Anaesthesia and neurological disorders in pregnancy

Abstract

Learning objectives•Describe common neurological conditions encountered in the peripartum period.•Identify the interplay of the physiologic changes of pregnancy with specific neurological disorders.•Explain the anaesthetic implications of common neurological conditions in the peripartum period.Key points•Neurological conditions are the third leading cause of maternal mortality in the UK.•Epilepsy is the most common neurological disorder encountered in pregnancy.•Neuraxial anaesthesia is not contraindicated in patients with multiple sclerosis.•Physiological changes of pregnancy put women at higher risk of acute cerebrovascular disorders and the anaesthetist is best placed to coordinate the management of these in the immediate setting.•Spinal pathology may preclude the use of neuraxial techniques for labour analgesia and anaesthesia. •Describe common neurological conditions encountered in the peripartum period.•Identify the interplay of the physiologic changes of pregnancy with specific neurological disorders.•Explain the anaesthetic implications of common neurological conditions in the peripartum period. •Neurological conditions are the third leading cause of maternal mortality in the UK.•Epilepsy is the most common neurological disorder encountered in pregnancy.•Neuraxial anaesthesia is not contraindicated in patients with multiple sclerosis.•Physiological changes of pregnancy put women at higher risk of acute cerebrovascular disorders and the anaesthetist is best placed to coordinate the management of these in the immediate setting.•Spinal pathology may preclude the use of neuraxial techniques for labour analgesia and anaesthesia. Advances in the medical and surgical management of neurological disorders have improved quality and length of life, and reduced the incidence of severe disabilities. As a consequence, increasing numbers of women of childbearing age have existing neurological diseases. The normal physiological changes of pregnancy, such as alteration in the immune response, a procoagulant state and increased metabolic demands, may affect the progression and status of some neurological disorders. This may complicate what would otherwise be a normal pregnancy and delivery. In the latest Mothers and Babies: Reducing Risk through Audits and Confidential Enquiries across the UK (MBRRACE-UK) report, neurological conditions were found to be the second most common indirect and third overall cause of maternal death in the UK.1Knight M. Bunch K. Tuffnell D. et al.Saving Lives, Improving Mothers’ Care – Lessons Learned to Inform Maternity Care from the UK and Ireland Confidential Enquiries into Maternal Deaths and Morbidity 2015–17. National Perinatal Epidemiology Unit, University of Oxford, Oxford2019Google Scholar This represents a slight trend upwards compared with the previous triennium report.2Kelso A. Wills A. Knight M. on behalf of the MBRRACE-UK neurology chapter writing groupLessons on epilepsy and stroke.in: Knight M. Nair M. Tuffnell D. Shakespeare J. Kenyon S. Kurinczuk J.J. on behalf of MBRRACE-UK. Saving Lives, Improving Mothers’ Care – Lessons Learned to Inform Maternity Care from the UK and Ireland Confidential Enquiries into Maternal Deaths and Morbidity 2013–15. National Perinatology Epidemiology Unit, University of Oxford, Oxford2017: 24-36Google Scholar The increase in mortality from neurological disorders in pregnancy results predominantly from epilepsy and stroke-related causes.1Knight M. Bunch K. Tuffnell D. et al.Saving Lives, Improving Mothers’ Care – Lessons Learned to Inform Maternity Care from the UK and Ireland Confidential Enquiries into Maternal Deaths and Morbidity 2015–17. National Perinatal Epidemiology Unit, University of Oxford, Oxford2019Google Scholar Efforts to reduce and prevent morbidity and mortality associated with neurological conditions should start with planning before conception. Women should be counselled regarding the effects of their condition on pregnancy outcomes and vice versa. Early identification and referral to appropriate specialists helps women's understanding and active participation in the management of both