Migraine and Other Headache Disorders pdf

Migraine and Other

Headache Disorders

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NEUROLOGICAL DISEASE AND THERAPY

Advisory Board

Gordon H. Baltuch, M.D., Ph.D.

Department of Neurosurgery

University of Pennsylvania

Philadelphia, Pennsylvania, U.S.A.

Louis R. Caplan, M.D.

Professor of Neurology

Harvard University School of Medicine

Beth Israel Deaconess Medical Center

Boston, Massachusetts, U.S.A.

Mark A. Stacy, M.D.

Movement Disorder Center

Duke University Medical Center

Durham, North Carolina, U.S.A.

Mark H. Tuszynski, M.D., Ph.D.

Professor of Neurosciences

Director, Center for Neural Repair

University of California—San Diego

La Jolla, California, U.S.A.

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1. Handbook of Parkinson’s Disease, edited by William C. Koller

2. Medical Therapy of Acute Stroke, edited by Mark Fisher

3. Familial Alzheimer’s Disease: Molecular Genetics and Clinical Perspectives,

edited by Gary D. Miner, Ralph W. Richter, John P. Blass, Jimmie L. Valentine,

and Linda A. Winters-Miner

4. Alzheimer’s Disease: Treatment and Long-Term Management, edited by

Jeffrey L. Cummings and Bruce L. Miller

5. Therapy of Parkinson’s Disease, edited by William C. Koller and George Paulson

6. Handbook of Sleep Disorders, edited by Michael J. Thorpy

7. Epilepsy and Sudden Death, edited by Claire M. Lathers and Paul L. Schraeder

8. Handbook of Multiple Sclerosis, edited by Stuart D. Cook

9. Memory Disorders: Research and Clinical Practice, edited by Takehiko Yanagihara

and Ronald C. Petersen

10. The Medical Treatment of Epilepsy, edited by Stanley R. Resor, Jr., and Henn Kutt

11. Cognitive Disorders: Pathophysiology and Treatment, edited by Leon J. Thal,

Walter H. Moos, and Elkan R. Gamzu

12. Handbook of Amyotrophic Lateral Sclerosis, edited by Richard Alan Smith

13. Handbook of Parkinson’s Disease: Second Edition, Revised and Expanded,

edited by William C. Koller

14. Handbook of Pediatric Epilepsy, edited by Jerome V. Murphy

and Fereydoun Dehkharghani

15. Handbook of Tourette’s Syndrome and Related Tic and Behavioral Disorders,

edited by Roger Kurlan

16. Handbook of Cerebellar Diseases, edited by Richard Lechtenberg

17. Handbook of Cerebrovascular Diseases, edited by Harold P. Adams, Jr.

18. Parkinsonian Syndromes, edited by Matthew B. Stern and William C. Koller

19. Handbook of Head and Spine Trauma, edited by Jonathan Greenberg

20. Brain Tumors: A Comprehensive Text, edited by Robert A. Morantz

and John W. Walsh

21. Monoamine Oxidase Inhibitors in Neurological Diseases, edited by

Abraham Lieberman, C. Warren Olanow, Moussa B. H. Youdim, and Keith Tipton

22. Handbook of Dementing Illnesses, edited by John C. Morris

23. Handbook of Myasthenia Gravis and Myasthenic Syndromes, edited by

Robert P. Lisak

24. Handbook of Neurorehabilitation, edited by David C. Good

and James R. Couch, Jr.

25. Therapy with Botulinum Toxin, edited by Joseph Jankovic and Mark Hallett

26. Principles of Neurotoxicology, edited by Louis W. Chang

27. Handbook of Neurovirology, edited by Robert R. McKendall and William G. Stroop

28. Handbook of Neuro-Urology, edited by David N. Rushton

29. Handbook of Neuroepidemiology, edited by Philip B. Gorelick and Milton Alter

30. Handbook of Tremor Disorders, edited by Leslie J. Findley and William C. Koller

31. Neuro-Ophthalmological Disorders: Diagnostic Work-Up and Management,

edited by Ronald J. Tusa and Steven A. Newman

32. Handbook of Olfaction and Gustation, edited by Richard L. Doty

33. Handbook of Neurological Speech and Language Disorders, edited by

Howard S. Kirshner

34. Therapy of Parkinson’s Disease: Second Edition, Revised and Expanded,

edited by William C. Koller and George Paulson

35. Evaluation and Management of Gait Disorders, edited by Barney S. Spivack

36. Handbook of Neurotoxicology, edited by Louis W. Chang and Robert S. Dyer

37. Neurological Complications of Cancer, edited by Ronald G. Wiley

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38. Handbook of Autonomic Nervous System Dysfunction, edited by

