Hemiplegic migraines are one of the most severe and frightening types of migraines that come with aura. Their symptoms are identical to a stroke and this can be terrifying for all those who don’t know what’s happening. However, for patients who know what’s happening to them there is very little consolation. They usually occur in just 1 in 10000 people. These migraines are so rare there aren’t enough doctors who know ways to treat them-or even properly diagnose them. This means hemiplegic migraine patients have fewer resources at their disposal than patients with other types of migraines.
Just have a look at this patient account to visualize the terrifying, debilitating nature of this condition.
First Hand Account
I’ve been recently diagnosed with hemiplegic migraine but it’s such a rare condition that I’m having lot of trouble in finding a doctor who can recommend a proper treatment for my condition. In fact, many doctors don’t even know about this form of migraine and some who have knowledge lack depth to treat it effectively. I’ve to spend so much time and money to travel to another city just to talk to the right doctor who could properly treat it.
Most of my attacks are very long and usually I start experiencing the symptoms 2-3 days in advance before the actual attack happens. It starts with a dull but severe ache originating at the back of my head. I can feel heaviness in my head but can do nothing to escape this pain. There is no energy in me and I feel exhausted. All I want at this moment is some sleep. On the second or third day (before the actual attack) I usually have trouble in maintaining my balance. I stagger and stumble. My speech begins to get slurred.
When my attack starts, it gets really scary. The entire left side of my face droops gradually and I can feel the numbness in my face, arms and legs. I also feel difficulty in swallowing and get bit nauseous. There is some trouble seeing, and my vision is blurry and dim. During the course of attack, I start to feel paralyzed, and cannot move my legs and arms. In fact, some of my attacks are so severe my bladder fails to work and I have to immediately check into a hospital to get catheterized. I also lose consciousness with an attack.
The attack starts subsiding after couple of days, and I regain feeling in my face, arms and legs and my paralysis goes away. It doesn’t happen immediately and takes days, or even weeks. I also notice short term memory loss and learning problems. I am also tired most of the time and feel these migraines have completely hijacked my life.
Very few people get these hemiplegic migraines. In a 2002 study in Denmark, it was found that out of more than 27,000 patients who experienced headaches, just 147 had confirmed diagnosis of hemiplegic migraines.
This rarity, along with the complexities surrounding this form of migraine, makes it cumbersome for health professionals to properly diagnose and treat it. In many cases, its symptoms lead to a misdiagnosis of stroke. this makes it very difficult to treat so for the most part doctors try to use symptoms and pain management techniques including both prescription and over the counter medications.
Types of Hemiplegic Migraines
There are two types of hemiplegic migraines:
Familial hemiplegic migraines
These migraines are based on family history. If another member is also diagnosed with hemiplegic migraine then it is termed as familial.
Sporadic hemiplegic migraines
When there is no family history of hemiplegic migraines, but a person is diagnosed with it, it’s termed as sporadic. However, if later on other family members are diagnosed with hemiplegic migraine, then it will be termed as familial. The patient who was diagnosed with sporadic hemiplegic migraine initially will retain their original diagnosis.
Both these migraine types have same symptoms, but the types are characterized by the genetic component and incidence among immediate family members. The diagnosis will come after initial diagnosis about migraine has been made by the doctor.
What are the causes of Hemiplegic Migraine?
The causes of hemiplegic migraines are very complex. There are certain genes in the body that are involved and can also involve entire families. In the recent years, researchers have been successful in finding three genes directly linked with hemiplegic migraine:
Mutations, or defects, in any of these may ultimately result in breakdown of body’s ability to manufacture certain proteins. Without these proteins, cells won’t be able to send out or receive signals that go between them. One of these neurotransmitters is serotonin.
Most of those who suffer from hemiplegic migraine inherit the genes from a parent with the similar condition.
