Neurological Illness in Pregnancy E-Book

M. Angela O'Neal

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 658

Veröffentlichungsjahr: 2015

Beschreibung

This book is a practical and easily readable guide for neurologists, obstetricians, and primary care doctors treating female patients with neurological illness in their reproductive years.

• Offers wide ranging coverage, including family planning and lactation
• Presents information in approachable tables and summaries, focusing on high yield information useful for clinical consultation
• Is written by a team of experts and edited by recognized leaders in the field

Leseprobe

This book is dedicated to Autumn Klein’s daughter, Cianna.

Neurological illness in pregnancy

Principles and practice

EDITED BY

Autumn Klein

University of Pittsburgh Medical Center, Pittsburgh, PA, USA

M. Angela O’Neal

Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

Christina Scifres

University of Oklahoma College of Medicine, Oklahoma City, OK, USA

Janet F. R. Waters

Magee Women’s Hospital, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

Jonathan H. Waters

Magee Women’s Hospital, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

This edition first published 2016 © 2016 by John Wiley & Sons, Ltd

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Library of Congress Cataloging-in-Publication Data

Neurological illness in pregnancy : principles and practice / edited by Autumn Klein, M. Angela O'Neal, Christina Scifres, Janet F. R. Waters, Jonathan H. Waters.       p. ; cm.    Includes bibliographical references and index.    ISBN 978-0-470-67043-9 (cloth)    I. Klein, Autumn, editor.   II. O'Neal, M. Angela, editor.   III. Scifres, Christina, editor.

IV. Waters, Janet F. R. (Physician), editor.   V. Waters, Jonathan H., editor.    [DNLM: 1. Nervous System Diseases.   2. Pregnancy Complications.   WQ 240]    RG580.N47    618.3–dc23

2015015026

A catalogue record for this book is available from the British Library.

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

Contents

Notes on contributors

Preface

Chapter 1 The history and examination

Introduction

Past medical history

Medication considerations

Family history and genetics

Habits

Review of systems

Examination

Cases studies

References

Chapter 2 Hormonal and physiologic changes in pregnancy

Embryology

Menstrual cycle

Hormonal changes in pregnancy

Endocrine changes during pregnancy

Physiologic changes in pregnancy

Endocrine control of parturition

Endocrinology and physiology of the postpartum patient

Lactation

References

Chapter 3 Neuroimaging

Introduction

Anatomic and physiologic changes of pregnancy

Imaging the pregnant patient

Neuropathology and associated imaging findings

Wernicke's encephalopathy

Conclusion

References

Chapter 4 Neurologic complications in the obstetrical anesthesia patient

Anesthesia-related injury

Obstetrical birth trauma

Diagnosis and management of birth-trauma-related neuropathy

References

Chapter 5 Headaches during pregnancy and peripartum

Migraine headaches

Treatment of migraines during pregnancy and lactation

Other primary headaches

Secondary headaches

Other secondary headaches

References

Chapter 6 Stroke in pregnancy and the puerperium

Introduction

Physiological changes that affect blood vessels and blood flow

Epidemiology

Pregnancy-related conditions

Ischemic stroke

Hemorrhagic strokes

Safety and use of radiological studies during pregnancy

Maternal and fetal prognosis

References

Chapter 7 Selecting contraception for women treated with antiepileptic drugs

Introduction

Contraceptive use in women treated with AEDs

Contraception methods

Bidirectional interactions of hormonal contraceptives and AEDs

Preconception planning

Summary and conclusions

References

Chapter 8 Epilepsy

Introduction

Fertility

Contraception

Pregnancy

Increased seizure frequency

Complications in the offspring

Individual registries

Low birth weight

Developmental delay

Specific effects of antiepileptic drugs and other interventional therapies

Complications of pregnancy

Breastfeeding

Summary and conclusions

References

Chapter 9 Multiple sclerosis

Epidemiology

Hormonal influences

Susceptibility to develop MS

Prognosis of MS and hormonal factors

Menstrual cycle

Preconception planning

Genetics/inheritance

Fertility

Assisted reproductive techniques

Pregnancy

Disease-modifying therapies and pregnancy

Breastfeeding

Menopause

References

Chapter 10 Neuromuscular disorders

Acquired generalized neuromuscular disorders

Hereditary disorders

General recommendations for pregnancy in neuromuscular disease

References

Chapter 11 Anterior and posterior pituitary disease and pregnancy

Anterior pituitary

Posterior pituitary

References

Chapter 12 Movement disorders and pregnancy

Introduction

Movement disorders presenting during pregnancy

Influence of pregnancy on the signs, symptoms, and treatment of movement disorders

Genetic testing issues during pregnancy

Summary

References

Chapter 13 Brain tumors and pregnancy

Epidemiology of brain tumors in women

Tumors potentially influenced by pregnancy or hormonal changes

Coexistence of pregnancy and malignancy

Treatment associated considerations in the pregnant patient

Surgery

Radiation

Chemotherapy

Delivery considerations and post-partum setting

Impact of cancer treatment on fertility in female pediatric cancer survivors

Impact of cancer treatment on fertility in women of child-bearing age

Hereditary tumor syndromes necessitating prenatal counseling

Summary

Acknowledgements

References

Chapter 14 Neuro-ophthalmology in pregnancy

Introduction

Visual signs and symptoms during pregnancy and postpartum

Specific neuro-ophthalmologic complications of pregnancy and postpartum

Acknowledgements

References

Chapter 15 Neurological infections in pregnancy

Introduction

Acknowledgment

References

Chapter 16 Neurosurgery

Introduction

Intracranial hemorrhage during pregnancy

Trauma

Tumors

Chiari malformations

Conclusion

References

Chapter 17 Sleep disorders

Introduction

Sleep duration quality, and architecture in reproductive women

Primary sleep disorders in reproductive and menopausal women

Conclusions

References

Chapter 18 Neurourology of pregnancy

Introduction

A review of the neurophysiologic control of micturition

Changes to the urinary system during pregnancy

Disorders resulting from parturition

Evaluation and treatment

Conclusions

References

Index

EULA

List of Tables

Chapter 1

Table 1.1

Chapter 3

Table 3.1

Table 3.2

Table 3.3

Table 3.4

Table 3.5

Chapter 4

Table 4.1

Table 4.2

Table 4.3

Chapter 5

Table 5.1

Chapter 6

Table 6.1

Table 6.2

Chapter 7

Table 7.1

Table 7.2

Table 7.3

Chapter 8

Table 8.1

Table 8.2

Table 8.3

Chapter 9

Table 9.1

Chapter 10

Table 10.1

Table 10.2

Chapter 11

Table 11.1

Table 11.2

Table 11.3

Table 11.4

Table 11.5

Table 11.6

Table 11.7

Chapter 13

Table 13.1

Chapter 17

Table 17.1

Table 17.2

Table 17.3

Table 17.4

Table 17.5

Table 17.6

List of Illustrations

Chapter 1

Figure 1.1

The axial

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2

image on the left shows dilatation of the subarachnoid space around the optic nerves. On the right is a sagittal

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image depicting an empty sella.

