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Hemiplegic migraine is a severe headache which leads to weakness on one side of the body. As is typical with other migraine headaches, nausea and light sensitivity are also present. The average frequency of these headaches is three per year. Some patients experience a few hemiplegic migraines during a lifetime while others get them up to 250 times per year.
The symptoms of this headache type may lead the sufferer to believe they are having a stroke. The standard stroke symptoms are:
- Numbness on the side of the body affected
- Drowsiness
- Dizziness
- Visual disturbances, also known as an aura
- Slurred speech or other speaking difficulties
- Tingling or numbness on the face, arm or leg of the affected side of the body
In severe cases, patients may need to be hospitalized due to high fever, changes in consciousness or seizure activity.
Two Types Of Hemiplegic Migraine
Familial Hemiplegic Migraine
This type of migraine runs in families and can persist for several generations. Familial hemiplegic migraine is an autosomal dominant form of migraine. This means the patient may get the abnormal gene from only one parent. It is common for only a single parent to suffer from hemiplegic migraine.
The following genes are associated with hemiplegic migraine:
- ATP1A2
- CACNA1A
- PRRT2
- SCN1A
Mutations in these genes affect neurotransmitter release in the brain. Mutated genes interrupt communication between nerve cells. This lack of communication may lead to visual disturbances and severe migraine headaches.
Sporadic Hemiplegic Migraine
This type happens to only one individual and is not passed down to future generations. This migraine type is rare. If you experience weakness or other stroke symptoms described above with your headache, seek immediate medical attention. The headache may be a symptom of a more severe disorder.
Both types of hemiplegic migraine typically begin in childhood. The diagnosis may be difficult because other conditions such as stroke and certain seizure types have similar symptoms.
Patients should visit a neurologist who will obtain a thorough family history, perform imaging tests of the brain and get lab tests as necessary.
Hemiplegic Migraine Triggers
The triggers for these migraine types are thought to be similar to those of other migraines. These triggers include:
- increased stress
- consuming caffeine and alcohol
- intense emotions
- sleep disturbances
- not eating regular meals
- certain food types: processed foods, salty foods, aged cheeses, chocolate, and foods with added MSG
- changes in the weather
- bright or flashing lights
It is essential to be aware of the triggers that cause the headache as the most effective treatment for this condition is prevention.
Hemiplegic Migraine Treatment
Non-steroidal anti-inflammatory drugs (NSAIDS) such as ibuprofen (Motrin) and naproxen (Aleve) can be used for acute pain relief. If the pain does not respond to NSAIDS, narcotic analgesics such as Norco (hydrocodone/acetaminophen) or oxycodone may be utilized.
Ondansetron can be used for nausea associated with hemiplegic migraine.
Intranasal ketamine has been shown to decrease the severity and duration of visual disturbances associated with hemiplegic migraine.1
Ergotamines and triptans should not been used for hemiplegic migraine. The FDA has mandated that package labelling for ergotamines (DHE) and triptans (Imitrex) state a contraindication for use in basilar and hemiplegic migraine. This is due to their vasoconstrictive properties, which may lead to vessel spasm and concerns about precipitating a stroke.
A small study conducted by Mathew et al. in May of 2016 stated the following: BM refers to basilar migraine and HM refers to hemiplegic migraine in the following study conclusion:
“in this retrospective study, triptans and DHE were used with no reported, subsequent acute/subacute ischemic vascular events for the abortive treatment of migraines with basilar and hemiplegic-type features. Although the small sample sizes generated theoretical statistical event rates of 4.5% for BM and 23% for HM, there has been no clear evidence that BM and HM carry an actual elevated risk for vascular events compared with migraine with aura”.
This study raises the possibility of utilizing triptans and DHE in the future for hemiplegic migraine treatment. More studies are needed to confirm the safety of these agents for the treatment of this condition.
Prevention
Hemiplegic migraines can be debilitating. If these headaches occur often, it may be necessary to take daily prophylactic medication. There are no randomized controlled trials for hemiplegic migraine prevention, but there are some case reports available. The following drugs have the best evidence for hemiplegic migraine prevention at the current time:
Verapamil
Verapamil has been studied in a limited number of patients for the treatment and prevention of hemiplegic migraine. Four patients with sporadic hemiplegic migraine were given verapamil 120mg one to three times daily. The attacks resolved within two months in two of the patients. A third patient had greater than 50% reduction in headache severity and frequency,
and the fourth patient improved with 5mg verapamil IV followed by 120mg PO verapamil daily. The verapamil had to be discontinued in this patient due to side effects, and the headaches slowly returned to baseline frequency.