Hemiplegic migraines have same triggers as other migraines. They are:
Cerebral angiography (it’s a medical test carried out to generate images of the blood vessels in the brain, and is done using iodine and can cause a migraine in the patient)
Minor head trauma
Signs and Symptoms
Hemiplegic migraine signs and symptoms can vary in severity and incidence, depending upon the patient. Some of the most common ones include:
Reduced motor function (i.e. can’t perform some fine motor skill activities such as tying your shoes or buttoning a shirt, or having difficulty in controlling the pencil or pen while writing)
Some visual disturbances (i.e. double or blurry vision, blind spots, flashing lights, zig zag patterns in the field of vision, and light sensitivity).
Sensory loss (i.e. tingling or numbness in the extremity or face)
Hemiplegia (a form of paralysis that affects just one side of the body)
Dropping on one side of the face
Inability to speak, difficulty in speaking, or inability to properly comprehend speech (alphasisa)-mostly occurs with some weakness in the right side of the body
Hemiparesis (weakness that mostly occurs on one side of the body, affecting the entire right or left side-occurs with just one or more symptoms present during the aura.
- Attention and focusing difficulties
- Fatigue (i.e. extreme tiredness, drowsiness, , or inability to stay awake)
- Some memory loss
- Impaired consciousness
The hemiplegic migraine’s neurologic symptom can range in length from few hours to even days. They usually subside gradually until the patients returns to normal. Symptoms such as attention difficulties and memory loss can last much longer, from few weeks to even few months. However, language, visual, motor or sensory symptoms permanence is very rare.
Severe attacks come with many troubling symptoms but fortunately, they are quite rare. Symptoms of severe attacks of both types of hemiplegic migraine includes:
Prolonged hemiplegia (it’s a form of paralysis that occurs on just one side of the body and may takes days, or even weeks, to subside
Seizures (common in some forms of FHM)
- Analysis of Symptoms
- Before experiencing the actual headache pain, patient gets the signs that it’s coming. These early symptoms, mostly in the form of auras, can also include short-term trouble with muscle sensation and control:
- Numbness on one side of the body, which may include legs, arms and one half of face
- Intense pain one side of the head
- A pins-and-needle feeling, often moving up from hands to one of the arms
- Vertigo or dizziness
- Paralysis or weakness on one side of the body
- Nausea and vomiting
- Loss of balance and coordination
- Some patients may also have problem with their senses, drowsiness, and communication
- Extreme sensitivity to sound, light, and smell
- Seeing double vision, zigzag lines, blind spots and double vision
- Slurred speech
- Language difficulties, such as trouble remembering a word and mixing words
- Loss of conscious or coma (very rare)
- Auras in hemiplegic migraines usually last from 4 minutes to an hour or more. They can be more severe and last much longer than with other types of migraine.
- Hemiplegic migraines come with very specific symptoms and most of them occur early during the headache’s aura phase. Difficulty speaking, partial paralysis and drooping on just one side of the face may look like signs of a stroke, but in some cases, are symptoms of hemiplegic migraine. This can be very frustrating for patients who’ve to deal with these symptoms, but some doctors feel it’s better to be cautious right from the beginning and begin evaluation and treatment in case the patient is indeed experiencing a stroke. Unfortunately, this approach may bring little relief because origin and causes of both these conditions are different.
- The severity of the symptoms and rarity of the condition often complicate the diagnosis. As, most of the symptoms closely resemble that of a transient ischemic attack (mini-stroke) or a stroke, that is in fact the first diagnosis, although some patients are first diagnosed with severe sinus issues but later on they are identified as hemiplegic migraine patients. Most of the times, its only after further evaluation that hemiplegic migraines are considered.
Hemiplegic migraine diagnosis is done by assessing specific signs and symptoms as well as all incidences of attacks. However, in most cases there is no need to undergo genetic testing.
The exact hemiplegic migraine diagnostic criteria are:
/*747/Sensory, visual, speech, and/or language symptoms that fully reverse once the attack subsides
Also, people with hemiplegic migraine disorder should at have least two of these characteristics:
They should have had at least three attacks and they should have experienced and aura with some motor weakness that fully reverses once this attack is over
Non-motor aura symptom that last 4 to 70 minutes (counted individually, never as a group), and motor symptoms that last less than 74 hours (<74)
One or more aura symptoms that may spread gradually over 5 minutes or maybe longer (≥5), and/or at least two symptoms that immediately follow
A headache accompanies the aura or a headache which occurs within one hour of the aura
At least one aura symptom which is unilateral (one-sided), including hemiplegia, hemiparesis, and dropping on one side of the face.
