Neurology and Pregnancy: Pathophysiology and Patient Care (Volume 171) (Handbook of Clinical Neurology, Volume 171)

Neurology and Pregnancy provides a comprehensive multidisciplinary guide to best practices for research and practicing neurologists, as well as obstetricians and other specialists caring for women with an acute or chronic neurological disorder. The book encompasses preconception care, genetic counseling, pregnancy in patients with chronic neurological disorders, and acute pregnancy-related neurological complications. Postpartum care and complications, including lactation concerns, are also addressed, as well as the long-term effects of pregnancy and its complications on maternal brain health. Vol 1 summarizes the complex neurophysiological changes in pregnancy from a basic and translational science perspective. This includes neuroimaging, principles of neuro-obstetric critical care, and ethical and medicolegal concerns, describes normal fetal cerebral development and summarizes the management of the most common prenatal neurological diagnoses. Vol 2 focuses on chronic neurological conditions in pregnancy such as epilepsy, migraine, and multiple sclerosis, as well as acute neurological disorders including preeclampsia/ eclampsia, ischemic and hemorrhagic stroke, and CNS infections. Each chapter introduction includes data on epidemiology, when applicable. In discussing management, comments of safety profiles of medications preconceptionally, during pregnancy and lactation are summarized. Each chapter includes 1-2 illustrative cases. Discusses neuroendocrine, brain, PNS, and cerebro-vascular changes in pregnancy Includes neurological care of the pregnant patient Covers neuroimaging and monitoring techniques Reviews common neurological complications with pregnancy Supplies illustrative case study examples Cover Series Page Title Page Copyright Copyright Handbook of Clinical Neurology 3rd Series Available titles Foreword Foreword Preface Preface Contributors Contributors 1 Neuroendocrine mechanisms of reproduction Introduction The Role of the Gonadotropin-Releasing Hormone (GnRH) ``Pulse Generator ́ ́ in Initiation and Biosynthesis and action of GnRH Neuroanatomy of GnRH pulse generator Neuromodulation of the GnRH pulse generator Neuroendocrinology of Puberty Neuroendocrinology of the Menstrual Cycle Follicular phase Midcycle LH surge Luteal phase Functional Hypothalamic Amenorrhea/Anovulation Pathophysiology Behavioral contributors Differential diagnosis Behavioral management Medical management Fertility treatment in FHA Polycystic Ovary Syndrome Pathophysiology Behavioral contributors Differential diagnosis Behavioral management Functional Hypothalamic Hypogonadism (FHH) in Men Pathophysiology Behavioral contributors Differential diagnosis Behavioral management Medical management The Neuroendocrinology of Pregnancy Summary References 2 Neurophysiological and cognitive changes in pregnancy Introduction Changes in Hormones During Pregnancy Steroid hormones Prolactin and placental lactogen Changes associated with parturition Neurophysiological Changes in the Maternal Brain Oxytocin Tuberoinfundibular dopamine neurons and prolactin Adaptive Changes in Central Control of Maternal Physiology Central adaptations of maternal energy homeostasis Central modulation of body temperature and neuroimmune responses Suppression of the stress response Adaptive Changes in Behavior Maternal behavior Hormonal regulation of maternal behavior Maternal aggression Cognitive Changes Summary and Concluding Remarks References 3 Adaptations in autonomic nervous system regulation in normal and hypertensive pregnancy Introduction Longitudinal changes in hemodynamics and hormones Pregnancy increases SNA Mechanisms of increased basal SNA during pregnancy Brain sites and neurocircuitry underlying increased basal sympathoexcitation during pregnancy Potential factors that activate central sympathetic regulatory regions during pregnancy Gonadal steroids Metabolic hormones: Insulin and leptin Volume-retaining hormones (RAAS and vasopressin) Pregnancy Impairs Baroreflex Control of HR and SNA Potential mechanisms for attenuated baroreflex function in pregnancy Afferent limb Descending pathways Medullary baroreflex pathway Increased inhibition in the RVLM Potential role of neurosteroid metabolite of progesterone Potential mechanisms by which pregnancy increases basal SNA and induces sympathoexcitation, yet impairs responses to barorecep Other reflexes altered in pregnancy Atrial volume reflex Arterial chemoreflex Preeclampsia Elicits