BJA Education, 21(6): 210e217 (2021)

doi: 10.1016/j.bjae.2021.02.003
Advance Access Publication Date: 18 March 2021

Matrix codes: 1A01,

2B01, 2B02, 2B03,
2B05, 2B06, 3B00

Anaesthesia and neurological disorders in pregnancy

Y. Metodiev1,* and F. Braveman2
1
Cardiff & Vale University Health Board, Cardiff, UK and 2University of Minnesota, Minneapolis, MN, USA
*Corresponding author: yavor.r.metodiev@gmail.com

Keywords: maternal medicine; maternal morbidity; nervous system diseases; neurological disorders; obstetric
anaesthesia

Learning objectives Key points

 Describe common neurological conditions  Neurological conditions are the third leading
encountered in the peripartum period. cause of maternal mortality in the UK.
 Identify the interplay of the physiologic changes  Epilepsy is the most common neurological dis-
of pregnancy with specific neurological disorders. order encountered in pregnancy.
 Explain the anaesthetic implications of common  Neuraxial anaesthesia is not contraindicated in
neurological conditions in the peripartum period. patients with multiple sclerosis.
 Physiological changes of pregnancy put women
at higher risk of acute cerebrovascular disorders
Advances in the medical and surgical management of and the anaesthetist is best placed to coordinate
neurological disorders have improved quality and length of the management of these in the immediate
life, and reduced the incidence of severe disabilities. As a setting.
consequence, increasing numbers of women of childbearing  Spinal pathology may preclude the use of neu-
age have existing neurological diseases. The normal physio- raxial techniques for labour analgesia and
logical changes of pregnancy, such as alteration in the im- anaesthesia.
mune response, a procoagulant state and increased metabolic
demands, may affect the progression and status of some
neurological disorders. This may complicate what would with the previous triennium report.2 The increase in mortality
otherwise be a normal pregnancy and delivery. In the latest from neurological disorders in pregnancy results predomi-
Mothers and Babies: Reducing Risk through Audits and nantly from epilepsy and stroke-related causes.1
Confidential Enquiries across the UK (MBRRACE-UK) report,
neurological conditions were found to be the second most General principles of management
common indirect and third overall cause of maternal death in
the UK.1 This represents a slight trend upwards compared Antenatal period
Efforts to reduce and prevent morbidity and mortality
associated with neurological conditions should start with
planning before conception. Women should be counselled
Yavor Metodiev MD PhD FRCA is a locum consultant obstetric
regarding the effects of their condition on pregnancy out-
anaesthetist at University Hospital of Wales in Cardiff, UK. He is a
comes and vice versa. Early identification and referral to
member of the editorial board of the International Journal of Ob-
appropriate specialists helps women’s understanding and
stetric Anesthesia.
active participation in the management of both their
Ferne Braveman MD CM is a professor of anesthesiology at the pregnancy and neurological condition. Obstetric anaesthe-
University of Minnesota, where she is establishing the first division tists should be involved early to help mitigate peripartum
of obstetrical anesthesiology in the department. Previously she was complications and optimise maternal outcomes.
professor and vice chair at both the University of Southern California
and Yale School of Medicine. At Yale she also led the division of
obstetrical anesthesiology and she is an emeritus professor at that
institution.

Accepted: 5 February 2021

Crown Copyright © 2021 Published by Elsevier Ltd on behalf of British Journal of Anaesthesia. All rights reserved.
For Permissions, please email: permissions@elsevier.com

