Diseases information. Disorders. Treatment.

Now, let’s get to something a little less gruesome and frankly something a little more common, which is migraine. Migraine is very, very prevalent in the United States. Seventeen percent of women and 6% of men in the United States suffer from at least one migraine a year. So we are talking about a very prevalent problem. What’s spooky about the American Migraine Study is this: although it is very prevalent, the diagnosis rate is terrible. Look at this. Only 41% of women with migraine get diagnosed, only 29% of men with migraine get diagnosed in the United States. Common problem, terrible diagnosis rate. There seems to be a sentiment out there that men don’t get migraine.

Now migraine being with a prodrome. Very often migraine begin with a prodrome and that’s not very often spoken about. We talk about auras – I’ll talk about aura’s a little bit too – but prodromes are very common. Prodromes occur up to a day before a migraine begins, and I’ll give you some examples of what prodromes are like. There are often changes in mentation. Many people feel depressed before their migraine, but actually more people with migraine feel euphoric. Many people are actually in a great mood, feel very, very well the day of their migraine. Some begin to yawn. I’ll tell you that spouses are better than headaches sufferers at figuring this out. Someone’s walking down the street, three o’clock, they slept well, suddenly they are yawning because a migraine prodrome can be a mild encephalopathy. People don’t think very clearly. Cold hands and feet, and anyone who sleeps with someone with migraine will tell you that even interictally they have colder hands and feet than the general population, but often before their migraine, they get even colder. Then frequent urination can occur, neck stiffness can occur and food cravings can occur as a migraine prodrome. Now this is interesting because the food craving that seems to do it the most is a craving for chocolate. Now we are very familiar, all of us are familiar with food cravings and food aversions in pregnancy. Very neurobiologically mediated. We have to accept this. But food cravings for chocolate are a very common migraine prodrome. I don’t know if you realize this, but if you give migraineurs chocolate or a placebo, you can generally distinguish between who gets a migraine. Probably chocolate doesn’t trigger a migraine, but at the same time a chocolate craving can be a migraine prodrome, people act upon it and they relate eating the chocolate to triggering the migraine. But that’s probably not the mechanism.

New we divide migraine into migraine with and without aura and that changes the terminology that I learned in medical school, of classic and common migraine. Everyone learn this? Okay, forget it. You can’t use it anymore, so don’t ever say that word. It’s now migraine with or without aura. Now migraine without aura is more common. About 85% of people with migraine have migraine without aura. Now what is that? Basically it is a pain which, in adults, is usually unilateral. In children it’s usually bilateral. But most of the time, in adults, it’s usually unilateral, usually pulsatile and worse with routine movement. I’m sure there are people out here who have migraine; if you have a migraine and someone drops something on the floor, you will do anything you can to avoid leaning over and picking it up. That kind of maneuver is awful, terrible. Migraineurs don’t like to exert themselves. That enhances the pulsatile quality of their migraine. They last 4-72 hours, by definition and they are associated with autonomic symptoms; light sensitivity, sound sensitivity, and although the IHS doesn’t include this, smell sensitivity. Migraineurs don’t like you to be doing a whole lot of cooking when they are getting a migraine.

