Neurological Disorders due to Systemic Disease E-Book

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

Seitenzahl: 826

Veröffentlichungsjahr: 2012

Beschreibung

How do you identify which neurological syndromes occur due to systemic disease? 

Neurological problems commonly occur in the context of underlying systemic disease, and may even be the presenting symptom of a medical condition that has not yet been diagnosed. Consequently neurologists need to be aware when a neurological presentation might indicate an underlying systemic disorder.

 Neurologic Disorders due to Systemic Disease provides the tools you need to make these connections. The unique Neurologic presentations-based approach relates to the common clinical situations you encounter, including: 

• Headache
• Stroke
• Movement disorders
• Neuromuscular disorders
• Encephalopathies, Seizures, Myelopathies, Neuro-Ophthalmologic and Neuro-otologic disorders, Sleep disorders, and others

 Major categories of systemic illness are explored for each presentation to guide you towards a likely cause.  These include: 

• Endocrine, electrolyte, and metabolic disorders
• Systemic autoimmune disorders
• Organ dysfunction and failure, and critical medical illness
• Systemic cancer and paraneoplastic disorders
• Systemic infectious disease
• Complications due to drugs and alcohol
•  Vitamin and mineral deficiencies

 Written by a leading cast of experts, with a practical approach including ‘things to remember’ for each presentation, Neurologic Disorders due to Systemic Disease should be on every neurologist’s desk.

Leseprobe

Contents

Cover

Title Page

Copyright

Dedication

List of Contributors

Preface

Chapter 1: Introduction

Chapter 2: Headache due to Systemic Disease

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure (See Also Organ Transplantation)

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Transplantation

Complications of Critical Medical Illness

Drugs, Toxins (Including Alcohol), and Vitamin and Mineral Deficiencies

References

Chapter 3: Encephalopathy (Delirium) due to Systemic Disease

Encephalopathy due to Endocrine Disorders

Encephalopathy due to Systemic Autoimmune Disorders

Encephalopathy due to Organ Dysfunction and Failure

Encephalopathy due to Systemic Cancer

Encephalopathy due to Systemic Infectious Diseases

Encephalopathy due to Transplantation

Encephalopathy due to Critical Medical Illness

Encephalopathy due to Drugs, Nutritional Deficiencies, and Vitamin Deficiencies

Conclusion

References

Chapter 4: Dementia and Systemic Disorders

Introduction

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Transplantation

Complications of Critical Medical Illness

Drugs, Toxins (Including Alcohol), and Vitamin and Mineral Deficiencies

Conclusion

References

Chapter 5: Stroke due to Systemic Diseases

Introduction

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Organ Transplantation

Complications of Critical Medical Illness

Drugs, Toxins, Alcohol, and Vitamin and Mineral Deficiencies

References

Chapter 6: Seizures due to Systemic Disease

Introduction

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Seizures as a Complication due to Transplantation and its Medicines

Seizures as a Complication of Critical Medical Illness—Status Epilepticus

Drugs, Toxins (Including Alcohol), and Vitamin and Mineral Deficiencies

Conclusion

References

Chapter 7: Neuro-Ophthalmology of Systemic Disease

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications of Critical Medical Illness

Drugs, Toxins (Including Alcohol), and Vitamin and Mineral Deficiencies

References

Chapter 8: Neuro-Otology of Systemic Disease

Endocrine Disease

Hyperlipidemia and Hyperviscosity Syndromes

Metabolic Disorders

Mitochondrial Cytopathies

Systemic Autoimmune Disorders

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Disease

Complications of Transplantation

Drugs and Toxins

References

Chapter 9: Movement Disorders due to Systemic Disease

Introduction

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Transplantation

Complications of Critical Medical Illness

Drugs, Toxins, and Vitamin and Mineral Deficiencies

Acknowledgment

References

Chapter 10: Myelopathies due to Systemic Disease

Introduction

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Drugs, Toxins, and Vitamin and Mineral Deficiencies

