I was recently diagnosed with hemiplegic migraines but it is such a rare condition I had a lot of trouble finding a doctor to diagnose and treat me. Many doctors don’t even know anything about it or not enough to treat it effectively. I had to drive several hours to another city just to talk to a doctor who could treat it.
My attacks are very long and I start having symptoms about two days before the attack. It will start with a dull but severe ache in the back of my head. My head feels very heavy and there is nothing I can do to escape the pain. I have no energy and am exhausted. All I want to do is sleep. The second day (the day before the attack) I usually start to have trouble with my balance. I stumble and stagger. My speech also begins to get slurred.
When the attack starts, it gets really scary. The whole left side of my face starts to droop and I get a numbness in my face, arms, and legs. I also have a hard time swallowing and get nauseous. I have a lot of trouble seeing, my vision is dim and blurry. As the attack goes on, I will start to feel paralyzed, unable to move my arms and legs. Some attacks are so severe my bladder won’t work so I have to check into the hospital and be catheterized. I have also lost consciousness with an attack.
After a couple of days the attack will start to subside. I regain the feeling in my face, arms, and legs and the paralysis goes away. It isn’t all at once though. Sometimes it takes three weeks for all the symptoms to go away completely. I do notice that I have learning problems and some short term memory loss. I am also tired all the time. Sometimes it feels like these migraines have hijacked my life.
“Margaret”Hemiplegic migraines are very rare. In fact, a study in Denmark in 2002 found that out of more than 27,000 people who experienced headaches, only 147 had a confirmed diagnosis of hemiplegic migraine. This rarity, coupled with the complexities surrounding this type of migraine, make it difficult for doctors to diagnose and treat. In fact, the symptoms of hemiplegic migraine often lead to a misdiagnosis of stroke. This also makes it difficult to treat so for the most part doctors use symptom and pain management techniques including both over the counter and prescription medication.
What is Hemiplegic MigraineHemiplegic migraine is an extremely rare type of migraine with aura. Researchers estimate that it is present in only about 0.005% of the population. Dr. Mark Khorsandi, a migraine surgeon at the Migraine Relief Center in Houston, Texas, says, “A hemiplegic migraine occurs when the migraineur experiences paralysis on one side of the body. The attack may also cause aura and visual changes, difficulty in speech, and confusion. Some have likened the symptoms of this type of migraine to that of a stroke.” This video, posted by a hemiplegic migraine patient, was shot during one of her attacks. You will notice she has paralysis on her left side – she is dragging her left leg and her left arm is completely limp. She is having to be helped to the restroom by her mother and sister. The extent of the paralysis shown here may be upsetting for some viewers.
Types of Hemiplegic MigrainesThere are two types of hemiplegic migraines, familial and sporadic. While the symptoms are the same, the types are characterized by the genetic component and incidence among family members. The diagnosis of type comes after the initial diagnosis of the migraine itself.
Familial hemiplegic migraine (FHM) – This is based solely on family history. If another family member has been diagnosed with a hemiplegic migraine, then others in the family who are diagnosed later with the same thing will be typed as familial.
Sporadic hemiplegic migraine (SHM) – When a person is diagnosed with hemiplegic migraine, yet there is not family history, they are typed as sporadic. However, if any family members are later diagnosed with hemiplegic migraine, those family members will be typed familial. The patient diagnosed with sporadic hemiplegic migraine will retain their original diagnosis.