their pregnancy and neurological condition. Obstetric anaesthetists should be involved early to help mitigate peripartum complications and optimise maternal outcomes. A cohesive anaesthetic plan based on shared decision-making with the patient and made well in advance of delivery may alleviate the often highly charged circumstances of the intrapartum period. The anaesthetist must be notified as soon as a woman with a neurological condition presents in labour or for an elective Caesarean delivery. Discussion of any status changes or modification of anaesthetic management, obstetric management, or both should occur at this time. New changes occur after delivery of the baby, including hormonal imbalances, breastfeeding demands, sleep deprivation and other physical and mental demands on a new mother. Some neurological disorders tend to worsen postpartum, especially autoimmune diseases. Newly developed neurological symptoms in the postpartum period need to be thoroughly investigated and promptly acted upon as consequences of delayed management can be disastrous. Epilepsy is the most common serious neurological disorder in pregnant women with an estimated global incidence of 6.85 per 1000 women.3Stephen L.J. Harden C. Tomson T. Brodie M.J. Management of epilepsy in women.Lancet Neurol. 2019; 18: 481-491Abstract Full Text Full Text PDF PubMed Scopus (57) Google Scholar Every year there are approximately 2500 births among women with epilepsy in the UK.2Kelso A. Wills A. Knight M. on behalf of the MBRRACE-UK neurology chapter writing groupLessons on epilepsy and stroke.in: Knight M. Nair M. Tuffnell D. Shakespeare J. Kenyon S. Kurinczuk J.J. on behalf of MBRRACE-UK. Saving Lives, Improving Mothers’ Care – Lessons Learned to Inform Maternity Care from the UK and Ireland Confidential Enquiries into Maternal Deaths and Morbidity 2013–15. National Perinatology Epidemiology Unit, University of Oxford, Oxford2017: 24-36Google Scholar The early input of a specialist in epilepsy is extremely valuable, as the choice of medications and dosing of medications may need to be altered during pregnancy (increasing doses, change of antiepileptic drugs [AEDs] regimen).4Battino D. Tomson T. Bonizzoni E. et al.Seizure control and treatment changes in pregnancy: observations from the EURAP epilepsy pregnancy registry.Epilepsia. 2013; 54: 1621-1627Crossref PubMed Scopus (162) Google Scholar The primary management goals are control of seizures, concerns related to dose-dependent teratogenicity of AEDs, the management of status epilepticus, the prevention of sudden unexplained death in epilepsy (SUDEP) and differentiating epileptic from non-epileptic convulsions (especially eclampsia). The analysis of 3806 pregnancies in the European and International Registry of Antiepileptic Drugs and Pregnancy (EURAP) study, involving women with primarily idiopathic generalised epilepsy, reported that two thirds of women remained seizure-free throughout pregnancy.4Battino D. Tomson T. Bonizzoni E. et al.Seizure control and treatment changes in pregnancy: observations from the EURAP epilepsy pregnancy registry.Epilepsia. 2013; 54: 1621-1627Crossref PubMed Scopus (162) Google Scholar For about 15% of women there was deterioration in seizure control, which was more likely to occur among women with focal epileptic disorders.4Battino D. Tomson T. Bonizzoni E. et al.Seizure control and treatment changes in pregnancy: observations from the EURAP epilepsy pregnancy registry.Epilepsia. 2013; 54: 1621-1627Crossref PubMed Scopus (162) Google Scholar Management of epilepsy in pregnancy with AEDs must balance the need to protect the mother from the detrimental effects of seizure activity against the risk of congenital malformations in the fetus. All AEDs possess some teratogenic potential (Table 1), but growing evidence suggests that monotherapy with certain AEDs such as lamotrigine or levetiracetam may be safer for both fetus and mother.5Bromley R.L. Weston J. Marson A.G. Maternal use of antiepileptic agents during pregnancy and major congenital malformations in children.JAMA. 2017; 318: 1700-1701Crossref PubMed Scopus (32) Google Scholar Patients should be counselled before and in early pregnancy regarding the risks of congenital malformations associated with AEDs as poor understanding of the specific risks may lead to poor adherence with therapy and deterioration in seizure control. Hormonal changes, increased volume of distribution and protein binding of AEDs, hyperemesis gravidarum, insomnia and psychological stress may result in the need of frequent adjustments of therapy to maintain seizure control and prevent SUDEP.6Voinescu P.E. Pennell P.B. Management of epilepsy during pregnancy.Expert Rev Neurother. 