Amos D. Korczyn

39. Handbook of Dystonia, edited by Joseph King Ching Tsui and Donald B. Calne

40. Etiology of Parkinson’s Disease, edited by Jonas H. Ellenberg, William C. Koller

and J. William Langston

41. Practical Neurology of the Elderly, edited by Jacob I. Sage and Margery H. Mark

42. Handbook of Muscle Disease, edited by Russell J. M. Lane

43. Handbook of Multiple Sclerosis: Second Edition, Revised and Expanded,

edited by Stuart D. Cook

44. Central Nervous System Infectious Diseases and Therapy, edited by Karen L. Roos

45. Subarachnoid Hemorrhage: Clinical Management, edited by Takehiko Yanagihara,

David G. Piepgras, and John L. D. Atkinson

46. Neurology Practice Guidelines, edited by Richard Lechtenberg

and Henry S. Schutta

47. Spinal Cord Diseases: Diagnosis and Treatment, edited by Gordon L. Engler,

Jonathan Cole, and W. Louis Merton

48. Management of Acute Stroke, edited by Ashfaq Shuaib and Larry B. Goldstein

49. Sleep Disorders and Neurological Disease, edited by Antonio Culebras

50. Handbook of Ataxia Disorders, edited by Thomas Klockgether

51. The Autonomic Nervous System in Health and Disease, David S. Goldstein

52. Axonal Regeneration in the Central Nervous System, edited by

Nicholas A. Ingoglia and Marion Murray

53. Handbook of Multiple Sclerosis: Third Edition, edited by Stuart D. Cook

54. Long-Term Effects of Stroke, edited by Julien Bogousslavsky

55. Handbook of the Autonomic Nervous System in Health and Disease, edited by

C. Liana Bolis, Julio Licinio, and Stefano Govoni

56. Dopamine Receptors and Transporters: Function, Imaging,

and Clinical Implication, Second Edition, edited by Anita Sidhu, Marc Laruelle,

and Philippe Vernier

57. Handbook of Olfaction and Gustation: Second Edition, Revised and Expanded,

edited by Richard L. Doty

58. Handbook of Stereotactic and Functional Neurosurgery, edited by

Michael Schulder

59. Handbook of Parkinson’s Disease: Third Edition, edited by Rajesh Pahwa,

Kelly E. Lyons, and William C. Koller

60. Clinical Neurovirology, edited by Avindra Nath and Joseph R. Berger

61. Neuromuscular Junction Disorders: Diagnosis and Treatment,

Matthew N. Meriggioli, James F. Howard, Jr., and C. Michel Harper

62. Drug-Induced Movement Disorders, edited by Kapil D. Sethi

63. Therapy of Parkinson’s Disease: Third Edition, Revised and Expanded, edited by

Rajesh Pahwa, Kelly E. Lyons, and William C. Koller

64. Epilepsy: Scientific Foundations of Clinical Practice, edited by Jong M. Rho,

Raman Sankar, and José E. Cavazos

65. Handbook of Tourette’s Syndrome and Related Tic and Behavioral Disorders:

Second Edition, edited by Roger Kurlan

66. Handbook of Cerebrovascular Diseases: Second Edition, Revised and Expanded,

edited by Harold P. Adams, Jr.

67. Emerging Neurological Infections, edited by Christopher Power

and Richard T. Johnson

68. Treatment of Pediatric Neurologic Disorders, edited by Harvey S. Singer,

Eric H. Kossoff, Adam L. Hartman, and Thomas O. Crawford

69. Synaptic Plasticity : Basic Mechanisms to Clinical Applications, edited by

Michel Baudry, Xiaoning Bi, and Steven S. Schreiber

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70. Handbook of Essential Tremor and Other Tremor Disorders, edited by

