3
The Primary Headache
Syndromes: When Is a Headache
“Just a Headache?”
The primary headache disorders account for the vast major- ity of the episodic and chronic head pain that is so perva- sive in our society. Unlike the secondary headaches described in the last chapter, these primary headaches do not point to- wards the presence of serious, underlying disease. Even so, the financial, physical and emotional cost imposed by these disorders is staggering, and it is no exaggeration to state that migraine in particular has been and remains a chronic
45
public health care emergency. In this chapter, we will begin our effort to provide the reader with the education and tools he or she requires to cope more effectively with migraine, the intimate enemy.
Migraine
Let’s get to the most likely cause of your headaches.
First, there is no such thing as a “regular headache.”
Studies have shown that 90% of all chronic, recurring headaches are either migraine or tension-type head- ache, and we thus would do well to understand the na- ture of those disorders. There are literally hundreds of other causes for chronic, recurring headache, but only 10% of cases involve one of these causes. Most of these other causes are either secondary headaches (see Chap- ter 2) or other rarer primary headache disorders, such as cluster. Studies show that patients try nearly 5 treat- ment options before finding one that works, and spend, on average, 3
1⁄
2years to find that successful treatment.
One reason is that often the correct diagnosis is not made.
While tension-type headache is more prevalent in the
general population, migraine is the most important pri-
mary headache disorder and the most common cause of
headache in patients presenting to a clinic or ED with the
chief complaint of head pain. In the past, we divided mi-
graine into two types, classic and common, based on the
presence or absence of aura symptoms; those terms now
have been replaced by migraine with aura and migraine
without aura.
Epidemiology
It frequently comes as quite a surprise to patients when they are told they have migraine. Studies have shown that there are more than 21 million migraine sufferers in the United States, but only 11 million have been diag- nosed; thus slightly less than half of all migraineurs wan- der about ignorant as to the source of their recurrent headaches and are either practicing benign neglect, pop- ping OTC medications for “sinus headache,” buying new eyeglasses or terrified they have a brain tumor. Perhaps reflecting the “machismo” underpinnings of our culture, the misconception that men do not get migraine or some other unidentified variable, men are less likely to be di- agnosed than women are. Regardless, the failure to se- cure an accurate diagnosis often results in failure to find effective treatment and thus, unnecessary suffering.
Migraine headaches most typically begin in child-
hood or the teenage years, often worsening until around
age 40 or 50 before they begin to improve. This pattern
is very different from most other physical afflictions,
which tend to increase as one advances to the “senior
years.” Migraine is especially prevalent during the years
that people ordinarily are most productive in their ca-
reers and are actively raising their families. As a conse-
quence, the disability imposed by migraine is costly. As
you read these words, over one million Americans are suf-
fering from an acute migraine attack that is inhibiting or
prohibiting their usual activities. Of these acute attacks,
over 60% will last 3 to 12 hours, and 12% will last longer
than a day. In addition to the personal suffering experi-
enced by the millions of Americans with migraine and
their families, migraine provokes more than 6 billion lost working days and 74 million days of restricted activity yearly. Americans spend 3 million days per month bed- ridden due to acute migraine. If you have migraine, you are likely to have many attacks; the average migraineur experiences three and a half attacks monthly. All of this translates to $17 billion annually in lost income and pro- ductivity and if you include the cost of caring for these attacks and their related health problems, the economic burden for society soars to almost $50 billion annually.
It is expensive to have migraine. Your missed work- days or impaired functioning during an attack mean that you may be perceived as a less desirable employee. When it comes to promotions and raises, people with migraine fare poorly; migraine sufferers have lower incomes than those who are migraine free.
Statistics on lost working days do not describe for us how migraine affects the quality of life (QoL) of those af- flicted. Experts now have tools to measure QoL data and have applied it to the migraine population. The results are astounding. The quality of life for those with migraine is poor even when compared to individuals with other chronic medical disorders that at first glance would seem more devastating; QoL scores are lower for migraine than for depression, chronic back problems, recent heart at- tack, and congestive heart failure. Only active AIDS scores worse.
Clinical Features
Fine, so migraine is common. And costly. And conveys
inestimable suffering. So what is “migraine” anyway?
Where does it come from? And, given the cold-hearted nature of natural selection, what if any evolutionary ad- vantage could be derived from a biologic condition that renders one intermittently disabled and vulnerable to competitors? All are good questions. Taking the last first, one can conjecture that man simply has not existed long enough for the dead branch of migraine to be pruned from the evolutionary tree. Alternatively (and more op- timistically for those of us who are migraineurs), one can envision that a tribe of Sioux living on the western plains would benefit from counting among its members an in- dividual or two whose brains were unusually sensitive and so attuned to imminent changes in weather, the ar- rival of a buffalo herds, and so on. What attributes this hypersensitivity would convey within our modern soci- ety is perhaps a bit more difficult to surmise.
As the biologic origin of migraine will be dealt with subsequently in this chapter, let us address the first ques- tion: what is “migraine?” The International Headache So- ciety (IHS) has provided clinicians and scientists with a dense ecuminium that describes in excruciating detail how we are to identify each of the multitudinous primary and secondary headache disorders. Remarkable for its brevity, clarity, and clinical utility is the IHS definition of migraine, and that definition is outlined in Table 3.1.
