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Genetic and Rare Diseases Information Center (GARD)

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Hemiplegic migraine


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Your Question

I experienced a headache followed by left sided numbness. I believe my doctor said I had hemiplegic migraine. During these episodes the left side of my body will feel like tiny pinpricks and go completely numb. When it gets really bad it spreads over to my right side and i cannot operate my car. When this happens I do not always get headaches. Is there any information you can give me?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is hemiplegic migraine?

Hemiplegic migraine (HM) is a rare type of migraine with aura that occurs with motor weakness during the aura. There are two types of HM which are distinguished based on the family history: familial hemiplegic migraine (FHM, in which at least one other family member has the condition) and sporadic hemiplegic migraine (SHM, in which there is no family history).[1] Signs and symptoms vary but may include visual disturbances, sensory loss, difficulty with speech, weakness on one side of the body, confusion, and impaired consciousness.[2] Severe attacks may occur in both types with prolonged hemiplegia, coma, fever and/or seizures.[1] FHM may be caused by a mutation in any of several genes and is inherited in an autosomal dominant manner.[1][2]
Last updated: 11/17/2014

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:[3]
  • 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.[3]
Last updated: 11/17/2014

What triggers hemiplegic migraine?

Minor head trauma, catheter angiography, exertion, and emotional stress have been reported by patients as triggers of hemiplegic migraine.[3]

Last updated: 8/26/2013

What causes hemiplegic migraine?

There are two types of hemiplegic migraine (HM), which are distinguished based on the family history in affected people: familial hemiplegic migraine (FHM, in which at least one other close family member has the condition) and sporadic hemiplegic migraine (SHM, in which there is no family history).[1]

FHM may be caused by changes (mutations) in the CACNA1A, ATP1A2, SCN1A, and PRRT2 genes. The first three of these genes give the body instructions for making proteins involved in moving charged atoms (ions) across cell membranes. This process is important for for normal signaling between nerve cells (neurons) in the brain and other parts of the nervous system. Researchers think that mutations in these genes may upset the balance of ions in neurons, affecting the release and uptake of certain neurotransmitters in the brain. The exact function of the PRRT2 gene is not yet understood but is also thought to play a part in controlling signals between neurons. When the genes involved in FHM do not work correctly, the signaling abnormalities between neurons lead to the development of severe headaches and auras in affected people.[4] The genetic cause in many families with FHM remains unknown.[3]

While SHM occurs in people with no history of the condition in their family, most cases are due to new (de novo) mutations that occur for the first time in the affected person. Some people with SHM do inherit a mutation from a parent who has the mutation but is not affected. The ATP1A2 and CACNA1A genes have been found to cause SHM in addition to FHM. Many people with SHM do not have a mutation in one of these genes; however, researchers think that mutations in other, unidentified genes are also involved in the condition.[5]
Last updated: 11/18/2014

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.[3] 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.[3]

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.[3]

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

References
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.