Kelly E. Lyons and Rajesh Pahwa

71. Handbook of Peripheral Neuropathy, edited by Mark B. Bromberg

and A. Gordon Smith

72. Carotid Artery Stenosis: Current and Emerging Treatments, edited by

Seemant Chaturvedi and Peter M. Rothwell

73. Gait Disorders: Evaluation and Management, edited by Jeffrey M. Hausdorff

and Neil B. Alexander

74. Surgical Management of Movement Disorders (HBK), edited by Gordon H. Baltuch

and Matthew B. Stern

75. Neurogenetics: Scientific and Clinical Advances, edited by David R. Lynch

76. Epilepsy Surgery: Principles and Controversies, edited by John W. Miller

and Daniel L. Silbergeld

77. Clinician's Guide To Sleep Disorders, edited by Nathaniel F. Watson

and Bradley Vaughn

78. Amyotrophic Lateral Sclerosis, edited by Hiroshi Mitsumoto, Serge Przedborski,

and Paul H. Gordon

79. Duchenne Muscular Dystrophy: Advances in Therapeutics, edited by

Jeffrey S. Chamberlain and Thomas A. Rando

80. Handbook of Multiple Sclerosis, Fourth Edition, edited by Stuart D. Cook

81. Brain Embolism, edited by Louis R. Caplan and Warren J. Manning

82. Handbook of Secondary Dementias, edited by Roger Kurlan

83. Parkinson's Disease: Genetics and Pathogenesis, edited by Ted M. Dawson

84. Migraine, Russell Lane and Paul Davies

85. Migraine and Other Headache Disorders, edited by Richard B. Lipton

and Marcelo E. Bigal

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Richard B. Lipton

Albert Einstein College of Medicine

New York, New York, U.S.A.

Marcelo E. Bigal

Albert Einstein College of Medicine

New York, New York, U.S.A.

New York London

Migraine and Other

Headache Disorders

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Published in 2006 by

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Preface

With this first edition of Migraine and Other Headache Disorders, we celebrate the

remarkable progress in the art and science of headache during the last decade. With

32 chapters by 54 leaders in the field, the book provides health care professionals

with practical approaches to patient care and reviews the scientific foundations of

headache. We emphasize migraine because of its high prevalence, enormous burden,

and the increasing availability of effective management strategies. At the same time,

we provide broad coverage of all the primary headache disorders. Finally, although

not focusing on specific subtypes of secondary headaches, we discuss strategies for

diagnosing and excluding the ominous causes of headache, based both on clinical

evaluation and, when appropriate, the use of diagnostic testing.

Our understanding of headache and the approach to treatment have been

transformed by insights from many places. Based on the Second Edition of the Inter￾national Classification of Headache Disorders, the book provides a series of diagnos￾tic algorithms intended to simplify clinical practice. We also present up-to-date

epidemiologic information on the primary headache disorders. Epidemiologic stud￾ies show that the overwhelming majority of headache sufferers who seek treatment in

primary care settings have migraine. Diagnosis becomes more efficient when that

fact is taken into account. Doctors should avoid oversimplifying the differential

diagnosis of the primary headaches, however.

Our understanding of migraine as a disorder has significantly evolved over the

past decade, based on genetic, epidemiologic, and translational studies. Once consid￾ered an episodic pain problem, treating the pain seemed like a sensible strategy. In the

past few years, many lines of evidence have suggested that migraine and other head￾ache disorders are best understood as chronic disorders with episodic manifestations.

Painful episodes are the most prominent manifestation of migraine. Nonetheless,

between attacks, there is an enduring predisposition to headache that characterizes

the migraine brain. Furthermore, migraine is not only a chronic disorder with epi￾sodic manifestations, it is sometimes a disorder that progresses in several ways. Pro￾gression may be clinical, as attacks increase in frequency until chronic or transformed

migraine develops. This clinical progression is sometimes accompanied by the devel￾opment of allodynia with sensitization as its presumed substrate. In addition, in some

individuals, morphological progression takes the form of deep white matter lesion or

posterior circulation strokes that increase with migraine attack frequency, probably

reflecting neuroplastic changes in the brain. Herein we highlight the emerging data

on progression and on the modifiable risk factors for migraine progression.

Progress in treatment has also taken several forms. Since 1990, ten new acute

treatments with a multiplicity of formulations and two preventive drugs have been

iii

approved. Many studies show that acute treatments work best if given early in the

attack. Combining acute treatments may improve treatment response in some indi￾viduals. In addition, recent epidemiologic data shows that, based on frequency and

disability criteria, preventive treatment should be offered or considered in about

40% of migraine sufferers. The same studies show that only 12% currently receive

preventive therapy. Preventive treatment decreases attack frequency and severity

and possibly prevents migraine progression. The use of specific acute agents that

act on the neural pathways of migraine pain, such as the triptans, dramatically

improve patient outcomes.

Migraine and Other Headache Disorders highlights the treatment approaches

developed at some of the best headache clinics in the world. It also reflects many

of the strategies adopted at The Montefiore Headache Center. The Montefiore

Headache Center was the first headache specialty care center in the world, founded

in 1945 by Dr. Arnold Friedman, and it is where we are both proud to be.