It is important to note that other studies have found little to no benefit of verapamil for hemiplegic migraine treatment. 4
Flunarizine
Flunarizine has some evidence for its use in hemiplegic migraine but is unavailable in the United States and Japan. It is a calcium channel blocker and was originally used to improve blood flow. It is marketed as the brand name Sibelium.
Acetazolamide
Acetazolamide was shown to improve hemiplegic migraine in several case reports. Acetazolamide was dosed at 250 mg twice a day in these studies.
Lamotrigine
Lamotrigine is an anticonvulsant medication used to treat certain types of seizures. It is also used to treat bipolar disorder. Lamotrigine must be started at low doses and titrated to the target dose slowly due to the danger of life-threatening rashes.
Lamotrigine showed benefit in a majority of patients suffering from migraine with aura in a case series of 47 patients. In this study conducted in 2004, Lamotrigine was reported to be beneficial in the majority of patients, including two who had hemiplegic migraine.
In other studies, lamotrigine has had mixed evidence regarding its usefulness in decreasing migraine attack frequency and intensity.
Based on the data above, the drugs with the best evidence for hemiplegic migraine prevention are verapamil and acetazolamide. Flunarizine may also be used if available. If these agents are ineffective, lamotrigine is an alternative, although evidence is limited to support its use in this condition.
Hemiplegic Migraine Disability
Patients who suffer from hemiplegic migraine headaches may qualify for social security benefits. The Social Security Administration (SSA) does not specify exact guidelines to qualify for benefits regarding migraines. If you can prove that your headaches prevent you from performing necessary work duties regularly, you may be eligible for SSA benefits.
Some factors the SSA will take into consideration are the following:
- Ability to concentrate
- Ability to walk, stand, and lift items
- Ability to interact with other employees
- Ability to understand instructions
It is critical to convey the frequency and duration of attacks and the symptoms experienced, which limit the ability to perform any job.
The SSA will also evaluate the severity of the headaches, other medical conditions present, and how often the conditions keep the individual from performing a job.
This is accomplished by evaluating medical records and a functional report completed by the patient and physician. It is essential that this form contains a detailed description of how headaches affect job performance.
Keep in mind that the SSA will make their determination based on the ability to perform any full-time job, not necessarily the current position held. If the SSA determines the patient is capable of holding a full-time position, the claim will be denied.
Hemiplegic migraine is a rare condition that can be detrimental to a patient’s life. There are two types, familial which is the genetic form and sporadic. Both types have symptoms similar to a stroke. If this is a new condition experienced, the sufferer should seek medical attention immediately.
As with classic migraines, there are triggers which may cause headaches. Hemiplegic migraines can be challenging to treat.
For this reason, I suggest keeping a headache journal. Keep track of activities and food intake before the headache occurred. If specific foods, drinks or activities are found to precipitate migraines, these can be avoided in the future.
Pay attention to the time of day the headaches occur. Are there more headaches at different times of the year or when the weather changes? Do the headaches seem to happen after experiencing loud noises or bright lights? This information can be important in helping reduce the number of attacks.
Some patients may experience debilitating headaches frequently. It may not be possible for these individuals to perform primary job duties. It is possible to qualify for SSA disability benefits in some cases.
As with any medical condition, if the headaches become debilitating, seek medical attention. There may be medications available that can help patients live happier, healthier lives.
Michael J. Brown, RPh, BCPS, BCPP
Mr. Brown is a Clinical Pharmacist specializing in pharmacotherapy and psychiatry.
Feel free to send Michael a message using this link.
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Kaube H, Herzog J, Käufer T, et al. Aura in some patients with familial hemiplegic migraine can be stopped by intranasal ketamine. Neurology 2000; 55:139.
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Headache. 2016 May;56(5):841-848. doi: 10.1111/head.12804. Epub 2016 Apr 8.
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Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol 2011; 10:457.
- Peer Mohamed B, Goadsby PJ, Prabhakar P. Safety and efficacy of flunarizine in childhood migraine: 11 years' experience, with emphasis on its effect in hemiplegic migraine. Dev Med Child Neurol 2012;54:274.
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