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What is a migraine aura?

By Ely Simon, MD

Amy[i], a 34-year-old woman with migraine, gets strange visual episodes that are usually 10-20 minutes of bright lights that move around in her visual periphery. She often gets headaches after these ‘auras’, but sometimes she does not get pain. Last week, she had an episode that felt like a strange sensation of objects that she saw as distorted and smaller in size than they really are. This lasted 35 minutes, and her doctor told her that this was also a type of migraine aura. Amy wants to know what is a migraine aura – what does it have to do with headaches, and should she be worried.

Aura is a strange type of short-lasting neurological event

Approximately 25% of people with migraine experience auras before or during their headaches. Auras can be thought of as transient neurological deficits or brief brain dysfunction. The most common symptoms are visual, including blind spots or bright lines or flashing light. Other types of auras may be numbness, weakness, or speech difficulty. More unusual auras can involve strange sensations, such as déjà vu or distortion of body size (known as Alice in Wonderland syndrome). As a rule, all types of aura develop gradually and last a few minutes and under an hour.

Auras can occur by themselves – without pain

Since we are used to thinking about auras associated with migraine headaches, it may be strange to hear that auras can occur without pain. That is, the same type of “migraine” aura that occurs preceding or during a painful headache can occur in some people without any pain. We still consider these painless auras to be part of a migraine because the physiological process that underlies a full-blown migraine headache is complex and has multiple phases – one of those phases relates to aura, whether they are associated with pain, or not.

What happens in the brain during an aura?

From our understanding, auras are a result of an electrical brain activity process known as ‘spreading depression’ that is also related to dilatation of blood vessels. In animal experiments, Leão’s cortical spreading depression (a slow propagation of inhibition of brain activity that spreads across sections of the surface of the brain) is observed when certain types of stimulation are applied to the brain. (According to legend), the connection between this phenomenon and migraine was made by observations correlating the rate of movement of migraine aura across the visual field to the rate of spread of the depression across the visual cortex, suggesting that spreading depression may cause the aura symptoms[ii]. A breakthrough came with successful functional neuroimaging studies of patients during an aura, demonstrating a moving wave of decreased blood flow[iii]. Still, our knowledge is partial and more studies are required.[iv]

Should I take any treatment during my aura?

Good question. Neurologists are uncertain if taking migraine medication during the aura is advisable, since the studies on these treatments have been done focusing on the headache pain. Since aura usually leads to a headache, it may make sense to take a medication. On the other hand, it would not be advisable to take a strong migraine medication, such as one of the triptans, if there is no headache – the aura may not develop into a painful headache at all. Further, the triptan medications are limited to just a few dosages per day and per week due to safety considerations, and you would not want to ‘waste’ a dose if it is not needed. So, it is really not clear – it is ultimately your decision to make. If you do decide to take any type of treatment during the aura, make sure to record the treatment and its effect in your migraine diary.

How do I know if my neurological symptoms are ‘just’ and aura?

Generally, if you experience sudden neurological symptoms, it is shocking and alarming, and possibly a signal of a stroke. So, why not get alarmed by an aura? Why is this considered a benign experience? Why is this not a warning of an impending stroke?

The answer is in the context of the symptoms. It is true that you will be alarmed the first few times that you experience an aura. You may even decide to go to your nearest emergency room to be evaluated. Eventually, if you experience migraine auras, they tend to be of similar type and you will grow comfortable with the knowledge that they go away and that you will not be left with brain dysfunction. Needless to say, if you think that your symptoms are not typical or there is lingering dysfunction after 30 minutes, you should go to the hospital to get evaluated.

I personally experience migraine auras and find them bothersome, at times. But, really, I have gotten used to them.

[i] This ‘textbook’ case is based on collective clinical experience, and is not based on a specific patient.

[ii] Lancet. 1985 Oct 5;2(8458):763-6.

[iii] http://www.migraine-aura.com/content/e27891/e27456/e27721/e46684/index_en.html

[iv] Neurol Sci (2013) 34 (Suppl 1):S47–S50

How do I know if my memory is normal?

By Ely Simon, MD

Sam[i], a 60-year-old plumber with hypertension and diet-controlled diabetes, has noted difficulty in remembering the addresses of his clients. He used to remember them all, and is concerned that he is losing memory. Sam has no problems doing all of his usual activities, and has not had problems that are noticeable by his family. Sam wants to know if his memory is normal. How can he know?

