportive management and monitoring'.)

Hereditary peripheral neuropathies — Hereditary neuropathies that can affect women of childbearing age include hereditary motor sensory neuropathy (HMSN), also known as Charcot-Marie-Tooth (CMT) disease, and Friedreich ataxia.

CMT disease is a spectrum of disorders caused by a specific mutation in one of several myelin genes that result in defects in myelin structure, maintenance, and formation. Affected individuals show progressive distal limb atrophy and weakness, often with gait disturbance and deformity of feet and hands (see "Charcot-Marie-Tooth disease: Genetics, clinical features, and diagnosis"). An observational study including 108 births to mothers with CMT reported these patients had more operative deliveries, malpresentations, and postpartum bleeding than the general obstetric population [19]. Gestational age at birth and birth weights were comparable to those in unaffected pregnancies. It was not clear whether the increased prevalence of malpresentation was related to fetal disease, although the disorder typically does not present until later in childhood. Postpartum bleeding from atony may have been related to the effect of the disease on uterine adrenergic nerves.

Friedreich ataxia is an autosomal recessive neurodegenerative disease affecting the cerebellum, peripheral nerves, and the heart (see "Friedreich ataxia"). Limited data suggest that women with Friedreich ataxia are not at high risk for complications of pregnancy or worsening of disease symptoms during pregnancy (see "Friedreich ataxia", section on 'Pregnancy implications'). In a case report, epidural and spinal anesthesia were used for caesarean birth without complication in a woman with Friedreich ataxia [20].

Postpartum compression neuropathies — Peripheral neuropathy has been reported postpartum in 1 to 58 per 10,000 deliveries [21]. The true incidence is not known since most series relied upon patient surveys or medical record discharge coding. The femoral and lateral femoral cutaneous nerves are most often affected.

●Patients with significant femoral neuropathy develop weakness involving the quadriceps muscle group with sparing of adduction. In addition to muscle weakness, sensory loss over the anterior thigh and most of the medial thigh is typical.

●The lateral femoral cutaneous nerve does not contain motor fibers; thus, neurologic symptoms are restricted to sensory changes. Lateral hip pain accompanied by paresthesias (burning pain) or hypesthesias (numbness and tingling) over the upper outer thigh is the classic presentation of compression of this nerve. (See 'Meralgia paresthetica' above.)

●Peroneal nerve compression results in foot drop. It can be caused by prolonged squatting, sustained knee flexion, or pressure on the fibular head from stirrups or palmar pressure during pushing.

●Obturator neuropathies are an uncommon complication of delivery and present with medial thigh pain and adductor weakness.

Factors that appear to predispose pregnant women to neuropathy postpartum include fetal macrosomia or malpresentation, sensory blockade (can impair recognition of discomfort), prolonged lithotomy position, prolonged second stage, and improper use of leg stirrups and retractors. However, many of these factors are interdependent (eg, prolonged lithotomy position and prolonged second stage). The prognosis for complete recovery is excellent; however, weakness may persist for several weeks or, rarely, months.

The clinical features, diagnosis, and treatment of peripheral nerve syndromes involving the leg are discussed in detail separately. (See "Overview of lower extremity peripheral nerve syndromes".)

MYASTHENIA GRAVIS — Myasthenia gravis (MG) is an autoimmune disorder resulting in dysfunction of the neuromuscular end plate of skeletal muscle. Affected patients are often young women who usually present with fatigable weakness after repetitive muscle use. Pregnancy has a variable effect on the course of MG. This topic is discussed separately. (See "Management of myasthenia gravis in pregnancy".)

MUSCLE DISEASE — Muscle disease affects women in the childbearing years. Pregnancy may influence the course of muscle disease, and vice versa. Experience with pregnancy in the setting of muscular dystrophy has been documented in several case series.

●Myotonic dystrophy (MD). This disorder generally has a benign course through pregnancy [12,22]. A minority of women experience increased weakness, which resolves after delivery. However, pregnant women with MD have an increased incidence of fetal loss, prematurity, and polyhydramnios, likely because of inherited disease in the fetus. A minority of women have prolonged labor due to poor uterine contraction. General anesthesia should be avoided in these patients because of specific complications associated with MD. (See "Myotonic dystrophy: Treatment and prognosis", section on 'Risk of anesthesia'.)

