The bright lights of the brain extinguished one by one like lamps.
-James Joyce

He has seen but half the universe who has never been shown the house of pain.
-Ralph Waldo Emerson

As an aside, visual auras have been a topic of fascination for neurologists for a very long time. Visual episodes were noted in antiquity. More recently the famous neurologist-author Oliver Sacks has written extensively about them. Lewis Carroll, author of ALICE IN WONDERLAND, suffered from migraine and unusual visual distortions, heralding his headaches still known today as the "Alice in Wonderland syndrome". These visions were employed in his writings. He might never have been the creative person he was if not for his migrainous visions. Undoubtedly migrainous visual phenomena are behind the miraculous apparitions in Macbeth, and Ezekiel’s brilliant visions in the Bible, and, very likely a good number of other religious hallucinations. We owe it all to migraine. To be fair, some of these visionaries had good imaginations, some had other disorders causing visual distortions and hallucinations such as schizophrenia, but some were migraneurs.

An aura may be non-visual. Some migraine sufferers lose the ability to talk and become confused or there may be numbness on one side of the body or weakness. A migraine attack can simulate a stroke. Very rarely, and this is most consistent with the theory that auras are really a sign of spasming blood vessels, visual or other neurological deficits may be permanent, in other words end in a stroke, or at least a permanent of semi-permanent deficit, something referred to as Complicated migraine.  Stroke-like abnormalities are frequently seen on MRI scans of migraineurs.  This makes eminent sense to neurologists who view the aura stage of migraine as being due to vasospasm.  Why couldn't vasospasm persist long enough to cause a stroke?  Some very rare familial forms of migraine predispose to stroke and are associated with hemiplegia and others with multiple MRI abnormalities.  Birth control pills and cigarettes are thought to increase the risk of stroke in migraineurs. But nowadays we know that many strokes attributed to migraine in the past really have other causes, for example cardiac abnormalities forming emboli. The widespread use of trans-esophageal echocardiography (TEE) and recognition of hypercoagulable states have markedly diminished the number of strokes attributed to migraine. Cardiac anomalies such as patent foramen ovale commonly cause strokes. Still there is little doubt that migraine causes a slight predisposition to stroke, particularly in young women who also use birth control pills, the estrogen in those pills acting to promote coagulation.

Usually the aura, visual or non-visual, recedes as the headache phase starts. After the pain phase there may still be a dull ache and various psychological effects that persist. Some people have high energy, others feel demoralized. The whole process can be serious and even disabling. Some vague psychological phenomena don't precisely coincide with a migraine attack but may happen considerably before or after the headache. These  prodromes or postdromes happen considerably before or after the attack.

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Migraine Classification

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If a person has an aura, by which is meant a specific neurological event before that headache phase, that defines Classic Migraine. This is known nowadays more simply as Migraine with Aura. If there is no aura, then we are dealing with Common migraine, migraine without aura. While the Classic vs common migraines terminology has now been abandoned it reveals that neurologists feel more certain diagnosing migraine when there is a distinct aura, especially a visual aura which is so highly characteristic. The truth of the matter is that the vast majority of recurrent severe headaches (as opposed to neuralgias representing a distinct type of pain) are almost certain to be migraines in that they respond to anti-migraine drugs.

Rarely symptoms relate to the brainstem and may have vertigo and double vision even lose consciousness as a prelude to their headache. Typical pain is occipital or in the back of the head. Alterations of consciousness go with this headache type affecting the brainstem implicated in the maintenance of arousal but is a worrisome feature of this type of headache called a Bickerstaff migraine originally recognized in teenage girls. Other features of Bickerstaff migraine go with involvement primarily of the brainstem including clumsiness and gait unsteadiness.  Children sometimes get abdominal pain or cyclic vomiting. Rarely an adult may even have a stroke or some permanent damage done as a result of migraine. In other words there may be very significant manifestations apart from the headache designated Complicated migraine. Other related headache syndromes also seem to involve the brainstem. There is ophthalmoplegic migraine that implicates the oculomotor (IIIrd) nerve with ptosis and double vision that may or may not involve the pupil or sometimes the VIth or abducent nerve.  Any person who has these more worrisome symptoms relating to the brainstem or cranial nerves nowadays is going to be investigated with an MRI and MRA scans and bloodwork.at the very least. Interestingly, after investigation, we rarely find any abnormality.

