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What Is Hemiplegic Migraine?


Updated: October 5, 2004

To understand Hemiplegic Migraine, we have to understand that Migraine attacks are episodic manifestations of a genetic neurological disease. Migraine can present in a variety of ways. Hemiplegic Migraine is a rare form of Migraine disease, made more confusing by there being two variations: Familial Hemiplegic Migraine (FHM) and Sporadic Hemiplegic Migraine (SHM).

Both FHM and SHM often begin in childhood and cease during adult years. Diagnosing FHM and SHM can be difficult as the symptoms are also indicative of vascular disease. and can be thought to be stroke, epilepsy, or other conditions. A full neurological work up and careful review of medical history and symptoms are necessary to rule out other causes and confirm a diagnosis of FHM or SHM. Family medical history is especially helpful in diagnosing FHM.

FHM and SHM share the same symptoms, which will vary among different Migraineurs. The difference between the two is that FHM can be traced back in the family history and has been linked to mutations of specific genes on chromosomes 1 and 19. SHM is FHM without the familial connection and that particular genetic mutation.

Symptoms of FHM and SHM:

  • Episodes of prolonged aura (up to several days or weeks)

  • Hemiplegia (paralysis on one side of the body)

  • Fever

  • Meningismus (symptoms of meningitis without the actual illness and accompanying inflammation)

  • Impaired consciousness ranging from confusion to profound coma

  • Headache, which may begin before the hemiplegia or be absent

  • Ataxia (defective muscle coordination)

  • The onset of the hemiplegia may be sudden and simulate a stroke.¹

  • Nausea and/or vomiting

  • Phonophobia and/or photophobia

Treatment of FHM and SHM:
Treatment of Hemiplegic Migraine can be challenging. The symptoms are greater in number and more difficult to treat. Those who experience Hemiplegic Migraines absolutely need to educate themselves about their disease and treatment. It's very common to need to seek the care of an excellent Migraine specialist with an established track record for treatment as many other doctors have never treated a case of Hemiplegic Migraine.

  • Abortive and Pain Relief: Migraine-specific abortives, the triptans and ergotamines, are currently contraindicated in the treatment of Hemiplegic Migraine because of their vasoconstrictive properties 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. Since the triptans and ergotamines aren't options, other treatments such as NSAID's, antiemetics, and narcotic analgesics are generally used for relief of Hemiplegic Migraine.

  • Preventive: Given the severity of the symptoms and the contraindication of abortive medications, preventive regimens are considered especially important in the treatment of Hemiplegic Migraine. As noted above the genes for FHM are mapped on chromosomes 1 and 19, These code for the calcium channel. "The mutant calcium channel does not open and close properly and cannot regulate the amount of calcium coming into the cell, so calcium influx and efflux regulation goes awry. This in turn leads to neurons firing too easily." For this reason, calcium channel blockers are sometimes especially effective preventive medications for FHM.

A Word of Caution:
Migraineurs with Hemiplegic Migraine should give special consideration to wearing some kind of medical identification at all times since an attack can lead to impaired consciousness and an inability to speak. Medical identification can save valuable time in an emergency and assure that proper treatment is received far more quickly.

>>For a more in-depth version of this article, please click HERE.<<

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