Conclusion

References

Chapter 11: Peripheral Nerve Disorders in Systemic Disease

Endocrine Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Transplantation

Complications of Critical Medical Illness

Drugs, Toxins, and Vitamin and Mineral Deficiencies

References

Chapter 12: Neuromuscular Junction Disorders due to Systemic Disease

Introduction

Endocrine Disorders

Systemic Autoimmune Disorders

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Drugs and Toxins

References

Chapter 13: Myopathies due to Systemic Disease

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Transplantation

Complications of Critical Medical Illness

Drugs, Toxins (Including Alcohol), and Vitamin and Mineral Deficiencies

References

Chapter 14: Autonomic Manifestations of Systemic Disease

Introduction

Autonomic Testing

Anatomy of the Autonomic Nervous System

Diabetic Autonomic Neuropathy

Amyloidosis

Rheumatologic Disorders

Sarcoidosis

Metabolic Disorders

Paraneoplastic Autonomic Syndromes

Infectious Diseases

Drug-Induced Autonomic Neuropathy

Alcohol

Secreting Neoplasms Mimicking Autonomic Disorders

Conclusion

References

Chapter 15: Sleep Disorders and Systemic Disease

Endocrine Disorders

Electrolyte and Other Metabolic Disorders

Systemic Autoimmune Disorders

Organ Dysfunction and Failure

Systemic Cancer and Paraneoplastic Disorders

Systemic Infectious Diseases

Complications due to Transplantation

Complications of Critical Medical Illness

Drugs, Toxins (Including Alcohol), and Vitamin and Mineral Deficiencies

References

Index

This edition first published 2013 © 2013 by Blackwell Publishing Ltd.

Blackwell Publishing was acquired by John Wiley & Sons in February 2007. Blackwell's publishing program has been merged with Wiley's global Scientific, Technical and Medical business to form Wiley-Blackwell.

Registered office: John Wiley & Sons, Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK

Editorial offices: 9600 Garsington Road, Oxford, OX4 2DQ, UK

The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK

111 River Street, Hoboken, NJ 07030-5774, USA

For details of our global editorial offices, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell

The right of the author to be identified as the author of this work has been asserted in accordance with the UK Copyright, Designs and Patents Act 1988.

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher.

Designations used by companies to distinguish their products are often claimed as trademarks. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book. This publication is designed to provide accurate and authoritative information in regard to the subject matter covered. It is sold on the understanding that the publisher is not engaged in rendering professional services. If professional advice or other expert assistance is required, the services of a competent professional should be sought.

The contents of this work are intended to further general scientific research, understanding, and discussion only and are not intended and should not be relied upon as recommending or promoting a specific method, diagnosis, or treatment by physicians for any particular patient. The publisher and the author make no representations or warranties with respect to the accuracy or completeness of the contents of this work and specifically disclaim all warranties, including without limitation any implied warranties of fitness for a particular purpose. In view of ongoing research, equipment modifications, changes in governmental regulations, and the constant flow of information relating to the use of medicines, equipment, and devices, the reader is urged to review and evaluate the information provided in the package insert or instructions for each medicine, equipment, or device for, among other things, any changes in the instructions or indication of usage and for added warnings and precautions. Readers should consult with a specialist where appropriate. The fact that an organization or Website is referred to in this work as a citation and/or a potential source of further information does not mean that the author or the publisher endorses the information the organization or Website may provide or recommendations it may make. Further, readers should be aware that Internet Websites listed in this work may have changed or disappeared between when this work was written and when it is read. No warranty may be created or extended by any promotional statements for this work. Neither the publisher nor the author shall be liable for any damages arising herefrom.

Library of Congress Cataloging-in-Publication Data

Neurological disorders due to systemic disease / edited by Steven L. Lewis.

p. ; cm.

Includes bibliographical references and index.

ISBN 978-1-4443-3557-6 (hardback : alk. paper)

I. Lewis, Steven L.

[DNLM: 1. Nervous System Diseases–etiology. 2. Diagnosis, Differential. 3. Neurologic Manifestations. WL 141]

616.8′0471–dc23

2012014818

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.