Further Increases in SNA Preeclampsia and sympathetic overactivity Potential mechanisms for sympathetic overactivity in preeclampsia The sympathetic baroreflex RAAS AT1R-AA Inflammation The endothelin system The natriuretic peptide system Summary and Future Directions Acknowledgments References 4 Physiology of the cerebrovascular adaptation to pregnancy Introduction Vasomotor Responses to Circulating Factors The Cerebral Endothelium and BBB CBF Autoregulation and Hemodynamics Function and Structure of the Cerebrovasculature Conclusions References 5 Neurobiology of maternal mental illness Introduction Neuroplasticity in Pregnancy Neuroendocrinology and Immune Changes in Pregnancy Perinatal Depression Perinatal Anxiety and OCD Postpartum Psychosis and Bipolar Disorder Trauma and Posttraumatic Stress Disorder Discussion References 6 Epidemiology of neurologic disease in pregnancy Cerebrovascular Disease Ischemic stroke Hemorrhagic stroke Preeclampsia/eclampsia Vascular malformations and aneurysms Subarachnoid hemorrhage Cerebral venous sinus thrombosis Posterior reversible encephalopathy syndrome Reversible cerebral vasoconstriction syndrome Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Moyamoya disease Dissection Epilepsy Demyelinating Diseases Multiple sclerosis Neuromyelitis optica Acute disseminated encephalomyelitis Progressive multifocal leukoencephalopathy Central pontine myelinolysis Neuromuscular Disease Neuromuscular junction disorders Myasthenia gravis Lambert-Eaton myasthenic syndrome Botulism Motor neuron diseases Amyotrophic lateral sclerosis Spinal muscular atrophy Acquired muscle conditions Inflammatory muscle disease Hereditary neuromuscular disorders Inherited myopathies Myotonias Myotonic dystrophy Nondystrophic myotonias Muscular dystrophy Limb girdle muscular dystrophy Hereditary peripheral neuropathies Charcot-Marie-Tooth disease Hereditary neuropathy with liability to pressure palsies Peripheral neuropathies Postpartum neuropathy Femoral neuropathy Obturator neuropathy Lumbosacral plexopathy Carpal tunnel syndrome Neuralgic amyotrophy Meralgia paresthetica Lumbosacral radiculopathy Other neuropathies Diabetic neuropathy Neuropathy due to nutritional deficiency Autoimmune neuropathies Acute inflammatory demyelinating polyneuropathy Chronic inflammatory demyelinating polyneuropathy Multifocal motor neuropathy Headache Primary headache disorder Migraine Tension-type headache Trigeminal autonomic cephalalgias Secondary headache disorders Spontaneous intracranial hypotension Idiopathic intracranial hypertension Movement Disorders Parkinson's disease Chorea Chorea gravidarum Wilson disease Sydenham chorea Myoclonus Ataxia Tourette syndrome Dystonia Stiff person syndrome Cranial neuropathies Bell's palsy Ophthalmologic Disorders Neuro-Oncology Pituitary tumors Meningioma Glioma Choriocarcinoma Hemangiomas Paraneoplastic disorders Sleep Disorders Sleep-disordered breathing Obstructive sleep apnea Central sleep apnea Narcolepsy Restless leg syndrome Insomnia Parasomnias Neurologic Infectious Disease Bacterial meningitis Fungal meningitis Toxoplasmosis Viral encephalitis Varicella-zoster virus Cytomegalovirus West nile virus Brain abscess Infectious myelopathy Zika virus Neurologic Intensive Care Irreversible brain injury Traumatic brain injury Spinal cord injury Neurologic Complications of Systemic Disease Sarcoidosis Systemic lupus erythematosus Psychiatric Disease Conclusion References Further reading 7 Neuro-obstetrics: A multidisciplinary approach to care of women with neurologic disease Introduction Preconception Care: Special Considerations General lifestyle advice for women prior to and during pregnancy Alcohol Tobacco Caffeine Illicit drugs Preconception risk factor assessment Specialist Individual Preconception Care Coordination of care Cerebrovascular disease Management considerations for obstetricians Hypertension Management considerations for neurologists Management considerations for anesthesiologists Epilepsy Management considerations for obstetricians Management considerations for neurologists Management considerations for anesthesiologists Demyelinating diseases Management considerations for obstetricians Management considerations for neurologists Management considerations for anesthesiologists Neuromuscular diseases Management considerations for obstetricians Myasthenia gravis Myotonic dystrophy Management considerations for neurologists Myasthenia gravis Myotonic dystrophy Management considerations for anesthesiologists Myasthenia gravis Myotonic dystrophy Headache