Ancient writings testify to the fact that headache has always been a problem of man. From the earliest manuscripts of the Greeks and Romans to the modern novel, there have been references to headache in literature as well as in art, music, and legend. Ii has played a role in fictional plots and folk tales and probably in the course of history. Plato's paragraph on headache is good enough to serve as an introduction to any general medical, neurologic, or psychiatric text published today. Shakespeare in Romeo and Juliet, Mark Twain in Following the Equator, Lewis Carroll in Through the Looking Glass, Cervantes in Don Quixote, and Steinbeck in The Wayward Bus have shown the effects of headache on their characters and writings.
Throughout history the siege of headache has been suffered by men and women of varied origins and professions. For some the recurring attacks had a profound influence on their ability to function and to cope with professional, creative, or political activities. Famous people who have had migraine include Frédéric Chopin, Richard Wagner, John Calvin, Immanuel Kant, Friedrich Wilhelm Nietzsche, Edgar Allan Poe, Leo Tolstoy, Thomas Jefferson, Ulysses S. Grant, Virginia Woolf, Charles Darwin, Karl Marx, and Sigmund Freud; they have offered representative descriptions of the miseries of their affliction and its interference with their lives.
From vague descriptions of head pain in the primitive practice of trepanation to modern attempts at classification and the use of psychotherapy and pharmacotherapy, we can trace the emergence of a specific body of knowledge and its incorporation into medical practice.
The clinical psychiatrist is most often concerned with chronic headache that is not associated with serious underlying disease. The symptom always demands careful differential study, however, since it may signal the presence of an underlying disease process that demands treatment and may even constitute a threat to life. The fact that a patient is in psychotherapy or seeing a psychiatrist does not necessarily mean that the cause of his headache is psychologic. Determination of the cause of chronic headache requires a careful and systematic history, a thorough physical and neurologic examination, psychologic evaluation, and the use of selected laboratory tests.
Migraine is a cumulative term used by many physicians for a variety of types of headache. There is considerable disagreement about the proper scope of the word, since some include in the migraine group all cases of chronic headache, including tension headaches and those for which no adequate cause can be found. Other physicians confine their use of the term to cases that show a complete syndrome of classic migraine, which embraces a relatively limited group of headaches. Although we have a broader knowledge of migraine than ever before and now recognize additional signs and symptoms that may constitute a migraine attack or a migraine equivalent, we have yet to formulate a comprehensive definition of migraine. Such a definition would incorporate biochemical, vascular, electrophysiologic, genetic, neurologic, and psychologic parameters.
Until then, a working definition that is mainly descriptive must suffice. From a clinical and experimental standpoint, we prefer to follow the classification of migraine provided by the Ad Hoc Committee of the National Institute of Neurological Diseases and Stroke,1 which provides a useful outline for discussion of migraine headaches, their diagnosis, and differential diagnosis.
The term migraine, like the Old English word megrim, comes through French from the Greek hemikrania. The classical concept of migraine is that of a paroxysmal disturbance of cerebral function with recurring attacks of pain, commonly unilateral in onset (though not always on the same side) and usually associated with nausea and vomiting. In some patients the pain is preceded by or associated with sensory, motor, and mood disturbances. There is often a family history of headache. Physical examination and laboratory data are of little value in diagnosing migraine or in following its course.
CLINICAL CLASSIFICATION OF MIGRAINE
Clinically, migraine is characterized by periods of increased or decreased activity. It is polysymptomatic, as it involves the central, peripheral, and autonomic nervous systems, as well as other body systems. The principal feature of migraine attacks is not always pain; ophthalmologic, sensory, motor, and mental symptoms may be prominent, with or without the headache. In early childhood, motion sickness and cyclical vomiting are frequently present. Women are more frequently affected by migraine than men, and menopause does not necessarily signal the end of migraine attacks.
There are a number of migraine variants, but certain features follow a recognizable sequence and permit classification into the following types.