●Facioscapulohumeral muscular dystrophy (FSHD). Among 38 women with FSHD with 105 gestations and 78 live births, rates of low birth weight, forceps-assisted deliveries, and operative deliveries were higher than the national average. In 24 percent of pregnancies, women experienced worsening muscle disease that did not resolve postpartum [23]. (See "Facioscapulohumeral muscular dystrophy".)

●Limb-girdle muscular dystrophy (LGMD). Permanent worsening of weakness is common in one-third to one-half of patients with LGMD, who also experience higher rates of fetal loss and have more operative deliveries [22,24]. (See "Limb-girdle muscular dystrophy".)

●Worsening of weakness is also reported in many women with other congenital myopathies [24]. Prematurity and operative deliveries are also common.

Issues related to pregnancy and polymyositis are discussed separately. (See "Initial treatment of dermatomyositis and polymyositis in adults", section on 'Pregnancy'.)

MOVEMENT DISORDERS

Chorea gravidarum — Chorea is characterized by irregular, brief, unpredictable, jerky movements involving multiple parts of the body in a random sequence. Chorea from any cause beginning during pregnancy has been called chorea gravidarum. Important causes of chorea in women of reproductive age include acute rheumatic fever and the antiphospholipid syndrome [25,26]; less common causes include Wilson disease, hyperthyroidism, vascular disease, other hypercoagulable states, and toxins. In some patients with chorea gravidarum, a specific cause cannot be identified. The evaluation of a patient with chorea is described separately. (See "Overview of chorea", section on 'Evaluation for the cause of chorea'.)

Chorea typically begins during the second to fifth month but occasionally presents postpartum [26]. Symptoms typically resolve spontaneously within several weeks to months or may subside shortly after delivery. If warranted, symptomatic treatment may be tried. (See "Overview of chorea", section on 'Management of chorea'.)

Restless legs syndrome — Pregnancy appears to be a risk factor for the occurrence or worsening of restless legs syndrome; the cause is unclear. (See "Management of restless legs syndrome and periodic limb movement disorder in adults", section on 'Pregnancy and lactation' and "Clinical features and diagnosis of restless legs syndrome and periodic limb movement disorder in adults", section on 'Pregnancy'.)

SEIZURES AND EPILEPSY — There are a number of important issues to be addressed by the physician when a woman with epilepsy becomes pregnant:

●Are antiseizure medications necessary?

●What effect do antiseizure medications have on the fetus?

●What effect does maternal epilepsy have on the fetus?

●What effect does pregnancy have on seizures?

●How should the patient be managed during pregnancy and delivery?

●How should the patient be managed during the postpartum period?

These issues are discussed separately. (See "Risks associated with epilepsy during pregnancy and the postpartum period" and "Management of epilepsy during preconception, pregnancy, and the postpartum period".)

Occasionally, a woman presents with a first seizure in pregnancy. The approach to evaluation and management is discussed separately. (See "Management of epilepsy during preconception, pregnancy, and the postpartum period", section on 'Approach to a first seizure in pregnancy'.)

BRAIN TUMORS — The incidence of brain neoplasms does not increase during pregnancy, and the types of tumors are similar to those seen in nonpregnant women of the same age. Low-grade and high-grade gliomas and meningiomas each represent approximately one-third of cases. No particular systemic neoplasm with brain metastases is associated with pregnancy with the exception of choriocarcinoma, in which approximately 3 to 20 percent of patients have brain metastases at the time of diagnosis [27]. Meningiomas, vestibular schwannomas (acoustic neuromas), and pituitary tumors may enlarge during pregnancy and become symptomatic. A small increase in tumor size can have major consequences depending on its location.

The changes that occur during pregnancy can have a significant effect on symptoms and tumor growth [27-29]. Fluid retention, for example, can increase tumor edema and enlarge vascular tumors such as meningiomas and vestibular schwannomas [29]. In addition, both meningiomas and vestibular schwannomas have sex hormone receptors, which may play a role in accelerating tumor growth during gestation [30]. A study of 12 pregnancies in 11 women with grade II gliomas found significant radiologic expansion of the tumor during pregnancy as compared with prepregnancy and postdelivery time periods [31]. In another small case series of eight patients with a glioma, pregnancy appeared to be associated with adverse effects (clinical and/or radiographic worsening) in six [32].