During diagnosis, the doctor must rule out every possible potential conditions that are responsible for the symptoms. This means eliminating any possibility of stroke or transient ischemic attack (mini-stroke). It should be noted here that although diagnostic criteria calls for all symptoms that are reversible, it does not exclude or discount the possibility that some patients who experience severe attacks of this kind may experience many neurological issues that are not reversible and remain with them for the rest of their lives.
A sporadic hemiplegic migraine attack requires the patient is the first one in the family to experience an attack, meaning that there is no first and second degree relative has had an attack that meets the diagnostic criteria.
A familial hemiplegic migraine requires that the patient has one or more second or first degree relatives who have experienced attacks meeting the required diagnostic criteria. Also with FHM, now genetic testing for this condition is available.
To completely clarify first and second degree relatives:
A first degree relative is someone Sibling, child, or parent
A second degree relative normally shares 20-25 percent of someone’s genes-uncle, aunt, nephew, niece, grandparent, grandchildren, and half-sibling.
Hemiplegic migraine treatment can be a tricky process. It generally focuses on efficient symptom management as opposed to eliminating this condition. Doctor should take into account variety of factors, including:
Identifying and then focusing on various symptoms that are more problematic for the patient
The actual severity of the symptoms
As with other types of migraines, the causes and treatments of hemiplegic migraine can vary from person to person. For all those who’ve the familial type, it’s at least possible to trace it through family and then a good treatment plan can be created by the doctor.
Medications are often used for managing hemiplegic migraine symptoms, but it has been found that common migraine management drugs are effective for this condition as well. These FMH and SHM treatments are often reserved for patients whose attacks are frequent, severe, and long lasting. Still, they just offer symptomatic support during the attack.
There is little correlation between the type of hemiplegic migraine and the patient’s real response to the specific drug therapy being used. The common drugs that may be used are:
- Tricyclic antidepressants
- Beta blockers
- Calcium channel blocker
- Intravenous verapamil
- Oral Verapamil
- Triptans for treatment of aura
- Nasal administration of ketamine
Managing Lifestyle and Environmental Triggers
Since common migraine triggers can also trigger hemiplegic migraines, their elimination will definitely help in efficient management of this condition. Common hemiplegic migraine triggers include:
- Lights – bright lights, sun glare, light glare at night, fluorescent lighting, LED lights
- Odors – perfume, secondhand smoke, gasoline, diesel, paint thinner, and others
- Noise – repetitious or loud sounds, heavy bass in any music
Beverages like drinks and diet sodas with excessive caffeine as well as wines and different types of alcohol
Artificial sweeteners like aspartame (found in may “diet” sodas) and food additives like monosodium glutamate (MSG)
Sleep issues-too much sleep, sleep deprivation, apnea, interrupted sleep, jet lag
Processed foods and aged cheeses and foods with high salt content
Medications –vasodilators and nitroglycerin as well as some oral contraceptives and other drugs
Exertion – extensive physical exertion, intense exercise, extreme sexual activity
Environmental changes- weather changes, barometric pressure changes, climate changes (moving to different geographical location) intense cold, intense heat
Once you identify migraine triggers and learn ways to manage them, you will notice amazing improvement in the frequency of your migraines and symptoms. In fact, some may even find their headaches or migraines are eliminated altogether.
Natural Hemiplegic Migraine Treatments
Many migraine patients opt for more natural options to treat their migraines. While some of these can relieve symptoms and may even prevent attacks, they may not be of any help in more severe attacks. In addition, most of the alternative treatments are not backed by extensive research. However, thousands of migraineurs around the world have found relief, and maybe, some of them may even work for you.