Classic migraine occurs in approximately 10 per cent of patients with migraine. The prodromes are sharply defined contralateral neurologic manifestations - usually visual, though in some patients the effects are sensory, motor, or a combination of these. The pain is unilateral and pulsatile, and anorexia, nausea, and vomiting are concomitant features.
The prodromes of common migraine are not sharply defined, and they may precede the attack by several hours or days. They include psychic disturbances, gastrointestinal manifestations, and changes in fluid balance. Symptoms common to both types include local or generalized edema, irritability, pallor, dizziness, and sweating. The actual headache is frequently longer than in the classic type, lasting for many hours to days, and may be bilateral. This is the commonest form of migraine.
Hemiplegie and ophthalmoplegic migraine are closely related, rare types that usually occur in young adults. The pain is moderate and ipsilateral to the paralysis, and in the ophthalmoplegic type is accompanied by extraocular muscle palsies in the third cranial nerve (internal and external ophthalmoplegia) and other oculomotor nerves. Often the paralysis becomes evident as the intensity of the pain diminishes, usually three to five days after onset of a persistent headache.2 The third cranial nerve may occasionally be permanently injured if the attacks of ophthalmoplegic migraine are frequent. The hémiplégie migraine complex is characterized by neurologic deficits, hemiparesis, or hemiplegia. The neurologic phenomena of both types may persist for some time after subsidence of the headache.
Some of these patients may have an intracranial aneurysm of the main trunk of the internal carotid or of its junction with the posterior communicating artery. The pathogenesis of the ocular symptoms may be in segmental narrowing of the basilar artery between the origin of the posterior cerebral and superior cerebellar arteries. Such narrowing may be due to edema of the posterior cerebral vessel, which causes a compression of the oculomotor nerve.
Bickerstaff 3 has distinguished another type of migraine called basilar artery migraine. Its prodromal symptoms include visual loss, disturbance of consciousness, and a variety of brain-stem symptoms, including vertigo, ataxia, dysarthria, and paresthesias, followed by a severe throbbing occipital headache with vomiting. This type of migraine occurs in young women and girls and often has a striking relationship to menstruation.
Cluster headache or periodic migrainous neuralgia occurs in a series of closely spaced attacks that may be followed by remissions of months or even years.4 The nature of the attack, pattern of recurrence, and lack of family history are so characteristic that this syndrome can be distinguished from migraine of the classic, common, or ophthalmoplegic type. The question still remains whether this syndrome belongs in the migraine spectrum or should be classified as a nonmigrainous vascular headache. In cluster headache the onset is sudden and frequently nocturnal, with each attack lasting from 20 to 90 minutes. The associated symptoms are a prominent feature of the attack: conjunctival injection, lacrimation, nasal congestion, bradycardia, and a Horner's syndrome that occurs on the same side as the headache in about 20 per cent of the patients. The attacks are precipitated by vasodilator agents - notably alcohol, nitroglycerin, and histamine. The facial thermogram reveals spotty areas of dense coolness in the supraorbital region, always ipsilateral to the patient's headache. This characteristic thermographic picture is not found in other types of vascular headache. It has been suggested that parasympathetic discharge in the seventh cranial nerve or deficient activity of the sympathetic nervous system or both may evoke this type of vascular headache. These headaches have been described in a variety of terms, including ciliary neuralgia and histaminie cephalalgia.
The transient neurologic disturbances of migraine have become permanent in a few patients. Lesions in the retina, the cerebral hemispheres, and the brain stem have been found in some of our patients, suggesting a neurologic or vascular cause. The average age of these patients is well below the usual age of onset for cerebrovascular disease, and as a rule the occipital cortex is most frequently affected, resulting in a permanent hemianopsia. Recent arteriographie studies have excluded the presence of malformation while indicating that these areas may be encephalomalacic.