Cover images © iStockphoto.com: Left: ‘Human body with internal organs’ © Max Delson Martins Santos. Right: ‘Neuron Energy’ ©ktsimage.

Cover Design: Sarah Dickinson

To my sons David, Michael, Adam, and Elliot, who teach me so much.

List of Contributors

Brandon R. Barton, MD, MS

Assistant Professor

Department of Neurological Sciences

Movement Disorder Section

Rush University Medical Center

Chicago, IL, USA

Eduardo E. Benarroch, MD, FAAN

Professor

Department of Neurology

Mayo Clinic

Rochester, MN, USA

José Biller, M.D. FACP, FAAN, FAHA

Professor and Chairman

Department of Neurology

Loyola University Chicago

Stritch School of Medicine

Maywood, IL, USA

Hannah R. Briemberg, MD, FRCPC

Clinical Assistant Professor

Department of Medicine

Division of Neurology

University of British Columbia

Vancouver BC, Canada

Ted M. Burns, MD

Professor

Department of Neurology

University of Virginia

Charlottesville, VA, USA

Terry D. Fife, MD, FAAN

Director, Balance Disorders & Otoneurology

Barrow Neurological Institute

Professor of Neurology

University of Arizona College of Medicine

Phoenix, AZ, USA

Christopher G. Goetz, MD, FAAN

Professor

Department of Neurological Sciences

Head, Movement Disorder Section

Rush University Medical Center

Chicago, IL, USA

Brent P. Goodman, MD

Assistant Professor

Department of Neurology

Mayo Clinic Arizona

Phoenix, AZ, USA

Matthew T. Hoerth, MD

Assistant Professor

Department of Neurology

Mayo Clinic Arizona

Phoenix, AZ, USA

Jaffar Khan, MD, FAAN

Associate Professor and Vice Chair for Education

Department of Neurology

Emory University School of Medicine

Atlanta, GA, USA

Kevin A. Kahn, MD

Director, Clinical Care Center

Carolina Headache Institute

Clinical Associate Professor

Department of Psychiatry

Adjunct Associate Professor

Department of Anesthesiology

UNC School of Medicine

Adjunct Clinical Associate Professor

UNC School of Dentistry

Chapel Hill, NC, USA

Brendan J. Kelley, MD

Assistant Professor

Department of Neurology

University of Cincinnati

Cincinnati, OH, USA

Steven Lewis, MD, FAAN

Professor and Associate Chairman

Head, Section of General Neurology

Rush University Medical Center

Chicago, IL, USA

Michelle L. Mauermann, MD

Senior Associate Consultant

Assistant Professor of Neurology

Mayo Clinic

Rochester, MN, USA

Jennifer R. Molano, MD

Assistant Professor

Department of Neurology

University of Cincinnati

Cincinnati, OH, USA

Sarkis Morales-Vidal, MD

Assistant Professor

Director of Telemedicine

Department of Neurology

Loyola University Chicago

Stritch School of Medicine

Maywood, IL, USA

Sital V. Patel, MD

Fellow

Rush University Medical Center

Chicago, IL, USA

Janet C. Rucker, MD

Associate Professor

Departments of Neurology and Ophthalmology

Mount Sinai Medical Center

New York, NY, USA

Erik K. St Louis, MD

Consultant, Center for Sleep Medicine

Division of Pulmonary and Critical Care Medicine

Departments of Medicine and Neurology

Mayo Clinic and Foundation

Associate Professor of Neurology

Mayo Clinic

Rochester, MN, USA

Joseph I. Sirven, MD, FAAN

Professor and Chairman

Department of Neurology

Mayo Clinic Arizona

Phoenix, AZ, USA

Matthew J. Thurtell, MBBS, FRACP

Assistant Professor

Department of Ophthalmology and Visual Sciences

University of Iowa

Departments of Ophthalmology and Neurology

Iowa City Veterans Affairs Medical Center

Iowa City, IA, USA

Allison Weathers, MD

Assistant Professor

Department of Neurological Sciences

Rush University Medical Center

Chicago, IL, USA

Preface

The aim of this book is to provide an overview of the clinical presentation, pathophysiology, diagnosis, and management of the various neurological syndromes that occur in the clinical context of an underlying systemic disease or its treatment.