A completely developed migraine attack may in- volve four distinct phases: prodrome, aura, headache, postdrome.
Prodrome Many migraines begin with a prodrome
which often occurs well before the attack begins . . . even
the day before. If you can identify that you are experi-
encing a prodrome, you may be able to preemptively treat your attack. Prodromes are often very subtle, and unless the symptomatology is identified for what it rep- resents, you probably will not realize you have them. Pro- dromes commonly involve changes in mood. Depression is particularly common, but, interestingly, euphoria (a positive, happy mood) is even more common; ironically, many individuals feel particularly well before their overt migraine attack begins. Irritability and restlessness can occur, and yawning often heralds the onset of an attack;
we find that spouses are often more adept than patients in appreciating that yawning is going to lead to a head- ache. Other prodromal symptoms can include neck stiff- ness, which may be earliest sign of the headache to fol- low. Cold hands and feet are common prodromal symptoms. Migraineurs tend to have cold hands and feet even between attacks, and the extremities become in- creasingly cold during an attack. This clinical phenome- non is what led to the development of biofeedback (i.e., Table 3.1 IHS Diagnostic Criteria for Migraine
At least 5 attacks fulfilling the following:
1. Headache attacks lasting 4–72 hours
2. Headache has at least 2 of the following characteristics:
1. a. unilateral location 1. b. pulsating quality
1. c. moderate or severe intensity
1. d. aggravation by walking stairs or similar routine physical activity
3. During headache at least one of the following:
1. a. nausea and/or vomiting 1. b. photophobia and phonophobia
4. No evidence of “organic” disorder causing chronic headaches
teaching people to change the skin temperature of their hands) as a migraine treatment.
You already may be familiar with the food cravings and food aversions commonly associated with preg- nancy. Migraine sufferers do not want pickles and ice cream, but during a prodrome they may covet other foods. These cravings are neurobiologically mediated, and one food craving that has drawn particular attention is the acute desire for chocolate. Many physicians and cit- izens have assumed that chocolate triggers migraines, but
Yawning as a migraine prodome
several recent studies have thrown a monkey wrench into this long-lived belief. In those studies, the investigators found that when migraineurs were given chocolate ver- sus placebo, one could not predict who would develop an acute migraine. To be fair, however, other studies have suggested chocolate to be an actual trigger for some at- tacks in some migraineurs. So chocolate lovers, take your chances according to whose research you believe.
Aura Migraine with aura (previously “classical” mi- graine) involves the same type of headache and associ- ated symptoms that occur in migraine without aura, but, in addition, some type of specific neurologic symptoma- tology. Only approximately 20% of migraineurs ever ex- perience an aura, and only a very few always will have an aura with their migraine attack. Although auras most often precede the headache phase of the attack, some mi- graineurs report that their auras occur at peak headache intensity. Most auras last 30 minutes or less, but occa- sionally the neurologic symptoms will persist for hours or, in rare cases, for weeks or months.
Case 3.1
A 17-year-old boy reports that while playing baseball in the
bright sunlight he suddenly develops a shimmering light on
the right side of his vision. Over the next 20 minutes, the
area of flashing light enlarges, and he notes that he is un-
able to see the left side of his coach’s face. Although he is re-
lieved when his visual symptoms resolve after a half hour,
he then develops a pounding headache behind his left eye
and begins to vomit.
The most common aura symptoms are visual, but numbness and/or tingling on one side of the body, in- ability to find words or speak (aphasia), or an actual paralysis on one side of the body all may occur. Visual auras generally last about 20 minutes but may persist up to an hour or even, in rare cases, for weeks or months.
Their onset is generally gradual, with both “negative”
and “positive” symptoms evolving and then receding.
They often begin with a small blind spot or hallucination of vision (see Fig 3.1 for examples) which enlarges over 10 to 30 minutes before fading away. Patients under- standably may be concerned that the problem is in their eye, but that is rarely the case. The generator of aura, in fact, is located in the portion of the brain that subserves vision, and vision in both eyes usually is involved. When you look out into space during a visual aura, you likely will see the disturbance towards one side or the other, but the aura will persist no matter which eye you cover.
The positive component of visual aura often involves bright flashing lights (photopsias), geometric hallucina- tions (teichopsias) or both. The leading edge of teichop- sias is often shimmering, and as the hallucination en- larges, it often leaves in its wake a blind spot that enlarges. Visual auras can be as bright flashing lights only, or one entire visual field on the left or right side may be missing or impaired. One’s vision may become fragmented, breaking down into a mosaic of small pieces.
With some visual auras, objects are perceived to be very
large, very small, or very distorted and such aura symp-
toms are what we believe may have inspired the vivid
descriptions of Lewis Carroll; we often refer to these
symptoms as the “Alice in Wonderland” syndrome.
Most physicians and many of the lay public are fa- miliar with visual aura, but sensory aura, less common but still quite prevalent, is less well recognized. With a typical sensory aura, numbness and tingling develop in the hand or face and, over minutes, “march” to involve other parts of the body, at times extending to the leg or
Figure 3.1 Visual aura often begins with a blind spot.
even the opposite side of the body. Involvement of the lips and tongue is common. Sensory auras may occur in- dependently or accompany visual aura within the same attack; when the latter occurs, the sensory symptoms typ- ically follow the visual. Sensory auras often are misdi- agnosed as transient ischemic attacks (TIAs ⫽ episodes warning of impending stroke) or focal seizures.
