The Curtis Center 170 S Independence Mall W 300E Philadelphia, Pennsylvania 19106 Mechanism and Management of Headache 0-7506-7530-6 Copyright ©2005 by James Lance. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Permissions may be sought directly from Elsevier’s Health Sciences Rights Department in Philadelphia, PA, USA: phone: (+1) 215 238 7869, fax: (+1) 215 238 2239, e-mail: [email protected]. You may also complete your request on-line via the Elsevier homepage (http://www.elsevier.com), by selecting ‘Customer Support’ and then ‘Obtaining Permissions’. NOTICE Medicine is an ever-changing field. Standard safety precautions must be followed, but as new research and clinical experience broaden our knowledge, changes in treatment and drug therapy may become necessary or appropriate. Readers are advised to check the most current product information provided by the manufacturer of each drug to be administered to verify the recommended dose, the method and duration of administration, and contraindications. It is the responsibility of the licensed prescriber, relying on experience and knowledge of the patient, to determine dosages and the best treatment for each individual patient. Neither the publisher nor the author assumes any liability for any injury and/or damage to persons or property arising from this publication. Previous editions copyrighted 1998, 1993, 1982, 1978, 1973, 1969 by Judith J.Lance International Standard Book Number: 0-7506-7530-6 Acquisitions Editor: Susan F. Pioli Developmental Editor: Joan Ryan Printed in the United States of America Last digit is the print number: 9 8 7 6 5 4 3 2 1 Preface to the Seventh Edition It is now 35 years since the first edition of this book appeared. In the 6 years since the last edition new headache entities have been described, new treatments have appeared, and research into the pathophysiology of migraine and cluster headache has intensified. Our aim is to retain the most important findings in the older literature as a background for the presentation of new developments and to combine the practi- cal approach to managment of previous editions with a critical analysis of the most recent information available. The format broadly follows the guidelines provided by the revised classification of the International Headache Society pub- lished in 2004. This classification introduced a new section on “Headaches attributable to psychiatric disorder.” Professor Jes Olesen pointed out in his introduction to the new classification, “research illuminating this field is extremely scarce, so the chapter is very brief.” For this reason we have not devoted a separate chapter to it. The important role of psychological factors in many forms of headache is considered in other chapters. Chronic daily headache has been given a chapter to itself because it presents a common therapeutic challenge. We trust that this new edition will prove to be a good companion for all those who suffer from headaches or treat them. J.W.L. P.J.G. xv Preface to the First Edition About once a month, until the age of 70 years, George Bernard Shaw suffered a devastating headache which lasted for a day. One afternoon, after recovering from an attack, he was introduced to Nansen and asked the famous Arctic explorer whether he had ever discovered a headache cure. “No,” said Nansen with a look of amazement. “Have you ever tried to find a cure for headaches?” “No.” “Well, that is a most astonishing thing!” exclaimed Shaw. “You have spent your life in trying to discover the North Pole, which nobody on earth cares tup- pence about, and you have never attempted to discover a cure for the headache, which every living person is crying aloud for.”* It is easy for a person who has never been troubled with headaches to lose patience with those who are plagued by them. The reaction of the virtuous observer may pass through a phase of sympathetic concern to one of frustrated tolerance and, finally, to a mood of irritation and resentment in which the recur- rence of headaches is attributed to a defective personality or escape from unpleasant life situations. The sound sleeper is traditionally intolerant of the insomniac and the speedy of bowel is just a little contemptuous of the consti- pated. In short, we tend to consider ourselves as the norm and to look quizzically at those whose physiological or psychological processes are at variance with our own. Such an attitude often persists in spite of years of advanced education and scientific training. To make it clear that I am not numbering myself among the righteous, I must state that I am not subject to headache and that my spirits often sink when confronted with a succession of patients whose contorted expressions testify to a lifetime of headache misery. This is about the only circumstance which I find likely to provoke headache in myself—I suppose on the principle that if you can’t beat them, join them! It would be foolish to deny that the workings of the mind are of great impor- tance in the production of headache, but they are only part of the story. My interest in migraine was first aroused when working at the Northcott Neurological Centre in Sydney. Each