Although written primarily with the neurologist (generalist neurologist, subspecialist neurologist, or neurologic trainee) in mind, the material in this book should also be accessible and of interest to internal medicine and other primary care physicians, internal medicine subspecialists, and medical students. It is my hope that both neurologist and non-neurologist readers of this text will find that the unique neurologic-syndrome-based approach in the following pages will provide clinically useful insight into the wide variety of neurological disorders that occur due to systemic disease, and provide practical clinical clues to the neurological, and the underlying systemic, diagnosis and management of these patients.

I would like to thank the neurology residents and my general neurology attending colleagues at Rush University Medical Center for creating a stimulating clinical and academic milieu in our daily “work” in the inpatient and outpatient diagnosis and management of the many patients with neurologic disorders from systemic disease. Special gratitude goes to our patients with these disorders for entrusting their care to us.

I would also like to thank my publishers at Wiley-Blackwell, in particular Martin Sugden, PhD, for getting this book off the ground, and to Julie Elliott and Rebecca Huxley, for their expertise in seeing the project through to completion. Finally, a very special thank you to my wife, Julie, for all of her support.

Steven L. Lewis, M.D.Chicago, IllinoisSeptember, 2012

1

Introduction

Steven L. Lewis

Rush University Medical Center, Chicago, IL, USA

Neurological problems commonly occur in the context of an underlying systemic disease, and these neurologic presentations are a frequent source of inpatient and outpatient neurological consultation. In many patients, the neurological disorder is a manifestation of a previously diagnosed systemic illness or its treatment, but in still many others the neurological disorder is the presenting manifestation of a medical condition that has not yet been diagnosed. The aim of this book is to provide the physician with an overview of the clinical presentation, pathophysiology, diagnosis, and treatment of the various neurological syndromes that occur in the setting of systemic illnesses.

In this book, “systemic disease” and “medical disease” are used interchangeably, and refer to the kind of disease or syndrome in which the primary dysfunction involves an organ or system other than the nervous system, with the nervous system disorder occurring as a secondary—though in many cases, a potentially major—consequence. This book, therefore, focuses mostly on the neurological illnesses that occur in the setting of those primary illnesses that are typically considered to be under the purview of general internal medicine or its subspecialties. In addition, this book discusses the neurological complications that occur due to medications and other therapies typically used to treat these systemic illnesses. Conversely, this book does not focus on diseases—such as many genetic disorders—with multisystem manifestations that include both neurological and systemic complications, but where the neurological disease is not considered a complication of the systemic disease.

Unlike most books on this subject, the chapters of this book are organized and defined by neurological clinical scenarios, rather than by medical diseases. Specifically, each chapter focuses on a particular category of neurological presentation (e.g. movement disorders) and discusses the various systemic illnesses, or their treatment, that can cause dysfunction within that category of neurologic disorders. This organizational scheme, I propose, especially parallels the very common clinical scenario where the medical illness underlying the neurological syndrome is unknown; in these scenarios, the clinician needs to have some knowledge and understanding of the various systemic illnesses that can lead to these neurologic presentations.

The following major neurologic presentations define the chapters of this book: headache, encephalopathy, dementia, stroke, seizures, neuroophthalmologic disorders, neurootologic disorders, movement disorders, spinal cord disorders, peripheral nerve disorders, neuromuscular junction disorders, disorders of skeletal muscle, autonomic nervous system disorders, and sleep disorders. Each chapter, in turn, is subdivided into major categories of systemic illness that can lead to neurologic dysfunction: endocrine disorders; electrolyte and other metabolic disorders; systemic autoimmune disorders; organ dysfunction and failure; systemic cancer and paraneoplastic disorders; systemic infectious diseases; complications due to transplantation, complications of critical medical illness; drugs, alcohol, and toxins; and vitamin and mineral deficiencies. In individual chapters, some of these subheadings are excluded when they are not particularly relevant to that chapter's neurologic topic.