What is normal memory?

Memory for learning new facts is called “semantic memory”, and it generally peaks in our 20’s and starts to decline thereafter. That does not mean that we become dysfunctional as adults, it just means that you should not be surprised if a 12-year-old will beat you consistently at a memory game. Like most things in biology, there is no one number that is normal. Rather, people who are neurologically intact perform memory tests across a range of scores that generally fits a ‘normal’ distribution (see the graph below). That is, you could be considered normal and have memory scores on standard tests that are either a little higher or a little lower than the average for the entire population of healthy people. Although the exact cutoffs may be different for different tests and for different purposes, we can consider the range of normal as between the -1 and 1 (standard deviations) on the x-axis of the graph below.

If memory declines with age, then how can I compare to a 20-year-old?

Actually, memory scores need to be corrected for age if the purpose of the test is to determine whether you are normal for your age. There are mathematical methods that are used when scoring standard tests of memory to make sure that your scores are compared to what would be expected for your age. In addition, most tests are also corrected for years of education, since that also turns out to strongly influence memory scores.

We have been discussing memory. What about attention, language skills, and other cognitive abilities?

Yes, other cognitive skills can be tested, as well. In fact, if your concern is about developing memory loss, it is important to test multiple cognitive abilities. People commonly refer to thinking ability and other cognitive skills as ‘memory’, so you should not be surprised to find that more than one cognitive skill may be affected.

How do I get tested for memory and cognition?

There are 3 types of tests that are commonly used by healthcare professionals:

Screening tests – These are brief, general under 10 minutes and cover one or a few cognitive abilities. The most popular is the MMSE, but the MoCA has gained a lot of use for its validity and greater range of testing. These are convenient, but provide limited information.
Full neuropsychological evaluations – These are paper-based tests that are administered by highly-trained psychologists or psychometricians. These have the advantage of greater depth and breadth, but are long and expensive. Typical batteries take several hours and can cost in the thousands of dollars. Interpretation of the results requires a trained neuropsychologist, cognitive neurologist, or other competent professional.
Computerized cognitive assessments – These have become popular for in-office testing, and play a role between the brief screening tests and the long neuropsychological evaluations. Computerized cognitive batteries can be between 30-60 minutes and cost in the range of common laboratory tests. Some assessments produce interpretations relating to whether the patient has cognitive impairment or abnormality. Other products simply produce graphs and tables of cognitive testing data similar to that produced by the paper tests. In both cases, a neuropsychologist, cognitive neurologist, or other competent professional should be trusted with clinical interpretation of the results.

Are computerized cognitive tests as reliable as psychologist-administered tests?

That is a good question, and one that certainly depends on the specific test. There are tests available that have been validated to the standards of paper based tests, and there are other newer tests that do not yet have such track records. It is best to be tested by a psychologist or neurologist, as they are likely to be sticklers for good validity. In any case, it is a good idea to ask which test (developed by which company) is being administered and if it has been well validated.

Should I get tested? What happens if my test shows something abnormal?

It is a good idea to ask your doctor whether you should be tested. The results of that test could provide objective evidence for a true abnormality in memory and other cognitive abilities. Some specialists also offer other tests that may be more specific for certain disease, such as Alzheimer’s disease. These include brain MRI, PET, SPECT, and some genetic testing. In general, you should be sent for these tests only by specialists who are expert in dementia and only if your clinical data justify the need for these tests.

Empowering patients with digital data about their brain health

One final note is in order. One of the big advantages of computerized cognitive testing is that it could be repeated multiple times easily and inexpensively. You could have a record of your cognitive health that could serve as a baseline for the future, as it may be hard to determine subtle changes if there were no prior testing results. Many experts consider home-based testing as the way of the future, but we are not there yet. As of now, it is best to tell your doctor about any cognitive symptoms or concerns, and decide together if you should get tested.

[i] This ‘textbook’ case is based on collective clinical experience, and is not based on a specific patient.

I am forgetting names – Do I have Alzheimer’s?

