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Genetic and Rare Diseases Information Center (GARD)

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Hemiplegic migraine


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Overview



What is hemiplegic migraine?

What are the signs and symptoms of hemiplegic migraine?

What causes hemiplegic migraine?

How might hemiplegic migraine be treated?

What triggers hemiplegic migraine?


What is hemiplegic migraine?

Hemiplegic migraine refers to migraine with aura accompanied with motor weakness.[1] Signs and symptoms vary but may include visual disturbance, sensory loss, difficulty with speech, weakness on one side of the body, confusion, and impaired consciousness. Frequency of hemiplegic migraine can vary considerably. It can take time for symptoms to resolve, but permanent motor, sensory, language, or visual symptoms are extremely rare.[2] It can occur as a sporadic or genetic disorder. When inherited, it is passed through families in an autosomal dominant fashion.[1]
Last updated: 8/26/2013

What are the signs and symptoms of hemiplegic migraine?

Symptom and symptom severity can vary considerably between people with hemiplegic migraine. Signs and symptoms of hemiplegic migraine are outline below.

Signs and symptoms associated with aura:[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) 
Signs and symptoms associated with motor weakness:[2]

  • Hemiparesis (weakness of one limb or one side of the body)
Neurologic symptoms:

  • Confusion 
  • Drowsiness 
  • Impaired consciousness 
  • Coma
  • Psychosis
  • Memory loss

Motor weakness is always associated with at least another aura symptom. People with hemiplegic migraine often describe a typical sensory aura that starts as tingling in one of the fingers and gradually progresses to the other fingers, up to the arm, and then affects the face, tongue, and later the body and leg.[2] In some patients, paresthesias (e.g., tingling) is followed by numbness. For other people sensory loss (e.g., numbness) is always predominant.[2] 

Motor weakness involves areas affected by sensory symptoms and varies from mild clumsiness to complete deficit. Sensory-motor symptoms usually start in one hand and gradually spread up to the arm and the face. These symptoms can be restricted to one limb or can spread all over one side of the body. They can affect both sides of the body, occurring simultaneously or in succession, or remain unilateral, switching side from attack to attack, or always involving the same side.[2] Speech disturbances mostly affect expression, with rare comprehension impairment.[2]

Neurologic signs and symptoms can last for hours to days. These symptoms can outlast headache. Attention and memory loss can last weeks to months. Permanent motor, sensory, language, or visual symptoms are extremely rare. Some people with the familial form of hemiplegic migraine develop mild ataxia, usually in adulthood.[2]

Last updated: 8/26/2013

What causes hemiplegic migraine?

A mutation in the gene CACNA1A, ATP1A2, or SCN1A can cause familial hemiplegic migraine (types FHM1, FHM2, and FHM3 respectively). Familial hemiplegic migraine is passed through families in an autosomal dominant fashion.[1]

De novo
mutations
in the ATP1A2 and CACNA1A have been discovered in a few cases of sporadic hemiplegic migraine. In general, mutations in CACNA1A, ATP1A2, or SCN1A seem to be more common in families with multiple affected members (large families), and in familial or sporadic cases with early onset disease.[2] 

The underlying cause in some families with familial hemiplegic migraine and in most cases of sporadic disease has yet to be identified.[2]

Last updated: 8/26/2013

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

What triggers hemiplegic migraine?

Minor head trauma, catheter angiography, exertion, and emotional stress have been reported by patients as triggers of hemiplegic migraine.[2]

Last updated: 8/26/2013

References
  1. Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol. May, 2011; http://www.ncbi.nlm.nih.gov/pubmed/21458376. Accessed 8/26/2013.
  2. Jen JC. Familial Hemiplegic Migraine. GeneReviews. September 2009; http://www.ncbi.nlm.nih.gov/books/NBK1388/#fhm.Clinical_Description. Accessed 8/26/2013.
  3. Migraine with aura. MayoClinic. June 2013; http://www.mayoclinic.com/print/migraine-with-aura/DS00908/DSECTION=all&METHOD=print. Accessed 8/26/2013.