2015; 15: 1171-1187Crossref PubMed Scopus (25) Google Scholar Common antiepileptics, dosing adjustment requirements with pregnancy and their teratogenic potential are listed in Table 1.Table 1Antiepileptic drugs, dose adjustment required during pregnancy, and potential for teratogenic effects (adapted from Voinescu and Pennell6Voinescu P.E. Pennell P.B. Management of epilepsy during pregnancy.Expert Rev Neurother. 2015; 15: 1171-1187Crossref PubMed Scopus (25) Google Scholar)Antiepileptic drugDose adjustmentTeratogenic potentialAssociated congenital malformationsPhenobarbitalNeeds monitoring and adjusting6–7% (with dose >150 mg)CardiacOral cleftsPhenytoinMay need increasing2.4–7%CardiacUrogenitalOral cleftsCarbamazepineNo need of adjusting3%MicrocephalyGrowth restrictionValproic acidNeeds monitoring9–10% (increasing with dose)Spina bifidaAtrial septal defectCleft palateHypospadiasPolydactylyCraniosynostosisLower IQOxcarbazepineNeeds adjusting2.5%Autism?LamotrigineNeeds adjusting (mostly higher doses)1.3–4.6%CardiacHypospadiasGabapentinUncertain1.2%UncertainTopiramateNeeds increasing2.4–6.8%CardiacHypospadiasOral cleftsLevetiracetamNeeds increasing1.7%Growth restriction? Open table in a new tab Antiepileptic drugs should be taken regularly throughout pregnancy, especially during labour when an intravenous formulation may be required because of variable intestinal absorption intrapartum. Seizures in the intrapartum period should be treated as eclamptic in origin, especially in the presence of risk factors such as pre-eclampsia or hypertension. Magnesium sulphate is the drug of choice in these circumstances.7Royal College of Obstetricians and Gynaecologists Epilepsy in pregnancy.Green-top Guideline No. June 2016; 68Google Scholar The doses recommended by the National Institute for Health and Care Excellence (NICE) are 4 g i.v. over 5–15 min followed by infusion of 1 g h−1 over 24 h.8National Institute for Health and Care Excellence (NICE)Hypertension in pregnancy: diagnosis and management (NICE guideline 133).https://www.nice.org.uk/guidance/ng133Date accessed: January 3, 2021Google Scholar The American College of Obstetricians and Gynecologists suggest a more liberal dosing regimen – 4–6 g loading dose followed by 1–2 g h−1.9American College of Obstetricians and Gynecologists Gestational hypertension and preeclampsia. ACOG Practice Bulletin No. 222.Obstet Gynecol. 2020; 135: e237-e260Crossref PubMed Scopus (354) Google Scholar Eclamptic seizures are usually short and self-limiting generalised tonic–clonic convulsions. In women with poorly controlled epilepsy, benzodiazepines may need to be given simultaneously to reduce the duration of the seizure and prevent maternal and fetal hypoxia and fetal acidosis. A maternal history of epilepsy should not dictate the mode of delivery. Neuraxial analgesia for labour is recommended to reduce sleep deprivation and labour pain-induced stress and dehydration.7Royal College of Obstetricians and Gynaecologists Epilepsy in pregnancy.Green-top Guideline No. June 2016; 68Google Scholar If opioids are used, pethidine should be avoided because of its epileptogenic potential. General anaesthetic drugs are all acceptable to use in epileptic women, except for etomidate, which may lower the seizure threshold in susceptible individuals. Hepatic metabolism of neuromuscular blocking agents (NMBAs) is increased by the enzyme-inducing effect of AEDs, and this shortens their duration of action.10Hopkins A.N. Alshaeri T. Akst S.A. Berger J.S. Neurologic disease with pregnancy and considerations for the obstetric anesthesiologist.Semin Perinatol. 