We are extremely grateful to our mentors. Among them, Dr. Lipton wants to

thank Dr. Seymour Solomon, who directed The Montefiore Headache Center for a

quarter of a century, for being a wonderful mentor and teacher. He’d also like to

thank his mentors and collaborators in research, particularly Drs. Philip Holzman,

W. Allen Hauser, and Walter F. Stewart. Dr. Bigal wants to acknowledge

Drs. Speciali and Bordini, from Brazil, and the teams at The New England Center

for Headache (Rapoport, Sheftell, and Tepper) and at Montefiore (Lipton and

Solomon) for their help and direction. We also want to thank the authors of the

chapters in this book for their excellent work.

Finally, we owe special thanks to our families, particularly our wives (Amy

Natkins Lipton and Janaı´na Maciel Bigal) and children (Lianna Lipton, Justin

Lipton, Luı´sa Bigal, and Hanna Bigal) for supporting us through evenings and week￾ends spent writing and editing as we prepared this book.

Finally, to our readers, we hope this book furthers your efforts to improve the

lives of headache sufferers. These common and disabling disorders are tremendously

gratifying to treat. In a field where cures are rare, we can nonetheless help patients by

empowering them with tools that relieve pain, restore their ability to function, and,

perhaps, prevent disease progression.

Richard B. Lipton

Marcelo E. Bigal

iv Preface

Contents

Preface . . . . iii

Contributors . . . . xv

1. Headache—Classification . . . . . . . . ........................ 1

Marcelo E. Bigal and Richard B. Lipton

Introduction . . . . 1

An Overview of the ICHD-2 . . . . 1

Classification of the Primary Headaches . . . . 5

Secondary Headaches . . . . 14

Headache Attributed to Head and/or Neck Trauma . . . . 14

Headache Attributed to Cranial or Cervical

Vascular Disorders . . . . 14

Headache Attributed to Nonvascular Intracranial Disorders . . . . 15

Headache Attributed to a Substance or Its Withdrawal . . . . 15

Headache Attributed to Infection . . . . 15

Headache Attributed to Disorders of Homeostasis . . . . 15

Headache or Facial Pain Attributed to Disorders of Cranium,

Neck, Eyes, Ears, Nose, Sinuses, Teeth, Mouth, or

Other Facial or Cranial Structures . . . . 16

Headache Attributed to Psychiatric Disorders . . . . 16

Cranial Neuralgias and Central Causes of Facial Pain . . . . 16

Controversies in the Classification of Primary Chronic Daily

Headaches of Long Duration . . . . 16

References . . . . 17

2. The Epidemiology and Impact of Migraine . . . ................ 23

Richard B. Lipton and Marcelo E. Bigal

Introduction . . . . 23

The Epidemiology of Migraine . . . . 23

The Burden of Migraine . . . . 31

Probable Migraine—An Important Migraine Subtype . . . . 32

Conclusions . . . . 33

References . . . . 34

v

3. Progressive Headache: Epidemiology, Natural History, and

Risk Factors . . . ..................................... 37

Ann I. Scher

Introduction . . . . 37

Classification . . . . 37

Chronic Daily Headache Epidemiology and Natural History . . . . 38

Demographic Factors Associated with Chronic

Daily Headache . . . . 38

Other Factors Associated with Chronic Daily Headache

Prevalence or Incidence . . . . 39

Conclusion . . . . 42

References . . . . 42

4. Comorbidity of Migraine . .............................. 45

Nancy C. P. Low and Kathleen Ries Merikangas

Introduction . . . . 45

Methodology of Comorbidity Studies . . . . 45

Evidence for Migraine Comorbidity . . . . 46

Conclusion . . . . 53

References . . . . 54

5. Pain Sensitivity: Intracranial and Extracranial Structures . . . . . . . . 61

Todd D. Rozen

Anatomy of Head Pain . . . . 61

References . . . . 65

6. Pathophysiology of Aura . .............................. 67

M. Sanchez del Rio and U. Reuter

Introduction . . . . 67

Neurophysiological Mechanisms . . . . 67

Imaging Studies . . . . 72

Molecular Mechanisms . . . . 72

Occipital Cortex Excitability . . . . 74

Genetics . . . . 75

Conclusions . . . . 76

References . . . . 77

7. Pathophysiology of Migraine . . . . . ....................... 81