Migraine equivalents. Other body disturbances may replace the headache of migraine attacks in some patients. These disturbances sometimes take the form of gastrointestinal complaints (abdominal migraine), such as nausea, vomiting, and diarrhea. At other times psychic symptoms predominate: confusion, lethargy, sleep disturbances, and mood and behavior changes. Additional manifestations include attacks of tachycardia, benign paroxysmal vertigo, bouts of fever, cyclical edema, and pain in the thorax, pelvis, or extremities.
PATHOGENESIS OF MIGRAINE
The exact origin of migraine attacks remains unknown. Evidence has accumulated that migraine is of vascular origin and that the symptoms of migraine are related to a disturbance of the central vasomotor centers, the extracranial and intracranial blood vessels, and the microcirculation. The pain of migraine is caused by dilatation of cranial arteries, in most instances the extracranial or scalp arteries. Associated with the vascular distention and increased blood flow is a "sterile inflammation" of the arterial wall that probably depends on humoral agents with vasoactive properties and local accumulation of pain-producing substances.5,7 The focal symptoms of migraine preceding the headache are probably produced by an initial phase of arterial vasoconstriction, largely intracranial and associated with a slowly spreading process of cortical depression that accounts for the gradual progress of neurologic symptoms.8-10
However, the view that migraine consists of phases of vasoconstriction and vasodilation is far too simplified; it is apparent that migraine is a complex vasomotor disorder.
No conclusion can yet be drawn about the specific role of humoral agents - vasoactive, chemical, or enzymatic - in the origins of migraine. Recent evidence suggests that the mechanism of migraine involves at least three or four biochemical keys: serotonin, catabolites of norepinephrine, prostaglandins, and bradykinin. No convincing evidence implicates acetylcholine or histamine in this mechanism.
It has been recognized for centuries that emotional factors can trigger migraine, but their various and relative roles in the cause and chronicity of the disease are still open to question. Psychologic factors may operate in a variety of ways:
- Directly, by precipitating changes in specific physiologic function, such as cranial vascular changes, sustained muscle contraction, and increased or decreased activity of various glands.
- As conversion phenomena, translating an emotional conflict into a physical dysfunction.
- As a result of delusional or hypochondriacal states.
The migraine patient's personality structure has been described as compulsive, with outstanding performance due to the emphasis on perfectionism, overconscientiousness, and ambition. He tends to become immersed in details and requires from himself and from others a consistently high performance that can be maintained only so long as energy reserves are high, as in youth. No doubt many migraine patients do have these characteristics, as do some headache-free persons; but other migraine patients have anxiety reactions, neurotic depressive symptoms, conversion, and dissociative and phobic reactions." These emotional characteristics may be precipitating factors, but again they are in no way specific for migraine.
Most studies on psychologic factors in migraine have been done on selected groups of patients who were attending a migraine clinic or were deemed suitable for psychiatric treatment by their family physician. It is likely that these patients had a higher incidence of such personality traits as anxiety, obsession, overconscientiousness, and overconcem about health than those who did not seek special aid for their migraine. There are no published studies on migraine patients as compared with control groups of similar age, sex, and socioeconomic background.
Psychologic studies of migraine patients indicate that many of them were not permitted to express emotions of anger or rage as children and that love and approval could be won only by suppression of their true feelings. These observations indicate that hostile impulses are basic to neurotic conflicts in patients with migraine. They do not handle their aggressive energy appropriately, and their attacks may erupt from unconscious hostile feelings associated with sustained resentment, anxiety, frustration, and energy depletion.12,13
In marked contrast to their typical behavior, which is usually controlled and outwardly calm, migraine patients during an attack may display irritability, withdrawal, poor judgment, and open hostility. When such behavior is encountered, it is important that the physician distinguish between a syndrome of depression and depression of mood, the latter often being an integral part of the migraine attack. The headache that continues for weeks or months should not be readily accepted as migraine; its persistence suggests that it is more likely caused by underlying anxiety or depression.