2014; 38: 359-369Crossref PubMed Scopus (31) Google Scholar Multiple sclerosis (MS) is an immune-mediated, chronic, demyelinating disorder of the central nervous system (CNS) with an immune-mediated aetiology. It is three times more common in women; the mean age at diagnosis is 30 yrs.11Brownlee W.J. Hardy T.A. Fazekas F. Miller D.H. Diagnosis of multiple sclerosis: progress and challenges.Lancet. 2017; 389: 1336-1346Abstract Full Text Full Text PDF PubMed Scopus (265) Google Scholar The overall prevalence in the UK is about 0.1–0.2%.12Kevat D. Mackillop L. Neurological diseases in pregnancy.J R Coll Physicians Edinb. 2013; 43: 49-58Crossref PubMed Scopus (6) Google Scholar The hallmark of the disease is periventricular inflammatory lesions, which eventually cause demyelination and axonal damage. The plaques can be observed anywhere in the CNS, hence the myriad of symptoms in the clinical presentation.11Brownlee W.J. Hardy T.A. Fazekas F. Miller D.H. Diagnosis of multiple sclerosis: progress and challenges.Lancet. 2017; 389: 1336-1346Abstract Full Text Full Text PDF PubMed Scopus (265) Google Scholar,13Dobson R. Giovannoni G. Multiple sclerosis — a review.Eur J Neurol. 2019; 26: 27-40Crossref PubMed Scopus (457) Google Scholar Symptoms of MS are usually intermittent (relapsing–remitting), but in about 10–15% it will have a slowly progressive course. Women may present with a variety of neurological symptoms, including sensory and motor deficits, bladder dysfunction, visual disturbance, mood changes and cognitive decline. The number of relapses usually reduces during pregnancy, especially in the third trimester, and most women will not require any treatment. It is thought that this effect results from the hormone-induced reduction in cell-mediated immunity during pregnancy, which may also explain the higher incidence of relapses in the first 3 months postpartum.10Hopkins A.N. Alshaeri T. Akst S.A. Berger J.S. Neurologic disease with pregnancy and considerations for the obstetric anesthesiologist.Semin Perinatol. 2014; 38: 359-369Crossref PubMed Scopus (31) Google Scholar,14Confavreux C. Hutchinson M. Hours M.M. Cortinovis-Tourniaire P. Moreau T. Rate of pregnancy-related relapse in multiple sclerosis. Pregnancy in Multiple Sclerosis Group.N Engl J Med. 1998; 339: 285-291Crossref PubMed Scopus (1222) Google Scholar Nevertheless, the anaesthetic antenatal assessment should focus on a thorough history and neurological examination and documentation of existing neurological deficits. It has been suggested in the past that neuraxial techniques, specifically spinal anaesthesia, may contribute to increasing relapses after delivery because the demyelinated spinal cord is exposed to neurotoxic local anaesthetic agents.15Neal J.M. Barrington M.J. Brull R. et al.The second ASRA practice advisory on neurologic complications associated with regional anesthesia and pain medicine: executive summary 2015.Reg Anesth Pain Med. 2015; 40: 401-430Crossref PubMed Scopus (201) Google Scholar However, the Pregnancy in Multiple Sclerosis study (PRIMS) found no correlation between the use of neuraxial techniques and relapses of MS in the postpartum period.14Confavreux C. Hutchinson M. Hours M.M. Cortinovis-Tourniaire P. Moreau T. Rate of pregnancy-related relapse in multiple sclerosis. Pregnancy in Multiple Sclerosis Group.N Engl J Med. 1998; 339: 285-291Crossref PubMed Scopus (1222) Google Scholar Numerous subsequent reports support these findings.16Vukusic S. Hutchinson M. Hours M. et al.Pregnancy and multiple sclerosis (the PRIMS study): clinical predictors of post-partum relapse.Brain. 2004; 127: 1353-1360Crossref PubMed Scopus (443) Google Scholar,17Bove R. Alwan S. Friedman J.M. et al.Management of multiple sclerosis during pregnancy and the reproductive years: a systematic review.Obstet Gynecol. 