Sometimes aura may occur without any accompany- ing headache. We call these acephalgic (i.e., “without head pain”) migraines. These are particularly likely to occur in the elderly and may be mistaken to be the warning symptoms of an impending stroke. If you have a long history of migraine with aura and then begin to experi- ence auras without headache, the attacks are still mi- graines. Treatment usually is not necessary. When head- ache does accompany aura, it may be severe, pounding, one sided, and accompanied by nausea, vomiting and light and sound sensitivity. Or it may be mild and lack one or more of these associated symptoms. Migraine is a spectrum, extending all the way from aura but no head- ache (“acephalgic migraine”) to a mild attack reminiscent of tension-type headache to a commode-hugging night- mare of excruciating head pain. If you have “regular”
headaches in between your attacks of full-blown mi- graine, those “regular” headaches are simply mild migraines.
Other auras are even more frightening but fortu- nately are rare. A syndrome called ophthalmoplegic mi- graine involves changes in the movements of the eye.
Another variant of migraine with aura is called basilar
migraine, and with these attacks, the individual may ex-
perience double vision, difficulty in swallowing or speak-
ing clearly, dizziness/imbalance, weakness or numbness on one or both sides of the body, confusion or lethargy.
Afflicted patients may feel that they are walking like a drunk; they may become sleepy or, on rare occasions, even comatose.
Headache It is, or course, the headache phase that has contributed most to migraine’s notoriety.
Case 3.2
A 27-year-old woman comes to my office complaining of a one-sided pounding headache, which awakens her each Sat- urday morning. The pain usually involves the right side of her head but occasionally is left sided or bilateral. The right- sided headaches are her worst, and within an hour of their onset, she will develop severe nausea. After drinking wine with dinner the night before, the headaches seem worse. She tells me she thinks that she must be allergic to alcohol. Dur- ing headaches, she is bothered by lights and sounds and finds that the pain becomes worse if she attempts to get out of bed and carry on with her routine activities. The headache usu- ally improves by the evening but never goes away completely until she sleeps that night.
This patient is describing a typical case of migraine without aura (previously “common” migraine). Migraine without aura comprises about 85% of migraine attacks.
The pain of migraine is often on one side but can be bi-
lateral; bilateral migraine headaches are particularly
likely to occur in children. The pain of migraine tends to
be pounding in quality. If it is not pounding when you
are standing, leaning forward may enable you to “ap-
preciate” the pulsatile quality of your pain. Finally, the head pain tends to worsen with movement, and if you exert yourself, the pain will increase yet more. Clinicians rightfully are uncomfortable about making the diagnosis of migraine when there have only been one or two at- tacks, but if the attacks become recurrent, the chance of the headaches being migrainous is very high. Migraines are indeed “sick” headaches. By sick, we mean that the attacks often are accompanied by nausea, vomiting, light sensitivity, sound sensitivity and a general feeling of mis- ery and “unwellness.” When suffering an acute migraine, you do not want to smell dinner cooking, you certainly do not want to eat what is cooked and you instinctively seek the desensitization offered by solitude and a quiet, dark place.
Postdrome Phase Many migraineurs suffer a post- drome following the conclusion of the peak headache phase. The postdrome feels much like a hangover and may involve some lingering headache of relatively mild intensity, melancholy, and a general feeling that one is
“washed out.” The postdrome may persist up to several days and in patients with chronic migraine may blend into a subsequent attack.
Pediatric Migraine
Migraine is not confined to teenagers and adults.
Case 3.3
An 8-year-old boy comes home from school once or twice a
month complaining of “stomach ache”. He would open his
windows, turn off the lights, and sleep for an hour. Period- ically he will vomit. After an hour of sleep, he awakens hun- gry and ready for dinner.
Another myth is that children do not get headaches, but unfortunately, they do. Migraine is a familial disease that probably is also hereditary. If you have migraine, there is a fair chance that your kids are going to have mi- graine. While it’s not easy to acknowledge that we’ve passed on something painful to our children, early iden- tification and treatment of the disorder both may help re- lieve suffering in the here and now and, possibly, lead to a better long-term experience with headache.
We often do not recognize pediatric migraine when it first occurs because childhood migraine may not in- volve head pain. Remember that migraine is a brain dis- order, and a variety of brain symptoms can be part of the disorder. One hint that migraine is present in childhood is the symptom of carsickness; a high percentage of mi- grainous kids are prone to motion sickness. As they grow up, we eventually recognize that they have migraine.
Many children will come home from school from time to time looking pale, nauseated, and very fatigued. As with the little boy in the case described, they fall asleep and then awaken an hour later feeling fine. Such episodes could be migrainous in origin, even in the absence of headache. Episodic bouts of abdominal (stomach) also pain can be migrainous in origin.
A baby who experiences repetitive spells of vomiting
with no other cause found could have migraine. Unfor-
tunately, there is no way of proving it at the time, but
your pediatrician may suspect migraine is the culprit. As the child grows up and his or her migraine matures into a more recognizable form, it often becomes quite clear that the doctor was correct.
Estrogen Levels and Migraine
Treating migraine in women has additional challenges since migraines are often influenced by hormonal factors.