Far and away the most frequent area of the brain affected in migraine is the occipital lobe which subserves vision. This is why migrainous visual phenomena are so prominent.  The term "ocular migraine" is much overused. The retina is truly part of the brain and migraine can certainly affect the retina except that the vast majority of visual symptoms in migraine implicate the occipital lobes and not the retina.

You can also have an aura, ordinarily visual sensations, without a headache. Why not? They may just have a vasospastic phase without pain ("migraine without headache" otherwise called acephalgic migraine). Persons with common migraine may have, in general, a far less typical attack than is described above. The headaches may not be on one side only and actually may be more like typical tension headaches

Another migraine type is Cluster.. This headache type is in men perhaps nine tenths of the time and is named because headaches tend to happen in clusters or groups over weeks to months and then the problem becomes inactive for a time, possibly years. Clusters are more common in women than previously thought. The pains are around or near one eye, the same eye within a particular cluster of headaches. There is no pounding. Instead the pains are lancinating or like a knife blade being driven into the eye, associated with tearing of that eye, almost as if there is an irritant or allergy, hence an old designation: Horton's Histamine Headache.  An abandoned treatment, histamine desensitization has seemed to benefit some sufferers. Clusters are sometimes associated with a Horner Syndrome, sympathetic denervation of the affected eye, miosis and ptosis during the headache attack or even  between attacks. This suggests that a structural lesion. So-called Raeder's paratrigeminal neuralia is an old medical concept implying that a lesion should be searched for about the cavernous Carotid artery.  Many patients were subjected to angiograms in the past, today to MRA investigations. Typically nothing is found. Rarely have I seen clusters occur in in non-smokers. We don’t know if smoking cessation will eliminate them, except to say that I've had patients who quit smoking without headache remission. Alcohol often sets off a severe cluster headache during the 6 to  8 or so weeks that clusters typically occur.  Otherwise once a particular cluster of headaches has run its course alcohol may not do anything.  One fascinating phenomenon associated with cluster headaches is that some fellows will have headaches at an exact time, for instance exactly 90 minutes after falling asleep. This implicates hypothalamic regulatory mechanisms providing support for the concept of an internal clock. Clinicians who see cluster headache sufferers have the distinct impression that something must be awry about the eye or in immediate retro-orbital region about the pituitary or hypothalamus or paracavernous region, but no one has been able to prove anything. Some cluster sufferers are hard smoking and drinking aggressive men with swarthy complexions so that one gets the distinct impression again that something may be off in the limbic system or hypothalamus in the brain. The old literature spoke of men with so-called leonine facies,  but some fellows seem to be just ordinary persons. 

Treatment of clusters is somewhat different than migraine but with some overlap of medications. Are clusters migraines or not? I feel they may be more of an "extreme migraine" as I've certainly seen a lot of people who have either both types of headaches or whose headaches seem to be a cross between the two headache types. With so many patients benefiting from triptans  and ergots in both headache types there is little reason to put the headaches into two completely separate categories. Many patients suffer needlessly because of being prescribed second line drugs which don't work for them instead of using triptans or Oxygen therapy which have a higher likelihood of success. Some persons develop chronic clusters which don't remit within weeks and pose a difficult problem. Like migraineurs, there is often "more than meets the eye" in terms of psycho-social issues. So-called Paroxysmal Intermittent Hemicrania characteristically is somewhat like a cluster headache with headaches happening more frequently throughout the course of a day and these more often occur in women. These are characteristically miraculously responsive to NSAID's but especially Indomethacin.