patient with migraine gave a history that was a little different from the others, but all were variations on a clearly *Pearson, H. (1942). Bernard Shaw. pp. 242-243. London, Collins. xvii xviii Preface recognizable theme. It seemed that all the clues were there to point the way to the understanding of the mechanism of migraine. These thoughts led to studies of the clinical features and natural history of migraine and, later, to laboratory work which now suggests that migraine is an hereditary recurrent metabolic distur- bance. If this be the case, a patient cannot be held responsible for having migraine attacks any more than a woman for having menstrual periods. The treatment of migraine has improved with better understanding of the syndrome, but knowledge of the migraine mechanism and its treatment still leave much to be desired. Mysteries remain in the problem of tension headache, although the place of psychological factors is much more obvious in this group than in migraine and an association with chronic over-contraction of muscle is almost universal. However, many tense, frowning people do not get headaches, and the explana- tion for those that do must go beyond a catalogue of undesirable personality traits and bad luck in cards or love. Migraine and tension headache are given most space in this small book because they are common complaints, not always easy to diagnose and treat, and worry patients and their medical attendants. Other common forms of headache such as those arising from eye-strain or sinusitis are not emphasized as much, because their mechanism and manage- ment are more straightforward. Serious acute headaches which betoken some hazardous intracranial condition are described sufficiently to assist in diagnosis, but not dealt with at length since their management usually becomes the prerog- ative of the specialist neurological unit. This book is designed to be relatively easy armchair reading for the general practitioner, senior medical student, or others who may be interested in the mechanism of headache or concerned with the practical management of headache problems. The neurologist may find something of interest in the chap- ters on tension headache and migraine. References are listed for those who wish to read in greater depth. The present concept of headache mechanisms depends to a great extent on the work of the late Harold G. Wolff and his colleagues, which is described in Wolff’s monograph Headache and Other Head Pain. The reader is referred to this work for aspects of headache which are passed over lightly here. The subject may not have all the excitement of a detective story but the talents of the great detectives of fiction would not be lost in trying to unravel some of the complexi- ties of headache. J.W.L. Acknowledgments Studies of clinical aspects, pathophysiology and treatment of headache have been conducted at The Prince Henry and Prince of Wales Hospitals, Sydney, and more recently at the National Hospital for Neurology and Neurosurgery, London, for over forty years. Those who have been or are presently engaged in the research programme include: R. D. Adams A. Gantenbein M. Matharu S. Afridi N. Giffin J. Michalicek S. Akerman V. Gordon J. Misbach H. Angus-Leppan C. Hadini D. Mitsikostas M. Anthony P. Hammond E. Mylecharane A. Bahra M. Hellier B. Olausson A. Bergerot H. Hinterberger J. Peralta R. Bhola P. Holland R. D. Piper P. Boers K. L. Hoskin F. Ragaglia C. Boes H. Kaube K. Shields N. Bogduk Y. E. Knight T. Shimomura E. Cittadini G. A. Lambert B. W. Somerville J. Classey M. Lasalandra P. J.Spira D. A. Curran G. D. A. Lord R. J.Storer B. Daher A. Lowy A. Srikiatkhachorn P. D. Drummond J. Marin K. M. A. Welch J. W. Duckworth N. Marlowe A. S. Zagami S. Evers A. May We are most grateful to this happy blend of neurologists, pharmacologists, anatomists, physiologists, biochemists and psychologists for many years of stimulating association, and to our neurosurgical colleagues for their friendly collaboration in managing headache and other problems. The research programme has been supported by the National Health andMedical Research Council of Australia and has been generously assisted by grants from the J. A. Perini Family Trust, the Adolph Basser Trust, TheAustralian Brain Foundation, The Migraine Trust, Sandoz AG, Basel (now Novartis), Glaxo-Wellcome (now GlaxoSmithKline) and Warren and Cheryl Anderson. Peter J.Goadsby particularly wishes to acknowledge the generous support of the Wellcome Trust, and the privilege of contributing to this book. xix xx Acknowledgments The manuscript has been prepared by Patricia Miller, Carol Flecknoe andSophie Ryan. The anatomical diagrams were drawn by Dr. N. Bogduk and Mr. Marcus Cremonese. All photographs were prepared by the Department of Medical Illustration, University of New South Wales. We are grateful to the editors of the following publications for permission to reproduce tables and figures from papers by our colleagues and ourselves: Annals of Neurology; Archives of Neurology; Brain; Cephalagia; Clinical and Experimental Neurology (Sydney, Adis Press); Headache; Journal of Neurological Sciences; Journal of Neurology, Neurosurgery