By Ely Simon, MD

Bill[i], a 70-year-old salesman with hypertension and high cholesterol, has noted difficulty in remembering the names of his clients. He used to remember them all, and is concerned that his work is suffering from worsening memory. He told his family doctor, who asked a few questions about whether he gets lost finding his way home (answer: No!) and if he has left the stove on allowing food to burn (answer: No!). Bill was surprised when told that there is nothing to worry about, and he is simply getting old. Still, he is worried and wants to know if there is something he can do about his poor memory.

What is Alzheimer’s disease?

Alzheimer’s disease is a degenerative neurological disease that affects 1 in 9 people over the age of 65[ii]. According to Marist poll, Americans rank Alzheimer’s as the most feared disease[iii]. So, lots of people are concerned in general, and many become very worried when they notice subtle signs that their memory is not as good as used to be. Many people even assume that they are developing Alzheimer’s based on the fact that they are forgetting things. However, Alzheimer’s is not just about getting older – it is an actual disease process that starts with the death of certain brain cells and build-up of abnormal protein complexes, and results over time in destruction of connections between nerve cells and ultimately in brain shrinkage. These pathological changes, which can be seen in the brain, are accompanied by progressive decline in memory and other cognitive abilities, resulting in greater dependence on others for help in performing activities of daily living, and ultimately death. So, a diagnosis of Alzheimer’s disease has specific meaning with regard to destruction of the brain and predicts a steady decline in function.

Age-related memory decline

We all start to forget things as we get older. In fact, research has shown that our ability to remember new facts, such as on memory games and puzzles, start to decline at age 25 and continues to decline throughout life. The decline may be subtle and is not to the degree that compromises daily function. Therefore, forgetting things a little bit is not a sure sign of Alzheimer’s.

What is “cognitive reserve”?

Because our brains are different from each other, we have different capacities to withstand damage before symptoms of cognitive decline start showing. This capacity is called by brain researchers, “Cognitive Reserve”[iv]. Someone with lots of cognitive reserve may appear normal for a period of time while his brain is actually undergoing changes of dementia. Someone with little cognitive reserve starts to show symptoms much earlier in the disease course, generally at a younger age. Certainly, even someone with a lot of cognitive reserve will eventually develop memory and cognitive decline if his disease progresses long enough – in fact, he may seem to decline much faster than someone whose disease had started earlier. Overall, having more cognitive reserve is thought to be better, since it results in more symptom-free years.

How do I build cognitive reserve?

That is a very good question that is not fully known. It seems that a large part has to do with your genetics, but it certainly helps to live a brain-healthy lifestyle and to be involved in learning. Things you could do include regular exercise, social activity, mental exercise, keep a healthy diet, and carefully managing vascular risk factors such as hypertension, diabetes, smoking, and obesity.

Should I get tested for Alzheimer’s?

It is a good idea to ask your doctor whether you should be tested. In all likelihood, you will be given a short screening test, and if suspicious for a problem, would be referred out for a longer cognitive testing session that could last 1 or more hours. The results of that test could provide objective evidence for a true abnormality in memory and other cognitive abilities. Some specialists also offer other tests that may be more specific for Alzheimer’s disease. These include brain MRI, PET, SPECT, and some genetic testing. In general, you should be sent for these tests only by specialists who are expert in dementia and only if your clinical data justify the need for these tests.

What can I do about my anxiety about getting Alzheimer’s?

So, your doctor told you that you don’t have to worry, but you are still anxious about getting Alzheimer’s.

First, remember that we all decline in memory ability as we get older. So, a little decline does not mean that you are getting Alzheimer’s.
Second, remember that there are things you can do to improve brain health:

Make an appointment for another evaluation by the same doctor in one year to see if there is a decline.
If you are really worried, ask to be referred to a memory specialist for a second opinion.
Make an appointment with your internist or family doctor to review management of your vascular risk factors, including lipids, blood pressure, body mass, and heart function.
Maintain a program of regular physical exercise.
Make an effort to remain active mentally with challenging work, games, or activities.
Try to lower your stress level by learning techniques such as relaxation or meditation.
Follow a healthy diet, such as the Mediterranean diet.

Summary: Tell your doctor and follow steps for good brain health

It makes sense that you are concerned about Alzheimer’s, but the best way to know if there is real reason to worry is to tell your doctor and, if needed, get checked out by a specialist – either a neurologist or a memory disorders specialist. In any case, it is worth following the recommendations for good brain health.