Okay, another case. Sixty-seven-year-old woman with a new holocranial headache with exposure to cold, tenderness in her scalp and pain upon chewing. That’s a classic history of giant cell arteritis. Scalp pain. Bothers them to touch the scalp. If they chew, they get jaw claudication or tongue claudication, and they are very typically associated with worsening upon exposure to cold. Now fortunately everybody doesn’t walk in here with a little arrow at their superficial temporal artery saying, “Look here.” Many of these people actually don’t have pain or tenderness over their superficial temporal artery. Very few of these people are under 60. It’s rare, rare, rare under that age, but most of them have polymyalgia rheumatica. So they are not well people. They come in, they feel terrible, they are achy. They have this new scalp pain and often it’s worse when they chew. Now you do a sedimentation rate and it’s generally high. There are cases where sed rates are normal. Fortunately they are relatively rare. I generally recommend not just doing a sed rate, but a C-Reactive protein, and a serum viscosity, because what you often find is, as you are treating them, one of these or more than one of these becomes better markers for the activity of their disease. In other words, just counting on sed rates is often not as good. You will find an individual patient where their serum viscosity turns out to be a better marker of how they are doing.
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Now I’ll tell you about another case. This is a case of a 46-year-old woman who came in with the abrupt onset of a severe frontal headache. She actually got better over a few hours. I’ll tell you a secret. Actually she was given sumatriptan, someone gave her sumatriptan and her headache went away so she was sent home, and someone did find that she had a stiff neck in the emergency room. The problem here is that she has a subarachnoid hemorrhage. All that white stuff is blood, she had a huge subarachnoid hemorrhage, responded beautifully to sumatriptan and she went home. Remember, 50% of these people die, 50% mortality, so you can imagine the problems. I will also mention in that, that I saw a patient with meningitis who came in with a fever and a stiff neck. Someone gave them DHE intravenously, it all went away. Sent home, did fine for about three hours and came in moribund. We’ll talk about that in these migraine drugs. But this is a subarachnoid hemorrhage and what makes a history is this: these are people who have the abrupt onset of a headache, terrible headache. It doesn’t come on gradually. It comes on apoplectically. So if someone comes in and says, “I have the abrupt onset of a terrible headache” you think of subarachnoid hemorrhage until proven otherwise. You get a CAT-scan. You don’t do an MRI, you do a CAT-scan and usually the blood will be seen. Occasionally it’s missed, so if you think about that, you do a lumbar puncture. Very, very important. Again, 50% mortality. You don’t want to miss this.
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Subhyaloid hemorrhages are usually seen in the optic nerves. These are puddle-shaped hemorrhages and they are generally within a disc diameter, so you are not going to see them way out here, you are going to see them within a disc diameter.

This is a case of a 31-year-old male who came to see me who had a 15 year history of recurrent right-sided throbbing headaches, that were always preceded by distortions in his left visual field. Now he should have had migraine, and I’ll tell you that the overwhelming majority of people like this have migraine. The only reason we thought anything else is because the headaches were always in the same place and the visual aura was always in the same place, so we did an MRI. This is a big arterial venous malformation in the occipital lobe. Now had he come in and said, “They are almost always on that side, but every once in awhile they switch to the other”, don’t scan them. It’s not worth it. I’ve never seen a person in 20 years of practice who had anything else that imitated migraine, where it shifted, even if it’s 1% or 2% of the time.

Now I’m going to go through some cases.This is a case, sad case, of a 61-year-old male who came in with a six-week history of headaches. The pain awakened him out of sleep and would get better as the day went on. When he would strain at stool, the pain would get worse, and when he would sit up he developed something called visual obscuration. This is a classic brain tumor history. Every neurology book you ever pick up will say, “Brain tumors cause early morning headaches, wake you up out of sleep, get worse if you cough or sneeze.” I think we all learned this in school. Three-quarters of these people with brain tumors had headaches that sounded like tension headaches, and about 9% had migraine-like headaches. About 14% of these people had kind of unclassifiable headaches. But look at this, 8% of all the people with brain tumors had what every neurology book you ever pick up says they are supposed to have. So, so much for that rule.
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The other thing is, it seems that if you have a previous history of headaches and you develop a brain tumor, you are more likely to develop headaches than if you didn’t have a previous history of headaches. It’s very common that what you’ve got now, with your brain tumor, is simply more of what you used to have. What I mean by that is, if you have a history of migraine and you develop a brain tumor, you are likely to develop more, more severe, more persistent migraines than you had before but not necessarily a new headache. If you had cluster headaches, or you had tension headaches, you are more likely to develop more to those, but again, not necessarily a new headache.