Management considerations for obstetricians Migraine Idiopathic intracranial hypertension (IIH) Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vascular syndrome (RCVS) Cerebral venous sinus thrombosis (CVST) Management considerations for neurologists Migraine Idiopathic intracranial hypertension (IIH) Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vascular syndrome (RCVS) Neuro-oncology Management considerations for obstetricians Management considerations for neurologists Management considerations for anesthesiologists Conclusion References 8 Perinatal pharmacology and safety profiles Introduction: Perinatal Pharmacology Reflects Perinatal Physiology Maternal Pharmacokinetics Placental Transfer and Fetal Safety Lactation and Newborn Safety How to Improve the Current Setting Knowledge implementation: Translation of knowledge into practice and guidance Knowledge generation: Structured data collection and integration Acknowledgments and conflict•of interest References 9 Neuroimaging and radiation exposure in pregnancy Introduction Safety of Neuroimaging in Pregnancy and Lactation Computed tomography (CT) Deterministic effects Stochastic effects As low as reasonably achievable (ALARA) principle Magnetic resonance imaging (MRI) Safety of contrast-enhanced imaging During pregnancy During lactation Radiographic Characteristics of Neurologic Conditions Associated With Pregnancy Cerebrovascular complications Ischemic stroke Amniotic fluid embolism Cerebral venous thrombosis (CVT) Subarachnoid hemorrhage (SAH) Preeclampsia, eclampsia, and HELLP syndrome Posterior reversible encephalopathy syndrome (PRES) Postpartum angiopathy (PPA)/reversible cerebral vasoconstriction syndrome (RCVS) Demyelinating diseases of the central nervous system Multiple sclerosis (MS) Neuromyelitis optica spectrum disorders (NMOSD) Neoplasms Meningiomas Gestational trophoblastic disease (Choriocarcinoma) Pituitary disorders Pituitary adenomas Pituitary apoplexy (PA) Sheehan syndrome (SS) Lymphocytic hypophysitis (LH) Back pain Epidural processes Others Epilepsy Intracranial hypotension Wernicke's encephalopathy Summary References 10 Neuro-anesthesiology in pregnancy Introduction Basic Considerations Related to the Provision of Anesthesia in the Obstetric Patient Neuraxial anesthesia General anesthesia Chronic Neurologic Conditions and Anesthetic Management of the Pregnant Patient Requiring Surgery Epilepsy Multiple sclerosis Myasthenia gravis Acute Neurologic Diseases That May Require Anesthetic Care in Pregnant Women Lumbar disc herniation Spine surgery Neuraxial labor analgesia and cesarean delivery anesthesia Guillain-Barre syndrome Intracranial mass lesions Neurosurgery (craniotomy) Neuraxial labor analgesia and cesarean delivery anesthesia Intracranial vascular pathology Neurosurgery (craniotomy/embolization) Neuraxial labor analgesia and cesarean delivery anesthesia Stroke Neuraxial labor analgesia or cesarean delivery anesthesia Traumatic brain injury Fetal and Neonatal Considerations in the Pregnant Patient Undergoing Neurosurgery Conclusion References 11 Neurocritical care of the pregnant patient Introduction General Consideration for Neurocritical Care in the Pregnant Patient Surgical considerations Timing Positioning Diagnostic imaging Fetal monitoring Acute episodes Surgical management Antenatal steroids Ventilation considerations Patient location Delivery planning Hypertensive Disorders of Pregnancy: Preeclampsia, Eclampsia, HELLP Syndrome Epidemiology Diagnosis Management Stabilization of blood pressure Management/prevention of eclampsia Delivery considerations Intracranial Neoplasms Epidemiology Diagnosis Management Medical management Surgical management Delivery considerations Noneclamptic Seizures Epidemiology Diagnosis Management Supportive care Treatment Cerebrovascular Disorders Epidemiology Diagnosis Management General management Ischemic stroke Cerebral venous thrombosis Hemorrhagic stroke Ventriculoperitoneal Shunt Malfunction Epidemiology Diagnosis Management Shunt management Delivery considerations Conclusions References 12 Neurologic complications of medical conditions in pregnancy Sickle Cell Disease Neurologic manifestations Preconception counseling Effects of pregnancy on maternal SCD Effects of SCD on pregnancy outcomes Management of SCD preconception and during pregnancy Antiphospholipid Antibody Syndrome Etiology of the obstetric complications Effect of APLS on pregnancy outcomes Effects of pregnancy on neurologic complications Management in pregnancy Cardiac Valve Disease Neurologic manifestations