210
 Anaesthesia and neurological disorders in pregnancy

Intrapartum period Antiepileptic drugs should be taken regularly throughout

pregnancy, especially during labour when an intravenous
A cohesive anaesthetic plan based on shared decision-making
formulation may be required because of variable intestinal
with the patient and made well in advance of delivery may
absorption intrapartum. Seizures in the intrapartum period
alleviate the often highly charged circumstances of the
should be treated as eclamptic in origin, especially in the
intrapartum period. The anaesthetist must be notified as soon
presence of risk factors such as pre-eclampsia or hyperten-
as a woman with a neurological condition presents in labour
sion. Magnesium sulphate is the drug of choice in these cir-
or for an elective Caesarean delivery. Discussion of any status
cumstances.7 The doses recommended by the National
changes or modification of anaesthetic management, obstet-
Institute for Health and Care Excellence (NICE) are 4 g i.v. over
ric management, or both should occur at this time.
5e15 min followed by infusion of 1 g h 1 over 24 h.8 The
American College of Obstetricians and Gynecologists suggest
Postpartum period a more liberal dosing regimen e 4e6 g loading dose followed
by 1e2 g h 1.9 Eclamptic seizures are usually short and self-
New changes occur after delivery of the baby, including hor-
limiting generalised toniceclonic convulsions. In women
monal imbalances, breastfeeding demands, sleep deprivation
with poorly controlled epilepsy, benzodiazepines may need to
and other physical and mental demands on a new mother.
be given simultaneously to reduce the duration of the seizure
Some neurological disorders tend to worsen postpartum,
and prevent maternal and fetal hypoxia and fetal acidosis.
especially autoimmune diseases. Newly developed neurolog-
A maternal history of epilepsy should not dictate the mode
ical symptoms in the postpartum period need to be thor-
of delivery. Neuraxial analgesia for labour is recommended to
oughly investigated and promptly acted upon as
reduce sleep deprivation and labour pain-induced stress and
consequences of delayed management can be disastrous.
dehydration.7 If opioids are used, pethidine should be avoided
because of its epileptogenic potential. General anaesthetic
Specific neurological conditions drugs are all acceptable to use in epileptic women, except for
etomidate, which may lower the seizure threshold in sus-
Epilepsy
ceptible individuals. Hepatic metabolism of neuromuscular
Epilepsy is the most common serious neurological disorder in blocking agents (NMBAs) is increased by the enzyme-inducing
pregnant women with an estimated global incidence of 6.85 effect of AEDs, and this shortens their duration of action.10
per 1000 women.3 Every year there are approximately 2500
births among women with epilepsy in the UK.2 The early input
Multiple sclerosis
of a specialist in epilepsy is extremely valuable, as the choice
of medications and dosing of medications may need to be Multiple sclerosis (MS) is an immune-mediated, chronic,
altered during pregnancy (increasing doses, change of anti- demyelinating disorder of the central nervous system (CNS)
epileptic drugs [AEDs] regimen).4 The primary management with an immune-mediated aetiology. It is three times more
goals are control of seizures, concerns related to dose- common in women; the mean age at diagnosis is 30 yrs.11 The
dependent teratogenicity of AEDs, the management of status overall prevalence in the UK is about 0.1e0.2%.12 The hallmark
epilepticus, the prevention of sudden unexplained death in of the disease is periventricular inflammatory lesions, which
epilepsy (SUDEP) and differentiating epileptic from non- eventually cause demyelination and axonal damage. The
epileptic convulsions (especially eclampsia). plaques can be observed anywhere in the CNS, hence the
The analysis of 3806 pregnancies in the European and In- myriad of symptoms in the clinical presentation.11,13 Symp-
ternational Registry of Antiepileptic Drugs and Pregnancy toms of MS are usually intermittent (relapsingeremitting), but
(EURAP) study, involving women with primarily idiopathic in about 10e15% it will have a slowly progressive course.
generalised epilepsy, reported that two thirds of women Women may present with a variety of neurological symptoms,
remained seizure-free throughout pregnancy.4 For about 15% including sensory and motor deficits, bladder dysfunction,
of women there was deterioration in seizure control, which visual disturbance, mood changes and cognitive decline.
was more likely to occur among women with focal epileptic The number of relapses usually reduces during pregnancy,
disorders.4 especially in the third trimester, and most women will not
Management of epilepsy in pregnancy with AEDs must require any treatment. It is thought that this effect results
balance the need to protect the mother from the detrimental from the hormone-induced reduction in cell-mediated im-
effects of seizure activity against the risk of congenital mal- munity during pregnancy, which may also explain the higher
formations in the fetus. All AEDs possess some teratogenic incidence of relapses in the first 3 months postpartum.10,14
potential (Table 1), but growing evidence suggests that Nevertheless, the anaesthetic antenatal assessment should
monotherapy with certain AEDs such as lamotrigine or lev- focus on a thorough history and neurological examination
etiracetam may be safer for both fetus and mother.5 Patients and documentation of existing neurological deficits. It has
should be counselled before and in early pregnancy regarding been suggested in the past that neuraxial techniques, specif-
the risks of congenital malformations associated with AEDs ically spinal anaesthesia, may contribute to increasing re-
as poor understanding of the specific risks may lead to poor lapses after delivery because the demyelinated spinal cord is
adherence with therapy and deterioration in seizure control. exposed to neurotoxic local anaesthetic agents.15 However,
Hormonal changes, increased volume of distribution and the Pregnancy in Multiple Sclerosis study (PRIMS) found no
protein binding of AEDs, hyperemesis gravidarum, insomnia correlation between the use of neuraxial techniques and re-
and psychological stress may result in the need of frequent lapses of MS in the postpartum period.14 Numerous subse-
adjustments of therapy to maintain seizure control and pre- quent reports support these findings.16,17
vent SUDEP.6 Common antiepileptics, dosing adjustment Extra caution should be taken with the use of NMBAs when
requirements with pregnancy and their teratogenic potential general anaesthesia is considered. Hyperkalaemia may occur
are listed in Table 1. with depolarising NMBAs because of MS-induced denervation