Chapter 3

Figure 3.1

34 y/o female, 1-week postpartum, with severe headache. Physiologic hypertrophy with a sagittal

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-weighted image demonstrating a homogenous, enlarged pituitary gland with convex superior margin, measuring 1.0 cm in height. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.2

33 y/o peripartum female with acute onset right-sided weakness and difficult speaking. Axial FLAIR through the basal ganglia (a) demonstrates abnormal

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signal within the left basal ganglia and internal capsule; diffuse weighted image (b) also demonstrates increased signal while the corresponding ADC (c) and exponential-ADC (d) maps confirm restriction of water diffusion, signifying acute infarction. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.3

33 y/o female, 10 days postpartum, with persistent headaches. Sagittal

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(a) demonstrates flattening of the pituitary gland and settling of the posterior fossa contents. Axial

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images (b,c) demonstrate an “empty sella” as well as optic nerve tortuosity and circumferential CSF space prominence. Imaging findings of increased intracranial pressure, in combination with a lumbar puncture opening pressure of 45 mm water and papilledema on ophthalmologic examination, were consistent with pseudotumor cerebri. In a different patient, 21 y/o female with pseudotumor cerebri, MRI demonstrates tortuous optic nerves with marked dilatation of the CSF spaces with flattening of the optic disc (d). Reproduced from Reference 2 with permission of Elsevier.

Figure 3.4

31 y/o female, 1 day following cesarean section for arrested labor and chorioamnionitis, with sudden onset headache with rapid deterioration of mental status. Examination revealed fixed and dilated pupils. Non-contrast CT images (a,b) demonstrate a hyperdense, lentiform extra-axial (subdural) collection over the left hemisphere with marked mass effect and midline shift. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.5

30 y/o female with preeclampsia developed acute onset severe headaches following childbirth. Non-contrast CT images (a,b) demonstrate linear sulcal hyperdensity consistent with acute subarachnoid hemorrhage; corroborating MR images demonstrate loss of normal FLAIR CSF suppression (c,d) as well as blooming artifact on susceptibility-weighted images (e,f), all findings of subarachnoid blood products. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.6

45 y/o postpartum female with 9 days of headache. Axial (a) and reconstructed sagittal (b) non-contrast CT images demonstrate a hyperattenuating, expanded sagittal dural venous sinus consistent with acute thrombosis. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.7

22 y/o female, first trimester, presenting with headache. Axial

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MR through the sagittal (a) and transverse (b) sinuses demonstrate a normal flow void. Three-dimensional phase-contrast (non-contrast) MR venography (c–e) confirms normal flow-related signal, signifying patent venous sinuses. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.8

Non-contrast MR with axial proton density (a) and sagittal

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(b,c) MR images demonstrate loss of the expected flow void within the left transverse sinus, compared to the contralateral side; axial susceptibility-weighted images (d–f) demonstrate “blooming” artifact from acute thrombosis of the vein of Galen and transverse sinus with additional blooming within the deep cerebral and small cortical veins, likely from additional thrombosis or extremely slow flow; left occipital parenchymal hemorrhage is also present; axial (g) and sagittal (h)

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-weighted images demonstrate a filling defect with the dural venous sinuses. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.9

Non-contrast MR angiography in a 42 y/o female, 4 weeks pregnant, with acute-onset right arm weakness and numbness. Diffusion-weighted imaging (a), with ADC maps (not shown) confirmed acute infarction; axial

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through the skull base (b,c) demonstrate loss of the expected left internal carotid artery (ICA) flow void, while

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fat-suppressed image through the distal internal carotid (d) demonstrates crescentic hyperintense mural thrombus; maximum-intensity projection (MIP) reformatted image from 2D time-of-flight imaging through the neck (e) demonstrates loss of the expected flow-related signal from the left internal carotid with “string sign;” axial 3D time-of-flight images (f,g) confirm loss of flow-related signal from the left internal carotid; MIP reformatted image (h) demonstrates rapid flame-shaped tapering of the distal ICA. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.10

Non-contrast 2D phase-contrast MR venography with axial (a) and sagittal (b) MIP-reformatted images demonstrate signal gaps (lack of flow-related enhancement) within the sagittal and transverse venous sinuses due to in-plane flow artifact; 2D time-of-flight venography demonstrates flow-related signal within the sagittal sinus (c), however, a signal gap is seen within the right transverse sinus (d). Reproduced from Reference 2 with permission of Elsevier.

Figure 3.11

42 y/o postpartum female, diagnosed with preeclampsia, presenting with dizziness. Axial FLAIR MR image (a) demonstrates the expected dural venous sinus flow voids (open arrow); MIP reformatted images from 2D phase-contrast MR venography through the sagittal (b) and transverse venous sinuses (c,d) demonstrates absent flow-related signal from the right transverse sinus; similar findings on 3D phase-contrast venography with sagittal (e), coronal (f), and axial (g) MIP images, likely due to slow flow within normal a variant; subsequent contrast-enhanced CT venography with axial (h) and MIP reformatted images (i,j) demonstrate a patent sinus. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.12

45 y/o female with 9 day history of a persistent postpartum headache. Sagittal

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MR (a) demonstrates loss of the expected sagittal sinus flow void; axial susceptibility-weighted images (b,c) demonstrate blooming artifact within the sagittal sinus; 3D phase-contrast venography with sagittal (d) and coronal (e) MIP reformatted images demonstrate loss of flow-related signal from the sagittal sinus; axial (f,g), coronal (h), and sagittal (i) post-contrast MR venography demonstrate filling defect within the sagittal sinus, confirming thrombosis. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.13

33 y/o female with acute-onset confusion and tachycardia during labor, cardiovascular collapse ensued and patient became comatose. An emergent cesarean section was performed and the patient was presented for imaging. FLAIR MR images (a–c) demonstrate multiple cortical and subcortical regions of