Prolonged stressful situations - such as puberty, leaving home, seeking work, and meeting the responsibilities of job, marriage, or parenthood - can trigger a series of migraine attacks in susceptible persons. When the headache occurs regularly during holidays, weekends, or periods of relaxation, it is probably coinciding with stressful interpersonal situations in which the patient, although appearing comfortable and relaxed, is repressing his anger towards someone whom he feels he should love or respect. Associated with this hostility is guilt, which is usually unconscious and may in itself be responsible for the pain. The headache associated with the "Sunday neurosis" described by Ferenczi14 is occasionally seen in patients who fear loss of impulse control on weekends and holidays.
Various nonspecific stimuli can produce a migraine attack. These include fatigue; bright or flickering lights; irregular eating patterns, inducing a mild hypoglycemic state; ingestion of food products containing tyramine, phenylethylamine, or monosodium glutamate; exposure to high altitudes; meteorologie changes; various drugs, including reserpine and vasodilators; and changes in endocrine balance during the menstrual cycle. The migraine patient shows a tendency to overreact to physiologic changes, rendering him sensitive to a wide range of stimuli or situations that become stressful and produce an attack of headache. ,5
An important problem is the occurrence of migraine in the presence of cerebrovascular disease. Headache with mild hypertension has no specific diagnostic features and is usually either a vascular headache or one caused by sustained muscle contraction (tension), as in the normotensive patient. In severe hypertension (readings in the range of 220/120), frequent and intense headaches are common; they are often occipital but may be felt elsewhere in the head. The headache is noticed on awakening and usually diminishes or disappears several hours after the patient arises. In most of these patients, a relationship exists between the intensity of the headache and the degree of hypertension. Reduction in the patient's blood pressure is followed by reduction in the frequency and severity of the headache. In the presence of hypertension, additional symptoms - paroxysmal pulsatile headache, severe tachycardia, pallor, nausea, and vomiting - may suggest the diagnosis of pheochromocytoma. Appropriate pharmacologic and chemical tests, including urine assay for catecholamines and their methoxy derivatives, are helpful in diagnosis.
Reportedly, patients with migraine frequently develop hypertension in the later years of life. It is difficult to assess this alleged development with accuracy, since migraine is not always clearly defined in these studies.
Our work has indicated that migraine is independent of the présense or absence of aneurysm or angioma. Aneurysm of the posterior communicating or internal carotid artery may cause unilateral headache that recurs in the orbital arc of the frontal region on the same side as the aneurysm. The presence of persistent defects in the visual field and sudden or increasing paralysis of the third and other extraocular cranial nerves suggest dilation of an aneurysm. It should be emphasized that if the pupil is spared at the onset of an isolated oculomotor palsy, regardless of the severity of the associated pain, the problem is not an aneurysm. A medical problem, usually diabetes, is indicated, and arteriography is not recommended, since diabetic ophthalmoplegia is essentially a mononeuritis, probably of an ischemic vascular basis.
The occurrence of chronic, recurrent headache with unruptured angioma and its relation, if any, to migraine is of importance. In patients with angioma who also had a history of migraine or attacks resembling migraine, both the aura (if present) and the headache were on the same side in all attacks, or the aura was prolonged or remained after the headache. Convulsions, focal signs on the contralateral side outlasting the headache, and a cranial bruit are among the neurologic signs of angioma. Auscultation of the skull should be a routine procedure in examining a patient with migraine. In the late stages of vascular malformation, the headache becomes constant and may be associated with subarachnoid hemorrhage, as in ruptured aneurysm with blood in the cerebrospinal fluid and coma.
We have also seen a number of older patients with head pains from temporal arteritis, carotid and basilar insufficiency, and glaucoma that simulate migraine, but we have not established any relationship between the migraine and these disorders.
Tumors of the occipital lobe can also stimulate migraine, causing headache, vomiting, and homonymous visual hallucination. Careful examination of the visual fields will usually reveal the presence of a persistent defect, which may increase in severity. The presence of focal neurologic signs, choked disks, and abnormalities in skull x-rays, electroencephalogram, brain scan, and cerebrospinal fluid aids in directing diagnostic attention to an intracranial mass and may establish the need for further diagnostic studies, such as arteriography or pneumoencephalography.