[i] This ‘textbook’ case is based on collective clinical experience, and is not based on a specific patient.

[ii] http://alz.org/facts/overview.asp

[iii]http://www.helpforalzheimersfamilies.com/alzheimers-dementia-care-services/alzheimers_feared_disease/

[iv] http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3024591/

Why am I writing the Brain Doc blog?

By Ely Simon, MD

I am a neurologist. Many people don’t know exactly what a neurologist does. Some assume that I do surgery on people’s brain – No, that is a neurosurgeon. Other people think that a neurologist’s job is close to a psychiatrist – That is also not exact, but an interesting topic worthy of its own blog. Actually, a neurologist is a medical doctor who specializes in diagnosing and treating diseases of the brain, spinal cord, nerves, and muscles. That is a lot of conditions, and includes Alzheimer’s disease, traumatic brain injury, Parkinson’s disease, multiple sclerosis, epilepsy, stroke, muscular dystrophy, diabetic neuropathy, and many others. Interestingly, the most common reason that people see a neurologist is to diagnose and treat migraine headaches – a full 20% of visits to neurologists in the US are for migraine. So, neurologists treat people of all ages. And, yes, most of what we deal with are concerns about or actual problems of the brain.

Educating and communicating

Although there are many ways a neurologist can actually help you with medications and other treatments, I feel that our most important role is in educating patients and families about their concerns, conditions, and treatments. Too many people have talked to me after seeing their doctor or after a hospitalization confused about their disease and their treatments. That leads to undue anxiety and unease. There seems to be not enough time for doctors to fully explain all of the details to the patients and families. Really, part of the issue is that patients are not fully able to listen to all of the details while still dealing with the shock of being told that they have a brain disease or due to the effects of the disease itself. Let’s not forget that good communication and education requires that the patient asks the right questions and that the physician provides answers that are informative but are at the level that could be understood and remembered. So, it is complex. No matter how you look at it, there is a need for better education and communication.

We use the Internet for information, so why not give the answers that patients need?

Most Internet sites for brain disease are informative, but general. I decided to write Brain Doc blog to answer questions and concerns that patients have. As a neurologist, there are common questions that we hear – so why not provide a forum for information and discussion that addresses real questions from the patient’s perspective?

Push the envelope – empower patients with data

I have another agenda that is driving me to write this blog. That is to talk about how new digital and mobile technologies can improve care and overall well-being. I strongly feel that providing patients with data about their own conditions will lead to better management and more efficient use of health resources. More importantly, the sense of security that comes with accessible personalized information can reduce stress and anxiety, and likely will improve overall brain health. Part of my entrepreneurial and research interest involves developing and testing new technologies to provide such solutions. In 2000, I founded a company to provide computerized testing of memory and other cognitive functions for clinical and research use. Now, I am developing smartphone technology to help people with migraine, where studies have shown that over 40% of an estimated 37 million people in the US are not even diagnosed correctly and even more are not optimally treated. Basically, I am a firm believer in using technology to provide data can empower patients to become active partners in their care and will ultimately lead to less suffering. I will be writing about that, as well.

I hope you enjoy the blog and provide great feedback…

Is my frequent headache really migraine?

Lisa[i] is a 38-year-old woman who has been having frequent headaches as long as she can remember. Her headaches which feel like her head is ‘splitting’, occur several times per year, and seem to be more frequent when she is under stress. Her mother also had severe headaches as long as she could remember, so Lisa thought these were something she just had to live with. Once she mentioned the headache to her family doctor and she was told to take ibuprofen, without much relief. Lisa recently talked to a friend with migraine and now really wants to know if she has migraine.

Most people with migraine are not diagnosed or treated

In general, migraine is greatly under-recognized and under-treated. Based on large population studies, just over 56% of people with migraine are ever diagnosed and treated, mostly with over the counter medications[ii]. Only 20% of diagnosed patients are treated with specific drugs for migraine, probably because only around one in three of these patients are treated by neurologists.

How do I know if I have migraine?

The best way to find out if you have migraine is to get diagnosed by your doctor according to the best practice guidelines of the International Headache Society and the American Academy of Neurology. While any doctor can arrive at the right diagnosis and treatment, usually neurologist or headache specialists are most skilled at diagnosing migraine.