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Anaesthesia and neurological disorders in pregnancy

Table 1 Antiepileptic drugs, dose adjustment required during pregnancy, and potential for teratogenic effects (adapted from Voinescu
and Pennell6)

Antiepileptic Dose Teratogenic Associated congenital

drug adjustment potential malformations

Phenobarbital Needs monitoring 6e7% Cardiac

and adjusting (with dose >150 mg) Oral clefts

Phenytoin May need increasing 2.4e7% Cardiac

Urogenital
Oral clefts

Carbamazepine No need of adjusting 3% Microcephaly

Growth restriction

Valproic acid Needs monitoring 9e10% Spina bifida

(increasing with dose) Atrial septal defect
Cleft palate
Hypospadias
Polydactyly
Craniosynostosis
Lower IQ

Oxcarbazepine Needs adjusting 2.5% Autism?

Lamotrigine Needs adjusting 1.3e4.6% Cardiac

(mostly higher doses) Hypospadias

Gabapentin Uncertain 1.2% Uncertain

Topiramate Needs increasing 2.4e6.8% Cardiac

Hypospadias
Oral clefts

Levetiracetam Needs increasing 1.7% Growth restriction?

or misuse myopathy. Non-depolarising NMBAs may have and mortality.2,18 Commonly, subarachnoid haemorrhage
prolonged effects secondary to decreased muscle mass. (SAH) and ICH in the peripartum period are a result of
Conversely, reports of resistance to NMBAs have also been ruptured cerebral aneurysms or arteriovenous malformation
reported, perhaps as a result of a proliferation of extra- (AVM). Almost half of the women with ICH of any cause have a
junctional receptors. history of pre-eclampsia, eclampsia or haemolysis, elevated
Most disease-modifying treatment options (biological liver enzymes and low platelets (HELLP) syndrome.19
agents, monoclonal antibodies, cytostatic drugs) are dis-
continued before conception and during gestation. Should
Cerebral aneurysms
relapses occur during the antenatal period, they are treated
Pregnancy-related physiological changes such as cerebral
with high doses of steroids, i.v. immunoglobulins or plasma
vasodilation, increased plasma volume and hypertension do
exchange.17
not increase the risk of aneurysmal ruptures.19 Therefore,
obstetric and anaesthetic management does not need to be
Cerebrovascular disorders modified in asymptomatic women with known brain aneu-
rysms. If an aneurysm bleeds, early intervention (e.g. clipping
Cerebrovascular disorders cause significant morbidity and
or coiling) improves maternal and fetal outcomes and if
mortality amongst patients in the peripartum period. Multi-
antenatal treatment is successful obstetric and anaesthetic
disciplinary expertise is essential during the acute presenta-
management during labour is as normal.19
tion, and if still pregnant, to choose the safest mode of delivery,
consider options for anaesthesia, and prevent deterioration of
maternal and fetal condition, as applicable. The coordination of Arteriovenous malformations
long-term follow up of patients is also important. The risk of AVM haemorrhage is increased during pregnancy
The discussion in this section will be limited to acute (up to 8.1%), requiring more proactive management of women
ischaemic stroke (AIS), cerebral venous sinus thrombosis with AVMs. Non-pregnant women who have experienced a
(CVT), intracranial haemorrhage (ICH), posterior reversible bleed from an AVM should undergo an intervention (coiling,
encephalopathy syndrome (PRES) and reversible cerebral clipping, surgery) before becoming pregnant. If AVMs are first
vasoconstriction syndrome (RCVS) in the peripartum period discovered during pregnancy the risks of intervention
(Table 2). compared with expectant management or voluntary termi-
nation of pregnancy should be considered given the signifi-
Intracranial haemorrhage cant risk of haemorrhage during pregnancy.20
Intracranial haemorrhage represents more than half of all Whatever the cause of ICH, the initial management of the
pregnancy-related strokes and is a major cause of morbidity woman should focus on her well-being, airway protection,

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 Anaesthesia and neurological disorders in pregnancy

Table 2 Cerebrovascular disorders in pregnancy. AIS, acute is chaemic stroke; ICH, intracranial haemorrhage; CVT, cerebral venous
sinus thrombosis; RCVS, reversible cerebral vasoconstriction syndrome; PRES, posterior reversible encephalopathy syndrome; MRI,
magnetic resonance imaging; SAH, subarachnoid haemorrhage; triple-H, hypertension, hypervolaemia, haemodilution

Condition AIS ICH CVT RCVS PRES

Time of onset Any time in Any time in Third trimester, Abrupt, postpartum Rapid (hours),
pregnancy, pregnancy, postpartum postpartum
postpartum postpartum

Clinical features Focal neurology Thunderclap Headache, seizures, Thunderclap Seizures, dull
headache, loss of focal neurology headache, transient headache, visual
consciousness, focal deficit, disturbances
focal neurology seizures

Imaging features CT/MRI angiogram CT evidence of SAH CT venogram is Usually normal CT Vasogenic oedema
is sensitive or ICH usually sensitive in occipito-parietal
regions

Treatment Thrombolysis, Supportive, Anticoagulation, Calcium channel Seizure control, BP

anticoagulation interventional, or seizure control blockers, triple-H, control
surgical clipping/ seizure control
coiling