and Psychiatry; Neurology; Migraine and Other Headaches (New York, Charles Scribner’s Sons); Migraine: a Spectrum of Ideas (Oxford, Oxford University Press); Medical Journal of Australia; Research and Clinical Studies in Headache (Karger of Basel and New York); Wolff/Wolff’s Headache and Other Head Pain (5thEdn, New York, Oxford University Press). Chapter 1 The History of Headache Among the beautiful frescoes in the Brancacci chapel in Florence, Italy, painted by Masaccio in the 15th century is the Expulsion from the Garden of Eden. Adam and Eve are shown with expressions of agony, Adam clasping his head with both hands, as the sad couple walk away. Was this Masaccio’s idea of the First Headache? To adopt an evolutionary approach, one might speculate that human suscepti- bility to headache developed when we assumed an upright posture. The discov- ery of trepanned skulls with bone regrowth shows that some Stone Age patients survived the trepanning procedure but does not tell us whether headache was the indication for operation. We have heard reports of a gorilla in the Toronto Zoo who sometimes curls up into a ball, shielding her eyes from the light and refus- ing to eat normally. Written accounts date from about 3000 BC: Alverez (1945) quotes a couplet from a Sumerian poem in which, in some sort of heaven or Abode of the Blessed, The sick-eyed says not “I am sick-eyed.” The sick-headed (says) not “I am sick-headed.” Also poetic, and more specific, is a reference to headache from Babylonian lit- erature of about the same period (Sigerist, 1955; McHenry, 1969). Headache roameth over the desert, blowing like the wind, Flashing like lightning, it is loosed above and below; It cutteth off him who feareth not his god like a reed, Like a stalk of henna it slitteth his thews. It wasteth the flesh of him who hath no protecting goddess, Flashing like a heavenly star, it cometh like the dew; It standeth hostile against the wayfarer, scorching him like the day, This man it hath struck and Like one with heart disease he staggereth, Like one bereft of reason he is broken, 1 2 Mechanism and Management of Headache Like that which has been cast into the fire he is shrivelled, Like a wild ass . . . his eyes are full of cloud, On himself he feedeth, bound in death; Headache whose course like the dread windstorm none knoweth, None knoweth its full time or its bond. The Atharvaveda, one of the four sacred Vedas of the Hindus, was written some time around the 10th century BC. One hymn reads, O physician, do thou release this man from headache, free him from cough which has entered into all his limbs and joints. One should resort to forests and hills for relief from diseases resulting from excessive rains, severe wind and intense heat (Chand, 1982). The main source of information about ancient Egyptian medicine is the Ebers Papyrus, said to have been found between the legs of a mummy in the necropolis of Thebes. The purchase and translation of this momentous and lengthy docu- ment (30 cm wide and 20.23 meters long) was arranged by George Ebers, pro- fessor of Egyptology in Leipzig, after whom the papyrus was named (Von Klein, 1905). Experts estimate that the Ebers Papyrus, which mentions migraine, neu- ralgia, and shooting head pains, was written or transcribed from earlier medical documents in approximately 1550 BC. The papyrus is obviously based on earlier writings, because one prescription was written for King Usaphais who reigned in 2700 BC(Major, 1930). Another six papyri on medical matters are in existence. I am indebted to Dr. John Edmeads for an artist’s impression of the advice given to headache sufferers in those days (Figure 1.1). A clay crocodile was firmly bound to the head of the patient, with herbs being placed in the mouth of the crocodile. Nowadays we dispense with the clay crocodile and put the magic herbs directly into the patient’s mouth. Greek medicine may be saidto have started with Aesculapius, who probably lived about 1250 BCand became so successful in his practice as a physician that the supply of souls to the underworld was imperiled. For this reason, at the request of Hades, Zeus destroyed Aesculapius with a thunderbolt. After his death, he became revered as a god and temples dedicated to healing of the sick were known through- out Greece as asklepia.Guthrie (1947) mentions that one of the cures recorded on stone tablets at Epidaurus was Case Number 29, a young man named Agestratos, who suffered from insomnia on account of headaches. He fell asleep in the temple and dreamed that Aesculapius cured him of his headache and then taught him to wrestle. The next day he departed cured and, shortly afterward, was crowned vic- tor in wrestling at the Nemean games. Egyptian medicine was probably known to Hippocrates (460 BC), who first described visual symptoms associated with migraine (Critchley, 1967): Most of the time he seemed to see something shining before him like a light, usually in part of the right eye; at the end of a moment, a violent pain supervened in the right temple, then in all the head and neck, where the neck is attached to the spine. . . . Vomiting, when it became possible, was