Clinical manifestations — Although nausea and vomiting is a common symptom of both pregnancy and cerebral neoplasms, pregnancy-related nausea and vomiting occurs very early in pregnancy and tends to improve across gestation, while tumor-related nausea and vomiting is more likely to arise late in gestation, gradually worsens, and may be accompanied by headache.

New onset of seizures can be a symptom of eclampsia or associated with a brain tumor. Eclamptic seizures are typically generalized. Tumor-related seizures may be focal and associated with focal neurologic findings. However, secondary generalization may occur rapidly, and the focal onset therefore may be unapparent to observers. In one case series, increased seizure frequency was noted in 5 of 12 pregnancies in women who harbored grade II gliomas [31]. An overview of the clinical manifestations and diagnosis of brain tumors can be found separately. (See "Overview of the clinical features and diagnosis of brain tumors in adults".)

Management — The treatment and prognosis of brain neoplasms seen during pregnancy is highly dependent upon the particular cell type involved, as well as the clinical manifestations and the stage of pregnancy [33]. Magnetic resonance imaging (MRI), which does not involve radiation, can be safely performed in the pregnant patient, but gadolinium is typically not recommended. (See "Diagnostic imaging in pregnant and lactating patients".)

Surgery during pregnancy is indicated in patients with malignant tumors or tumors causing severe symptoms [33]. Brain irradiation when indicated may be performed during pregnancy.

Symptomatic treatment with anticonvulsants or corticosteroids may be necessary and should be used with appropriate caution. Neither should be used prophylactically in this setting. Treatment of seizures during pregnancy is discussed separately (see "Management of epilepsy during preconception, pregnancy, and the postpartum period"). Considerations regarding the use of corticosteroids in pregnancy are also discussed separately. (See "Safety of rheumatic disease medication use during pregnancy and lactation", section on 'Glucocorticoids'.)

Maternal blood pressure and fluids should be monitored closely. Antihypertensive medication should be given sparingly to avoid hypotension, which could result in cerebral hypoperfusion. Excessive hydration, which could worsen cerebral edema, should also be avoided [34]. (See "Management of vasogenic edema in patients with primary and metastatic brain tumors".)

A potential problem can occur in patients with brain tumors during the second stage of labor. Increased cerebrospinal fluid pressures have been observed in normal women during painful uterine contractions [35]. This could potentially lead to neurologic compromise in a woman who already has a baseline elevation in intracranial pressure. Thus, careful consideration should be given to the type of delivery. Depending upon the location and size of the mass, a cesarean delivery under general anesthesia may be warranted [36]. There are insufficient data to assess risk and make a general recommendation; advice of subspecialists in neurology and neurosurgery is advised in each case. Anesthetic issues related to labor and delivery are discussed separately. (See "Obstetric and nonobstetric anesthesia for patients with neurologic disorders", section on 'Brain tumors'.)

Pituitary adenoma — Pregnancy also promotes the growth of lactotroph adenomas (prolactinomas). Neurologic symptoms most often develop in patients with macroadenomas (13 to 36 percent) but also occur in a few patients (less than 2 percent) with microadenomas (defined as less than 10 mm in diameter). Pregnant women with known pituitary adenomas may present with new-onset headache or visual changes that can be difficult to differentiate from migraines and other headache disorders. MRI can be used when necessary during all trimesters of pregnancy to rule out growth of the adenoma or pituitary apoplexy from ischemia or hemorrhage [37]. (See "Diagnostic imaging in pregnant and lactating patients", section on 'Magnetic resonance imaging'.)

It is preferable to reduce tumor size with a dopamine agonist and/or surgery prior to pregnancy in women with a macroadenoma. (See "Management of lactotroph adenoma (prolactinoma) before and during pregnancy".)

VENTRICULOPERITONEAL SHUNT — Shunt malfunction complicates up to 25 to 50 percent of pregnancies in women with ventriculoperitoneal (VP) shunts [38,39]. Functional occlusion of the shunt most often occurs in the third trimester. The rise in intra-abdominal pressure caused by the enlarging uterus may exceed the pressure within the peritoneal catheter, resulting in the cessation of cerebrospinal fluid drainage with a subsequent elevation in intracranial pressure [33]. Symptoms include confusion, lethargy, nausea, vomiting, headache, nystagmus, and cranial nerve palsies. These symptoms require urgent evaluation, neuroimaging, and neurosurgical consultation.

Preconception counseling should include description of the risks and magnetic resonance imaging (MRI) for establishing baseline ventricular size and to verify adequate shunt function.