cardiovascular support and neuroprotection. Should delivery timing of anticoagulant and antiplatelet drug dosing, and the
of the fetus become necessary in the acute phase, anaesthetic patient’s neurological status. Regional anaesthesia remains
options may be limited. Reduced level of consciousness, the preferred technique if it is safe to perform. Neuro-
confusion, severe haemodynamic complications (hypo-/hy- protective strategies, such as elevation of the head, preven-
pertension, arrhythmias), or need of neurosurgical interven- tion of major swings in arterial pressure, avoiding
tion may necessitate the use of general anaesthesia with hypercapnia and hypoxia, and hyperosmolar therapy, are
simultaneous stabilisation. Total intravenous anaesthesia important for maternal outcome.18 The risk of postpartum
may provide smoother control of blood pressure and prevent haemorrhage may be increased by anticoagulation and anti-
exacerbation of cerebral oedema. Care should be taken during platelet therapy, and should be managed aggressively. Inten-
laryngoscopy and extubation, to prevent spikes in blood sive care admission should be strongly considered after
pressure. Magnesium sulphate or lidocaine infusions in delivery. Prevention of recurrence of AIS should start as soon
addition to remifentanil boluses may be useful.18 If delivery is as safe, and includes increased physical activity, lifestyle
decided in women who have minor symptoms and are hae- modifications, long-term antiplatelet therapy and treatment
modynamically stable and conscious, neuraxial techniques of comorbidities.18
might be considered. Most women with an ICH, even those
with minor symptoms, will require management in a critical Cerebral venous sinus thrombosis
care unit after delivery. Cerebral venous sinus thrombosis is a rare cause of stroke
with a peripartum incidence of 11.6 per 100,000 deliveries.12
Acute ischaemic stroke Three-quarters of the cases occur in the postpartum period.
Ischaemic stroke is the rarer form of stroke in pregnancy and Pregnancy is usually the sole risk factor for developing CVT,
is usually related to risk factors such as preeclampsia or but screening for thrombophilia is recommended should it
eclampsia, hypertension, Caesarean delivery, prothrombotic occur. CVT can present with sudden-onset headaches, sei-
states (factor V Leiden, sickle cell disease, antiphospholipid zures or deteriorating level of consciousness and the diag-
syndrome, systemic lupus erythematosus), older age, black nosis is confirmed by CT scan or MRI. Anticoagulation is the
ethnicity, greater parity and multiple gestation.19 Clinical mainstay of therapy, and the prognosis is better than in CVT
signs of AIC include motor and sensory deficit consistent with not related to pregnancy.19
an ischaemic vascular lesion, but atypical symptoms, such as
headache, nausea, dizziness and visual disturbances, may be Reversible cerebral vasoconstriction syndrome
present.18 Treatment focuses on preserving brain tissue by Reversible cerebral vasoconstriction syndrome, also known as
identification of the affected vessel, reperfusion, and pre- postpartum angiopathy, is a rare condition with unknown
vention of recurrence. Therapy may include thrombolysis, prevalence. It is associated with significant morbidity, and
anticoagulation and antiplatelet medications.18 Thrombolysis this may be attributed to the lack of clear understanding of its
in pregnancy is controversial and carries the additional risks pathophysiology and effective treatment. RCVS is most
of placental abruption, systemic bleeding and fetal loss. Blood commonly observed in postpartum women with pre-
pressure control, especially avoidance of hypotension, is eclampsia and autoimmune disease or in those using recre-
paramount for both maternal and fetal outcomes. ational drugs. The usual presentation is thunderclap head-
Obstetric management is usually expectant unless there aches lasting minutes to days and segmental stenosis with
are difficulties in achieving haemodynamic targets to improve post-stenotic dilatation on cerebral angiography (‘string of
cerebral perfusion, and fetal delivery is judged to be of benefit beads’ appearance). The clinical picture may be varied and
to the mother. Anaesthetic management must consider the non-specific and the syndrome may last 2e3 months.

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Anaesthesia and neurological disorders in pregnancy