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signal abnormality across multiple vascular territories; DWI (d) demonstrate confluent restricted diffusion within the posterior left hemisphere with a contralateral punctate focus of signal abnormality—findings concerning for an embolic event; (e) ADC map demonstrates both infarction by decreased pixel values and vasogenic edema by increased pixel values. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.14

35 y/o, G3P2, postpartum day 9 from twin delivery, re-admitted with tonic–clonic seizures. History of hypertension and several days of headache. Axial (a–c) and sagittal (d) FLAIR MR images demonstrate patchy areas of juxtacortical, primarily white matter

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hyperintensity involving the parietal and occipital lobes and cerebellum, typical locations for hypertensive encephalopathy or PRES/PRLS. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.15

36 y/o female, one day following cesarean section at 33 weeks for severe preeclampsia, presenting with cortical blindness. Magnetic resonance imaging confirms findings of hypertensive encephalopathy with axial FLAIR through the lateral ventricles (a) demonstrates abnormal

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signal within the occipital periventricular and juxtacortical white matter, as well as within the left internal capsule; diffusion-weight image (b) demonstrates associated increased signal within the occipital regions, however, the corresponding ADC map (c) reveals facilitated diffusion, as opposed to infarction. A follow-up MRI (d) demonstrates complete signal alteration resolution. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.16

27 y/o male presented with abdominal pain at outside facility. Rapid mental status deterioration necessitated transfer. Axial FLAIR (a–d) demonstrates abnormal increased

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signal within the posterior frontal cortex, medial thalami, periaqueductal gray matter, and floor of the fourth ventricle. Areas of true restricted diffusion (e,f) were confirmed by ADC maps (g). Patient became comatose, was unresponsive to medical management, and died. Postmortem examination demonstrated macrophage-rich infiltration of the periaqueductal brainstem, medial thalamus, hypothalamus, and mammillary bodies consistent with Wernicke's encephalopathy. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.17

76 y/o female with 6 months of blurry vision, bilateral temporal hemianopsia. Axial (a), sagittal (b), and coronal (c) post-contrast

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MR images demonstrate a homogenously enhancing supra-sellar meningioma exerting upward mass effect upon the optic chiasm. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.18

38 y/o female with left facial numbness, facial droop, and jaw pain. Axial

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MR (a), as well as 3D thin-slice gradient echo (b,c) demonstrates a part solid, part cystic mass along the expected course of the left trigeminal nerve, expanding Meckel's cave; post-contrast axial (d) and coronal (e)

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MR images demonstrate homogenous enhancement of the soft tissue component, again filling Meckel's cave, with lack of enhancement within the cystic portion. Biopsy confirmed a trigeminal schwannoma. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.19

Photograph from an endonasal trans-sphenoid sellar dissection (Image courtesy Dr. Juan Fernandez-Miranda MD) demonstrating an opening in the dural reflections covering the pituitary gland, the adjacent cavernous carotid, the abducens nerve laterally, and the lateral-most aspect of the cavernous sinus containing cranial nerves III, IV, V2, and V3. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.20

21 y/o female with multiple left-sided cranial neuropathies, most prominently CN III, worsening during pregnancy. Coronal

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MR (a) demonstrates a hypointense, well-defined, lobulated extra-axial supra-cellar mass. Axial

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MR (b) confirms a hyperintense supra-sellar extra-axial mass that is fairly homogenous in signal, though exerts region mass effect upon the pons and left cavernous sinus structures; post-contrast axial (c) and coronal (d)

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MR imaging demonstrates robust homogenous enhancement. Subsequent biopsy revealed a cavernous hemangioma. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.21

Bell's palsy confirmed by contrast-enhanced

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MR with axial (a) and coronal (b) images demonstrate abnormal, asymmetric enhancement involving the labyrinthine facial nerve segment and geniculate ganglion. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.22

50 y/o female with persistent, progressive disequilibrium, diagnosed with a petroclival meningioma. Axial

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MR image through the level of the internal auditory canal (a) demonstrates a lobulated extra-axial cerebellopontine angle mass compressing the pons; contrast-enhanced

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MR image at the same level (b) demonstrates a homogenously enhancing mass with dural tail, a typical finding of meningioma; 3D gradient echo image (c) demonstrates the masses' proximity to the abducens nerve as well as the facial and vestibulocochlear nerve complex. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.23

23 y/o with known prolactinoma, 25 weeks pregnant, presenting with persistent headaches and new left visual field disturbance. Magnetic resonance examination 10 months prior with post-contrast

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sagittal (a) and coronal (b) imaging demonstrates a lobulated, slightly hypo-enhancing and heterogeneous intra-pituitary lesion with partial encasement of the right cavernous carotid. Non-contrast imaging performed during pregnancy with sagittal (c) and coronal (d)

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MR images demonstrate marked enlargement of the sellar mass with circumferential encasement of the cavernous carotid. A 2-year postpartum MRI demonstrates marked size reduction of the pituitary lesion with less regional mass effect (e,f). Reproduced from Reference 2 with permission of Elsevier.

Figure 3.24

27 y/o female with diabetes insipidus and hypothyroidism. Contrast-enhanced sagittal (a) and coronal (b)

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-weight images demonstrate abnormal enlargement and enhancement of the infundibulum and hypothalamus. Infectious causes were excluded and lumbar puncture was only significant for elevated protein. A presumptive diagnosis of hypophysitis was made and the patient improved with steroid therapy. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.25

35 y/o G2P0 female with hypothyroidism, 31 weeks pregnant, presenting with worsening dizziness. Sagittal

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MR (a) demonstrates an empty or partially empty sella; in a 31-week pregnant female one would expect physiologic hypertrophy;

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(b) and FLAIR MR images (c) also demonstrate an empty sella with a “central dot” representing the non-displaced pituitary infundibulum. Imaging findings are consistent with the clinical diagnosis of Sheehan's. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.26

22 y/o female with history of infrequent and irregular menses and “pituitary tumor” presented to outside facility with headache, subsequently transferred for surgical management. Final pathology revealed a hemorrhagic adenoma. Sagittal (a) and coronal (b) non-contrast

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MR images demonstrate an intrinsically

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hyperintense lesion within the pituitary, consistent with acute blood products; post-contrast

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imaging (c–e) demonstrates appropriately enhancing pituitary parenchymal with a hypo-enhancing rim surrounding the region of hemorrhage presumably relate to the underlying adenoma. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.27