Is there a simple test that I can do at home?

Researchers have found that a 3-question test can give pretty good indication if you have migraine. The test is called “ID Migraine”, and is highly reliable for screening people for migraine[iii]. Many primary care offices use this tool, and there are on-line versions as well. Keep in mind that a positive ID-Migraine test is not strictly a diagnosis, but rather should cause you to ask your doctor if you have migraine and if you should be treated with migraine treatments.

The ID Migraine Test

If you answer “Yes” to two out of the following three questions, then there is a high chance that you have migraine.

Has a headache limited your activities for a day or more in the last three months?
Are you nauseated or sick to your stomach when you have a headache?
Does light bother you when you have a headache?

Empowering patients with better knowledge

There are many types of headache and many different forms of migraine. The ID Migraine test is good for screening, but it may not good enough for diagnosing your personal headache. I am an advocate for providing patients with good, professionally-qualified information and personalized data so that better decisions can be made. As I have emphasized here, the goal is not to replace the traditional in-office medical evaluation, but rather to drive patients to ask more informed questions and to have better tools at home to make informed decisions.

[i] This ‘textbook’ case is based on collective clinical experience, and is not based on a specific patient.

[ii] Diamond S, Bigal ME, Silberstein S, Loder E, Reed M, Lipton RB. Patterns of diagnosis and acute and preventive treatment for migraine in the United States: Results from the American Migraine Prevalence and Prevention study. Headache.2007 Mar;47(3):355-63. Erratum in: Headache. 2007 Oct;47(9):1365. PubMed PMID:17371352.

[iii] Lipton RB, Dodick D, Sadovsky R, Kolodner K, Endicott J, Hettiarachchi J, Harrison W; ID Migraine validation study. A self-administered screener for migraine in primary care: The ID Migraine validation study. Neurology. 2003 Aug 12;61(3):375-82. PubMed PMID: 12913201.

What really triggers my migraine?

Anat[i], an executive at a medical start-up company, experiences migraine headaches 3-4 times per month. She believes that her headaches are triggered by eating foods with monosodium glutamate, drinking wine, and eating chocolate, so she tried to minimize these in her diet. However, Anat also notices that she sometimes gets a migraine the day after a late night. Recently, she had eaten hamburgers at a barbeque the day of her migraine, so she is now concerned that hamburgers may be a trigger – and she really does not want to avoid hamburgers. Anat wants to know what really triggers her headaches.

Most migraine sufferers have things that they think bring on their migraines

A recent review of many research studies that reported on migraine triggers concluded that a majority of people with migraine think they know what triggers their episodes and also that they can predict when a migraine will occur based on certain warning signs, otherwise known as premonitory symptoms[ii]. While the frequency of reported triggers varies from study to study, the common triggers can be categorized as:

Physical: Stress, strenuous exercise
Hormonal: Pre-menstrual, menstrual
General health: Sleep deprivation, fasting, missing meals, dehydration
Food: alcohol, chocolate, hard cheese, Chinese food
Other: Weather changes, perfume

What’s the difference between a ‘cause’, a ‘trigger’, and a ‘premonitory symptom’?

Many people report factors that may be associated with a headache with words that may be confusing: cause, trigger, and premonitory signs. To keep it straight:

A ‘cause’ is the real reason for the headache, and it is best reserved for the scientific mechanisms that underlie the migraine phenomena. An individual suffering from migraine usually has no way of knowing the true cause of the headaches. We also use the word ‘cause’ in a broader sense to indicate a factor that has a strong connection with a result. So, if drinking beer always (or almost always) resulted in a migraine, then we could conclude that it is a cause of the migraine. We usually do not have that level of certainty.
A ‘trigger’ is some real-world factor or circumstance that can predispose a migraine sufferer for a new migraine episode. You think they know about their triggers after observing that migraine episodes occurred in association with certain factors.
A ‘premonitory symptom’ is some feeling that is not specific to migraine, but that you think commonly comes hours or days before your migraine episodes. Premonitory symptoms are generally thought of as warning signs that could predict a migraine.

Why is it important to find out about my triggers and warning signs?