Peter J. Goadsby

Introduction . . . . 81

Migraine—Explaining the Clinical Features . . . . 81

Genetics of Migraine . . . . 82

Familial Hemiplegic Migraine (FHM) . . . . 82

Migraine Aura . . . . 83

Headache—Anatomy . . . . 83

Headache Physiology—Peripheral Connections . . . . 84

vi Contents

Headache Physiology—Central Connections . . . . 86

Central Modulation of Trigeminal Pain . . . . 88

What Is Migraine? . . . . 89

References . . . . 90

8. Allodynia and Sensitization in Migraine . . . . . ................ 99

William B. Young, Avi Ashkenazi, and Michael L. Oshinsky

Introduction . . . . 99

Sensory Processing by the Nervous System . . . . 100

Sensitization of the Dorsal Horn . . . . 100

Rat Model of Migraine Headache and Allodynia . . . . 102

Human Studies of Allodynia in Pain Disorders

Other Than Migraine . . . . 103

Human Studies of Allodynia in Migraine . . . . 104

Time Course of Sensitization in Migraine . . . . 106

The Effect of Allodynia on Treatment Outcome . . . . 106

The Effect of Treatment on Allodynia . . . . 107

Allodynia in Headache Disorders Other Than Migraine . . . . 107

Conclusion . . . . 108

References . . . . 108

9. Genetics of Migraine and Other Primary Headaches . . . ........ 113

Gisela M. Terwindt, Esther E. Kors, Joost Haan, Kaate R. J. Vanmolkot,

Rune R. Frants, Arn M. J. M. van den Maagdenberg, and

Michel D. Ferrari

Introduction—Genetic Studies on Headache . . . . 113

The Clinical Spectrum of the CACNA1A Gene Mutations . . . . 114

The Clinical Spectrum of the ATP1A2 Gene . . . . 118

Sporadic Hemiplegic Migraine . . . . 120

Genetic Susceptibility in Migraine . . . . 120

Cluster Headache . . . . 123

Tension-Type Headache . . . . 123

Concluding Remarks . . . . 123

References . . . . 124

10. Identification or Exclusion of Secondary Headaches . . ........ 131

Randolph W. Evans and R. Allan Purdy

Introduction . . . . 131

General Indications for Neuroimaging for Headaches . . . . 132

Neuroimaging for Headaches with a Normal

Neurological Examination . . . . 132

Neuroimaging for Migraine . . . . 133

Evaluation of the Acute Severe New-Onset Headache (‘‘First or

Worst Headaches’’) . . . . 135

Headaches Over the Age of 50 Years . . . . 138

New Daily Headaches . . . . 140

References . . . . 141

Contents vii

11. Differential Diagnosis of Primary Headaches:

An Algorithm-Based Approach . . . ...................... 145

Richard B. Lipton and Marcelo E. Bigal

Introduction . . . . 145

Approaching a Patient with Headache . . . . 145

Conclusions . . . . 152

References . . . . 152

12. Diagnostic and Severity Tools for Migraine . ............... 155

Marcelo E. Bigal and Richard B. Lipton

Introduction . . . . 155

Screening for Migraine . . . . 156

Assessing Migraine-Related Disability . . . . 159

Assessing Psychological Comorbidity . . . . 163

Assessing Ongoing Treatment . . . . 163

Conclusion . . . . 165

References . . . . 165

Appendix: The PRIME-MD Questionnaire . . . . 167

13. Migraine Without Aura . ............................. 173

Fred Sheftell and Roger Cady

Introduction—Migraine Without Aura: An Underdiagnosed and

Undertreated Disorder . . . . 173

The ICHD-2 Criteria for Migraine Without Aura . . . . 174

Migraine in Clinical Practice . . . . 177

The Convergence Hypothesis . . . . 181

Menstrually Related Migraine . . . . 182

Conclusion . . . . 183

Illustrative Case History . . . . 184

References . . . . 184

14. Migraine with Aura . . . . ............................. 189

Malene Kirchmann Eriksen and Jes Olesen

Introduction . . . . 189

Classification . . . . 189

Migraine with Typical Aura . . . . 192

Familial and Sporadic Hemiplegic Migraine . . . . 197

Basilar-Type Migraine . . . . 198

Differential Diagnoses . . . . 199

References . . . . 199

15. Childhood Periodic Syndromes . . . ...................... 203

Vincenzo Guidetti, Federica Galli, Azzurra Alesini, and Federico Dazzi

Introduction . . . . 203

Cyclical Vomiting . . . . 204

RAP and Abdominal Migraine . . . . 206

BPV of Childhood . . . . 207

viii Contents