57 y/o male with 6 days of severe headache and third cranial nerve palsy. Axial non-enhanced CT (a) demonstrates hyperattenuating sellar soft tissue consistent with acute blood products. Axial pre-contrast

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(b),

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(c), and FLAIR (d) MR images demonstrate mixed-signal sellar material; axial susceptibility-weighted gradient echo image (e) demonstrates sellar blooming artifact; constellation of findings consistent with acute blood products. Post-contrast sagittal (f) and coronal (g)

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MR images demonstrate marked pituitary enlargement with heterogeneous enhancement; note mass effect upon the optic chiasm. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.28

30 y/o male with “pituitary tumor” presenting with severe headaches. Axial

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(a) and FLAIR (b) MR images through the sella demonstrate a fluid–fluid level within the sella with

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hyperintense fluid layering non-dependently, consistent with acute hemorrhage; sagittal pre- (c) and post-contrast (d)

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MR images demonstrate intrinsically

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-hyperintense non-dependent hemorrhagic fluid; note a normally enhancing pituitary gland displaced posteriorly. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.29

50 y/o female with migraines and progressive lower extremity weakness. Non-contrast sagittal (a) and contrast-enhanced sagittal (b) and axial (c)

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MR images demonstrate abnormal nerve root enhancement consistent with Guillain–Barre syndrome. Reproduced from Reference 2 with permission of Elsevier.

Figure 3.30

30 y/o female, 5 days postpartum, with persistent, severe headaches. Sagittal

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MR image (a) demonstrates subtle settling of the posterior fossa structures; axial

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images through the basal cisterns demonstrate effacement and crowding (b,c); axial

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(d) demonstrates thin, bi-hemispheric extra-axial CSF-equivalent collections; axial (e) and coronal (f) post-contrast

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MR images demonstrate smooth pachymeningeal enhancement. Constellation of findings are consistent with intracranial hypotension. Reproduced from Reference 2 with permission of Elsevier.

Chapter 6

Figure 6.1

Illustrative case posterior reversible encephalopathy syndrome. FLAIR MR sequence with axial cuts. Arrows indicate vasogenic edema predominantly affecting the posterior cerebral regions with sparing of the paramedian occipital cortex.

Figure 6.2

Illustrative case of reversible cerebral vasoconstriction syndrome. Sagital CT angiogram (maximum intensity projection images). Arrows indicate segments of constriction and dilatation (“beading”) of the left posterior inferior cerebellar artery.

Chapter 7

Figure 7.1

Progestin-releasing intrauterine device, containing levonorgestrel. Courtesy of Association of Reproductive Health Professionals (AHRP). Reproduced under the Creative Commons License: http://creativecommons.org/licenses/by/3.0/

Figure 7.2

Copper T 380A Intrauterine Device. Courtesy of Association of Reproductive Health Professionals (AHRP). Reproduced under the Creative Commons License: http://creativecommons.org/licenses/by/3.0/

Figure 7.3

The contraceptive implant elutes etonogestrel. Courtesy of Association of Reproductive Health Professionals (AHRP). Reproduced under the Creative Commons License: http://creativecommons.org/licenses/by/3.0/

Chapter 9

Figure 9.1

Relapse during pregnancy and postpartum.

Chapter 11

Figure 11.1

Coronal and sagittal MRI scans of an intrasellar prolactin-secreting macroadenoma in a woman prior to conception (above) and at 7 months of gestation (below). Note the marked tumor enlargement at the latter point, at which time the patient was complaining of headaches. Reproduced from Reference 17 with permission of Elsevier.

Chapter 13

Figure 13.1

(a) T1 axial post contrast MRI showing an enhancing meningioma in the left cavernous sinus (white arrow). (b) T1 axial post contrast MRI 5 years later showing enlargement of the meningioma (white arrow).

Vignette 1:

A 47-year-old peri-menopausal woman presented to her primary care physician with pain in her left eye. A brain MRI with contrast revealed a meningioma in the left cavernous sinus (Figure 13.1a). Given the challenging surgical location, unknown growth rate of the meningioma, and mild symptoms that were not necessarily related to the meningioma, follow-up MRI was recommended. Unfortunately, the patient failed to follow-up until approximately 5 years later when she presented with progressive diplopia and was found to have a third nerve palsy. Repeat brain MRI showed an increase in the size of the meningioma (Figure 13.1b). Debulking surgery was recommended given the expansion of the meningioma close to the optic chiasm and pressure on surrounding normal brain. Although this growth happened during menopause, an association is hard to prove.

Figure 13.2

(a) FLAIR MRI showing no evidence of tumor. (b) FLAIR MRI 3 months later showing new hyperintensity in the right frontal lobe with significant mass effect and brain shift consistent with progressive glioma as shown by the white arrow. The patient was pregnant so did not receive gadolinium contrast.

Vignette 2:

A 38-year-old woman with a known low-grade (WHO grade II) astrocytoma was being followed with surveillance MRIs (Figure 13.2a) when she presented with a seizure. She was 3 months pregnant at the time so a non-contrast MRI was done and revealed significant disease progression (Figure 13.2b). Given the degree of mass effect, she underwent a subtotal resection of the recurrence, and the pathology was consistent with a glioblastoma (WHO grade IV). She received involved field radiation without the standard chemotherapy regimen because of her pregnancy. At approximately 32 weeks, she had a successful caesarian section and delivered a healthy baby. Chemotherapy was started after delivery.

Chapter 14

Figure 14.1

Worsening of diabetic retinopathy during pregnancy. Retinal hemorrhages (straight white arrows) and cotton wool spots (white arrowheads) are present in both eyes. The right eye (left panel) also has retinal exudates nasal to the optic disc (gray arrow). The left eye (right panel) has neovascularization on the optic nerve (curved arrow).

Figure 14.2

Hypertensive retinopathy. There is optic disc edema, blood vessel tortuosity, and pre-retinal hemorrhage (arrow).

Figure 14.3

Cerebral venous sinus thrombosis in the immediate postpartum period. (a) Magnetic resonance venogram with contrast showing a filling defect consistent with thrombosis from the torcula to the mid left transverse sinus (arrow). (b) Severe bilateral papilledema secondary to raised intracranial pressure from venous sinus thrombosis. Both optic discs are elevated and there are bilateral hemorrhages and exudates.

Figure 14.4

Moderate bilateral papilledema in idiopathic intracranial hypertension. The disc margins are blurred and elevated, and the vessels are obscured.