Some persons have highly paroxysmal headaches happening only with sexual intercourse or  even orgasm.  This headache type occurs and then seems to remit on its own out of the blue, designated benign coital headaches. It is more typically a sudden severe non-pulsatile pain that doesn't last very long and often there is more concern about its affecting sexual response. Everyone thinks of an aneurysm because some subarachnoid hemorrhages occur during sex.  It is important to take take this headache type seriously especially when evaluating the first such instance in an emergency room. Valsalva, ecstatic straining  or another mechanism may be responsible for the rupture of an intracranial aneurysm.  Since CT does not exclude SAH entirely,  a lumbar puncture is often required. CT will show evidence of Subarachnoid hemorrhage about 95% of the time if gotten just after the bleed. But the sensitivity falls off rapidly thereafter so that by day 3 the accuracy falls to about 80%. Lumbar puncture will show yellow spinal fluid (xanthochromia) nearly 100% of the time for up to 2 weeks after the hemorrhage. Lumbar puncture should be considered seriously in anyone having their first unusually severe headache, persons with "thunderclap" sudden headaches, those with subacute progressive headaches, association with fever confusion, meningismus or seizures or if high pressure is suspected (papilledema on eye exam) or those where a specialist suspects low pressure. Data from a spinal tap can also confuse the clinician,  if the fluid is bloody due to a traumatic tap. We tell residents who obtain bloody spinal fluid to go and spin the fluid immediately to determine if there is xanthochromia.  One  rare error in an ER is to give a triptan agent which may work temporarily then assume the headache which is really due to a subarachnoid hemorrhage is a migraine. Triptans shouldn't be used to make a diagnosis.  Where a pattern of such headaches has developed in an individual they are likely benign. The vast majority of such headaches are not serious.  Again, many of them seem to respond to NSAID's  or ergots and the problem seems fortunately to be short-lived. Some observers have related such headaches to obesity and poor physical condition.

In Summary a Migraine Headache has some or all of the following features: Duration of 4-72 Hours, Unitlateral Throbbing Pain aggravated by movement of moderate to severe intensity with nausea or vomiting, photo/phonophobia. There may or may not be a neurological Aura.. 

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The older vascular model  of migraine is most useful as a conceptualization of migraine phases. A migraine attack has three phases best defined by the status of blood vessels during a particular phase. First is the aura phase, which, as we have seen, not everyone has. At this time, arteries constrict (vasoconstriction). Some of the brain may not get a sufficient supply of blood in this phase and that may be the for the focal symptoms which are part of the Aura phase. There will be a neurological deficit of some kind. In some instances and typically in the visual area in the back of the brain, Brain tissue that is not getting enough blood reacts by being more active electrically. (Tissue suffering from severe oligemia will in due course become less electrically active and die) This is because lacking a sufficient energy supply, cells have more trouble controlling the flow of electrically charged ions and the tissue becomes hyper-excitable (depolarize). That is probably why some people have visions or distortions. Most persons just have negative visual phenomena areas in their field of vision of relative blindness (scotomata).  For all intents and purposes aura = vasospasm. Nowadays this vascular model is looked down upon in favor of more pharmacologic physiology, but it is still the best way to conceptualize the phases of a migraine attack. It may be that a focal diminishment of cerebral metabolism occurs first and that blood supply is only later reduced in the back of the brain.

Some older investigations relate these vascular stages to Serotonin. In the first or vasospastic phase of the headache, there is an overabundance of Serotonin. Some of this serotonin seems to come from platelets, blood clotting elements in the blood that contain a lot of it. Serotonin can act as a vasoconstrictor - a chemical that promotes vasoconstriction. Nowadays we know the story is more specific as will be described below.

The second phase of a migraine headache is the pain phase. At this time arteries dilate. Cranial arteries are pain sensitive structures whose walls contain tiny nerve endings that go into the trigeminal nerve. As the pulse beat goes through a dilated sensitive artery, you may feel a pounding severe headache.  That is why, classically, the aura ceases as you get into the pain phase, not always, but most often. This pain is often accompanied by nausea and vomiting defining a so-called, sick headache.

Almost imperceptibly the headache attack marches into the third or inflammation-muscle contraction phase. In this third phase, pain becomes more diffuse. There are inflammatory chemicals and cells, (white blood cells) that come into the nervous system and covering of the brain, the meninges. There is photophobia, fear or avoidance of light. The person wants to be in a dark room. There can be a clouding of thought or mental functioning and prolonged persistent misery. In truth, photo and phonophobia are not phase specific in migraine. Avoidance of stimuli does not only apply to the pain phase of a headache. A good many migraineurs avoid all sorts of stimuli especially light but even loud noises and smells even while not in the throes of a headache. So some of them betray an overall hypersensitivity to stimuli.

For some persons irritation and pain can persist even for days while the classic blood vessel spasm only lasts a much shorter time. Spinal fluid may show a sterile or aseptic meningitis pattern. consistent with inflammation.. The signs of this meningitis include irritation from light, the photophobia and neck stiffness. The stiff neck also may be due to anxiety that tightens neck muscles.