Muscle-contraction (tension) headache usually causes no problem in diagnosis and is clearly differentiated from migraine. Most patients with migraine, however, also have muscle-contraction headaches between attacks of migraine. These headaches have no prodromal signs, are nonpulsatile, and are usually located in the occipital nuchal region, but may cover the head like a cap or band. They may be accompanied or preceded by anxiety or dizziness.
Basic to the treatment of migraine is the understanding that we are dealing with a chronic disease that may well represent a lifelong problem. Short-term approaches are usually at best a temporary help and are doomed to failure in most instances. A judicious mixture of longrange, structured planning and concurrent attempts at the relief of symptoms is needed, and development of a good physician-patient relationship must underlie the entire approach."5
Treatment of migrarne must begin with a consideration of the patient as a whole, not just his symptoms. First, precipitating or provoking factors, which vary from patient to patient, must be ascertained and alleviated. Overloading environmental demands or emotional tensions, which are often specific, and fatigue should be avoided. Physical disturbances (dentai, allergic, metabolic) should be eradicated. The most effective treatment of the disorder is a dual approach using pharmacotherapy and psychotherapy.
Pharmacology of the acute attack. In mild attacks, the commonly used analgesics are sometimes effective if administered early enough. Most patients, however, have severe pain, for which ergotamine tartrate is the most effective drug. It can be given by inhalation or by the sublingual, oral, rectal, or parenteral route. For treatment to be effective, the drug must be administered early and in adequate dosage. Caffeine may be combined with ergotamine to increase the vasoconstrictive effect of the latter and enhance its action. In our experience, this combination reduces the amount of ergotamine necessary to control the attack. Antispasmodics and antiemetics help control nausea and vomiting.
Habituation to ergotamine can occur in patients who use the drug daily in increasing dosage over long periods. These patients usually have underlying depression, and for them withdrawal of the drug and management of the depression form the basis of treatment. The physician must watch for ergotism, which is a rare but dangerous side effect of ergotamine therapy.
Prophylaxis or interval treatment. Wim few exceptions, results of drug prophylaxis have been found to be about the same regardless of the drug prescribed, and few drugs have more than a placebo effect. Methysergide (Sansert®), a serotonin antagonist, is effective in reducing the frequency and severity of migraine. Side effects include abdominal discomfort, muscle cramps, depression, and peripheral vascular and fibrotic reactions (retroperitoneal and pulmonary).17 It should therefore be used only in migraine refractory to other therapy. After six months the drug should be discontinued for two months before therapy is started again. The recommended dosage is 6 mg. daily, but this varies with the patient.
Barbiturates and the minor and major tranquilizers are of temporary benefit in migraine prophylaxis, reducing anxiety and improving the patient's ability to handle stress. In migraine with underlying depression, antidepressants are the treatment of choice. These include the benzodiazepine derivatives, imipramine hydrochloride (Tofranil®) and amitriptyline hydrochloride (Elavil®); dosage is 75 to 150 mg. daily. There is a tendency to overuse psychotherapeutic drugs, both in dosage and in duration of treatment. They should be used as an adjunct to psychotherapy and discontinued as soon as the patient can do without them.
Psychotherapy. Since psychologic factors are the most frequent précipitants of a migraine attack, the treatment of psychologic problems is of major importance. Many migraine patients exhibit the same qualities of anxiety and depression that characterize the sufferer from tension headache or any number of other functional complaints. However, psychotherapy rarely changes the natural course of the disorder. The physician who is not a psychotherapist should restrict himself to supportive therapy, guidance, counseling, and situational insight. He may be able to provide symptomatic relief by altering behavior patterns and emotional responses to life situations. It is unlikely, however, that he will be successful in changing a hard-driving, compulsive, successful migraine patient into a "take it easy" personality. Interpretative insight and long-term intensive therapy for the migraine patient, with or without a severe neurosis or psychosis, should be left to the qualified psychiatrist.