Among young children, boys are more likely to have mi- graine than girls. Girls soon catch up and pass the boys in developing migraine at puberty. This is the first bit of evidence that hormones are involved with the develop- ment of migraine. When women become pregnant, their migraines are very likely to improve or resolve, at least in the second and third trimesters. For women taking birth control pills, headaches often worsen. Women are most likely to have a migraine problem in their early 40s, and this tends to improve after menopause. So it seems that hormones, and in particular, estrogen, are an im- portant influencing factor for migraine. The major way estrogen influences migraine is to trigger an attack when the levels of estrogens fall. These are referred to as es- trogen withdrawal headaches. This is what happens be- fore the period begins, although the actual menstruation is an effect of the withdrawal of progesterone and not estrogen.
When migraine occurs with the period (menstrual mi-
graine), episodes are often longer in duration compared
to other attacks. The principles and medications used in
treating this are the same as in all migraine, although
there are a few additional tricks. Some women do better if they take a nonsteroidal antiinflammatory medication daily for a few days before the period (or the expected time of the headache). If you are on a prophylactic med- ication that works reasonably well except around the time of the period, transiently raising the dose before the period begins might help. Occasionally, estrogen patches are used before the period to blunt the fall in estrogen levels triggering the headache. Magnesium, discussed elsewhere, can also help. Diuretics (water pills) have been used widely. They reduce the bloating you might get with your period, but do not do very much to reduce the headache. Prophylactic triptans, such as sumatriptan, naratriptan and frovatriptan seem to be effective in re- ducing menstrual migraines.
Many women are willing to have a hysterectomy to rid themselves of headaches. This is usually a mistake.
First, it is not the menstrual period itself, but the estro-
gen effect that is the problem. The ovaries manufacture
estrogen, not the uterus, so removing it does not make
any sense. The problem is that if the ovaries are pro-
ducing their own estrogen, even if we give estrogen
medications, there still may be rising and falling levels
that can trigger an attack of migraine. If desperate and
convinced that estrogen withdrawal is the problem, a
chemically induced menopause can be precipitated us-
ing drugs called GnRH analogs, although this type of
chemically induced menopause is reversible when the
drug is discontinued. The estrogen level can then be con-
trolled, not allowed to fall, by using long-acting estro-
gen preparations. None of this is done often, and most
of the time the migraines are treated by more conven- tional methods.
Case 3.4
A 52-year-old woman has a long history of migraine with her periods. They are difficult to treat. She is delighted that after menopause last year, they went away. Wanting estro- gen replacement therapy, she is placed on Premarin. Now her migraines are back, and she does not know what to do.
After menopause, the levels of estrogen remain low and estrogen withdrawal is less likely. Frequently, when a woman who had severe menstrual migraines becomes menopausal, the whole problem resolves. Then she starts taking estrogen replacement therapy (ERT), and the headaches return. Whether it is good or bad for you to take estrogens after menopause is controversial, and we are not going to discuss all of the arguments. Is it possi- ble to take estrogen and still benefit from a natural re- duction of migraine that being postmenopausal brings?
The answer is “maybe”.
You cannot predict what will happen to your mi- graine if you take estrogen replacement therapy. Usually something happens, however. The most common form of estrogen replacement therapy is the use of Premarin, which is a mixture of multiple forms of estrogen. It ap- pears that estrogen patches, which release estrogen grad- ually, therefore preventing precipitous falls, seem least likely to worsen the problem and the most likely to help.
Other estrogen preparations may also be acceptable; par-
ticularly those which also contain male sex hormones.
Look also for implantable forms, which give very even, continuous levels, to be available in the future.
Case 3.5
A 28-year-old woman has migraines that are particularly severe when she has her periods. She just found out that she is pregnant. She has stopped all her medications far her head- aches, but is worried about what she can do if she gets a migraine.
While we are on the subject of hormones, what about pregnancy? What will happen to your migraines, and what can you take to relieve them? The good news is that mi- graines usually, but not invariably, improve during preg- nancy, particularly in the second and third trimesters. The bad news is we do not know very much about the safety of drugs during pregnancy, so we employ as many of the nonmedication methods as we can. Some drugs are a par- ticular concern for a pregnant migraine patient. Ergots can make the uterus contract, leading to miscarriages. Dival- proex sodium (Depakote) can cause severe malformations.
The simple painkillers with butalbital and acetaminophen
(Fioricet, Fiorinal, Esgic, Phrenilin) might be safe for occa-
sional use, but it is important to understand the limitations
of our knowledge of drug safety during pregnancy. On oc-
casion, the risks of a migraine with its accompanying nau-
sea, vomiting, and dehydratic could be substantial during
pregnancy. Treatment, for example with Imitrex injection,
may be beneficial. When we quote side effects to you, it is
from well-designed scientific studies where these are mea-
sured. In pregnancy, we certainly do not test the situation
purposely by giving drugs to pregnant women. Our only
knowledge comes from retrospective or prospective ob- servations of women who took them. Scientifically, this way of studying risk is flawed. Therefore, we will never be able to fully assess the safety of any drug in pregnancy.
Tension-Type Headache
The other exceedingly common primary headache disor- der is tension-type headache. This clumsy term has an in- teresting history. When the authors were in medical school (yes, this was after the introduction of electricity), these were called tension headaches. As they are wont to do gen- erally, doctors argued over what the term implied. Some believed that the muscles were tense, while others who used this term believed the person was tense; regardless, the term “tension” clearly was ambiguous. Experts then coined the term muscle contraction headache, explaining that what was tense were the muscles of the scalp and head.