Figure 14.5

Goldmann visual fields showing severe peripheral visual field constriction due to idiopathic intracranial hypertension.

Figure 14.6

Pituitary apoplexy in the postpartum period (Sheehan syndrome). (a) Coronal T1 MRI with gadolinium shows a sellar/suprasellar mass with peripheral rim enhancement. The optic chiasm is draped over the mass lesion (arrow). This patient presented with acute onset of headache, diplopia, and visual loss. (b) Automated visual field (24–2 SITA standard) showing a bitemporal hemianopia worse in the right eye (the dark areas are not seen).

Figure 14.7

Posterior reversible encephalopathy syndrome in a patient with preeclampsia and cerebral blindness. Axial T2 MRI reveals hyperintense areas in the occipital lobes bilaterally (arrows). The patient's vision was normal within a few days after delivery and three weeks later, the MRI abnormalities had resolved.

Figure 14.8

Valsalva retinopathy in the right eye. There is a large boat-shaped pre-retinal hemorrhage obscuring the macula (arrow).

Chapter 15

Figure 15.1

Tuberculous meningitis. (a) Axial T1-weighted post contrast MRI demonstrating multiple nodular enhancing regions (arrows) throughout the cerebral hemisphere. (b, c) Axial T2 FLAIR MRI demonstrating diffuse sulcal and cisternal hyperintensities, respectively (dashed arrows).

Figure 15.2

Neurocysticercosis. Axial T2 FLAIR image demonstrates cystic lesion with scolex in the left Sylvian fissure (arrow).

Chapter 16

Figure 16.1

(a) Noncontrast head CT showing large right cerebellar hematoma from a ruptured arteriovenous malformation (AVM). (b) CT angiogram (CTA) demonstrating an AVM with feeders from the posterior inferior cerebellar artery.

Figure 16.2

(a) Noncontrast head CT showing parietal ICH from a ruptured arteriovenous malformation (AVM). Cerebral angiography ((b) anteroposterior and (c) lateral views) shows an AVM with a feeding artery from the angular branch and drainage into the superior sagittal sinus. (d) Postoperative angiogram showing complete resection.

Chapter 18

Figure 18.1

Peripheral innervation of the female lower urinary tract. Reproduced from Reference 1 with permission of Elsevier.

Figure 18.2

The role of the autonomic and somatic nervous systems in bladder filling and storage, as well as voiding. Reproduced from Reference 1 with permission of Elsevier.

Guide

Cover

Table of Contents

Preface

Pages

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Notes on contributors

Valérie BiousseProfessor of Ophthalmology and Neurology Emory University School of Medicine Atlanta, GA, USA

Yvette M. BordelonAssociate Clinical Professor of Neurology David Geffen School of Medicine at the University of California, Los Angeles Los Angeles, CA, USA

Benjamin M. BruckerAssistant Professor of Urology New York University School of Medicine New York, NY, USA

Anil CanDoctoral Research Fellow Department of Neurosurgery Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Louis R. CaplanProfessor of Neurology Beth Israel Deaconess Medical Center Harvard Medical School Boston, MA, USA

Kathy ChuangClinical Fellow in Neuromuscular Medicine Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Anne DavisAssociate Professor of Obstetrics and Gynecology Columbia University Medical Center New York, NY, USA

William T. DelfyettAssistant Professor of Neuroradiology University of Pittsburgh School of Medicine Pittsburgh, PA, USA

Rose DuAssociate Professor of Neurosurgery Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Kimberly L. FerranteAssistant Professor of Obstetrics and Gynecology New York University School of Medicine New York, NY, USA

David T. FetzerAssistant Professor of Radiology University of Texas Southwestern Medical Center Dallas, TX, USA

Nancy Foldvary-SchaeferProfessor of Neurology Cleveland Clinic Lerner College of Medicine Cleveland, OH, USA

Elizabeth R. GerstnerAssistant Professor of Neurology Massachusetts General Hospital Harvard Medical School Boston, MA, USA

Diogo C. HaussenAssistant Professor of Neurology Emory University School of Medicine Atlanta, GA, USA

Aiden HaghikiaScientific Assistant Department of Neuroanatomy and Molecular Brain Research Ruhr University Bochum, Germany

Kerstin HellwigSenior Consultant Department of Neurology St. Josef Hospital Ruhr University Bochum, Germany

Sally IbrahimAssistant Professor of Medicine Cleveland Clinic Sleep Disorder Center Cleveland Clinic Lerner College of Medicine Cleveland, OH, USA

Linda P. KelleyFellow, Neuro-ophthalmology Emory University School of Medicine Atlanta, GA, USA

Jennifer L. LyonsAssistant Professor of Neurology Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Olajide KoweConsultant Anesthesiologist Yorktown Regional Hospital Saskatchewan, Canada

Mark E. MolitchMartha Leland Sherwin Professor of Medicine Division of Endocrinology, Metabolism and Molecular Medicine Northwestern University Feinberg School of Medicine Chicago, IL, USA

Shibani S. MukerjiClinical Fellow in Neurology Massachusetts General Hospital Harvard Medical School Boston, MA, USA

Page B. PennellAssociate Professor of Neurology Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Sathiji NageshwaranDivision of Brain Sciences University College London School of Medicine London, UK

Nancy J. NewmanProfessor of Ophthalmology, Neurology and Neurological Surgery Emory University School of Medicine Atlanta, GA, USA

Victor W. NittiProfessor of Urology and Obstetrics and Gynecology New York University School of Medicine New York, NY, USA

M. Angela O'NealInstructor in Neurology Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Mohammad Kian SalajeghehAssistant Professor of Neurology Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Soma SenguptaInstructor, Division of Neuro-Oncology Beth Israel Deaconess Medical Center Harvard Medical School Boston, MA, USA

Huma SheikhInstructor in Neurology Faulkner Headache Division Brigham and Women's Hospital Harvard Medical School Boston, MA, USA

Marsha SmithDepartment of Neurology Southern Ohio Medical Center Portsmouth, OH, USA

Janet F. R. WatersClinical Assistant Professor in Neurology Magee Women's Hospital University of Pittsburgh Medical Center Pittsburgh, PA, USA

Jonathan H. WatersProfessor, Department of Anesthesiology and Bioengineering Magee Women's Hospital University of Pittsburgh Medical Center Pittsburgh, PA, USA

Judith M. WongPediatric Fellow Department of Neurosurgery University of California Los Angeles Los Angeles, CA, USA

Mark S. YerbyAssociate Clinical Professor of Public Health North Pacific Epilepsy Research Oregon Health Sciences University Portland, OR, USA

Preface

Dr. Autumn Klein

The creation of this textbook was initiated by Dr. Autumn Klein, a pioneer of women's neurology. She developed the format and carefully chose the authors from an elite group of specialists from across the United States and abroad. She unexpectedly passed away on April 17, 2013, before the completion of the book. In her memory, the authors of the book chose to complete the textbook as a legacy to her passion for the field of women's neurology.