The book begins with the chapter on headache (Chapter 2) by Kevin Kahn, MD, from the Carolina Headache Institute, who discusses secondary headache syndromes that can be associated with systemic disease, as well as the interface between systemic illness and primary headache syndromes. Chapter 3, written by Allison Weathers, MD, from Rush University Medical Center provides an overview of the diffuse encephalopathy (delirium) syndromes that (by definition, arguably) occur within the setting of systemic dysfunction. In contrast, in Chapter 4, Jennifer Molano, MD, and Brendan Kelley, MD, from the University of Cincinnati tackle the interaction of systemic dysfunction and neurologic syndromes that more resemble dementia than typical toxic-metabolic encephalopathies; these authors also provide additional insights into the interface between the primary degenerative dementias and systemic illnesses.

In Chapter 5, Sarkis Morales-Vidal, MD, and José Biller, MD, from Loyola University Medical Center review the many systemic disorders that can be associated with, and potentially cause, cerebrovascular disease and stroke that the clinician should keep in mind in addition to the usual and well-known medical stroke risk factors. In Chapter 6, Matthew Hoerth, MD, and Joseph I. Sirven, MD, from the Mayo Clinic, Scottsdale, discuss the many medical problems that can lead to seizures; typically, recognition of these systemic causes of seizures can avoid unnecessary, or prolonged, antiepileptic drug therapy in these patients.

In Chapter 7, Matthew Thurtell, MBBS, from the University of Iowa and Janet Rucker, MD, from the Mount Sinai School of Medicine review and illustrate the many neuroophthalmological signs and symptoms that occur due to, and give clue to, the presence of an underlying potentially serious and sometimes vision-threatening systemic illness. In Chapter 8, Terry Fife, MD, from the Barrow Neurological Institute in Arizona discusses the many—and probably underrecognized by many neurologists—auditory or vestibular neurootologic syndromes that can occur due to medical illness.

In Chapter 9, Brandon Barton, MD, and Christopher Goetz, MD, from Rush University Medical Center review the many movement disorders (including parkinsonism, dystonia, tremor, chorea, myoclonus, ataxia, and tics) that can occur due to systemic disease or its treatment. In Chapter 10, Sital Patel, MD, and I, also from Rush, discuss myelopathies (whether from extrinsic compression of the spinal cord or intrinsic noncompressive spinal cord dysfunction) that can occur as a complication of an underlying medical disorder.

In Chapter 11, Michelle Mauermann, MD, from the Mayo Clinic Rochester and Ted Burns, MD, from the University of Virginia review the many neuropathic syndromes, and their characteristic clinical patterns, that can occur due to systemic disorders. Extending the discussion further, in Chapter 12, Jaffar Khan, MD, from the Emory University School of Medicine discusses presynaptic and postsynaptic neuromuscular junction disorders and their association with underlying systemic illness. In Chapter 13, Hannah Briemberg, MD, FRCPC, from the University of British Columbia reviews the many myopathic disorders that can occur as a consequence of medical illness and certain medications.

In Chapter 14, Brent Goodman, MD, and Eduardo Benarroch, MD, from the Mayo Clinic, Rochester, review autonomic nervous system manifestations that can occur—with or without other signs of neurologic dysfunction—in the setting of systemic disease; the authors also review how to assess for these autonomic disorders. Finally, in Chapter 15, Erik St. Louis from the Mayo Clinic, Rochester, discusses the association of disorders of sleep, including the parasomnias, and underlying systemic illness.

Each chapter concludes with a list of the authors' suggestion of “Five things to remember about” that particular neurologic topic and its relation to systemic disease; these can be construed as suggested minimum “take home” points that provide some additional overall clinical perspective for the reader.