The problem with this is that many times tension head- aches are not associated with any more muscle contrac- tion than what occurs during a headache-free period. Fur- thermore, people with migraine seem to have as much tension in their muscles as do tension headache sufferers.
Therefore, we are left with this cryptic compromise:
“tension-type headache.” The confusion that has at- tended the naming of this disorder reflects well the un- certainty as to its origin. As with migraine, it is simple enough to define tension-type headache clinically (Table 3.2), but it is anybody’s guess as to what specific biologic process generates these symptoms.
As we have discussed, patients with migraine often
experience headaches that are tension-type in character.
Interestingly, medications intended to treat migraine acutely or to prevent migraine attacks also tend to be ef- fective for the “tension-type” headaches that migraineurs suffer. On the other hand, those same medications do not appear to help patients with “pure” tension-type head- ache. This observation, coupled with other research ex- amining the migraine and tension-type headache pop- ulations, suggests that these two primary headache disorders arise from very different biologic sources.
Tension headaches may still be the most common type of headache. These are almost always mild, easy to treat with over the counter drugs and therefore do not come to medical attention. When they are severe, they are usually part of the spectrum of migraine and are treated as any other type of migraine. If you really be- lieve that you have severe tension headaches, ask your- self a question: do I have other kinds of headaches. If the answer is “yes,” they are likely all part of this migraine spectrum.
Table 3.2 IHS Diagnostic Criteria for Tension-Type Headache At least 10 episodes occurring on ⬍1 day per month on average (⬍12 days per year) and fulfilling the following:
1. Headache lasting from 30 minutes to 7 days
2. Headache has at least two of the following characteristics:
1. a. bilateral location
1. b. pressing/tightening (non-pulsating) quality 1. c. mild or moderate intensity
1. d. not aggravated by routine physical activity such as walking or climbing stairs
3. Both of the following:
1. a. no nausea or vomiting
1. b. no more than one of photophobia or phonophobia
4. Cannot be attributed to another disorder
Neck pain can occur with migraine in two distinct ways. It is common for neck pain to occur early in a mi- graine or even before the head pain starts. This is your signal to take your medication. Massage of the neck might even help as well. There is another type of neck pain, which that occurs late in a migraine. In this setting, massage will make it worse and your treatments will not work very well. Remember this and you can use the sig- nal to treat early.
Case 6
A 51-year-old woman states that she has had an ache throughout her head for many years, and it rarely goes away.
She can
-live with that,” but every few weeks she has a head- ache that seems the same as the others, then develops a throb- bing quality and settles behind her left eye. She becomes very fatigued and nauseated with those headaches, which take up to 2 days to resolve.
In 1988, the concept was proposed that tension-type headaches and migraines represent in many ways the opposite ends of the spectrum of a continuum of chronic recurring headaches, and so reflect the same underly- ing process. In other words, all of these headaches are related. Other headache experts feel strongly that the terms migraine and tension need to be separated and are very different phenomena, with different causes.
Many say that their migraines start out like tension
headaches and end up like migraines. Others say that
their migraines wind up like tension headaches. It seems
that a large number of people have both, and there must
be a close relationship between these two types of head-
aches. Therefore, if you have migraine and tension headaches, you may not actually have two distinct types of headache. However, this does not mean that they feel the same or even that they should be treated the same way.
We have learned that people with migraine who ex- perience different kinds of headaches often respond to one migraine medication for all their headaches. A study done with Imitrex (sumatriptan) showed that these peo- ple responded equally well when they had typical mi- graines, typical tension headaches, and attacks with fea- tures of each. It seems, therefore, that in this setting we do best by choosing an agent based on the severity of pain, rather than trying to distinguish the type of head- ache. It is important to recognize that if you have recur- rent disabling headaches, you are most likely suffering from migraine. Look for more information about treat- ment in Chapter 4.
Medication for Tension-Type Headache
Tension-type headaches are very common, but, fortu- nately, the chronic variety is rare. The episodic ones of- ten require little treatment and respond to relaxation or the addition of a heat or ice pack to the back of the neck.
A mild, over-the-counter painkiller is fine to take as well.
If they are more severe, they often respond to one of a
host of medications that contain butalbital (Fiorinal,
Fioricet, Esgic, Phrenilin, etc.). If you take these, be aware
of rebound headaches, whereby overuse of the drug be-
comes the trigger of the next headache. Therefore, never
use these more often than three times each week or more
than 10 tablets each week. The major side effect is seda- tion, so you do not drive a car or operate a dangerous machine under the influence of these drugs.
When tension-type headaches become chronic (oc- cupy more than half your time), they become a much more difficult problem. You might want to take a painkiller every day, but all of those drugs will have un- safe side effects if you do this. The main treatments for chronic tension headaches are several drugs that are used to treat depression, tricyclic antidepressants. Whether they work has nothing to do with whether you are de-
Severe tension headache is rare
pressed. Some centrally acting muscle relaxants have also been used. They are not always effective, but they are the drugs most likely to work, and they are relatively safe to take for the months and years that you may need them.