Although there are no controlled studies examining the best method of delivery for pregnant women with VP shunts, there seems to be agreement that vaginal delivery can be attempted [38-40]. Shortening the second stage has been recommended by some but not all authors to reduce the rise in intracranial pressure that occurs during pushing. However, increased intracranial pressure should not occur with a properly functioning valve.

Caesarean delivery is recommended for neurologically unstable patients and those with obstetric indications for surgical delivery. Care should be taken to avoid dislodging or manipulating the abdominal tip of the shunt while the peritoneal cavity is open. Both regional and general anesthesia can be used in pregnant women with VP shunts [41]. Prophylactic antibiotics have usually been recommended for labor and delivery to avoid shunt infection [33,39]; however, the risk of infection is low, and this practice has been questioned [40]. The antibiotics given are the same as for prophylaxis against infective endocarditis. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures" and "Infections of cerebrospinal fluid shunts".)

SPINAL CORD INJURY — A significant proportion of spinal cord injuries occur in women of reproductive age [42]. In contrast to males with spinal cord injury, fertility is not usually compromised. This section discusses the management of pregnancy in a woman who has had a previous spinal cord injury.

Common complications of spinal cord injury include anemia (from iron deficiency, folate deficiency, anemia of chronic disease, and chronic renal insufficiency), urinary tract infection, decubitus ulcers, and respiratory problems. Serial urinary tract cultures or antibiotic suppression is recommended to avoid upper urinary tract infection and its associated morbidity [43]. Thermoregulation is impaired so that hypothermia can occur from cutaneous vasodilation and inability to shiver, while hyperthermia results from impairment of the normal sweating mechanism. Due to the tendency to spasticity and contractures, muscle-strengthening exercises for the upper extremities may be recommended for appropriate patients; range-of-motion exercises may be useful in others [43]. The management of chronic complications of spinal cord injury is discussed in detail separately. (See "Chronic complications of spinal cord injury and disease".)

Baseline pulmonary function studies should be performed in those who are contemplating pregnancy, and pulmonary function should be reassessed serially during pregnancy to identify women who might need ventilatory assistance in labor. (See "Respiratory complications in the adult patient with chronic spinal cord injury".)

There are insufficient data to recommend universal thromboprophylaxis during pregnancy or the puerperium for all patients with spinal cord injury; each case should be addressed individually [44].

Vaginal delivery is permissible. The ability to perceive labor pain is dependent upon the level of the injury. Women with cord injuries below T11 will be able to perceive labor pain, while those with lesions at T5 to T10 may have painless labors and deliveries. Such women should be monitored to avoid an unattended delivery from undetected labor. They may suspect labor from other clues such as spasticity and flexor spasm. They should also be taught uterine palpation to help detect contractions, and use of a home uterine monitor, weekly cervical examinations, and liberal hospitalization can be considered near term. Although some others have observed a higher frequency of preterm labor, most series report rates similar to that in the general obstetric population.

These precautions may be particularly important for women with lesions above T5-6 level for the following reasons [45]:

●They are prone to slightly earlier delivery (36 to 40 weeks rather than 38 to 42 weeks).

●Eighty-five percent develop autonomic dysreflexia during labor. The disorder can be precipitated by distension or irritation of the cervix, vagina, bowel, or bladder (table 2). The manifestations of this potentially life-threatening syndrome result from sympathetic hyperactivity below the level of the lesion, since this portion of the spinal cord has been isolated from hypothalamic control. Symptoms include malignant hypertension (which may be confused with preeclampsia), loss of consciousness, headache, nasal congestion, facial erythema, sweating, piloerection, bradycardia or tachycardia, and arrhythmia. Uteroplacental vasoconstriction may develop, leading to fetal hypoxia. The symptoms are exacerbated by contractions and wane between contractions. Continuous monitoring of the cardiac rhythm and blood pressure are warranted, as cerebrovascular accidents, intraventricular hemorrhage, or myocardial infarction may occur. (See "Chronic complications of spinal cord injury and disease", section on 'Autonomic dysreflexia'.)

Epidural anesthesia extending to the T10 level should be used to prevent these manifestations, even if the patient does not perceive pain; antepartum consultation with an anesthesiologist is important [43]. (See "Pharmacologic management of pain during labor and delivery".)

