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Genetic and Rare Diseases Information Center (GARD)

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Familial hemiplegic migraine type 1


Other Names for this Disease

  • FHM1
  • Hemiplegic migraine, familial type 1
  • MHP1
  • Migraine, familial hemiplegic 1, with progressive cerebellar ataxia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is familial hemiplegic migraine?

What are the signs and symptoms of hemiplegic migraine?

How might hemiplegic migraine be treated?

What is familial hemiplegic migraine?

Familial hemiplegic migraine (FHM) is a form of migraine headache that runs in families. Migraines usually cause intense, throbbing pain in one area of the head, often accompanied by nausea, vomiting, and extreme sensitivity to light and sound. These recurrent headaches typically begin in childhood or adolescence and may last from a few hours to a few days. People with familial hemiplegic migraine experience an aura that comes before the headache. The most common symptoms associated with an aura are temporary visual changes such as blind spots (scotomas), flashing lights, zig-zagging lines, and double vision. In people with familial hemiplegic migraine, auras are also characterized by temporary numbness or weakness, often affecting one side of the body (hemiparesis). An aura typically develops gradually over a few minutes and lasts about an hour. Researchers have identified three forms of familial hemiplegic migraine known as FHM1, FHM2, and FHM3. Each of the three types is caused by mutations in a different gene. [1]
Last updated: 11/30/2012

What are the signs and symptoms of hemiplegic migraine?

The symptoms and severity can vary considerably among people with hemiplegic migraine. Signs and symptoms associated with aura may include:[2]
  • Visual disturbance (e.g. blind spots, flashing lights, zigzag pattern, and double vision)
  • Sensory loss (e.g., numbness or paresthesias of the face or an extremity)
  • Difficulty with speech (which usually occur along with right-sided weakness)
Motor weakness involves areas affected by sensory symptoms and varies from mild clumsiness to complete deficit. Affected people may also experience neurologic symptoms such as confusion, drowsiness, impaired consciousness, coma, psychosis, and/or memory loss. Neurologic symptoms can last for hours to days. Attention and memory loss can last weeks to months. However, permanent motor, sensory, language, or visual symptoms are extremely rare.[2]
Last updated: 11/17/2014

How might hemiplegic migraine be treated?

Treatment of hemiplegic migraine varies depending on severity and which symptoms are most problematic for the patient. In general, treatments aim to manage symptoms. Drugs that are effective in the prevention of common migraines may be used in hemiplegic migraine.[2] Prophylactic management is applied to patients with frequent, long lasting, or severe attacks. Examples of migraine drugs that have been tried with variable success in people with hemiplegic migraine, include oral verapamil, acetazolamide, lamotrigine.[2]

There are a few articles describing the use of nasal administration of ketamine, intravenous verapamil, and triptans for treatment of aura in people with hemiplegic migraine. Use of triptans in hemiplegic migraine is controversial and may be contraindicated in people with severe attacks.[2]

For further information on these and other treatments, we recommend that you speak with your healthcare provider.
Last updated: 8/26/2013

References
  1. Jen JC. Familial hemiplegic migraine. GeneReviews. September 8, 2009; http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=fhm#fhm.Management. Accessed 9/12/2013.
  2. Jen JC. Familial Hemiplegic Migraine. GeneReviews. September 2009; http://www.ncbi.nlm.nih.gov/books/NBK1388/#fhm.Clinical_Description.


Other Names for this Disease
  • FHM1
  • Hemiplegic migraine, familial type 1
  • MHP1
  • Migraine, familial hemiplegic 1, with progressive cerebellar ataxia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.