The axe in the head

Charles Darwin, Vincent van Gogh, and Karl Marx all had one thing in common: they suffered from migraines. Today, more than 10 percent of the world's population shares this fate. Seemingly out of the blue, a migraine attack hits them more or less regularly. Suddenly the pain, the throbbing and pounding in the head, the nausea and the sensitivity to light set in. It is almost impossible to function in everyday life. For more than a hundred years, doctors have been searching for the triggers of the attacks and, more importantly, for the underlying causes of this oh-so-mysterious disease. There are now a handful of explanations and insights - but how they fit together is still only partially understood.

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It has been known since ancient times and affects people all over the world, but there is no cure. If you have migraines, you are likely to have them for the rest of your life. But at least you're not alone: about 8 percent of men and 15 to 25 percent of women suffer more or less regularly from nagging headaches, nausea, sensitivity to light, and many other accompanying symptoms.

When such a migraine attack begins, it triggers a veritable thunderstorm in a person's head within minutes: The brain's metabolism and blood flow are thrown out of control, causing stabbing and pulsating pain. Often concentrated on one side in the temple area or behind one eye, the pain reaches a level that can topple a person like an axe. Bright lights or loud noises are then pure torture. Violent movements can cause nausea and vomiting. Relief often comes only from rest and a darkened room, sometimes a cooling compress. On average, such an attack can last from four to 72 hours, but sometimes much longer. It is then impossible to work or function in everyday life.

No wonder the World Health Organization (WHO) ranks migraine as one of the top 20 causes of disability worldwide. According to one study, migraine sufferers lose an average of 17 days of work per year due to acute attacks, and the resulting costs and losses are estimated at around US$3 billion. However, the frequency of migraine attacks varies widely: While some people experience a severe attack only every few years, others suffer several times a month, some even every week or every day.

The unpredictability of the disease and its frequent occurrence in unfavorable situations is often bad enough for sufferers, but at the same time it has given them a bad image, because they have to deal not only with their pain, but also with society's misunderstanding of the disease. Migraineurs are often dismissed as neurotic whiners unable to cope with stress. Many have to live with the accusation that they are whiners, hysterics, or even shirkers, i.e., that they would use this type of headache as an easy excuse for their own purposes.

At least when it comes to work, this is not the case. One study found that the vast majority of migraine attacks do not occur during working hours, but on weekends or holidays. This does not diminish the reputation of the disease, however, because there is hardly a phenomenon of such magnitude that is as subject to myths, disinformation and mistreatment as migraine. At the same time, there are few conditions that leave you helpless during an acute attack.

The fact that migraines are much more than simple headaches is demonstrated by the phenomenon of aura. In about 10 percent of migraine sufferers, the attacks are accompanied by additional neurological phenomena. Usually beginning half an hour or an hour before the headache, the aura may be preceded by a visual disturbance. British neurologist Oliver Sacks described his first migraine experience at the age of four: "A bright, shimmering light appeared to my appeared to my left - blindingly bright, almost like the sun. It expanded and became a huge shimmering semicircle stretching from the ground to the sky, with sharp zigzag edges and in brilliant shades of blue and orange. Then, after the brightness, came blindness, a void in my field of vision, and soon I could see almost nothing on my left side. My vision returned to normal after a few minutes, but those were the longest minutes I had ever experienced."

This type of visual aura with a distinct border is called a scotoma. The zigzag pattern and the blank spot it creates are typical. Similar zigzag patterns were apparently seen more often by the famous astronomer John Herschel. In a letter to his colleague and friend George Airy in 1850, he wrote "I saw the pattern of the battlements twice in my eyes to-day. A sort of chess pattern filled it, and a sort of carpet pattern in the rest of the field of vision." Similar geometric patterns have been reported by other migraine sufferers. Oddly enough, so have patients who are drugged or who are awakening from hypoglycemic delirium.

In addition to vision, other senses can be affected by an aura. For example, some migraineurs experience distortions or hallucinations of smell or taste. How specific these phenomena can become can be roughly described as follows: The sign that a severe attack is approaching is, strangely enough, the distinct smell of microwave popcorn; you can smell it everywhere, but it is impossible to find the source of the smell. Sometimes the smell even seems to come directly from my fingertips. I don't own a microwave, and for obvious reasons, popcorn has no appeal to me.

In addition to these hallucinations, a person's sense of self may be altered or disturbed during a migraine aura. These can range from tingling sensations in the extremities or lips to speech problems or even paralysis on one side of the body. Some migraineurs also describe a feeling of ego dissolution, a temporary state of depersonalization. There are also experiences in which the body image is distorted, making the person feel very large or very small. Some see and feel only individual limbs of themselves as grotesquely inflated or shrunken. For example, when I sit down, I suddenly feel that my head is as big as the table, while my hands, feet, and torso become very small. This distortion effect, also known as the "Alice in Wonderland syndrome," may even have been the inspiration for the story that gave it its name. The author of the book, Lewis Carroll, suffered from migraines with aura. It is possible that his experiences with such aura phenomena gave him the idea for the size-changing potions and cookies in his book.