Numerous treatment modalities have been attempted with Increasing muscle weakness may occur in labour as a result of
various efficacy. Maintaining hypertension, hypervolaemia stress, sleep deprivation and pain. Clinical evaluation should
and haemodilution (triple-H therapy) with the addition of a include assessment of bulbar and respiratory muscle weak-
calcium channel blocker has been reported to be successful in ness. Epidural analgesia is recommended to minimise the risk
the management of more severe cases.19 The overall aim is to of myasthenic crisis caused by pain. Women with MG are
reverse cerebral vasoconstriction to reduce the impact of more likely than others to develop fatigue, especially in the
neurological injury (ICH, AIS), and this is best done in tertiary second stage, and are thus more likely to require instrumental
neurology and neurocritical care units.21 Most cases of RCVS or surgical delivery.10 Neuraxial techniques are safe in MG but
resolve spontaneously within 2e3 months, but the ones who extra caution needs to be taken with the level of sensory and
develop complications risk permanent neurological deficit motor block, especially during Caesarean delivery as higher
depending on the size and location of the infarction.21 levels can precipitate respiratory weakness and require
ventilatory support. Combined spinaleepidural (CSE) with a
Posterior reversible encephalopathy syndrome low-dose spinal component may be preferable to minimise
PRES is an uncommon condition that may occur without any this risk. If general anaesthesia is required, non-depolarising
identifiable risk factors but is more likely to develop in post- NMBAs need to be given judiciously and at reduced doses,
partum women with renal disease, pre-eclampsia, sepsis and starting at one-tenth of the normal dose. Depolarising NMBAs
exposure to immunosuppressant drugs. Symptoms usually such as suxamethonium may need increased dosing (up to
develop rapidly without prodromes and include headache, 2mg kg 1) as patients with MG tend to be resistant to its ef-
visual disturbances (visual field defects, diplopia, scotomata), fects. Sugammadex may be used to reliably reverse neuro-
seizures and confusion. Brain imaging (usually MRI) reveals muscular block from rocuronium or vecuronium. The dose
focal oedema mainly in the parieto-occipital and cerebellar should be titrated to effect according to neuromuscular
regions, which is reversible when appropriate treatment is monitoring.23 However, there is insufficient literature to
instigated. Many cases of PRES have overlapping features with evaluate the effects of sugammadex on lactation and expo-
pre-eclampsia and eclampsia, which makes blood pressure sure to the drug through breast milk.24
control particularly important. Seizures should be controlled Numerous other drugs given in the perioperative or peri-
with phenytoin and benzodiazepines, whereas magnesium partum period can contribute to maternal muscle weakness
should be reserved for cases with suspicion of eclamptic and respiratory failure. Magnesium sulphate, used for the
aetiology. Maintaining strict fluid balance, correction of elec- treatment of preeclampsia/eclampsia and for neonatal neu-
trolyte abnormalities, and removing other possible causes of roprotection, can lead to increased muscle weakness. The risk
PRES are some of the recommended treatment goals. Despite needs to be weighed against its benefits for the mother, the