Some persons have yet a fourth phase, a post-headache phase during which there are temporary psychological changes. Some feel a sort of release, some fatigue, and for others elation. Connected with a full-blown migraine attack is a host of chemicals that scientists have only recently started to detect and define. Some persons also have a prodrome, a non-specific psychic premonition or alteration of brain function that may herald a headache though occurring well before a clinical headache attack, so in essence there can be a pro or postdrome.

It is these three phases, the aura, the headache and post headache period and the discreet attacks of headache, ordinarily a pounding headache, worse on one side of the head, that distinguish migraine from other forms of head pain. The symptoms alone allow the doctor to make a diagnosis.

PHASES OF MIGRAINE ATTACK
I. Aura (vasospasm)
II. Headache (Compensatory Vasodilation)
III. Inflammatory-muscle contraction
IV Post-headache symptoms

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Headache is rarely a sign of more serious disease. It happens that the brain itself is insensitive to pain. What is sensitive includes the blood vessels inside the head that are invested with nerves that transmit pain signals and various other parts composing the skull, the muscles which can contract near nerves and cause intense pain, and the meninges a three layered covering of the brain and external structures such as sinuses and facial tissues and sensory nerves. All of these can respond with pain.

You can relax about a headache being a sign of a brain tumor as well. A brain tumor ordinarily does not produce lot of pain unless it begins to affect other structures that are not part of the brain such as the meninges or blood vessels because due to the tumor's tugging on these other structures. The pain of a brain tumor is ordinarily more constant and less paroxysmal. Classically brain tumor pain is worst every morning. As Carbon dioxide levels are allowed to rise in sleep, and also with recumbancy, the intra-cranial pressure rises. Some more serious problems such as bleeding from an aneurysm or weak arterial blood vessel, and inflammations such as meningitis, may cause pain but not in the form of recurrent attacks such as those we experience with migraine. This means that persons with severe painful attacks only rarely have anything that is life threatening. In fact when we do tests and scans to look at the brain, in a person with typical migraine, which today means a CT scan, or an MRI scan, we almost never find anything abnormal within the head. A lot of people ask about migraines and aneurysms. Aneurysms themselves actually are fairly common, but they seem to be no more or less common in migraneurs. Most aneurysms cause no problems or pain unless and until they burst and cause sudden bleeding in the head. As it turns out, only rare aneurysms rupture.  Aneurysms, it goes without saying, are better diagnosed earlier than later so that they can be clipped or subjected to intravascular coiling.  Subarachnoid hemorrhages occur in people with migraine and people without migraine. Migraine headache is not a sign of a brain hemorrhage.

As it happens this is a complicated issue. I hear stories about people who'd complained to their doctor for years about head pain (why didn't the doctor take them seriously?) then had a big brain hemorrhage which left them dead or disabled. Unscrupulous malpractice attorneys love these cases. They know as we do that the preceding headaches had nothing to do with the subarachnoid hemorrhage (SAH), that the person who bled just happened to have another very common condition, namely migraine.  Migraine is common and neither increases nor decreases the likelihood of  having a subarachnoid hemorrhage. Under some conditions even a careful clinician can be fooled.  A person with migraine may come into the emergency room with a headache not substantially different than their previous pains and turn out to have a bleed.  Sometimes a difference in their head pain syndrome might signal that something else is wrong  (medical jargon- raises the index of suspicion) but not always. The doctor is blamed for the hemorrhage.

Every experienced neurologist has had rare patients whose serious disease, typically cerebrovascular disease, is heralded by sometimes very typical migraine or vascular headaches. I have seen patients already with brain hemorrhages and bland strokes who had migraine headaches sometimes well before a life- threatening cerebrovascular event.  I suspect that vasospasm plays a critical role in these patients particularly when they have hemiplegic or focal episodes.  Since there are so few of them, and migraine is so very common, I cannot determine with any degree of certainty whether the cerebrovascular episode, be it hemorrhage or infarct, more frequently infarct, was a chance association. There must be some patients who are genetically predisposed to severe vasospastic events, whose syndromes we have yet to define completely. I exclude obvious associations such as cerebral vasculitis, dissections, cocaine and ephedrine use and other phenomena because there are some few patients who have none of these well defined entities. I again emphasize that the vast majority of migraine headaches are not life-threatening and are not harbingers or hemorrhages or strokes but a few migraines are. The differences between benign and serious headaches are not always patently obvious.