It is the year 1900 and the famous psychoanalyst Sigmund Freud is once again on forced leave at Berggasse 19 in Vienna. He suffers from migraine headaches. His six children, between the ages of 5 and 13, have already learned to be quiet during this time, so as not to disturb their father by playing too loudly. Research and patient meetings have been canceled. Although Freud continues to suffer from the agonizing headaches, he is also fascinated by his migraines with aura. In view of the manifold, not always tangible manifestations, especially during the aura, it was clear to him that this illness, which he called both hideous and magnificent, must have psychic roots. In the light of his theories, he interprets it as an outlet for unconscious conflicts. The Oedipus complex, the son's subliminal feelings for the mother, he speculated, could cause both the mental and physical manifestations of migraine.

Freud therefore tried to treat his own migraines through self-analysis, without success. Nevertheless, he stuck to his view of a psychological trigger. And in doing so, he was in line with the current trend, which sees massive feelings of guilt as the cause of suffering. Chronic headaches are thought to be a kind of unconscious self-punishment to deal with these feelings of guilt.

At present, the picture is not quite so neuroscientific, but the idea of migraine as a psychosomatic, stress-related disease is still widespread. We now know that migraine is a biologically based disease with the same valence as other medical disorders such as hypertension, angina, asthma and epilepsy. Unfortunately, many myths about this disorder have persisted. The most destructive of these are "It's all only in your head" and "It's all caused by stress".

But that is not true. What is true is that certain factors, such as stress, certain foods, or certain lifestyle choices, can trigger an acute migraine attack. But these triggers are not the actual cause of migraines, and they vary greatly from person to person. In fact, stress is an early indicator of an increased likelihood of a migraine attack, but it is not the primary cause. Rather, a change in stress level, from a stressful work week to a relaxing weekend, and a change in sleep-wake patterns are more likely to lead to a migraine attack. In addition, women are more likely to have a migraine attack when their hormone levels fluctuate.

Whatever it may be, one thing is certain: it sometimes takes a detective's intuition to figure out what factors are at work in your case. Coffee, cheese, and red wine are particularly common among potentially harmful foods. "I traced my sudden onset of migraines ten years ago to the opening of the first Starbucks store in Dallas. I went there every day after that," Vicki Amick told The New York Times Migraine Blog. "And also on a trip to Italy, after which I got into the habit of having a glass of red wine with dinner at night."

Chocolate, which has also long been considered a trigger, has now been exonerated. Research has shown that craving sweets immediately before an attack is not a trigger, but a symptom of migraine. But it is not always possible to avoid potentially triggering situations - and complete abstinence does not always help, according to a 2010 study. According to the study, avoiding migraine triggers altogether can increase sensitivity to previously harmless factors, creating new triggers. It appears that migraine sufferers have an overall increased sensitivity to environmental stimuli, the intensity and expression of which is flexible.

Almost as persistent as the idea of a psychological, psychosomatic model of migraine is the myth of the migraine personality. As early as 1734, the physician D. Junkerius noted that these individuals were full of silent and suppressed rage. Harold Wolff, a physician at Cornell University Medical College and one of the fathers of migraine research, described typical migraineurs in a 1937 study as ambitious, perfectionistic, rigid, obsessive and highly achievement-oriented people who, due to anxiety and insecurity, cannot adequately express their feelings and react appropriately to stressful situations.

Today, most doctors believe that this type of migraine personality does not exist. As early as 1980, a German study of 1,700 patients found that more than 60 percent of migraineurs had a "neuroticism score" within the normal range. This numerical value indicates the degree to which a person exhibits emotional instability, which can lead to neurotic and thus, for example, psychosomatic symptoms if the person is exposed to too much stress. With a score of 60 percent, migraine sufferers are in the average range for people with chronic illnesses, according to the researchers. The same study also showed a surprisingly low correlation between migraine and depression.

But there are other voices. For example, pain therapists Martin Krumbeck and Rolf Leeser report on their website: "Although studies claim to have disproved the theory of the 'migraine personality', it is noticeable in daily practice that the dutiful personality clearly predominates in migraine patients. For example, the typical migraine sufferer arrives on time for an appointment or is usually there before the appointed time. At work, the migraine sufferer makes sure that everything is done in an orderly fashion; he or she does not like unfinished business at the end of the day. It is also noticeable that migraineurs have very few sick days. The household of a migraine sufferer is generally very tidy and, above all, clean. It is not clear from these rather anecdotal observations, however, whether this "neatness" may have developed as a reaction to migraine. For many patients, sleep deprivation or irregular eating, for example, may be triggers. And while emotional stress or excessive demands are not considered migraine triggers, they can certainly increase the frequency of attacks. So making sure that unfinished business does not become a burden may just be a matter of prevention - it has nothing to do with a basic migraine personality.