Dr. Klein began her education by studying gender and neuroscience at Amherst College where she earned her BA magna cum laude. She went on to obtain a PhD in neuroscience and an MD from Boston University School of Medicine. After an internship in internal medicine at Brown University, she completed her residency in the Harvard Neurology Residency Program at Brigham and Women's Hospital and Massachusetts General Hospital, where she served as chief resident in her final year.

She completed a fellowship in clinical neurophysiology and epilepsy and then went on to establish the Division of Women's Neurology at Brigham and Women's Hospital. She subsequently moved to Pittsburgh where she founded the Division of Women's Neurology within the Departments of Neurology and Obstetrics at the University of Pittsburgh. From its inception, Dr. Klein served as the chief of this unique subspecialty of neurology. The division served and continues to serve as an interdisciplinary program bridging neurology with obstetrics, gynecology, and women's medicine. It focuses on gender differences in medical evaluation, diagnosis, and implementation of treatment and care. In addition to the creation of this division, she created an epilepsy monitoring unit for the treatment of pregnant women with epilepsy.

Autumn is fondly remembered for her self-less devotion to the patients for whom she cared. She made herself available for consultation on obstetrical patients 24/7. It was always reassuring to have her respond to an unexpected neurologic event. She educated her patients about their neurologic disease and about what to expect during pregnancy and motherhood. She collaborated extensively with obstetricians, anesthesiologists, and epilepsy staff to provide comprehensive patient care. With this first edition of Neurological Illness in Pregnancy, we hope that Autumn's vision will be fulfilled and that it will create a legacy that carries on for generations to come.

M. Angela O'Neal Christina Scifres

Janet F. R. Waters Jonathan H. Waters

CHAPTER 1The history and examination

Mary A. O'Neal

Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA

Introduction

The focus of this chapter will be on the information most helpful to understand, counsel, and treat female neurology patients in their reproductive years. The key elements of the neurologic history and examination will be systematically reviewed with emphasis on gender differences. It will conclude with a few clinical cases. The goal is to enable neurologists to develop the knowledge and skills to maximize care for their female patients with regard to family planning and pregnancy. The objective of this chapter is to help physicians to perform a history and examination that focuses on and identifies the specific family planning concerns of the female patient and how these concerns relate to their neurologic disease.

Many common neurological diseases preferentially affect young women. How we, as neurologists, approach treatment depends on our patients' needs at that point in her life cycle. This is different for each disease process.

Migraine is a very common disorder with lifetime prevalence in women of up to 25%. Because of hormonal influences, the ratio of affected women over men is 3:1 [*1]. The history should include usual triggers, of which menses and ovulation are common. Birth control pills (BCPs) have a variable influence on migraine frequency and in some women may aggravate the disorder [*2]. However, many women with menstrual headaches report that cycle suppression (which can be obtained using the subdermal implant, injectable contraception, a pill, patch, or ring) improves their symptoms. The type of migraine is important when discussing contraception. Women with classic migraines should be counseled to avoid estrogen-containing contraceptives (e.g., the pill, patch, or ring), given the increased risk of ischemic stroke. However, common migraine does not preclude use of estrogen-containing contraceptives unless associated with other cerebrovascular risk factors such as an underlying hypercoaguable state. [*3] Furthermore, when choosing medications (abortive or prophylactic), you should take into account, whether the woman are trying to get pregnant, or, if not trying to conceive, what birth control they are utilizing. For instance, topiramate in doses above 200 mg/day may reduce the effectiveness of oral contraceptives [*4]. Does the patient have regular menses? Could she have polycystic ovarian syndrome? If so, Valproate would not be a good choice as a prophylactic medication [*5]. Another concern with patients already predisposed to obesity is that many prophylactic medications can contribute to weight gain.

Multiple sclerosis is another example of a neurologic disease that affects women in their childbearing years [*6]. Many of these patients are on an immunomodulatory medication. Interferons are pregnancy class C, copaxone pregnancy class B, and methotrexate a pregnancy class D medication. Because immunomodulatory medications are not recommended during pregnancy, birth control should be discussed if the woman is not planning pregnancy. What should we recommend to our patients who would like to become pregnant? [*7] They should discontinue their immunomodulatory medication when they discontinue their hormonal or intrauterine contraceptive, as the only contraceptive that typically delays return to fertility is depot medroxyprogesterone acetate. They should be counseled that pregnancy does not worsen overall MS disability [*8] Treatment needs to be appropriately adjusted to best address our patient's needs at each particular point in her life cycle.

Past medical history

Patients' medical background allows us to frame a more accurate diagnosis for their current complaints. The disorders from which women suffer are different from those that affect men. A woman's reproductive desire adds an additional layer of complexity. The medical illnesses more common in women influence which is the most probable neurologic disorder. The following is a brief snap shot of some of the disorders that are more prevalent or occur exclusively in women and how that shapes their care.

The psychiatric problems of depression, anxiety, and borderline personality disorder are more frequent in women [9, 10]. Thus, their neurological problems may be a consequence of somatization, conversion, or have an overlay due to these conditions. These women are at risk for over testing and noncompliance, as well as poor maternal weight gain, poor infant bonding, substance abuse, and postpartum depression [11, 12]. The medications we choose need to take these factors into account.

Autoimmune disorders also affect women more frequently. They may have neurologic complications directly due to their rheumatologic problem, like lupus flares [13, 14]. In addition, their neurologic problem may be due to a result of an underlying hypercoaguable state or as a consequence of their immunosuppressant medications. The recommendations and concerns for these women during pregnancy are highly specialized.

Cardiovascular concerns are important though they are uncommon in premenopausal women. Although estrogen before the menopausal transition is likely protective against cardiovascular disease, we cannot ignore women with a strong family history of vascular disease especially if associated with other risk factors such as tobacco use and migraines [*15]. These women do suffer from cardiovascular complications and need advice about risk factor modification. Women with congenital or acquired cardiac disease will need specialized care to appropriately manage the physiologic changes that occur during pregnancy and labor.