Certain ominous headaches can predispose to brain hemorrhages and are worth mentioning. Uncontrolled hypertension leads to strokes and brain hemorrhage. The patient and physician need to join in a cooperative effort to aggressively manage hypertension. This involves drugs with side effects lifestyle changes and meticulously following blood pressure. Patients neglect hypertension at their own peril. Patients on Coumadin (warfarin) with new headaches need a CT scan. Infrequently certain brain lesions are apt to bleed such as arteriovenous malformations. AVM's may present with a combination of migraine like headache, seizures and cerebral bleeds or any combination of this triad but quite a few are assymptomatic.  Here you have to consider genetic disorders such as Von-Hippel Lindau disease.  Rarely aneurysms may start with a minor bleed a sentinel hemorrhage, a warning of the big bleed to come. The early headache from an aneurysm is sometimes described as "the worst headache of my life" something that sticks in doctors minds when they evaluate headaches but it isn't all that useful.  Many SAH headaches aren't that bad, and as we've seen migraine headaches can be very severe, but there is often, not always,  something else, neck stiffness, alteration of consciousness, other classic signs and in about 90% of cases an abnormal CT scan.  Some patients with SAH have none of these signs. The problem may go unrecognized, there may be an adverse outcome and a malpractice event for the hapless patient and doctor. Old folks with new headaches usually have other symptoms but we always consider temporal arteritis which is just about always excluded with a normal sed rate. That is an important diagnosis because it may produce visual loss.

Another headache which resembles migraine happens from low pressure. That causes a tugging on meninges and blood vessels when the person stands. The key to diagnosis is a postural orthostatic headache that remits when supine and occurs with standing very much like a post-spinal headache complicating lumbar punctures and spinal anesthesia. The head can pound and there is often photophobia. Unusual sensations about the head and neck are described. Some patients have congenital abnormalities or very rarely post-traumatic spinal fluid leaks which happen spontaneously. Some call this Schaltenbrand Syndrome.  The problem can be terrible and last for weeks to months. Treatment other than fluid and caffeine is to try to identify the site of the leak which is a tall order.  Radionuclide cisternagrams,  and/or neuroaxis MRI's may have to be done and then to neurosurgically close the leak. Many cases show meningeal thickening and enhancement on MRI.  Some few patients respond to blood patch treatments. So it is obvious that conditions causing traction or inflammation in extra-cerebral tissue, especially meninges and blood vessels, can mimic migraine headache. The headache of pseudotumor can be similar to migraine. In pseudotumor which usually occurs in overweight women, intracranial pressure is high because of diffuse interstitial brain edema. Pseudotumor  is a  brain tumors mimic because disc swelling, papilledema is noted, yet there is no tumor is otherwise known as benign intracranial hypertension.  Repeated lumbar punctures, acetozolamide, Prednisone and weight reduction are the mainstays of therapy.

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Erratic eating and sleeping patterns particularly and depression interact with headaches. It is very hard to convince most people about this but it seems the common habit of not eating almost at all during the day, then consuming most of your calories at night, (an obese eating pattern) may bring on migraine headaches. In migraneurs it is very common to find something that is unbalanced in their lives. There may be not enough exercise or often, given some quirks in personality, too much. There is a great debate in medical circles as to personality types and migraine. Every experienced clinician can attest to the fact that most often refractory migraineurs have psychosocial incongruencies or dietary or sleep indiscretions or a generalized ennui or disgust .with life. As with all chronic medical disorders one can make some association with depression. The question of whether depression is the cause or the effect of migraine (chicken or egg) as with so many other disorders has yet to be completely resolved.

Figure 3: Serotonin triad. Sleep disorders and depression occur disproportionately in migraine patients.