"Migraine is not a psychological or psychiatric condition, but one that results from biological and physiological changes." So says Joel Saper, a migraine specialist at the Michigan Headache and Neurological Institute. And it's true. However, there is a great deal of disagreement about the exact mechanisms of how the disease develops. There are several explanations for what exactly happens in the head during a migraine attack, and they are only partially compatible.

The so-called vascular theory can be traced back to the British physician Thomas Willis, who noticed in 1664 that the blood circulation of the head must play a central role in migraine. Since the brain itself is completely insensitive to pain, but the blood vessels, especially in the meninges, are peppered with pain sensors, he believed that the headache must originate in the veins. With the advent of modern imaging techniques, this assumption was confirmed: In 1981, Danish neurologists led by Jes Olesen showed that during the aura phase of an acute migraine attack, the vessels in the cerebral cortex first dilate considerably. Then there is a counter-reaction, and the veins contract again in a spreading wave. Recent studies show that nerve signals actually cause the cerebral vessels to dilate during a migraine attack. At the same time, the vessel walls become permeable to substances that cause inflammation in the surrounding tissue.

But what exactly is responsible for this vasodilation and inflammation? In trying to answer this question, some scientists have turned to two papers that have been forgotten for decades. In 1941, neuropsychologist Karl Lashley, himself a migraine sufferer, traced the course of his visual aura, a classic spreading peak pattern with an empty area within. He suspected that the empty area was due to a failure or severe inhibition of neurons in his visual cortex. This, he estimated, was spreading at a rate of about 3 millimeters per minute. He interpreted the flickering edge of the inhibited area as preceding strong excitation of the brain cells before they failed.

A few years later, Brazilian epilepsy researcher Aristides Leão unwittingly provided evidence for Lashley's hypothesis: While measuring the brain activity of rabbits, he discovered a wave of nerve cell inhibition that spread concentrically at three to five millimeters per minute. Starting from one point, the cell membranes of the neurons begin to depolarize one after the other, their ion balance is massively disturbed, and no electrical signals can be transmitted in the affected area. The function of the neurons collapses almost completely. Leão calls this phenomenon cortical spreading depression (CSD). When migraine researchers unearthed his study decades later, they quickly realized that the spreading wave Leão observed had a similar course to the changes in blood flow during an acute migraine attack. And the parallel to the visual aura postulated by Lashkey also fits the picture.

In 2001, scientists found definitive proof of the link between cortical depression and migraine with aura using magnetic encephalography (MEG). MEG maps changes in the brain's natural magnetic field, caused in part by electrical signals from nerve cells. Further research soon revealed that restoring nerve function requires enormous energy from the brain, which leads to oxygen deprivation and abnormal blood vessel dilation. A possible explanation is found.

But parallel to these findings, another line of research is developing. It too has its origins in the past. As early as 1961, the Italian researcher Federigo Sicuteri discovered that migraine sufferers have particularly high levels of serotonin in their blood during an attack. This biochemical messenger affects mood and the sleep-wake cycle, as well as pain sensation and vasodilation. An excess of serotonin could initially constrict blood vessels in the brain, causing the neurological symptoms during the aura. At the same time, however, the excess also triggers an excessive breakdown process of the neurotransmitter. The resulting deficiency then causes the painful dilation of the meningeal vessels. Further research confirms this. This link may explain, for example, why some women have migraines associated with their menstrual cycle. This is because estrogen, which fluctuates during the menstrual cycle, also affects serotonin levels.

Serotonin is also the target of the triptans used today to treat acute attacks. These drugs, which are only effective against migraine, have a chemical structure similar to that of the neurotransmitter and specifically bind to the serotonin receptors in the blood vessels. This causes the veins in the cerebral cortex to constrict, counteracting the painful dilation. But how do cortical depression and serotonin fit together? And what triggers both phenomena?

"Don't be like that. This little migraine attack is not life-threatening". This or something similar can be heard from people around migraine sufferers when they have another attack. This is not only insensitive, it is simply wrong. Migraineurs can very well die from an attack. In recent years, it has become clear that the abnormal vascular changes in the brain appear to have far more serious consequences than previously thought. This is because the constant constriction and dilation leaves permanent damage in the fine veins. French researchers discovered a clear difference between migraine sufferers and healthy people when they used fMRI to examine the brain vessels of nearly 800 people over the age of 65: They found significantly more damage to the microvessels in the brains of migraineurs. These patients were almost twice as likely to have a particularly large lesion.