Let me talk about another case. This is a case of a 22-year-old woman, came in with new frontal headaches which would increase when she strained at stool and began awakening her out of sleep, and her periods had become abnormal. Now if you look at this lady – she’s a little on the heavy side -if you look at her optic nerve, she has papilledema. What’s wrong with her? Anyone know? Pseudotumor, that’s right. Now we call this idiopathic intracranial hypertension or pseudotumor. Again, most of these are women, most of them are obese, most of them have menstrual irregularities. They have generally non-focal neurological exams, except for papilledema. They have papilledema. Although some don’t, which is spooky. Many of them have a recent weight gain. Some have endocrinopathy’s, and up where I live – I live in the New York area – actually infectious disease is really easy in New York because we actually have only one disease, which is Lyme disease. Everybody either has Lyme disease or thinks they have Lyme disease, and that includes the people who bring them into the office. Actually Lyme disease can cause pseudotumor, so it actually is a fairly important cause.
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So how do you treat these people? Generally we use carbonic and hydrase inhibitors, that’s probably the agent of choice. When I was a resident we used to do lumbar punctures, serially. You need to do one to diagnose these people, you show that their protein is either normal or low, they don’t have cells, their pressure is very, very high. And then we used to do these serial lumbar punctures. We basically used to do one or two a day, every day, until they signed out against medical advice. Which usually didn’t take very long. We came to realize that that is kind butcherous. Furthermore, when you start doing that you actually have a chance of implanting an epidermoid tumor into their spine. So you have to be very careful. If they are getting into trouble, if they have papilledema and they are losing their vision, we recommend doing optic nerve fenestrations and occasionally ventricular shunts.

Ninety percent of persons who seek medical advice for headache have migraine or tension-type headaches, and only 10% of all people have anything else wrong with them. Although there are many types of headaches, the overwhelming majority of people fit into only a very small category of headaches.
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Let’s discuss what doesn’t cause headaches, because these are things which are commonly blamed on causing headaches, which aren’t very important. The first is: refractive errors of the eye. Whether you need glasses or you don’t need glasses is nice, but usually doesn’t have a whole lot to do with why you have chronic recurring headaches. The second is: chronic sinus disease. We know that acute sinusitis causes headaches but that’s easy to recognize, but chronic sinus disease, whether your sinuses are a little thickened or not, generally has very little to do with whether you have, or why you have chronic recurring headaches. Then there are allergies. Allergies are not a very important cause of chronic recurring headaches. They can be a non-specific trigger of headaches in people, particularly migraineurs but by themselves, allergies don’t cause chronic recurring headaches.

The overwhelming majority of headaches are in the eye. People come in and they say, “My eye hurts in here” so they run off to the eye doctor. So the question that then comes up is, if the headache is in the eye, how do you know if you should see your eye doctor? Well, the answer is really simple. One is, you almost never to see your eye doctor because very few causes of headache have anything to do with the eye. The reality is that virtually everything that is sensitive to pain within the brain is supplied by the first division of the trigeminal nerve. V1 fibers are ubiquitous within the anterior middle fossa and even the superior part of the tentorium, so virtually everything that hurts in the head refers pain to the eye. Here’s a rule. All the ocular causes of pain cause changes in the external appearance of the eye. In other words, if you have a red eye, that’s acute glaucoma for example, the first few minutes of acute angle closure glaucoma and very few of these cases are going to be seen in the office, acutely. So basically, if someone comes into the office and they have a white eye and they say the pain is in the eye, forget about their eye doctor.
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Now the IHS, International Headache Society, classified headaches a number of years ago and they put out a very, very long doctrine about hundreds and hundreds and causes of headache. Here’s the Green simplification of the IHS classification, which is probably all it’s worth – one slide. It’s basically worth dividing headaches into primary and secondary headaches. Primary headaches are those in which the headache is the problem, and secondary headaches are those in which the headache is a symptom of another organic neurological disease.