An obstetrical and lactation history is extraordinarily important. The number of pregnancies, the gestational stage of the current pregnancy, and the history of either planned or spontaneous abortions predict which obstetrical and neurological diseases are most likely. These factors also determine how, if necessary, to image and what medications are appropriate. For instance, the association with antiphospholipid antibody syndrome and spontaneous miscarriages is well established [*16]. A history of eclampsia should be sought. There is good evidence that prior eclampsia predicts eclampsia in future pregnancies as well as increases risk of future maternal hypertension [17, 18]. Other obstetrical issues such as preterm premature rupture of membranes and placenta previa should be asked about directly as these patients are predisposed for recurrence in future pregnancies [*19]. A woman with a history of recurrent fetal loss needs an obstetrical referral to help planning/monitoring in future pregnancies.

Bone health is often neglected. The medications we choose should reflect this concern [*20]. In addition, many neurologic patients' disability may limit weight bearing [*21]. It is important to be aware of the effects of medication on bone health; as the long-term use of many medications increase the risk of osteoporosis [*22] (see Table 1.1). Examples of commonly used medications that promote bone loss include the anticonvulsants, phenytoin, and carbamazepine. Counseling about the benefits of exercise as well as recommending daily calcium and vitamin D intake is helpful to avoid these complications.

Table 1.1 Drugs that may have adverse effects on bone metabolism

Anticoagulants

   Warfarin

Cyclosporine

Steroids

Medroxyprogesterone acetate

Vitamin A and synthetic retinoids

Loop diuretics

Antiepileptic drugs

   Phenytoin

   Carbamazepine

   Phenobarbital

Chemotherapeutic drugs

   Aromatase inhibitors

   Methotrexate

   Ifosfamide

   Imatinib

Proton pump inhibitors

Antidepressants

   Selective serotonin reuptake inhibitors (SSRIs)

   Tricyclics

Thiazolidinediones

Antiretroviral therapy

A history of an underlying hypercoaguable disorder is an extremely important historical data in pregnancy planning. During pregnancy there is an increase in factors I, II, VII, VIII, IX, and X as well as a decrease in protein S. The net result is that normal pregnancy is a hypercoaguable state. If a woman has a preexisting hypercoaguable disorder, her chance of having a clotting complication is high and anticoagulation during her pregnancy should be recommended [*23].

Surgeries such as those involving the lower spine may make epidural anesthesia more challenging or complicated. For example, a lumbar peritoneal shunt depending on the location may preclude an epidural. Other patients with severe scoliosis, obesity, or lumbar fusion may make neuroaxial anesthesia challenging. A personal or family history of anesthetic complications is an additional historical piece to be obtained. A prior history of postdural headache should be inquired about as this increases a patient risk for recurrence [*24]. Anything that would make the patient at risk for anesthesia should warrant an early consult to an obstetrical anesthesiologist.

Medication considerations

Contraception is a topic that neurologists tend to neglect. It is important to provide patients with recommendations on which contraception options are most appropriate. The most effective contraceptives are the subdermal implant and intrauterine contraceptives, which have been estimated to be 20 times as effective as oral contraceptives and surgical sterilization. There is a myriad of contraceptive choices and they are generally chosen due to personal preference, efficacy, and safety. In our patients, efficacy may be affected by medication interactions (e.g., topiramate) or at times disability. For instance, young women whose disability involves the spinal cord may not be good candidates for certain barrier methods of contraception due to difficulty in positioning or with peroneal sensory loss. In other women, certain types of contraception are contraindicated by safety concerns. For women who have had a stroke, significant cardiovascular risk factors, an underlying hypercoaguable state, and migraine with aura, combined estrogen-containing pills, patch, or ring are not recommended [*3]. However, progestin-only methods including the subdermal implant, intrauterine contraceptive, the injectable contraceptive, or progestin-only pills (e.g., Micronor) are safe.

When choosing to prescribe medications to women of childbearing age, it is important counsel the patient on risks and benefits of treatment. We are prescribing medications to young women who may or may not be planning on pregnancy at the time of consultation. Knowledge of the pregnancy class of the medication prescribed and what that means to your patient is essential in order to counsel them on the risks of taking that medication during pregnancy and what to do with the medication if they get pregnant. The Mother to Baby website (www.mothertobaby.org) and hotline is a useful source for information on medication use during pregnancy; free information on medication use during lactation is available from Lactmed (http://toxnet.nlm.nih.gov/cgi-bin/sis/htmlgen?LACT). Is there any influence of the medication on their method of birth control? Should they be taking higher doses of folate (e.g., 5 mg/day)? The potential effect of in vitro fertilization on the underlying neurological disease may need to be discussed especially for women with migraines [*25]. The effects of medication on the long-term gender-specific health issues such as weight and bone health should also be considered.

The issues around fertility are complex. What are the potential risks of fertility treatment? Are there alternatives? The available options for these women require discussion, planning, and individualization for best care. A number of medications may affect fertility. The mechanisms are diverse, but weight gain is the most common. Additional weight contributes to the metabolic syndrome and polycystic ovarian syndrome. In addition, to weight gain, valproate may also influence androgen levels making contraception more problematic [*26]. It has also been associated with neural tube defects. Therefore, if there is a reasonable alternative medication that controls the neurological disorder in women of childbearing age that would be preferred.

Family history and genetics

Family history is a critical component to the history. It tells us which disease processes are likely, so we can appropriately screen and counsel patients to minimize risk. Women with a strong family history of coronary artery disease, hypertension, or diabetes need to be made aware of increased risk of vascular disease associated with obesity, sedentary life style, smoking, and estrogen. Pregnancy may add to risk factors.

Do they have a genetic disorder? What is the mode of inheritance? Is there a reliable genetic test? These are important factors to help women make informed decisions about pregnancy. The more information the patients have the better equipped they are to make appropriate choices. It is a mistake to assume a patient understands the disease, simply because it runs in their family. For example, a young woman presented to clinic with a family history of maternal Huntington's disease. Her mother had tested negative. She did not understand that she was not at risk for inheriting the disorder. Knowledge is a powerful tool.