A study conducted by the Mayo Clinic in the United States shows what this means. According to this study, 27 percent of all strokes in young patients under the age of 45 are caused by migraine. Migraine also probably plays a role in a quarter of all strokes. This is confirmed by one of the largest population studies of migraine mortality to date, published in August 2010 in the British Medical Journal. In this study, researchers examined the relationship between migraine and causes of death in approximately 470,000 men and women in Iceland. Analysis of data collected over 26 years shows that people with migraine with aura have a slightly increased risk of all causes of death studied. Significant increases were found for heart attack and stroke, among others.

Strokes caused by microbleeds and vascular injuries in the brain are particularly common, according to a second study in the same issue. According to the study, women with migraine aura have a 2.3 percent higher risk of such a hemorrhagic stroke and a 3.6 percent higher risk of fatal brain damage from bleeding and other events. When these women take the pill, the risk factors add up to an eight percent higher risk. There is at least one consolation: A study also published in 2010 shows that the consequences of strokes and cerebral hemorrhages are significantly less severe in migraineurs than in the general population. Women with migraine aura usually had few symptoms and no permanent damage from such events. According to neurologist Tobias Kurth, director of the INSERM research center in Paris, this may be due to the fact that migraine sufferers tend to have smaller blood vessels, which means that only small areas of the brain are damaged.

And the microlesions do not seem to affect mental abilities. Although there are indications that such damage to the brain could in principle play a role in Alzheimer's and other dementias, in tests of cognitive performance in people over 65 with and without migraine, the Paris researchers led by Kurth found no signs of impairment. The migraineurs with lesions performed just as well as controls without lesions. "This is a very reassuring result for many people who suffer from migraine," says Kurth. "Despite the increased presence of microvascular lesions in the brain, this disorder does not appear to confer a higher risk of cognitive decline." In other words, migraineurs' brains may be crawling with damage as they age, but that will not make them demented. At most, it will cause them to die of a stroke - especially if they are unlucky enough to be female and suffer from migraine with aura.

For most migraine sufferers, migraines are a real hassle: the excruciating headaches, the nausea, the need to immediately withdraw from everything during an attack and seek peace and darkness. In extreme cases, migraines can seriously disrupt daily life. The list is long and includes work, relationships, children, self-esteem, ambition, and identity. But there is something else: it gives sufferers the opportunity to develop extraordinary talents.

The neurologist Oliver Sacks reports in his blog about a patient, an extremely creative mathematician, who, despite great pain, cannot do without his migraines. Although he suffers weekly, starting on Wednesday and worsening until Sunday. Sunday, but when the attack subsides on Sunday evening, he feels as if he has been reborn. The migraine triggers a burst of energy and creativity that gives the scientist his best ideas on Monday and Tuesday. "I prescribed him a drug to prevent the migraines, thinking he would have seven migraine-free days to work creatively," Sacks says. "But he soon came back and reported that his creativity had disappeared along with the migraines. Apparently, in some strange, complex way, he even needed his migraines.

The English writer George Eliot, famous in the 19th century for her novels "Middlemarch" or "The Mill on the Floss," also experienced the invigorating influence of migraine. She wrote that she always felt "dangerously well" just before an attack. The famous medieval philosopher and mystic Hildegard von Bingen may have owed her extraordinary visions to her migraines. She attributed them to divine inspiration, but according to neurologists, her descriptions bear striking similarities to visual aura phenomena. to visual aura phenomena: "I saw a great star, beautiful, and with it a multiplying multitude of falling stars," she wrote. "And suddenly they were all extinguished and turned into black coals. and turned into black coals."

The fascinating shapes and colors of the visual aura may also have played an important role in some famous artists. For example, the Italian painter Giorgio di Chirico, one of the main proponents of "metaphysical painting," suffered from migraines with aura since childhood. His strangely distorted, dreamlike architectural views may also have been inspired by early experiences with visual migraine phenomena. The great surrealist Salvador Dali also suffered from migraines, but it is not known whether this influenced his art.

In music, migraine seems to have less of an impact on artistic creation - at least there are far fewer known cases. However, both Gustav Mahler and the French composer Charles Gounod are known to have suffered from migraine. However, there is no evidence that it influenced their compositions. Claude Debussy, on the other hand, described in an 1898 letter migraine-induced musical dreams he experienced while working on the piece "Pelléas et Melisande": "During the last crisis - it lasted eight hours - I had the most remarkable nightmares: I was assisting at a rehearsal of Pelléas, and suddenly Golaud transformed himself into a bailiff and adapted the phrases of his commission to the notes of the music". Modern New York composer Annie Gosfield uses music primarily to help with acute migraines. "Composing can sometimes get me out of a migraine: Working alone in a controlled environment can at least distract me from the ice pick in my head," she writes to Oliver Sacks on his blog. "I've found that the concentration I need to work during periods of intense pain sometimes brings me to a different approach."