These are very difficult headaches to treat; be patient if your doctor keeps trying to adjust or change your med- ications. Avoid taking painkillers frequently or you will never get anywhere with your treatment. Non-medica- tion techniques of all kinds should be employed, includ- ing physical therapy, chiropractic therapy, massage, and exercise to increase the range of motion of the neck and shoulders and reduce local triggers in these regions.
Biofeedback is a self-teaching tool where you are trained to reduce the amount of muscle tension in the face, head, neck, and shoulders. This may help, particularly if you are good at mental imagery.
Unfortunately, in this era of managed care, it is get- ting more and more difficult to get these services cov- ered under insurance plans. Stress management pro- grams can also be employed. The use of heat packs to the neck and shoulders makes sense, too. As with other forms of chronic pain, a regular exercise program helps control the pain through natural mechanisms.
Cluster Headache
Cluster headache is a much rarer but extremely vivid pri- mary headache disorder. Its defining characteristics are outlined in Table 3.3.
Although exact figures vary according to the study
you read, the female preponderance that exists in mi-
graine is reversed in cluster; there are roughly six times
as many male cluster sufferers as female. Again, in con- trast to migraine, the individual attacks of cluster are briefer and much more severe in intensity. Attacks tend to “cluster” in cycles that persist up to two or three months, and during such cycles the afflicted individual will suffer multiple attacks within a single day; one of these attacks typically will begin at the onset of REM (rapid eye movement) sleep, awakening the slumbering individual to a maelstrom of excruciating pain that may occur at various locations over the head but almost in- variably settles in or behind one eye. Almost without ex- ception, patients describe the pain as either “like a knife or hot poker is being stuck in my eye and twisted” or
“there is terrible pressure behind my eye, and it feels like the eye is going to pop out.” Attacks often are accompa- nied by tearing, nasal congestion, copious nasal dis- charge and swelling or drooping of the eyelid, all occur- ring on the same side as the pain. Often the pain is referred to the upper molars, and just as the nasal symp- toms may prompt an erroneous diagnosis of “sinus head- Table 3.3 IHS Diagnostic Criteria for Cluster Headache At least 5 attacks fulfilling the following criteria:
1. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15–180 minutes if untreated
2. Headache is accompanied by at least one of the following:
1. a. ipsilateral eye redness and/or tearing 1. b. ipsilateral nasal congestion and/or drainage 1. c. ipsilateral eyelid swelling
1. d. ipsilateral forehead and facial sweating 1. e. ipsilateral shrinking of pupil or eyelid droop 1. f. a sense of restlessness or agitation
3. Attacks have a frequency from one every other day to 8 per day
4. Cannot be attributed to another disorder
ache” or of a dental problem. This referral of pain to the teeth may provoke unnecessary dental extractions. Even the behavior of someone with cluster headaches is dif- ferent from someone with migraine. Migraineurs want to go to bed and remain motionless. They want to squeeze their temples into their pillow and hold still. Cluster
Tearing of the eye in cluster headache
headache sufferers cannot hold still. They rock, pace, and cry. Alcohol can trigger a migraine, but it is astounding how sensitive cluster headache sufferers are to drinking alcohol. A half glass of wine or a few sips of beer are likely to induce an agonizing attack during a cluster period. Even the minute amount of alcohol found in “nonalcoholic”
beers can be enough. Sleep can trigger a migraine, but with cluster, precipitation of headache during sleep is even more common. You may be awakened from sleep, often at the same time every night. This feature led to an old term for cluster headaches, alarm clock headaches.
The following case from Mobile, Alabama illustrates all too well how far off base the diagnosis and manage- ment of cluster may stray.
Case 3.7
A 47-year-old male presents for evaluation of episodic head- ache. At age 36, he experiences his first bout of headache at- tacks that occur daily for six weeks, with two to four attacks per day and each lasting about 30 minutes. The attacks in- variably involve severe and disabling pain behind the left eye. The pain is non-throbbing and accompanied by tearing, nasal congestion, nasal discharge and swelling of the eyelid.
During attacks he is unable to remain still and paces in- cessantly, at times pausing to strike his head against the wall. Almost every night he awakens at about 1
AMby an attack, and he develops severe insomnia due to his fear of these headaches.
Over the ensuing years, he continues to experience iden-
tical cycles of headache that would be separated by headache-
free intervals, which persist up to six to nine months. He
has seen multiple health care providers for his headaches,
and his efforts have resulted in both 17 sinus surgeries and dental extractions of all his upper molars on the left. De- scribing his symptoms to a friend at a party, the friend sug- gests that the patient might have cluster headaches. When the patient broaches this possibility with his primary care provider, his suggestion is summarily dismissed. This is fol- lowed shortly by another sinus surgery that, as with his pre- vious surgeries, produces no clinical improvement.
His past medical history is otherwise notable only for a long and extensive history of cigarette smoking. His physi- cal examination is normal.
He is told that his headache syndrome is typical of cycli- cal cluster, and because he had just begun a new cycle of at- tacks, he is begun on preventative therapy with verapamil and prednisone and given injectable sumatriptan (Imitrex) to use for acute headache treatment. The sumatriptan is ef- fective, and the preventative medications terminate his cy- cle within three days.
There is no excuse for the delay in diagnosis and in- appropriate treatment that this patient received. Cluster is a highly distinctive headache disorder that—unlike migraine—tends to be symptomatically consistent from attack to attack, cycle to cycle and even patient to patient.