Autoregulatory techniques. Biofeedback has been used for over 20 years but was named only five years ago; it has been investigated and applied clinically as a promising therapy for treatment of migraine. Biofeedback is a technique of instrumentation to give a person immediate and continuing information concerning changes in a body function of which he is not usually aware, such as blood pressure, brain wave activity, or muscle tension. The purpose of biofeedback is to use the information to bring a degree of conscious control to body systems usually thought to be under involuntary innervation.
For migraine headaches, the technique used at some medical centers is called "autogenic feedback training," which consists of temperature feedback combined with such relaxing autogenic phrases as "I feel very quiet," "My head is getting cool," and "My hands and arms are warm." Temperature sensors placed on the forehead and hands lead to a meter that registers in a manner immediately visible to the patient the temperature difference between hands and forehead. By warming the hands and at the same time cooling the head, while repeating a list of autogenic phrases and watching the temperature sensors, the patient can train himself to relax mentally and to control the muscle tension and blood flow in various areas of the extremities and head. After a month's practice, many patients no longer need the feedback device and are able to increase hand warmth or relax taut muscles without objective demonstration of success.
Present evidence seems to indicate that some patients can effectively improve their headache states in this way, while others are not helped at all. Adequate control studies are difficult to do, follow-up studies are not yet available, and no comparisons have been made with other autoregulatory techniques or treatment methods. However, the method is worth a trial, particularly in the younger migraine patient, as effective therapy usually becomes increasingly difficult the longer the migraine has persisted.
Related to biofeedback are a variety of other relaxation techniques, including those espoused by followers of Zen, Yoga, transcendental meditation, progressive relaxation, and hypnosis. Hypnosis has been used as a relaxant technique that must be continued by the patient through autohypnosis, practiced faithfully a number of times each day in order to reduce tension. It does appear that some persons who practice these techniques secure temporary relief from their tension states. In all these techniques, however, the underlying factors that produce the tensions are not usually understood by the patient; so, rather than getting at the cause of the tension, a reconditioning or covering technique is used instead of insight and working-through of problems.
Acupuncture evidently works in some patients with chronic recurring headache, but I have seen many patients who have taken a series of acupuncture treatments with no relief at all. At this point, insufficient data, including control studies, make it wise to withhold judgment as to the degree of temporary or permanent success of acupuncture with headache patients.
Surgical procedures for relief of chronic recurring headache are usually not helpful or recommended; these include dorsal column stimulators and sensory rhizotomy.
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3. BiCkerstaff. E. R. Basilar artery migraine. Lancet 1 (1961). 15.
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5. Cumings, J. N. The relationship of serotonin and platelets in migraine. In Symposium on Headache and Migraine. Barcelona: World Federation of Neurology. Sept. 12, 1973. (In press.)
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12. FromnvReichmann, F. Contributions to the psychogenesis of migraine. Psychoanal. Rev. 24 (1937). 26.
13. KoIb. L. C. Psychiatric and psychogenic factors in headache. In Friedman, A. P.. and Merritt, H. H. (eds.). Headache Diagnosis and Treatment. Philadelphia: Davis, 1959, pp. 266-272
14. Ferenczi, S. Sunday Neurosis (1919). Further Contributions to the Theory and Technique of Psychoanalysis, Second Edition. London: Hogarth Press. 1950, pp. 174-177.
15. Friedman, A. P. The infinite variety of migraine. In Cumings, J. N. (ed.). Background to Migraine. Volume III. London: Heinemann. 1970. pp. 165-181.
16. Friedman. A. P. Migraine headache. J. A.M. A. 222 (1972), 1400-1402.
17. Graham, J. R., et al. Fibrotic disorders associated with methysergide therapy for headache. N. Engl. J. Med. 274 (1966), 359-368.