Cause of Hemiplegic MigraineThe cause of hemiplegic migraines is very complex. There are certain genes involved that can involve entire families. Dr. Khorsandi explains, “There are two different types of hemiplegic migraines – familial (FHM) and sporadic (SHM). FHM is a genetic condition associated with gene mutations. SHM occurs in people without the family history of the disorder.” To further clarify, hemiplegic migraines tend to run in families, meaning that there is a genetic component. However, a SHM can occur in a patient with no family history and potentially initiate a FHM occurrence if other family members begin experiencing them. This means that a family can have no incidence of the migraine, then the gene mutates in one person. From that point on, that mutated gene has the potential to be passed on to children, grandchildren, and so on through the direct family line. A husband cannot “catch” it from his wife and it cannot be transmitted from one person to another except through heredity where the gene is passed to the children. It is also worth noting that the mutated gene can be passed on without any symptoms, only to show up a generation or two later. Hemiplegic migraines are also susceptible to the same typical triggers that provoke other migraines. These include:
- Minor head trauma
- Cerebral angiography (a medical test that produces images of the brain’s blood vessels – it is the contrast, which contains iodine, that is given to the patient that can cause a migraine)
Signs and symptomsSigns and symptoms of hemiplegic migraine can vary in incidence and severity, depending on the patient. Some of the most common include:
- Decreased motor function (i.e. clumsy, can’t perform fine motor skill activities like buttoning a shirt or tying your shoes, or difficulty controlling the pen while writing)
- Visual disturbances (i.e. blurry or double vision, flashing lights, blind spots, light sensitivity, and zig zag patterns in the field of vision)
- Hemiparesis (weakness that occurs on one side of the body, affecting that entire left or right side – occurs with one or more other symptoms present during the aura)
- Sensory loss (i.e. numbness or tingling in the extremity or face)
- Hemiplegia (paralysis that affects one side of the body)
- Aphasia (difficulty speaking, inability to speak, or inability to comprehend speech – often occurs with weakness on the right side)
- Drooping on one side of the face
- Neurologic symptoms
- Attention difficulties
- Fatigue (i.e. drowsiness, extreme tiredness, or inability to stay awake)
- Impaired consciousness
- Memory loss
- Prolonged hemiplegia (the paralysis that occurs on one side of the body takes longer – days or weeks – to subside
- Seizures (common in some forms of FHM)
Diagnosing Hemiplegic MigraineDoctors diagnose hemiplegic migraine by assessing specific symptoms and signs as well as incidence of attacks. For most people, it is not necessary to undergo genetic testing. The diagnostic criteria for hemiplegic migraine are:
- The patient must have had at least 2 attacks and the attacks must have had:
- Aura with motor weakness that fully reversed after the attack, and
- Visual, sensory, and/or speech/language symptoms that fully reversed after the attack
- The patient must also have at least 2 of these characteristics:
- One or more aura symptoms that gradually spread over 5 minutes or longer (≥5), and/or at least two symptoms that follow
- Non-motor aura symptom that last 5 to 60 minutes (counted individually, not as a group), and motor symptoms that last less than 72 hours (<72)
- At least one aura symptom that is one-sided (unilateral), including hemiparesis, hemiplegia, and drooping on one side of the face.
- A headache accompanies the aura or a headache occurs within one hour of the aura.
- A first degree relative is someone’s child, sibling, or parent.
- A second degree relative shares 25% of someone’s genes – aunt, uncle, niece, nephew, grandchildren, grandparent, and half-sibling.
Treatment of Hemiplegic MigraineHemiplegic migraine treatment is not usually a straightforward process. The treatments generally focusing on symptom management as opposed to elimination of the condition. The doctor must take into consideration a variety of factors, including:
- The severity of the symptoms
- Identifying and focusing on the symptoms that are the most problematic for the patient
Medication are often used to manage the symptoms of hemiplegic migraine, but it has also been found that drugs used to prevent common migraines may be effective for this condition as well. These treatments for SHM and FHM are often reserved for patients whose attacks are severe, long lasting, and frequent. Still, they only offer symptomatic support during the migraine attack.
There is very little correlation between the type of hemiplegic migraine and the patient’s response to the drug therapy that is used. Some of the types of drugs that may be used include:
- Beta blockers
- Tricyclic antidepressants
- Calcium channel blocker
- Oral Verapamil
- Intravenous verapamil
- Nasal administration of ketamine
- Triptans for treatment of aura
Management of Environmental or Lifestyle Triggers
Since typical migraine triggers can also trigger hemiplegic migraines, eliminating those triggers can also help to manage the condition. Common migraine triggers include:
- Sensory stimuli:
- Odors – secondhand smoke, perfume, gasoline, paint thinner, diesel and others
- Lights – sun glare, bright lights, light glare at night, LED lights, fluorescent lighting
- Noise – loud or repetitious sounds, heavy bass in music
- Beverages such as diet sodas and drinks that contain a lot of caffeine as well as wine and other types of alcohol
- Food additives like monosodium glutamate (MSG) and aspartame (artificial sweetener found in most “diet” sodas)
- Processed foods as well as aged cheeses and foods that are very salty
- Sleep issues – sleep deprivation, too much sleep, interrupted sleep, apnea, jet lag
- Exertion – intense exercise, extreme sexual activity, extensive physical exertion
- Medications – nitroglycerin and other vasodilators as well as oral contraceptives and other drugs
- Environmental changes- barometric pressure changes, weather changes, climate changes (moving from one geographical location to another), intense heat, intense cold
By identifying their migraine triggers and learning how to manage them, patients will often notice a significant improvement in the frequency of their migraines and symptoms, or they may find their headaches are eliminated altogether.