Precision tinted lenses or “migraine glasses”
Stress relieving exercises or activities
You can Help someone suffering from Hemiplegic Migraine
Migraines are referred to as “invisible disability” because its symptoms are usually not apparent. Most patients will exhibit only physical symptoms like hemiplegia or hemiparesis during a hemiplegic migraine attack. When they’re not experiencing an attack, they may look completely normal as if they don’t have any condition at all. However, even when symptoms are not there, a person who has been diagnosed with a hemiplegic migraine usually has the condition all the time and they can experience an attack anytime. It just may not be apparent until there is an attack and the symptoms actually occur.
If you, your family member or some friend is suffering from hemiplegic migraine, this is important to understand this factor. Educate yourself on this topic and get more information on symptoms, especially since few of them can be scary and patient has no control over an attack (even when it is being managed with medication).
Work with the affected person to have a solid plan of action when an attack occurs so that you know what to do, whether it’s taking them to the emergency room of the hospital or helping them get to the bed. It’s important to understand that patient may experience anxiety or depression-or maybe both-during, before, and even after an attack.
If you’re a business owner or manager who has an employee with this condition, try to understand their condition by interacting with them and how this condition affects them and if there are some special accommodations that can help such as providing flexible leave or telework or controlling migraine triggers. Institute a practical plan with the employee so that if they get an attack at work you know of the right steps to handle the situation, such as calling their family members to send someone to pick them up.
Prevention and risk factors
Hemiplegic migraine attacks often start early in childhood or young adulthood. It’s more likely for all those with family history of headaches. If one of your parents suffer from hemiplegic migraine, you have 50-60 percent change of getting these headaches.
It’s not possible for you to prevent hemiplegic headaches if they run in your family. However, you should take medicine to reduce the number of headaches you experience in a week or month.
Another effective way to prevent these migraines is to avoid all factors that trigger these headaches.
Some people start getting migraine attacks as they age. In others, the condition continues to stay.
Having migraine with aura may double your risk of getting some kind of stroke-especially in case of women. This risk increases manifold if you smoke (women and men) or take any kind of birth control pills (women). However, risk of stroke is minimal.
If you have Hemiplegic Migraines
Please calm down and keep in mind that you’re not responsible for this condition. You cannot control it, so the best thing you can do is to do everything to educate yourself and learn ways to manage your symptoms. If you’re working or go to school, check if reasonable accommodations are available there to manage your condition or take definitely steps to control things which you can.
You should create a rock solid support system. Studies in the past show that all those who suffer from this condition can benefit greatly from a robust support system. Be straightforward and tell all those who are close to you to provide emotional support and help when needed. This won’t make you weak; it makes you smart. We all live in a society, and need people in our lives. If you don’t have family members or friends nearby, find a good support group for hemiplegic migraineurs, migraines, or chronic conditions. Many online forums and groups are also available:
- Patients Like Me: Hemiplegic Migraine
- Hemiplegic Migraine Support Group on Facebook
- Support Group on Facebook
- The International Hemiplegic Migraine Foundation
- Hemiplegic Migraine UK Support Forum
- Healing Well: Hemiplegic Migraine
Is there any hope in future?
Hemiplegic migraines are very rare and currently there is limited research on treatments, but this disorder is slowly coming onto the radar of key players in the field of migraine research. A clinical stage biopharmaceutical company, Xenon is vigorously conducting research on hemiplegic migraines to understand the symptoms and the experiences of patients with this condition so that they can develop effective treatments. The condition is fast gaining attention and many more studies in this field are expected to start up as more awareness is brought to the condition. Meanwhile, there are few treatments that do work and provide at least some relief.
Your doctor can prescribe a heart medication or an anticonvulsant to widen blood vessels if you’re experiencing this type of migraine. There is little research on this type of migraine, but now doctors are slowly opening up to newer medications to handle these attacks. Try to keep a migraine diary to track your attack triggers, or consult a neurologist or a doctor specially trained in migraines.
In other words, there’s hope on the horizon.
Organizations that can help with migraines
- American Migraine Foundation (AMF)
- American Headache Society Committee for Headache Education (ACHE)
- National Headache Foundation
- Migraine Research Foundation
- The Migraine Trust
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