its reversible course PRES can cause neurological complica- fetus, or both.25 Opioids and aminoglycosides also need to be
tion such as AIS or ICH with permanent sequalae.18 given cautiously as they can also compromise respiratory
function. Regular therapy with anticholinesterases needs to
continue throughout labour and may need supplementation
Myasthenia gravis
with steroids, i.v. immunoglobulins, or both.
Myasthenia gravis (MG) is an autoimmune condition where Up to a third of neonates born to seropositive mothers with
the body produces antibodies against the alpha subunit of the MG may develop transient neonatal MG (TNMG), which pre-
nicotinic acetylcholine receptors in the neuromuscular junc- sents with generalised hypotonia, weak cry, poor feeding and
tion. This leads to fatigability of skeletal muscles which can respiratory compromise. In utero, a-fetoprotein blocks the
affect some muscle groups more than others making clinical acetylcholine receptor antibodies that have crossed the
symptoms variable e ptosis, diplopia, dysphagia, respiratory placental barrier. After delivery, neonatal a-fetoprotein con-
weakness.22 The incidence of MG is one in 5000 and it affects centrations decrease, and this may be responsible for the
twice as many women as men.10 It is not uncommon for pa- onset of TNMG. The placental transfer of the antibodies may
tients to first present during pregnancy as symptoms initially result in up to 20% of neonates at 12e48 h of life and may
appear most often in the third decade of life.22 Treatment of require temporary treatment until the infant recovers. All
MG is usually symptomatic and includes anticholinesterase babies born to mothers with MG need to be assessed by neo-
drugs such as pyridostigmine, and immunosuppressants such natologists as the occurrence of TNMG does not correlate well
as steroids, intravenous immunoglobulins, azathioprine, with the severity of the maternal condition.25
mycophenolate and methotrexate. The latter two are contra-
indicated in pregnancy because of teratogenic effects,
Hereditary neuromuscular disorders
whereas the other options have been reported to be successful
in managing symptoms and preventing myasthenic crisis and Myotonic dystrophy
respiratory failure during pregnancy.12 The presence of a Myotonic dystrophy (DM) is the most common muscular
benign thymoma, common in patients with MG, needs to be dystrophy in adults, and its main feature is myotonic con-
investigated and surgical management may be recom- tractions of both skeletal and smooth muscles. It is geneti-
mended. Thymectomy has been shown to reduce the fre- cally divided into two subtypes depending on the affected
quency of exacerbations and reduce the need for steroids.22 If gene. DM1 is caused by a mutation in dystrophia myotonica
indicated, it should ideally be performed before a planned protein kinase (DMPK) gene, whereas DM2 is a consequence
pregnancy, although the benefit of thymectomy may be of mutation in the cellular nucleic acid-binding protein
delayed up to several years. (CNBP) gene.25 Both subtypes result in multisystemic disor-
During pregnancy one third of women may have wors- ders involving the cardiovascular system (cardiomyopathy,
ening of symptoms (mainly in the first trimester and post- heart failure, conduction abnormalities), respiratory system
partum), one-third will have an improvement and the rest will (reduced lung capacity, restrictive respiratory failure),
have no change in frequency or severity of relapses. endocrine system and neurodevelopmental retardation,

