What You Need to Know About Hemiplegic Migraines

When Migraine Symptoms Resemble a Stroke

Woman with headache
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A hemiplegic migraine is a rare type of migraine that begins in childhood.

The significant feature of hemiplegic migraine is the motor aura, which causes weakness or paralysis on one side of the body

While alarming to experience a hemiplegic migraine, the good news is that there are treatments available.

Understanding Hemiplegic Migraines

Hemiplegic migraines are different from classic migraines with auras because of the motor aura.

Even so, a person with hemiplegic migraine typically experiences other auras besides the motor aura.

For example, a person undergoing a hemiplegic migraine attack may first experience a visual aura (for example, seeing flashing lights) followed by a sensory aura (for example, numbness and tingling), and lastly, a motor aura, which is weakness that usually begins in the hand, spreading into the arm and face. While the weakness traditionally occurs on one side, some people experience it on both sides of the body.

In addition to the aura symptoms, the duration of a hemiplegic migraine aura is different from that of a typical migraine with aura. In fact, the aura of a hemiplegic migraine attack often lasts more than one hour, and in around five percent of people, it lasts more than 24 hours.

Types

There are two types of hemiplegic migraine:

Familial Hemiplegic Migraine (FHM)

As the name suggests, the familial form is inherited.

Researchers first identified three gene mutations associated with familial hemiplegic migraine:

  • FHM1 is caused by mutations in the CACNA1A gene located on chromosome 19
  • FHM2 is caused by mutations in the ATP1A2 gene located on chromosome 1
  • FHM3 is caused by mutations in the SCN1A gene located on chromosome 2

    Since these gene mutations (when the DNA sequence is altered) are inherited in an autosomal dominant pattern, if a parent has a type of familial hemiplegic migraine, his or her child has a 50 percent chance of inheriting that type.

    As research evolves on hemiplegic migraine, more genetic mutations are being discovered. For example, mutations in the PRRT2 gene have also been linked to familial hemiplegic migraine.

    Sporadic Hemiplegic Migraine (SHM)

    This type is less common than familial hemiplegic migraine and is not inherited, which means that a person will not have a family history of hemiplegic migraine. Instead, the genetic mutations of a person with sporadic hemiplegic migraine occur spontaneously.

    Symptoms

    Symptoms of sporadic hemiplegic headaches are identical to those of familial headaches. In addition to the one-sided weakness experienced during the aura phase (called hemiplegia), a hemiplegic migraine attack may also include one or more of the following symptoms:

    • Severe headache
    • Nausea and/or vomiting
    • Sensitivity to light and/or sound
    • Typical aura symptoms like vision changes, numbness and tingling, and/or trouble speaking

    In more severe hemiplegic migraine attacks, a person may experience fever, seizures, and coma.

    Treatments

    To prevent hemiplegic migraine attacks, a headache doctor may prescribe Calan SR (sustained-release verapamil) or Diamox SR (sustained-release acetazolamide). These medications are taken every day.

    Treating an acute hemiplegic migraine attack can be a bit more challenging, according to the American Migraine Foundation. This is because there are medications that doctors want people with this disorder to avoid because they may increase their risk of having a stroke. 

    These medications include:

    • Triptans
    • Ergotamine derivatives
    • Beta-blockers

    Typically, nonsteroidal anti-inflammatories (NSAIDs), anti-nausea drugs, or opioids are used to ease a hemilegic migraine.

     A Word From Verywell

    If you believe you may be experiencing a hemiplegic headache, it's imperative you seek medical attention for a thorough evaluation. Hemiplegic migraine is rare and can mimic other serious conditions, like stroke or vascular disease. If you are diagnosed with hemiplegic migraine, it's important to see a headache specialist for treatment.

    Sources:

    Headache Classification Committee of the International Headache Society. "The International Classification of Headache Disorders: 3rd Edition (beta version)". Cephalalgia 2013;33(9):629-808.

    Pelzer N, Stam AH, Haan J, Ferrari MD, Terwindt GM. Familial and sporadic hemiplegic migraine: diagnosis and treatment. Curr Treat Options Neurol. 2013 Feb;15(1):13-27.

    Pelzer N et al. Recurrent coma and fever in familial hemiplegic migraine type 2. A prospective 15-year follow-up of a large family with a novel ATP1A2 mutation. Cephalalgia. 2017 Jul;37(8):737-55.

    Robertson CE. (2017). Hemiplegic migraine. In: UpToDate, Swanson JW (Ed), UpToDate, Waltham, MA.

    Russell MB, Ducros A. Sporadic and familila hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol. 2011 May;10(5):457-70.