Habits

The habits (good and bad) that women employ before and during pregnancy affect their cardiovascular risk. During pregnancy, these risks are magnified. Moreover, it is hard to over emphasize the benefits of exercise on managing stress, weight, depression, and sleep. The effects on heart and brain health are well documented. Barriers for routine monitoring including health screening like mammograms and Pap smears need to be recognized. These may be cultural, socioeconomic, or driven by the patient's disability. For example, consider a patient with multiple sclerosis who attempted to obtain a gynecologic examination. If she is paraplegic, she may be unable to transfer onto her internist's examination table.

Case 1

A 24-year-old woman comes in for evaluation of her headaches. She has two kinds of headaches. The first is a daily constant aching headache worst at the end of the day. It is aggravated by stress and is associated with bilateral neck pain. This headache has been present for past 1 year, but worse over the last several months. She takes acetaminophen or ibuprofen about six tablets of either everyday for this headache.

The second headache is hemicranial throbbing and much more severe. She has nausea, rare vomiting, and light sensitivity with this headache. She denies any other symptoms. During this headache she has to lie down. The frequency varies from one to three times a month. It occurs always 1–2 days prior to her menses. She has noted that red wine can trigger it. The headache lasts usually 1 day. These headaches began in her teens. Her mother and sister have similar headaches. She has never been treated for her headaches.

Her past medical and surgical history is unremarkable.

Medications include: acetaminophen prn, ibuprofen prn

She drinks four to six caffeinated beverages a day.

She recently stopped her oral contraceptives as she wants to become pregnant.

Pertinent social history is that she is engaged to be married in 2 months. They are planning on starting a family as soon as possible. She has no history of tobacco use, alcohol use, or illicit drugs, but she does not exercise on a regular basis.

Her family history is positive for migraine in mother and sister. In addition, her mother and an aunt had breast cancer.

On review of systems she endorses the following: She has lost ten pounds over the last 2 months. She has regular menses and normal breasts; she has not had an obstetrics and gynecology (OB/GYN) examination for 2 years. Her sleep is poor with difficulty falling asleep. She says she has been quite anxious about her upcoming wedding.

The history is consistent with a diagnosis of chronic daily headaches of the tension type and common migraine. Her triggers, factors that are provoking her headaches, and her desire to conceive all frame the therapeutic approach. In this case, education about analgesic rebound, overuse of caffeine, and poor sleep will need to be addressed. Stress management including a regular exercise program and its importance and relation to headache and sleep. She needs education on which medications are considered safe in pregnancy and the importance for women with a family history of breast cancer to have regular examinations. Prophylactic medication is less appropriate as she is actively trying to conceive. Abortive therapy may be an option, providing the medication is not contraindicated during pregnancy, it is also important to avoid the potential pitfall of analgesic rebound.

Review of systems

A review of systems there should include special attention to a number of issues for female patients. Are they at their ideal weight? Has there been weight loss or gain? Their weight influences their risks, as well as what medications should be chosen or avoided. Obesity increases the risk for gestational diabetes, hypertension, and eclampsia [27–29]. Irregular menses may indicate an underlying hormonal imbalance, influence fertility, and determine which medications are most appropriately employed. Menorrhagia and iron deficiency are common problems in young women that can be effectively treated with use of the levonorgestrel-containing intrauterine system. Iron deficiency is often compounded during pregnancy exacerbating conditions such as restless leg syndrome. Breast masses or discharge are key elements/components that deserve to direct query of our female patients.

Examination

Gender does not affect the neurologic examination. The areas on which to focus are determined by the patient's history. The history of the present illness, medical background, and physiologic state of the woman allows the physician to generate a list of the most likely possibilities. The findings on examination help to narrow and/or confirm this differential. The most important elements of the medical examination include blood pressure and weight. The other necessary pieces of the medical examination are patient-specific and dependent on the history.

Cases studies

Below are vignettes that demonstrate the importance of a gender-based history and how this will guide a therapeutic approach. These cases are based on actual patients.

Case 2

A 28-year-old woman gravida 7 para 3, 24 weeks pregnant with chronic hypertension comes in for evaluation of headaches. The headache began 3 weeks ago. They were initially intermittent, but over the last 10 days they have become constant. They are worse in the morning or if she coughs. She has no clear relieving factors. Her blood pressure has not been well controlled with systolic pressures recorded as high as 220. Her labetalol was increased and her blood pressures have improved. They have been running around 140–150/90. Over the last week, she has been having blurred vision and worsening headache. Over the last 2 days, she has noted some double vision with the images side by side. The diplopia is worse when she looks far away and while watching television. She denies any other neurologic problems. She does not usually suffer from headaches. She underwent a 24-hour urine protein which was normal, suggesting that preeclampsia was not the source of her headache. CBC including platelet count was normal. Liver enzymes were also normal. She had a brain MRI and MR venogram 10 days ago which were normal.

Her past medical history is remarkable for hypertension, renal stone, history of requiring a nephrostomy tube during her last pregnancy, and a history of herpes simplex virus

She is taking multivitamins and labetalol only.

Her review of systems is notable for weight gain of 30 pounds since the start of the pregnancy. She has no diabetes and no sleep problems.

The history is concerning for elevated intracranial pressure causing a sixth nerve palsy in the second trimester of pregnancy with a 30 lb. weight gain. Of note is that she had recent normal imaging. She had gained a significant amount of weight which makes idiopathic intracranial hypertension (IIH) a concern. The hypercoaguability associated with pregnancy causing cerebral venous thrombosis is also in this differential, but less likely given her normal venogram. Neurologic consequences of hypertension such as stroke (ischemic or hemorrhagic) are unlikely given her history and normal imaging studies. Posterior reversible encephalopathy syndrome would also be less likely given her history of worsening headache with improved blood pressure control. On examination, her blood pressure was 120/90. Her weight was 205 lbs. and height 61 inches. The neurological examination confirmed a left sixth nerve palsy and papilledema. A repeat brain MRI and MR venogram was done. The MR venogram was normal. The repeat brain MRI showed dilatation of the subarachnoid space around the optic nerve sheath a finding seen in IIH (Figure 1.1) Her CSF opening pressure was 550 mm. The testing confirms a diagnosis of IIH.

These cases illustrate how to individualize the history in order to consider gender differences and allow us to better treat our female patients. It is important to anticipate our patient's risks for disease based on their genetic makeup, lifestyle choices, and preexisting medical conditions. In this setting, clinicians must also be aware of the patient's desires for conception present and future. This will allow our patients to achieve their life goals with minimal health risk.

Figure 1.1 The axial T2 image on the left shows dilatation of the subarachnoid space around the optic nerves. On the right is a sagittal T1 image depicting an empty sella.

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