In describing this headache disorder, all cluster patients tend to sound as if they have read the same book.
Another interesting relationship with cluster is the in-
creased risk of developing a stomach ulcer. If you have
cluster headaches, particularly if you have cheek and
tooth pains with the attacks, you are at high risk of de-
veloping this problem as well. Unfortunately, the med-
ications used to treat the ulcer do not help the headaches.
Even though ingestion of alcoholic beverages typi- cally triggers an acute cluster attack during an active period, an astoundingly high proportion (more than 50%) of people with cluster abuse alcohol, a rate much greater than that found in the general population. Clus- ter headache sufferers usually smoke cigarettes as well, and through the effect of smoke on a ganglion (collec- tion of nerve cells) in the back of the throat, smoking may contribute to the development of the disorder in the first place. Cigarette smoking also places cluster suf- ferers at risk for developing heart disease. Given this and the fact that many cluster patients are males in their 40s and 50s, should they develop atherosclerotic disease involving the blood vessels of the heart (coronary artery disease), the most effective medication for treatment of acute cluster, injectable sumatriptan (Imitrex) could not be used.
Medication for Cluster Headache
The treatment of cluster headaches is another matter al- together. These headaches are not a variety of migraine, and what we told you about migraine does not neces- sarily apply to cluster headaches. They are severe and disabling attacks, and every attempt should be made to treat them aggressively. When treating cluster headaches, both preventive and acutely administered medications are needed.
Lithium carbonate, divalproex sodium, topiramate
and verapamil are used most often, sometimes in com-
bination. Corticosteroids, like prednisone, work well, but
have many problems associated with prolonged use, so
they are not often used. Only treat for the duration of the cluster, although that may be unpredictable. The best pre- dictor is how long past clusters have lasted, but that does not always hold up as an absolute predictor. A drug might work well for one cluster, then fail miserably on another, so do not refuse to retry a medication that failed you in the past. Often in the beginning and toward the end of a cluster, fragments of the headaches may occur that are mild and brief. However, the discomfort is in the same lo- cation as the regular attack, so if you have cluster head- aches, you recognize this only too well. Another treatment for cluster is a drug called “capsaicin” applied to the in- side of the nose. This is made from a hot pepper extract.
With repetitive use, it depletes a pain-producing chemical called substance P. This treatment may work only after a week or more of applications. It really burns, however, so if you try it, our phone numbers are unlisted.
These treatments for cluster headache are preventive and, at best, will relieve only a moderate percentage of attacks. Therefore, something needs to be available for acute attacks. Painkillers, even narcotics, are quite inef- fective at relieving the pain of cluster headache. The largest concern with narcotics is addiction, and there aren’t enough narcotics in the world to eliminate the pain of a cluster headache. Also, these headaches can occur several times a day for months, so you can imagine how many of these drugs you could wind up taking. Most im- portant, however, is that they do not even work well.
Oxygen may work well. You need to have a large
tank of pure oxygen with a special mask; breathe it in at
the first sign of an attack, and then continue to inhale un-
til your headache resolves. This treatment has a high suc-
cess rate, does not make you fatigued, and can be admin- istered at home or at the office. It is quite safe (unless someone smokes around it). Sumatriptan (Imitrex) by in- jection is amazingly effective within minutes. It is fine to try oxygen first and save the sumatriptan for headaches that do not respond to oxygen, or when you are away from home and do not have access to oxygen. One concern is that for an unknown reason, cluster headache patients commonly smoke and therefore have an increased risk of heart disease. Your doctor has to consider the safety issues of sumatriptan even more if you take this drug for clus- ter. It is interesting that oxygen treats only cluster head- aches and not migraine although sumatriptan treats both.
Want to Know More?
Migraine is the most commonly encountered headache disorder in clinical practice; in approximately 95% of cases involving patients who present nonemergently with the chief complaint of headache, migraine will prove to be the final diagnosis. As we have seen, migraine is not difficult to define or diagnose clinically, (Table 3.1) but its precise cause has proven to be elusive. The recent emergence of a “unified” theoretical model for migraine biogenesis has led investigators to develop drugs de- signed specifically to fit that model, and that certain of these drugs have been spectacularly successful in clini- cal practice suggests that current theory may be close to the mark in describing what underlies the clinical syn- drome we have labeled migraine.
A fair amount of evidence has accumulated to sug-
gest that the brains of migraineurs are hypersensitive, im-
plying that at least certain of their brain cells (neurons) are more likely than the neurons of nonmigraineurs to activate spontaneously or in response to a wide assort- ment of internal or external stimuli. Aside from bestow- ing a proclivity for headache attacks, this “brain hyper- sensitivity” may be manifested in a variety of ways that at first blush appear to have little connection to one an- other. For example, motion sickness is more prevalent in migraineurs than in the population at large, and mi- graineurs also are more prone to jet lag, difficulty initi- ating or terminating sleep and alcohol intolerance (in- cluding, sadly, a propensity for hangovers). Migraine is comorbid
1with a number of other conditions that imply brain hypersensitivity, including panic disorder, chronic anxiety disorder, bipolar (“manic-depressive”) disorder and epilepsy.