Psychological factors predominate in transformed migraine. In transformed migraine a person, typically in her 40's may have started out with typical migraine attacks while young. The headache morphs into a chronic daily headache situation.  The sufferer has  almost constant pain, is rarely pain free but also continues to have severe disabling headaches. Often they are using large quantities of marginally effective medication, bottles of Excedrin or Fioricet which give some minimal low grade relief.  Because they don't take the headache away,  and especially given the terror of having a full-blown attack of pain, these medicines tend to be used extensively.  The mechanism is in part incomplete headache relief compounded by phobia about a severe pain attack.  These folks just have to have something to treat their severe pain else they will die or so it seems to them.  It's as hard as anything to convince them to stop taking Excedrin, Fioricet or Midrin, to taper or stop these drugs.  Lo and behold, with a lot of patient explanation, if they ever do stop these prn or as needed drugs they find miraculously, nine times out of ten, their headaches are much better. Sure they have some headaches, but not every day and not nearly of the magnitude they had while abusing their drugs, withdrawing from them and overdosing at the same time. It would seem that their headaches change due to medicine overuse, but this is not obvious from the evidence and there is quite a bit of debate as to whether medicine overuse is the cause of transformed migraine or whether there is a biological tendency in some persons for their migraine attacks to spontaneously change over time to become less floridly paroxysmal attacks and morph into a constant pain syndrome. We see some folks who never abused medicines and yet have changed their headache pattern or who follow directions to stop using prn drugs yet still are refractory.  When you consider it this process occurs in a good number of diseases e.g. MS converts from relapsing remitting (i.e. involving discreet attacks) to a "secondary progressive" form, asthma converts from an attack mode in youth to a type of chronic COPD with constant shortness of breath. So the conversion of Migraine from the attack mode to chronic daily headache or transformed migraine may give testimony to the inadequacy of early treatment. Regardless their are profound accompanying psychological issues which may be either cause or effect but which have to be dealt with nevertheless.

Figure 4: The chronic daily headache, analgesic abuse cycle.

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So what can be done? Ideally there is a combined approach to migraine involving physical and emotional modalities. It is critical to identify all factors that might be exacerbating headaches. On a routine office visit we usually prescribe medicines that ameliorate the symptoms. It is not possible to mention all of the medicines used to treat migraine because of their great number but here are some of the highlights.

It's important to point out too that some of the most effective approaches to migraine don't involve drugs. Lots of folks with intractable headaches could control their situation better by taking control, most especially organizing their lives. A tremendous number of refractory migraneurs lead disorganized lives, tortured by children, husbands, boyfriends, bosses, juggling one or more careers and home life with haphazard eating and sleeping habits. As a first step I advise people to organize their lives and stop beating on themselves. Regular sleep and eating schedules are advised, rather than the binge and purge haphazard patterns that some people think they can get away with, regular exercise and decreasing caffeine which seems to up-regulate migraine hypersensitivity.

There are basically two groups of medicines, those that prevent the headache and some used to treat the headache attack itself.  (Prophylaxis vs. prn use.) Actually there's a third category in pre-emptive treatment in those few persons who are aware of specific times or triggers in their pattern of pain.  In the prn category (as needed) narcotics are to be avoided, if possible. True, they are effective over the short run, but they tend to sedate and may be habit forming especially for a frequent or chronic headache. And Narcotics cause nausea. Narcotics fail to address the headache mechanism. They are useful to stave off severe pain as an emergency measure. Demerol injections are used in the emergency room.  Many migraine combinations contain an agent that causes vascular spasm. This helps the pain phase of the headache. Caffeine and ergot derivatives (Ergots affect Serotonin) will cause some constriction of blood vessels during the pain phase. Caffergot, Wigraine, Ergomar, and Bellergal are some proprietary ergot  preparations from the past, very rarely used now.  DHE-45, heretofore available by injection only, has been released as a nasal spray, Migranal.  It is a very effective anti-migraine drug and an Ergot derivative. Ergots taken to excess can increase nausea and cause profound blood vessel spasm with tingling in the extremities and even blue or cold fingers and toes, or in rare cases angina or a heart attack ("ergotism"). for that reason we don’t like to use ergots in smoking men of a certain age. Ergots and vasoconstrictors are contra-indicated in older patients prone to angina, in persons with high blood pressure and in pregnant women.

Midrin is a mild combination that also causes blood vessels to constrict. Sometimes it is effective for milder headaches and in children although it is a large pill. Over the counter medicines almost always contain caffeine. Caffeine has been shown to increase the effect of analgesics such as aspirin, ibuprofen and acetaminophen (Tylenol). Along with narcotics we avoid Fiorinal, and Fioricet. As mentioned these drugs fall into the minimally effective category and very often patients end up using them in large quantity such that they almost seem to be addicted to them. They are not physically addicted but just emotionally dependent due to extreme fear of having a very bad headache. The fact that these preparations are only incompletely effective actually contributes to their abuse potential. Patients get into desperate situations where they use the drugs up then end up prevaricating with their physicians or even more than one doctor at a time.