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 Anaesthesia and neurological disorders in pregnancy

with DM1 being the more severe form. It is likely that these spontaneous recovery is observed.10 There has been little
women will pose significant challenges during their preg- experience with sugammadex in patients with DM but it
nancy, and it is essential that a neurologist, a cardiologist may reverse neuromuscular block more reliably in these
and an endocrinologist are involved early alongside the patients.27 In the postoperative period, women with DM are
obstetric team.26 likely to require close monitoring, respiratory support, or
Women with DM are not seen commonly on the delivery both on high-dependency or critical care unit, regardless of
suite as most patients are infertile. For the parturient with the anaesthetic technique used.10
DM, neuraxial techniques for labour analgesia and for sur-
gical anaesthesia are recommended to mitigate the risks of Spinal muscular atrophies
general anaesthesia, such as aspiration caused by pharyn- Spinal muscular atrophies (SMAs) are a group of four distinct
geal muscle weakness, oesophageal smooth muscle dys- neuromuscular disorders. The most common type that affects
motility, gastric distension and delayed emptying.10 Epidural women in childbearing age is type 2, commonly associated
analgesia should be established early if labour is to be with severe scoliosis, a variable degree of respiratory failure
attempted, and extended to surgical anaesthesia if this is and cardiac abnormalities. Type 3 and type 4 SMA are usually
required. Parenteral opioids should be used cautiously or diagnosed in the second year of life and early adulthood,
not at all, as these women are known to be overly sensitive respectively, and have variably severe clinical features,
to the sedative effects of opioids. Low-dose spinal opioids whereas babies with type 1 SMA do not generally survive
may be used with caution, and respiratory function moni- beyond the second year of life.
tored. Regional blocks such as quadratus lumborum and The obstetric outcomes in women with SMA are poor, and
transversus abdominis plane blocks are recommended for almost 80% of patients will experience complications, such as
post-surgical analgesia. preterm labour and worsening of muscle function, which may
General anaesthesia is extremely risky in patients with persist after pregnancy.10 Women with SMA are seldomly able
DM and should be avoided in order to reduce the risk of to deliver spontaneously and frequently require obstetric in-
pulmonary aspiration and difficult tracheal intubation, terventions requiring anaesthetic care. Neuraxial techniques
especially when myotonia is induced by anaesthetic drugs.26 are strongly recommended but may prove to be technically
Known triggers of myotonia are suxamethonium, neostig- challenging because of spinal deformities and surgical hard-
mine, hypothermia and shivering. Reversal of neuromus- ware.22 Opioids should be used cautiously or not at all.
cular block with neostigmine is not recommended, and Women with SMA are likely to require respiratory support in
controlled ventilation should be continued until full the peripartum period in the form of noninvasive ventilation,
both during and after surgery. The merits of tracheal intuba-
tion should be discussed well in advance as the risk of long-
term invasive respiratory support is significant, should the
Table 3 Classification of spinal dysraphisms. Adapted from
patient require intubation and ventilation.
Rossi and colleagues29
General anaesthesia drugs need to be carefully considered