Some migraine patients choose more natural treatment options for their migraines. While these may help relieve symptoms and even prevent some migraines, they may not help with more severe attacks. Additionally, most of these alternative migraine treatments do not have a lot of research behind them and some may be controversial. However, there are migraineurs who have found relief and, who knows, they may work for you too.
- Precision tinted lenses or “migraine glasses”
- Stress relieving exercises or activities
How to Help Someone Suffering from Hemiplegic MigrainesMigraines are often referred to as an “invisible disability or condition” because the symptoms are usually not physically apparent. The patient will likely only exhibit the physical symptoms like hemiparesis or hemiplegia during a hemiplegic migraine attack. When they are not having an attack, they may look and act as if they have no condition at all. However, even when the symptoms are not present, a person who has been diagnosed with hemiplegic migraine has the condition all the time and they could have an attack at any time. It just may not be apparent until there is an attack and the symptoms occur. If you have a family member or friend who has hemiplegic migraine, this is very important to understand. Take some time to educate yourself on hemiplegic and become familiar with the symptoms, especially since some of them can be pretty scary and the patient has no control over an attack (even when managing it with medication). Work with the person to have a plan of action when an attack occurs so that you know what to do, whether it is helping them to bed or taking them to the emergency department of the hospital. It is also important to understand that the person may experience depression or anxiety – or both – before, during, and after an attack. If you are a manager or business owner who has an employee with hemiplegic migraine, sit down with them to learn how the condition affects them and if there are any special accommodations that can help such as controlling migraine triggers or providing flexible leave or telework when the employee has a migraine. Institute a plan with the employee so that if they have an attack at work you know how to respond appropriately, such as calling a family member to pick them up.
If you have Hemiplegic MigrainesThe first and most important thing you need to know is that no part of your condition is your fault. You cannot control this condition so the best thing you can do for your own peace of mind is educate yourself and learn how to manage your symptoms. Learn about reasonable accommodations that are available to you at work or school, and take definitive steps to control the things that you can. It is also important to create a strong support system. Studies show that people who suffer from chronic health conditions benefit greatly from a good support system. Don’t be afraid to let the people who care about you in so they can provide emotional support and help when needed. This doesn’t make you weak; it makes you smart. None of us is an island. We all need people in our lives. If you don’t have friends or family nearby, find a support group for hemiplegic migraines, migraineurs, or chronic conditions. You can also join online groups or forums:
- Hemiplegic Migraine Support Group on Facebook
- The International Hemiplegic Migraine Foundation Support Group on Facebook
- Hemiplegic Migraine UK Support Forum
- Patients Like Me: Hemiplegic Migraine
- Healing Well: Hemiplegic Migraine
What is on the HorizonHemiplegic migraines are rare and there isn’t much research regarding treatments, but it is slowly coming onto the radar of key players in migraine research. Xenon, a clinical stage biopharmaceutical company is currently conducting research on hemiplegic migraines to better understand hemiplegic migraine patients’ symptoms and their experiences with the condition so that they can develop better treatments. The condition is gaining attention and more studies are expected to start up as more awareness is brought to the condition. In the meantime, there are treatments that do work and provide at least some relief. Dr. Khorsandi sums it up very well. “You may be prescribed an anticonvulsant or heart medication to widen blood vessels if you are suffering from this type of migraine,” he says. “There is little research on these migraines, but migraine doctors are opening up new types of medication that can help with these attacks. We recommend keeping a migraine diary to track triggers of an attack, or consult a doctor or neurologist specially trained in migraines.” In other words, there’s hope on the horizon.
Organizations for Resources and Help with Migraines
- American Headache Society Committee for Headache Education (ACHE)
- American Migraine Foundation (AMF)
- Migraine Research Foundation
- National Headache Foundation
- The Migraine Trust