NSAID's play some role. Among them, Naproxen is the most extensively used. They work some of the time, mostly for milder headaches and in children. By the time persons are seen in our practice, they are mostly long past the NSAID stage.

Before we get to triptans it's important to distinguish "step care" with "stratified care" and here I am borrowing from the lucid discussions of Dr. Stephen Silberstein. In Step care you try first the least expensive agents and see if they will work before embarking on more costly drugs. This is what the HMO's would have you do because they don't wish to pay for the more expensive agents. The problem with step care is that you'll end up trying and failing with a lot of patients, particularly the migraine patients who visit a neurologist, often some of the worst migraine sufferers. The other approach is to make a reasonable estimation about which persons are going to respond to what agents, so-called stratified care. Persons who have more severe headaches, who are more significantly disabled, possibly on the basis of MIDAS scale above or other criteria, ought to be started on triptans right off the bat and more effective preventive meds too and not bother with less effective drugs such as NSAID's.

It is important to address the issue of nausea and vomiting. That can impair the absorption of an oral medicine. Reglan and other antinausea drugs can be given or sometimes medicine can be used in suppository form. Other drugs are administered in other novel ways, under the tongue, via inhalation or intra-nasally.

Intravenous Depacon (valproate) has been a boon to the treatment of the acute intractable migraine headache and at this stage seems to be underused in emergency rooms. Toradol is also sometimes effective though less so in emergency room situations. Parenteral DHE still seems to be a very effective emergency room drug given with IV Reglan. Fentanyl transmucosal (Actiq) has recently been used with some refractory migraine sufferers to decrease emergency room visits, but it is a narcotic of course and needs to used very cautiously.

Other agents are used to prevent or prophylax migraine. They have to be taken as a regular dose daily and thus are only recommended in persons with frequent or very disabling headaches. If there are more than three significant headaches a month they are probably worth using. Some tricyclic antidepressants are of value especially when there is an accompanying sleep disturbance or depression. These include among others amitriptylene and nortriptylene. . These are old medicines and we now have a whole class of anti-depressants that act specifically on Serotonin, the selective serotonin reuptake inhibitors, called SSRI’s. These drugs block the neuron releasing serotonin from taking it up and degrading the chemical, in essence prolonging serotonin’s effect. They have much fewer side effects than older anti-depressants. We say they have a cleaner effect, because they act so much more specifically on serotonin itself, and less on other transmitters as the older medicines tend to do. Examples are Prozac, Paxil, Zoloft and others.

A lot of doctors use SSRI's for migraine prophylaxis, not because they are treating depression (these are all antidepressants) but because they want to affect serotonin. The problem is that these drugs most of the time don’t seem to work. In some persons they may even increase headaches!!  In situations where they do work, they seem to do so because the headache is a "depressive equivalent", an symptom accompanying depression. The whole topic is very tricky. Part of the confusion might relate to the fact that serotonin has different effects in the various phases of a migraine attack, also that there are so many responses that serotonin affects, so many different serotonin receptor types as we’ve discussed above. On the other hand some of the older antidepressants have a more "tried and true", reliable, anti-migraine affect. These include the older drugs mentioned above as well as nortriptylene, and trazodone which also have less side-effects.

