Hemiplegic Migraine

Sporadic and Familial Hemiplegic Migraine

The Basics

Migraine can present in a variety of ways. Hemiplegic migraine is a rare form of migraine where people experience weakness on one side of their body (hemiplegia) in addition to the migraine headache attack. The weakness is a form of migraine aura and occurs with other forms of typical migraine aura like changes in vision, speech or sensation. Hemiplegic migraine is divided into Familial hemiplegic migraine (runs in the family) or Sporadic hemiplegic migraine (happens only in one individual). This is a very rare migraine type so if you ever experience new or never-evaluated weakness with your headache, you should seek immediate medical evaluation and not assume you have hemiplegic migraine.

Both familial and sporadic hemiplegic migraines often begin in childhood. Diagnosing hemiplegic migraine can be difficult, as the symptoms can mimic stroke, seizures or other conditions. A full neurological work up, including obtaining imaging of the brain and vessels in the head, and careful review of medical history and symptoms are necessary to rule out other causes and confirm a diagnosis of hemiplegic migraine. Family medical history is especially helpful in diagnosing familial hemiplegic migraine.

Currently there are four genes related to familial hemiplegic migraine: CACNA1A, ATP1A2, SCN1A, and possibly PRRT2. These genes are related to channels on nerve membranes that control the movement of substances like sodium, calcium and potassium across the nerve. Mutations of these genes result in over-excitability of nerves. Genetic testing is available but not necessary for all people. Genetic testing may be of highest yield in people with early onset hemiplegic migraine associated with eye movement abnormalities (nystagmus), seizures or other persistent neurologic symptoms (ataxia).

Symptoms of Hemiplegic Migraine

  • Motor weakness on one side of the body (Hemiplegia)
  • Headache
  • Other typical aura symptoms – vision changes (sparkles, shimmers, visual field defects), numbness, tingling, trouble speaking
  • Fever
  • Impaired consciousness ranging from confusion to profound coma
  • Ataxia (defective muscle coordination)
  • Nausea and/or vomiting
  • Phonophobia (increased sensitivity to sound) and/or photophobia (increased sensitivity to light)

The symptoms can last for hours to days, or rarely weeks, but most resolve completely.

Please refer to the International Classification of Headache Disorders 3rd edition (beta version) website for more information on the criteria used to diagnosis hemiplegic migraine: https://www.ichd-3.org/1-migraine/1-2-migraine-with-aura/1-2-3-hemiplegic-migraine/

Treatment

Treatment of hemiplegic migraine can be challenging. The care of a headache specialist is often required, as many other doctors may never have treated a case of hemiplegic migraine.

Acute treatment:

Triptans and ergotamines are currently contraindicated in the treatment of hemiplegic migraine because of their vasoconstrictive properties (risk of vessel spasm) and concerns about stroke. One small study was conducted, safely using triptans with patients with hemiplegic migraine, but more trials are needed before they’re considered a safe option. Other treatments such as NSAIDs, antiemetics, and sometimes narcotic analgesics are used for symptomatic relief of hemiplegic migraine. Intranasal ketamine has been shown to shorten the duration of aura symptoms in patients with hemiplegic migraine.

Preventive treatment:

Given the severity of the symptoms and the contraindication of certain acute medications (triptans and ergotamines), preventive regimens (medications taken daily to prevent the attacks whether you have a headache or not) are considered especially important in the treatment of hemiplegic migraine. There are small studies reporting use of verapamil, acetazolamide, flunarizine, ketamine, lamotrigine and naloxone for treatment of hemiplegic migraine. Since hemiplegic migraine is a subset of migraine with aura, certain preventive medications commonly used to treat typical migraine with aura, including amitriptyline, topiramate, and valproic acid may be beneficial. Beta-blockers are generally avoided for people with hemiplegic migraine out of theoretical concern that it may affect the ability of vessels to dilate.

Summary

Because symptoms of hemiplegic migraine are also symptoms of other conditions such as stroke and epilepsy—a hemiplegic migraine attack can be quite frightening, both to the migraineur and to those witnessing the attacks. Proper diagnosis and treatment are especially essential with this form of migraine. Imaging studies and other testing should be performed to rule out other causes of the symptoms. It is important that people with hemiplegic migraine understand their migraines as well as possible. With continuing research, especially genetic research, more is being learned about hemiplegic migraine. As this research continues, living with hemiplegic migraine will become easier.

Resources:

The International Headache Society.  https://www.ichd-3.org/1-migraine/1-2-migraine-with-aura/1-2-3-hemiplegic-migraine/

Klapper, J., Mathew, N. & Nett, R. (2001) “Triptans in the Treatment of Basilar Migraine and Migraine With Prolonged Aura.“ Headache 41 (10), 981-984. doi: 10.1046/j.1526-4610.2001.01192.x

Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol 2011; 10:457.