Medications Preconception management of women with an MHV HIV Infection General principles Treatment-naïve women Women on antiretroviral treatment Mode of delivery and infant prophylaxis Neurologic complications Pregnancy outcomes Connective Tissue Disorders Systemic Lupus Erythematosus Diagnosis and sex-specific concerns Preconception management Neurologic and psychiatric manifestations Psychosis Stroke Seizures Headache Other neurologic manifestations Effects of pregnancy on maternal SLE Effects of SLE on pregnancy outcomes Preconception planning Fibromuscular dysplasia (FMD) Neurologic manifestations Summary References 13 The ethics of neurologically complicated pregnancies Introduction Ethical Theories The four principles Autonomy Beneficence and nonmaleficence Justice Casuistry Feminist ethics and ethics of care Virtue ethics Four-quadrant approach Bioethics of pregnancy Application of Ethical Frameworks at the Interface of Obstetrics and Neurology Deciding for others Determination of capacity and competence Surrogate decision-makers and advance directives Standards for making proxy decisions Substituted judgment Best interests Advance directives in pregnancy Reproductive Decisions in Intellectually Disabled Pregnant Women Background Sterilization and abortion for women with cognitive impairment Case study Treatment of Neurologic Conditions in Pregnancy: Shared Decision-Making Balancing benefits and burdens of treatment options in neurologic conditions impacting maternal and fetal health Background Shared decision-making Brain Death and Disorders of Consciousness in Pregnancy Medical facts Clarifying the medical facts: Disorder of consciousness and brain death How should decision-making proceed? Gestational age and fetal condition The Ethics Committee References 14 The developing brain by trimester The Developing Brain per11Abbreviations used in the chapter are listed at the end of the chapter before References section. Tr Germinal Matrix (GM) Germinal matrix development Germinal matrix hemorrhage (GMH) Antenatal cystic matrix regression (germinolysis, subependymal pseudocyst) Development of Arteries Vasculogenesis Phases of arterial development Intrinsic brain vessels Development of Veins Initial venous development (Streeter, 1915; Padget, 1956) Other developmental findings have clinical relevance Primary Gyration Normal development Abnormal development Gyrification theories and relation to the perinatal period Development of White Matter (WM) Cerebral mantle layers Major forebrain connections Development of Cerebellum Cerebellar structure and function Cerebellar development Cerebellar malformations Genetic defects of neurogenesis in cerebellar germinal zones Genetic defects in migration, synaptogenesis, and connectivity Prenatal cerebellar disruption Prenatal cerebellar hemorrhage Congenital cerebellar infection Fluid Production and Ventricular Size CSF space development Choroid plexus development Fetal ventriculomegaly (see also Chapter 15 of this volume) Definition Imaging aspects Diagnostic testing References 15 Diagnosis and management of congenital neurologic disease during pregnancy Fetal Ventriculomegaly Midline Abnormalities Abnormalities of the Corpus Callosum Holoprosencephaly Septo-Optic Dysplasia Agenesis of the Cavum Septum Pellucidum Posterior Fossa Abnormalities Dandy-Walker Malformation (DWM) Mega Cisterna Magna (MCM) Vermian Hypoplasia (VH)/Dysgenesis Blake's Pouch Cyst (BPC) Cerebellar Hypoplasia Joubert Syndrome (JS) Rhombencephalosynapsis (RS) Kinked Brainstem Neural Tube Defects (NTDs) Anencephaly and Craniorachischisis Myelomeningocele, Myelocele, and Meningocele Encephalocele Tuberous Sclerosis Complex (TSC) Schizencephaly Periventricular Nodular Heterotopia (PNH) Brain Tumors Fetal Stroke Conclusion References 16 Perinatal stroke Introduction Gestational Cerebrovascular Development Neonatal Cerebrovascular Development and Autoregulation of Cerebral Blood Flow Classification of Perinatal Stroke Perinatal arterial ischemic stroke (PAIS) Clinical presentation Diagnosis Further evaluation of the neonate with PAIS Etiology and pathogenesis Management and treatment Prognosis Cerebral sinovenous thrombosis (CSVT) Presentation Diagnosis Management and treatment Prognosis Hemorrhagic stroke Clinical presentation and diagnosis Etiology and pathogenesis Management and treatment Prognosis Risk Factors for Perinatal Stroke Maternal/Obstetric factors Intrapartum events Neonatal factors Prothrombotic factors Conclusion References Index Index A B C D E F G H I J K L M N O P R S T U V W X Z