Although written primarily with the neurologist (generalist neurologist, subspecialist neurologist, or neurologic trainee) in mind, the material in this book should also be of interest and accessible to internal medicine physicians, other primary care providers, internal medicine subspecialists, and even interested medical students. It is my hope that the reader of this text will find that the unique neurologic syndrome-based approach in the following pages will provide clinically useful insight into the wide variety of neurological disorders that occur in the context of systemic disease, and provide practical clinical clues to both the neurological diagnosis and the underlying medical diagnosis and management of these patients.

2

Headache due to Systemic Disease

Kevin A. Kahn

Carolina Headache Institute, NC, USA

The chief complaint of headache must always be considered to have an origin in medical illness before primary headache entities may be considered. The accepted criteria for migraine and other primary headache disorders by the International Headache Society (IHS) have at their core the mandate that secondary headaches must be excluded [1]. This chapter will review the potential secondary headaches that can occur as a consequence of medical illness.

An understanding of the mechanisms through which head pain is generated is critical to appreciating how systemic illness can generate headache. Sensation within the head depends upon afferent nerves from the anterior aspect of the head and the posterior aspect of the head that converge upon the trigeminal nucleus caudalis in the pons. This nucleus then sends further input to the thalamus and higher cortical structures to process new sensory information. During primary headache disorders such as migraine, this system is activated by either peripheral or central triggers to send electrical impulses efferently to peripheral structures. The depolarization of nerves ending in the periphery results in a release of inflammatory substances causing swelling, inflammation, and pain within peripheral structures. Such structures include meningeal arteries, sinuses, skin, and musculature within the head and neck. In addition to activation of peripheral structures, there is an increase of excitatory input or lack of inhibitory control centrally that results in increased sensitivity of all senses as well as activation of brainstem emesis/nausea centers. Thus, pain generated within the head is mediated through trigeminally innervated structures. The associated features of pain are generated by trigeminally related central activation and disinhibition. The pain from other primary headache disorders such as cluster headache, tension-type headache, and the trigeminal autonomic cephalgias all generate pain via these same trigeminal pathways. The manifestation of pain is often pulsatile or throbbing but can present as burning, stinging, aching, sharp, dull, pressure, squeezing, and so on. Associated features are typically sensitivity to light (photophobia) and noise (phonophobia), nausea, and vomiting, but can also present as sensitivity to smell (osmophobia) or touch, sinus congestion, lacrimation, rhinorrhea, scleral erythema, ptosis, neck pain/tension/stiffness, and worsening with position or activity.

Since the final common pathway of head pain is trigeminally mediated, many secondary headaches seem to have features in common with the primary headaches. For instance, meningitis, an infection of trigeminally innervated membranes around the brain, can present with light/sound sensitivity, nausea, throbbing pain, and stiff neck. It is the presence of other systemic features such as fever, in addition to guidance from the patient history, that help separate the primary from secondary headaches. Thus, it is important to remember that headaches that are new to an individual or are associated with abnormal signs on exam or are a dramatic change from preexisting headaches are red flags that mandate consideration of secondary headaches. The secondary headaches can often mimic migraine since anything that can irritate central or peripheral trigeminally innervated structures will affect the same pain mechanisms as the primary headaches.

Endocrine Disorders

Sex Hormones

Perhaps, the most common relationship between endocrine function and headache is evident in the phenomenon of hormonally mediated headache. The IHS divides hormonally mediated headaches into those from endogenous and those from exogenous hormones (see section on Drugs) [1].

Endogenous Estrogen-Related Headache

The IHS defines this headache as being related to estrogen cycling yielding patterns of pure menstrual migraine versus menstrually related migraine. Pure menstrual migraine occurs exclusively between 2 days before onset of menses (menstrual bleeding) and 3 days after onset of menses. This pattern should be present in at least two of the three menstrual cycles. Menstrually related migraine includes the time frame of pure menstrual migraine and other times of the entire cycle [1]. The phenomenon of hormonally exacerbated headache can be one of the factors implicated in migraine chronification, defined as an increase in headache frequency from less than 15 days per month to more than 15 days per month. In such patients, with >15 days/month of headache and a history of menstrually related migraine, prevention of estrogen withdrawal during the week of menstrual bleeding has been reported to be associated with resolution of chronic migraine [2].