The issue of migraine prophylaxis is in a state of flux. There is no way to obliterate migraine or eliminate it entirely. It's said that on average two thirds of patients may have a 50% reduction in headaches. Patients should not expect too much.  All too often a breakthrough headache leads patients to discontinue effective preventive drugs. Neurologists typically see patients who have "tried" long lists of medicines at the hands of family doctors. When questioned further we almost always find that they've used inadequate doses over a short length of time, that medicines weren't truly given a clinical trial at all.  As a general rule if a drug hasn't been given for at least two to three months, it has not been proven to be ineffective. The biggest problem with preventives is their high rate of being prematurely abandoned. Then we have a terrible time trying to convince the sufferer to take the medicines in a way that will be of help. There are now available good review articles comparing the effectiveness of various preventive medications. Two of them are NEJM 346:257, 2002 and Neurology 55(6):754.  Beta-blockers are effective. The most tried and true are propranolol, and Timolol, metoprolol. Calcium Channel blockers especially verapamil do work for a lot of patients but the consensus is that while they are better tolerated with far fewer side effects, they are somewhat less effective.  Beta-blockers don't seem to be effective in cluster headache whereas verapamil in doses up to 860 mg may work for cluster headache. Prednisone and Lithium also are specifically used for cluster headaches. Triptans and ergots are underused for clusters probably due to fear of vasoconstriction in middle aged male smokers. Tricyclic agents, amitriptylene is the most tried and true, seem be be effective especially where there is accompanying sleep disturbance and in children who also seem to respond well to Inderal providing they do not have asthma. NSAID's I have not found to be too effective. They may work better in children and in persons with less severe migraine headaches.  Indomethacin particularly may have a miraculous effect for paroxysmal intermittent hemicrania and hemicrania continua. Methysergide is rarely used anymore and is a drug of last resort because it can cause retroperitoneal fibrosis. Many authorities recommend that he choice of prophylaxis be based on co-morbidities. For instance if depression or a sleep disturbance accompanies migraine, a tricyclic agent might be the best choice.  If there is hypertension, beta or calcium channel blocking drugs may be of value.

There has been limited data over the years about simple measures use of Magnesium and Riboflavin or other B vitamins in migraine and we've had some few patients who have seemed to respond to these measures and they are definitely worth trying. Riboflavin recommendations seem mostly to stem from a small study by Schoenen (1998) of some eighty Belgian patients. The treatment group used 400 mg of riboflavin had a modest benefit. I don't know that there are any studies confirming these results. Magnesium is available in many forms and without prescription. Dosages betw 6 and 10 mg/Kg/D are sometimes recommended. However, I've yet to see very convincing data on Magnesium replacement.

Figure 5: Classes of foods affecting migraine

Long lists of foods which affect migraine have been published. These include foods rich in Tyramine (red wines, cheeses, chocolates), and nitrites that dilate blood vessels, and even MSG has been implicated, also Nutra-Sweet (Aspartame) in a few instances. A lot of people ask about Caffeine. Caffeine is important only if the dose is varied a lot but it does not seem to be a factor if used regularly. I have seen some rare patients who cured of their migraine when "dried out" from caffeine. This is rare, but it’s important to know that some people who cease using caffeine entirely may stop having headaches. Honestly this may be more connected with jettisoning other non-healthful habits along with caffeine. Caffeine causes vasospasm, and vasodilation as well in different arteries. Constant use may in some way, "up-regulate", increase the sensitivity, of muscular artery walls. Caffeine and smoking cessation are worthwhile considerations in chronic migraine.

My experience over the years is that these lists of foods play only a minor role. These specific foods are important only in selected individuals and they are few. It doesn't make sense to go crazy eliminating certain foods. Dietary changes are less emphasized now than years ago. Again, non-healthful dietary habits which are part poor habits in general seem to play a role, rarely specific foods. Dietary indiscretion, non-regular consumption of food, binge eating and starvation, erratic sleep contribute to headaches.  Moderation and regularization of eating, relaxation, and exercise is critical. This figures in with the personality profile of migraneurs. Sometimes they seem to enjoy delayed gratification, periods of self-denial, which are variably followed by binges. They need to regularize and moderate their lives.

Modern clinicians don't emphasize the categories of food as given above as being migraine trigger. In fact phenomena such as chocolate craving are thought of as part of an aura or prodrome of a migraine attack, far from being a trigger of a headache, viewed as more of a sign that a bad headache is about to occur.

Modalities such as counseling, biofeedback etc will be important. Migraine is a disorder with a broad spectrum. For some just prescribing the right medicine will have a dramatic effect, but for other situations the approach has to touch the soul as well as the body.

If you suffer from migraine, I would urge you to look at more than medicines that can be used to stave off or decrease pain. A multi-faceted approach may involve some soul-searching, counseling, changing one’s life-style, which is difficult to do but well worth it. Some kind of counseling may well be in order in refractory cases and the active participation of the sufferer which may not be so easy. A migraine "check-list" follows below incorporating non-medicine related factors that are well worth looking at.