2014; 124: 1157-1168Crossref PubMed Scopus (82) Google Scholar Extra caution should be taken with the use of NMBAs when general anaesthesia is considered. Hyperkalaemia may occur with depolarising NMBAs because of MS-induced denervation or misuse myopathy. Non-depolarising NMBAs may have prolonged effects secondary to decreased muscle mass. Conversely, reports of resistance to NMBAs have also been reported, perhaps as a result of a proliferation of extra-junctional receptors. Most disease-modifying treatment options (biological agents, monoclonal antibodies, cytostatic drugs) are discontinued before conception and during gestation. Should relapses occur during the antenatal period, they are treated with high doses of steroids, i.v. immunoglobulins or plasma exchange.17Bove R. Alwan S. Friedman J.M. et al.Management of multiple sclerosis during pregnancy and the reproductive years: a systematic review.Obstet Gynecol. 2014; 124: 1157-1168Crossref PubMed Scopus (82) Google Scholar Cerebrovascular disorders cause significant morbidity and mortality amongst patients in the peripartum period. Multidisciplinary expertise is essential during the acute presentation, 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 long-term follow up of patients is also important. The discussion in this section will be limited to acute ischaemic stroke (AIS), cerebral venous sinus thrombosis (CVT), intracranial haemorrhage (ICH), posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) in the peripartum period (Table 2).Table 2Cerebrovascular 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, haemodilutionConditionAISICHCVTRCVSPRESTime of onsetAny time in pregnancy, postpartumAny time in pregnancy, postpartumThird trimester, postpartumAbrupt, postpartumRapid (hours), postpartumClinical featuresFocal neurologyThunderclap headache, loss of consciousness, focal neurologyHeadache, seizures, focal neurologyThunderclap headache, transient focal deficit, seizuresSeizures, dull headache, visual disturbancesImaging featuresCT/MRI angiogram is sensitiveCT evidence of SAH or ICHCT venogram is usually sensitiveUsually normal CTVasogenic oedema in occipito-parietal regionsTreatmentThrombolysis, anticoagulationSupportive, interventional, or surgical clipping/coilingAnticoagulation, seizure controlCalcium channel blockers, triple-H, seizure controlSeizure control, BP control Open table in a new tab Intracranial haemorrhage represents more than half of all pregnancy-related strokes and is a major cause of morbidity and mortality.2Kelso A. Wills A. Knight M. on behalf of the MBRRACE-UK neurology chapter writing groupLessons on epilepsy and stroke.in: Knight M. Nair M. Tuffnell D. Shakespeare J. Kenyon S. Kurinczuk J.J. on behalf of MBRRACE-UK. Saving Lives, Improving Mothers’ Care – Lessons Learned to Inform Maternity Care from the UK and Ireland Confidential Enquiries into Maternal Deaths and Morbidity 2013–15. National Perinatology Epidemiology Unit, University of Oxford, Oxford2017: 24-36Google Scholar,18Miller E.C. Leffert L. Stroke in pregnancy: a focused update.Anesth Analg. 2020; 130: 1085-1096Crossref PubMed Scopus (16) Google Scholar Commonly, subarachnoid haemorrhage (SAH) and ICH in the peripartum period are a result of ruptured cerebral aneurysms or arteriovenous malformation (AVM). Almost half of the women with ICH of any cause have a history of pre-eclampsia, eclampsia or haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.19Shainker S.A. Edlow J.A. O'Brien K. Cerebrovascular emergencies in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2015; 29: 721-731Crossref PubMed Scopus (19) Google Scholar Pregnancy-related physiological changes such as cerebral vasodilation, increased plasma volume and hypertension do not increase the risk of aneurysmal ruptures.19Shainker S.A. Edlow J.A. O'Brien K. Cerebrovascular emergencies in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2015; 29: 721-731Crossref PubMed Scopus (19) Google Scholar Therefore, obstetric and anaesthetic management does not need to be modified in asymptomatic women with known brain aneurysms. If an aneurysm bleeds, early intervention (e.g. clipping or coiling) improves maternal and fetal outcomes and if antenatal treatment is successful obstetric and anaesthetic management during labour is as normal.19Shainker S.A. Edlow J.A. O'Brien K. Cerebrovascular emergencies in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2015; 29: 721-731Crossref PubMed Scopus (19) Google Scholar The risk of AVM haemorrhage is increased during pregnancy (up to 8.1%), requiring more proactive management of women with AVMs. Non-pregnant women who have experienced a bleed from an AVM should undergo an intervention (coiling, clipping, surgery) before becoming pregnant. If AVMs are first discovered during pregnancy the risks of intervention compared with expectant management or voluntary termination of pregnancy should be considered given the significant risk of haemorrhage during pregnancy.20Gross B.A. Du R. Hemorrhage from arteriovenous malformations during pregnancy.Neurosurgery. 