An indwelling Foley catheter will prevent bladder distention, and a bowel regimen is useful to prevent bowel distention from constipation [44]. If symptoms of autonomic dysreflexia occur, it is important to make sure the catheter is draining freely and the bowel is not impacted.

If the patient has not yet received anesthesia and is supine, having her sit up leads to pooling of blood in the lower extremities and may reduce blood pressure. Antihypertensive medications (such as intravenous labetalol or oral nifedipine) are given as needed. However, patients with spinal cord injury maintain systemic blood pressure by compensatory enhancement of the renin-angiotensin-aldosterone system. For this reason, they may be very sensitive to medications that inhibit the function of angiotensin-converting enzyme (ACE inhibitors). If severe hypertension persists despite anesthesia and intravenous antihypertensive medications, nitroprusside may be considered, but is relatively contraindicated since it may cause cyanide intoxication in the fetus. A case report noted a dramatic benefit from magnesium sulfate in a woman with autonomic dysreflexia [46]. (See "Evaluation and treatment of hypertensive emergencies in adults".)

SUMMARY AND RECOMMENDATIONS — Pregnancy can affect many neurologic diseases, while certain neurologic diseases or their treatments may have a significant negative effect on pregnancy, labor, or delivery.

●The management of cerebrovascular disease, epilepsy, low back pain, myasthenia, polymyositis, and restless legs syndrome are discussed separately in specific topic reviews devoted to these conditions in pregnancy. (See "Management of epilepsy during preconception, pregnancy, and the postpartum period" and "Cerebrovascular disorders complicating pregnancy" and "Maternal adaptations to pregnancy: Musculoskeletal changes and pain" and "Management of myasthenia gravis in pregnancy" and "Clinical features and diagnosis of restless legs syndrome and periodic limb movement disorder in adults" and "Initial treatment of dermatomyositis and polymyositis in adults", section on 'Pregnancy'.)

●There are a number of issues to consider for women with multiple sclerosis (MS) who are pregnant or planning pregnancy. These are discussed in separate topic reviews. (See "Multiple sclerosis: Pregnancy planning" and "Multiple sclerosis: Pregnancy and postpartum care".)

●A variety of neuropathies are common in pregnancy.

•Carpal tunnel syndrome (CTS) has an increased incidence in pregnancy, with symptoms commonly improving postpartum. Patients may benefit symptomatically from splinting. (See 'Carpal tunnel syndrome' above.)

•Bell's palsy is also more common in pregnancy; treatment recommendations are not changed during pregnancy. (See 'Bell's palsy' above.)

•Meralgia paresthetica is more common in late stages of pregnancy but typically does not require treatment and resolves after delivery. (See 'Meralgia paresthetica' above.)

•Postpartum compression neuropathies result from compression during labor and delivery. These include femoral neuropathy, peroneal neuropathy, and obturator neuropathy. The prognosis for complete recovery is excellent; however, weakness may persist for several weeks or longer. (See 'Postpartum compression neuropathies' above.)

●Muscle disease affects women in the childbearing years. Pregnancy may influence the course of muscle disease, and vice versa. (See 'Muscle disease' above.)

●Chorea gravidarum typically begins during the second to fifth month but occasionally presents postpartum. Symptoms typically resolve spontaneously within several weeks or may subside shortly after delivery. If warranted, the cautious use of haloperidol and corticosteroids may be beneficial. (See 'Chorea gravidarum' above.)

●The physiologic changes (eg, fluid retention, altered sex hormone levels) that occur during pregnancy can have a significant effect on symptoms and brain tumor growth. (See 'Brain tumors' above.)

●Shunt malfunction complicates up to 25 to 50 percent of pregnancies in women with ventriculoperitoneal (VP) shunts requiring urgent evaluation, neuroimaging, and neurosurgical consultation. Vaginal delivery can be attempted in stable women with VP shunt. Caesarean section is recommended for neurologically unstable patients and those with obstetric indications for surgical delivery. Prophylactic antibiotics are suggested during labor and delivery to reduce the risk of shunt infection. (See 'Ventriculoperitoneal shunt' above.)

●The management of pregnant women with a spinal cord injury should include consideration of thromboprophylaxis. Home monitoring may be required in those with lesions from T5 to T10 as they may have painless labor. In addition, patients with lesions above T5-6 may be prone to autonomic dysreflexia during labor and require additional anesthesia, cardiovascular monitoring, and antihypertensive treatments. (See 'Spinal cord injury' above.)