able to divert the pain and render it more moderate. The History of Headache 3 FIGURE 1.1 An interpretation of the treatment of migraine in 1200 BC. A clay crocodile with magic herbs in its mouth was bound to the patient’s head. (Drawing by P. Cunningham, reproduced by permission of J. Edmeads.) Other forms of headache, such as those caused by exercise and sexual inter- course, were noted by Hippocrates (Adams, 1939) and are discussed later in this text. The story of migrainewas elaborated by Roman authors. Cornelius Celsus, a friend of the Emperor Tiberius, described how headache could be induced by drinking wine or by the heat of a fire or the sun (Critchley, 1967). Aretaeus, born in Cappadocia (now a region of Turkey) in about AD80, enlarged on previous descriptions of migraine by commenting that pain is often restricted to one half of the head and that afflicted patients . . . flee the light; the darkness soothes their disease; nor can they bear readily to look upon or hear anything agreeable; their sense of smell is vitiated, nei- ther does anything agreeable to smell delight them, and they have also an aver- sion to fetid things; the patients, moreover, are weary of life and wish to die (Adams, 1841). Galen (AD131–201) introduced the term “hemicrania” for unilateral headache, attributing it to “the ascent of vapours, either...too hot or too cold.” “Hemicrania” was later transformed to the Old English “megrim” and French “migraine.” Galen’s view of four humors that govern health and disease persisted for many centuries. It was expressed, in terms that could be given a modern inter- pretation, by Paul of Aegina, a Greek physician at the medical school of 4 Mechanism and Management of Headache Alexandria, who wrote in AD600: “Headache, which is one of the most serious complaints, is sometimes occasioned by an intemperament solely; sometimes by redundance of humors, and sometimes by both” (Adams, 1844). Treating headache by applying hot iron or by applying garlic to an incision in the temple was advocated by the Arab physician Abu’l Oasim (Abulcasis), born in Spain in AD 936 (Critchley, 1967). Critchley also quotes the Persian Avicenna (AD980–1037) as saying, “Little does it concern the patient that there is an underlying cause to be treated if the practitioner proves unable to relieve his pain.” An illuminated manuscript of the 12th century by the Abbess Hildegard of Bingen describes recurrent visual phenomena that may well have been migraine equivalents: “I saw a great star, most splendid and beautiful, and with it an exceeding multitude of falling sparks with which the star followed south- ward...and suddenly they were all annihilated, being turned into black coals ...and cast into the abyss so that I could see them no more” (Critchley, 1967). She explained that her headaches were unilateral, because nobody could stand it if they were on both sides of the head (Isler, 1993). Transient sensory loss and weakness on the left side of the body accompa- nying severe headache was described by Charles Le Pois in 1618 (Riley, 1932). In 1684 Dr Willis’s Practice of Physicke,published in London 9 years after the death of Thomas Willis (1621–1675), includes two chapters on headache (reproduced by Knapp, 1963, 1964). Headache is “a disease which falls upon . . . sober and intemperate, the empty and the full-bellied, the fat and the lean, the young and the old, yea upon men and women of every age, state or condition.” Willis pointed out that the source of pain is not the brain, cere- bellum, or medulla, because “they want sensible fibres” but rather distension of the vessels, which “pulls the nervous fibres one from another and so brings to them painful corrugations or wrinklings.” He described the provocation of headache by wine, overeating, lying in the sun, passion, sexual activity, long sleeping, “scirrhous tumours growing to the meninges” and “other diseases of an evil conformation.” He comments on “ravenous hunger” as a premonitory symptom of migraine and on the nausea and vomiting that follows the headache. Willis’s views on therapy do not live up to the expectation aroused by his clinical observations. He starts with sage but joyless advice about avoiding wine, spiced meats, baths, sexual intercourse (“Venus”), and violent motions of the mind and body, before advocating enemas, the letting of blood, and application of leeches, and then proceeding to a bizarre polypharmacy. Although few would take umbrage at the use of vinegar, nutmeg, and rose- mary (a decoction of the dried leaves made with spring water), some would raise eyebrows at pills of amber, crab’s eyes and coral, and most would draw the line at millipedes and woodlice: “We ought not to omit or postpone the use of millipedes or woodlice, for that the juice of them, wrung forth, with the dis- tilled water, also a powder of them prepared, often bring notable help, for the curing of old and pertinacious headaches.” In fairness to Willis, he also administered anodynes and hypnotics to those in need. The Swiss physician Wepfer (1620–1695) noted the alternation of migraine from one side of the head to the other in some cases, attributing migraine to