2012; 71: 349-356Crossref PubMed Scopus (70) Google Scholar Whatever the cause of ICH, the initial management of the woman should focus on her well-being, airway protection, cardiovascular support and neuroprotection. Should delivery of the fetus become necessary in the acute phase, anaesthetic options may be limited. Reduced level of consciousness, confusion, severe haemodynamic complications (hypo-/hypertension, arrhythmias), or need of neurosurgical intervention may necessitate the use of general anaesthesia with simultaneous stabilisation. Total intravenous anaesthesia may provide smoother control of blood pressure and prevent exacerbation of cerebral oedema. Care should be taken during laryngoscopy and extubation, to prevent spikes in blood pressure. Magnesium sulphate or lidocaine infusions in addition to remifentanil boluses may be useful.18Miller E.C. Leffert L. Stroke in pregnancy: a focused update.Anesth Analg. 2020; 130: 1085-1096Crossref PubMed Scopus (16) Google Scholar If delivery is decided in women who have minor symptoms and are haemodynamically stable and conscious, neuraxial techniques might be considered. Most women with an ICH, even those with minor symptoms, will require management in a critical care unit after delivery. Ischaemic stroke is the rarer form of stroke in pregnancy and is usually related to risk factors such as preeclampsia or eclampsia, hypertension, Caesarean delivery, prothrombotic states (factor V Leiden, sickle cell disease, antiphospholipid syndrome, systemic lupus erythematosus), older age, black ethnicity, greater parity and multiple gestation.19Shainker S.A. Edlow J.A. O'Brien K. Cerebrovascular emergencies in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2015; 29: 721-731Crossref PubMed Scopus (19) Google Scholar Clinical signs of AIC include motor and sensory deficit consistent with an ischaemic vascular lesion, but atypical symptoms, such as headache, nausea, dizziness and visual disturbances, may be present.18Miller E.C. Leffert L. Stroke in pregnancy: a focused update.Anesth Analg. 2020; 130: 1085-1096Crossref PubMed Scopus (16) Google Scholar Treatment focuses on preserving brain tissue by identification of the affected vessel, reperfusion, and prevention of recurrence. Therapy may include thrombolysis, anticoagulation and antiplatelet medications.18Miller E.C. Leffert L. Stroke in pregnancy: a focused update.Anesth Analg. 2020; 130: 1085-1096Crossref PubMed Scopus (16) Google Scholar Thrombolysis in pregnancy is controversial and carries the additional risks of placental abruption, systemic bleeding and fetal loss. Blood pressure control, especially avoidance of hypotension, is paramount for both maternal and fetal outcomes. Obstetric management is usually expectant unless there are difficulties in achieving haemodynamic targets to improve cerebral perfusion, and fetal delivery is judged to be of benefit to the mother. Anaesthetic management must consider the timing of anticoagulant and antiplatelet drug dosing, and the patient's neurological status. Regional anaesthesia remains the preferred technique if it is safe to perform. Neuroprotective strategies, such as elevation of the head, prevention of major swings in arterial pressure, avoiding hypercapnia and hypoxia, and hyperosmolar therapy, are important for maternal outcome.18Miller E.C. Leffert L. Stroke in pregnancy: a focused update.Anesth Analg. 2020; 130: 1085-1096Crossref PubMed Scopus (16) Google Scholar The risk of postpartum haemorrhage may be increased by anticoagulation and antiplatelet therapy, and should be managed aggressively. Intensive care admission should be strongly considered after delivery. Prevention of recurrence of AIS should start as soon as safe, and includes increased physical activity, lifestyle modifications, long-term antiplatelet therapy and treatment of comorbidities.18Miller E.C. Leffert L. Stroke in pregnancy: a focused update.Anesth Analg. 