There is yet more objective evidence that migraineurs
possess brain hypersensitivity. Electroencephalograms
2performed on migraineurs often demonstrate findings in-
dicative of neuronal excitability, especially in the poste-
rior (visual) portions of the brain and particularly when
the individual undergoing the test is stimulated visually
with a strobe light. Furthermore, if the neurons of a mi-
graineur are stimulated by a pulsed wave of magnetic
energy, the individual may experience acute visual hal-
lucinations (called “phosphenes”) and abruptly develop
a typical migraine headache; a special type of brain MRI
scan performed at that same time will demonstrate an in-
triguing abnormality that may represent successive
waves of neuronal activation and deactivation spreading
across the posterior brain regions (so-called “spreading de-
pression”). Finally, examination of the cerebrospinal fluid
of migraineurs has demonstrated abnormally high con- centrations of excitatory neurotransmitters or their breakdown products.
Why is the migrainous brain hypersensitive, and how is this hypersensitivity linked to the familiar clinical syn- drome of episodic headache with associated nausea and light and sound sensitivity? Put in its simplest terms, the unified theory of migraine biogenesis goes something like this.
In response to a destabilizing trigger (for example, the drop in estrogen that occurs just prior to menses), ge- netically sensitized neurons of the brain’s visual cortex fire off (depolarize). The trap thus is sprung. A wave of depolarization spreads across the posterior regions of the brain, and following their burst of electrochemical exu- berance, the neurons lie exhausted and electrically silent for a period of time (the spreading depression phenom- enon). This cortical spreading depression in turn acti- vates the brain circuitry that signals head pain. Normally that signal passes from pain receptors located on the net- like web of blood vessels residing within the envelope of tissue (meninges) that encloses the brain; the brain tissue itself is insensitive to pain, relying instead on these meningeal pain receptors to serve as a “distant early warning system” for potential threats to its health and integrity.
In the case of migraine, however, this system oper-
ates in reverse, as the head pain signal arises within the
brain itself (Figure 3.2) traveling upstream—against the
typical direction of signal flow-down the brainstem and
out the trigeminal nerve to its terminus at the meningeal
blood vessels. The signal’s arrival at the junction between
nerve ending and blood vessel triggers the nerve termi- nals to release chemical messengers that in turn cause the vessels to leak proteins that produce an inflammatory re- action in the surrounding tissue. That inflammation fur- ther activates the trigeminal nerve endings, and a new head pain signal is born . . . this one traveling its typical downstream route to destinations located within the brain stem and brain proper.
Thus, a migraine attack involves a reverberating sig- nal of head pain, initiating within the brain, coursing out to the meninges and then ricocheting back from the pe- riphery to its central origin. This reverberating, volley of pain signaling will persist until the circuitry becomes spontaneously exhausted or is interrupted by some type of therapeutic intervention.
Figure 3.2 In a migraine headache, the pain signal travels up-
stream from the brain.
Want to Know Yet More?
How might one go about attacking this system to halt the to and fro volleys of escalating head pain signal? The op- tions are vast and extend from the simplistic to the highly complex and specific. During an acute migraine, the af- flicted individual instinctively may take steps to calm what essentially is an acutely agitated biologic system. A dark, quiet room and sleep may go a long ways towards bringing the migraine attack to a close; sleep in particu- lar involves activity of serotonin, the neurochemical mes- senger that appears to play a major inhibitory role in the head pain pathway. Some find that aerobic exercise will abort an attack; others find exercise intolerable, experi- encing with it a dramatic increase in the pain already present. Some have learned that ingestion of a caffeinated beverage will help quell an attack; others find that caf- feine serves not only to trigger attacks but also to am- plify migrainous pain once it is present.
Certain prescription and nonprescription drugs exert nonspecific or highly selective actions that may interrupt head pain transmission or at least nullify the effects of that transmission. Steroid medication and nonsteroidal anti-inflammatory drugs can be useful if taken early in an attack; their precise mechanism and site of action are unknown, but one possibility is that they counteract the meningeal inflammation produced by release of neu- ropeptide messengers
3at the junction of the trigeminal nerve endings and the blood vessels they supply.
Other, more selective agents have been synthesized
to mimic naturally occurring brain chemicals that influ-
ence inhibitory switches located along the head pain cir- cuit. If the agent can reach the inhibitory receptor and ac- tivate it, the transmission of head pain is effectively switched off. The triptan family, including its progeni- tor, sumatriptan (Imitrex), are “designer drugs” that re- semble serotonin and have a selective affinity for these inhibitory switches. Their optimal use is described in Chapter 4.
It should be noted that a sizeable proportion of mi- graine sufferers experience headaches on a chronic, per- vasive basis. Biologically, the head pain signaling system in these individuals has become chronically sensitized, and aggressive, persistent and consistent therapeutic in- tervention is required to desensitize those systems and restore them to a state that renders the individual head- ache free or nearly so. Strategies for accomplishing both acute and chronic desensitization are discussed in detail elsewhere in this book, but Appendix 3 (“Migraine: What You Can Do, What Your Doctor Can Do”) serves a con- cise summary that describes how migraineurs and clini- cians may cooperate to construct individualized and ef- fective management plans.
Notes
1
Comorbidity may be bidirectional; for example, migraineurs are prone to have epilepsy, and individuals with epilepsy are prone to have migraine.
2
“EEG,” a test that measures the waves of electrical energy pro- duced by neurons.
3