Of course, it would be great to prevent the migraine from coming. For one thing, if you know that you are at high risk for a migraine, you could be extra-careful to avoid the triggers that predispose you to an attack. Also, you may be careful to limit your risk of migraine episodes by avoiding multiple triggers at once. Certainly, you may also be diligent about taking your migraine medications with you if you know that there is a good chance that you will be getting a migraine.

How can I tell if certain foods really trigger migraine?

It is actually very hard to tell. Since you eat many types of food it could be hard to decide which food is the culprit. Also, it may not be the food, at all. Was it the hamburger, the ketchup, the fries, or none of the above? To make matters worse, you may be biased by what your friend thinks triggers her headaches. According to behavioral psychologists, we are not very good at figuring out causative relationships since there are always many possible interpretations of our observations, and we tend to favor interpretations that support our favorite theories (“Confirmation bias”)[iii]. So, if you already have in your mind that saccharine triggers your migraine, then you are likely to blame the diet soda rather than the Chinese food or the beer.

Solution: Collect and analyze data about your personal triggers

Scientists conduct prospective experiments to establish causative relationships. They systematically vary the suspected trigger while keeping other factors under control. It would be great if you could do that. However, in the real world the conditions are not controlled and there are lots of factors that may contribute to a migraine. So, what can be done?

Empowering patients with better data about their triggers

The best way to make meaningful connections between real-world factors is to scientifically observe and analyze a lot of data from the days preceding migraine episodes. While this was not practical before the advent of smartphones, it can now be done in an easy and effective way. I am leading a large project to log and analyze personal migraine experiences using a new smartphone app. Using advanced machine learning statistical techniques, this will not only provide valuable insight into the experiences of many migraine suffers as a group, it will also be able to provide you with insight into which of your triggers is really strongly associated with your migraine and which are not.

[i] This ‘textbook’ case is based on collective clinical experience, and is not based on a specific patient.

[ii] Pavlovic, J. M., Buse, D. C., Sollars, C. M., Haut, S. and Lipton, R. B. (2014), Trigger Factors and Premonitory Features of Migraine Attacks: Summary of Studies. Headache: The Journal of Head and Face Pain, 54: 1670–1679.

[iii] https://en.wikipedia.org/wiki/Confirmation_bias

What is the difference between “migraine” and just a regular “headache”?

Headache is the one of the most common reasons people go their doctors. People generally use the word “headache” for what neurologists call tension-type headache, and it is usually a mild to moderate pressure-like pain associated with stress and emotional tension. So, if this is what most people call “headache”, then what is “migraine”?

Migraine is a syndrome of neurological symptoms and pain

Migraine is not just about pain. In fact, there is an unusual type of migraine without pain. If so, what is migraine? It is really made up of repeated episodes of a sequence of neurological symptoms that last from one hour to several days. While people tend to experience migraine differently, overall you can see patterns variably characterized by symptoms such as:

vague warning signs before the migraine
auras – mostly visual disturbances, including enlarged blind spots and bright line configurations
pain on one side or in certain head locations
nausea
discomfort with bright lights or loud noises
Runny nose or tearing
Neck pain

Migraine is a genetic disease

Around 50% of people with migraine have a first degree relative who also has migraine. That means that some people are predisposed to migraine, and that migraine will manifest under certain conditions.

Real physiological changes happen in the brain

Research has found that a slow electrical wave of ‘spreading depression’ moves across the surface of the brain during a migraine. That could account for the symptoms of the aura, while the pain is linked to a cascade of events leading to expansion of blood vessels, release of inflammatory chemicals, and irritation of nerve endings. Also, serotonin, a brain chemical that transmit messages across cells, is decreased in migraine.

Migraine is not dangerous

Although people with migraine have a greater than normal risk of stroke later in life, that risk is small.

Even though migraine is associated with unusual activity of the brain cells, it is not a form of epilepsy.
Even though migraine can be accompanied by short-lasting visual phenomena and other neurological symptoms, it is not a form of stroke.

Summary

Migraine is a neurological condition that is different than a garden-variety ‘headache’. There is a series of physiological changes in the brain that parallel the symptoms, including the pain. Most importantly, there are now effective therapies developed specifically for migraine. So, it is important to ask your doctor if your “headache” is really “migraine”.