2020; 130: 1085-1096Crossref PubMed Scopus (16) Google Scholar Cerebral venous sinus thrombosis is a rare cause of stroke with a peripartum incidence of 11.6 per 100,000 deliveries.12Kevat D. Mackillop L. Neurological diseases in pregnancy.J R Coll Physicians Edinb. 2013; 43: 49-58Crossref PubMed Scopus (6) Google Scholar Three-quarters of the cases occur in the postpartum period. Pregnancy is usually the sole risk factor for developing CVT, but screening for thrombophilia is recommended should it occur. CVT can present with sudden-onset headaches, seizures or deteriorating level of consciousness and the diagnosis is confirmed by CT scan or MRI. Anticoagulation is the mainstay of therapy, and the prognosis is better than in CVT not related to pregnancy.19Shainker S.A. Edlow J.A. O'Brien K. Cerebrovascular emergencies in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2015; 29: 721-731Crossref PubMed Scopus (19) Google Scholar Reversible cerebral vasoconstriction syndrome, also known as postpartum angiopathy, is a rare condition with unknown prevalence. It is associated with significant morbidity, and this may be attributed to the lack of clear understanding of its pathophysiology and effective treatment. RCVS is most commonly observed in postpartum women with pre-eclampsia and autoimmune disease or in those using recreational drugs. The usual presentation is thunderclap headaches lasting minutes to days and segmental stenosis with post-stenotic dilatation on cerebral angiography (‘string of beads’ appearance). The clinical picture may be varied and non-specific and the syndrome may last 2–3 months. Numerous treatment modalities have been attempted with various efficacy. Maintaining hypertension, hypervolaemia and haemodilution (triple-H therapy) with the addition of a calcium channel blocker has been reported to be successful in the management of more severe cases.19Shainker S.A. Edlow J.A. O'Brien K. Cerebrovascular emergencies in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2015; 29: 721-731Crossref PubMed Scopus (19) Google Scholar The overall aim is to reverse cerebral vasoconstriction to reduce the impact of neurological injury (ICH, AIS), and this is best done in tertiary neurology and neurocritical care units.21Edlow J.A. Caplan L.R. O'Brien K. Tibbles C.D. Diagnosis of acute neurological emergencies in pregnant and post-partum women.Lancet Neurol. 2013; 12: 175-185Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar Most cases of RCVS resolve spontaneously within 2–3 months, but the ones who develop complications risk permanent neurological deficit depending on the size and location of the infarction.21Edlow J.A. Caplan L.R. O'Brien K. Tibbles C.D. Diagnosis of acute neurological emergencies in pregnant and post-partum women.Lancet Neurol. 2013; 12: 175-185Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar PRES is an uncommon condition that may occur without any identifiable risk factors but is more likely to develop in postpartum women with renal disease, pre-eclampsia, sepsis and exposure to immunosuppressant drugs. Symptoms usually develop rapidly without prodromes and include headache, visual disturbances (visual field defects, diplopia, scotomata), seizures and confusion. Brain imaging (usually MRI) reveals focal oedema mainly in the parieto-occipital and cerebellar regions, which is reversible when appropriate treatment is instigated. Many cases of PRES have overlapping features with pre-eclampsia and eclampsia, which makes blood pressure control particularly important. Seizures should be controlled with phenytoin and benzodiazepines, whereas magnesium should be reserved for cases with suspicion of eclamptic aetiology. Maintaining strict fluid balance, correction of electrolyte abnormalities, and removing other possible causes of PRES are some of the recommended treatment goals. Despite its reversible course PRES can cause neurological complication such as