and adjustments in doses and combinations made. Sux-
Open spinal dysraphisms
 Myelomeningocele
amethonium is contraindicated because of reports of pro-
 Myelocele ducing significant hyperkalaemia and death. Non-
 Hemimyelomeningocele depolarising NMBAs produce prolonged and profound
 Hemimyelocele neuromuscular block and should be used with caution.
Closed spinal dysraphisms Sugammadex has the potential to mitigate this issue.10
With subcutaneous mass
Lumbosacral
 Lipomas with dural defect Spinal dysraphisms
o Lipomyelomeningocele
Spinal dysraphisms are a group of congenital malformations
o Lipomyelocele
 Terminal myelomeningocele involving the vertebral arches, spinal cord and meninges.
 Meningocele These are among the most common of birth defects, an inci-
dence which may be as high as 10 in 1000 with isolated bone
Cervicothoracic
 Non-terminal myelocystocele abnormalities being the most common (spina bifida occulta,
 Meningocele which may be considered a normal variant).28 An under-
Without subcutaneous mass
standing of the nature of the lesions and anatomical abnor-
Simple dysraphic states malities is essential to providing safe anaesthesia/analgesia
 Intradural lipoma for parturients with spinal dysraphisms. A classification
 Filar lipoma combining clinical and radiologic findings to describe the
 Tight filum terminale deficits/defects is provided in Table 3.29
 Persistent terminal ventricle
 Dermal sinus
Women with repaired open dysraphisms (to provide
coverage of exposed neural elements) frequently have their
Complex dysraphic syndromes
phylum terminale released at the same time. These patients
 Disorders of midline notochordal integration
o Dorsal enteric fistula
are unlikely to have a patent, or even present, epidural space,
o Neurenteric cysts and their ligamentum flavum may not be present. Thus,
o Diastematomyelia epidural space identification and catheter placement may not
 Disorders of notochordal formation be possible. Spinal anaesthesia above the area of repair should
o Caudal agenesis
be considered only if a recent MRI scan suggests the sub-
o Segmental spinal dysgenesis
arachnoid space could be safely accessed without injuring the
spinal cord. Furthermore, Chiari malformation is common in

BJA Education - Volume 21, Number 6, 2021 215

Anaesthesia and neurological disorders in pregnancy

these patients, often with associated hydrocele, which may MCQs

have been treated with a shunt. Shunt malfunction symptoms
The associated MCQs (to support CME/CPD activity) will be
must be distinguished from those of pre-eclampsia in the
accessible at www.bjaed.org/cme/home by subscribers to BJA
pregnant patient.
Education.
Closed spinal dysraphisms are a group of abnormalities in
which overlying skin is present, but the spinal cord and other
structures are abnormal. Some are associated with the pres-
ence of masses, usually in the lumbosacral area. Most masses
References
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pomas are usually subcutaneous but extend through the bone 1. Knight M, Bunch K, Tuffnell D et al. Saving Lives, Improving
defect into the spinal canal and are often tethered to the Mothers’ Care e Lessons Learned to Inform Maternity Care
spinal cord. The anaesthetist should be aware that these ab- from the UK and Ireland Confidential Enquiries into Maternal
normalities are frequently associated with low-lying spinal Deaths and Morbidity 2015e17. Oxford: National Perinatal
cord and conus medullaris, a tethered spinal cord, or both. Epidemiology Unit, University of Oxford; 2019
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