Familial hemiplegic migraine: what is it?

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Familial hemiplegic migraine is a rare genetic form of migraine that causes weakness on one side of the body. Symptoms can include visual auras, headache, sensitivity to light or sound, and nausea or vomiting. It is passed on by an autosomal dominant gene and can be diagnosed through genetic testing. Treatment options include preventive, abortive, and rescue medications.

Familial hemiplegic migraine, also known as FHM, is a relatively rare type of migraine that causes weakness or impaired function on one side of the body, or hemiplegia. Migraine is considered familial because it has a genetic component and therefore runs in families; it should be noted, the symptoms are often indistinguishable from a non-genetic form of the disorder called Sporadic Hemiplegic Migraine (SHM). Familial hemiplegic migraine is among the more severe forms of migraine, with a wider range of symptoms and longer lasting residual effects.

Symptoms of familial hemiplegic migraine may be similar to those of a classic migraine, which may include pre-onset visual auras, headache, sensitivity to light or sound, and nausea or vomiting. Most classic migraines, however, do not include the hemiplegia typical of familial hemiplegic migraines. Symptoms may also include ataxia or general muscle weakness; mental confusion; visual impairment; and sometimes sudden onset, which can mimic a stroke. In a classic migraine, the symptoms usually stop after the attack ends, but some familial hemiplegic migraine symptoms can continue for hours or even days after the migraine ends. Migraine can also occur without headache. While most classic migraines begin around puberty or adolescence, familial hemiplegic migraine can also occur in children, usually presenting as seizures. This type of migraine can also begin to dissipate during menopause, while classic migraines can often last a lifetime.

The propensity for any type of migraine is usually genetic, but familial hemiplegic migraine is passed on by an autosomal dominant gene, meaning that only one parent with the dominant gene is needed to carry the disorder. Diagnosing familial hemiplegic migraine typically requires that a patient exhibit most of the symptoms associated with SHM and that at least one first-degree relative – a parent, child, or sibling – has symptoms. Genetic testing for familial hemiplegic migraine involves the identification of mutations in the ATP1A2, CACNA1A and SCN1A genes. Researchers have discovered three slightly different forms of migraine, known as FHM-1, -2 and -3. Each form corresponds to the mutation of a single gene.

Familial hemiplegic and SHM migraines are more serious and potentially more dangerous than classic migraines, so care and attention must be paid to their prevention and treatment. Most migraine attacks are caused by a combination of external and internal triggers. Some triggers, such as emotional stress, can be difficult to avoid; others, such as alcohol use, are easily avoided. Migraine medications typically fall into three categories: Preventive medications work to stop migraines before they start; abortive treatments attempt to stop a migraine during an attack; and rescue medication is usually a form of pain reliever and works to relieve or mask the pain until an attack has passed. Patients should discuss options with their doctors, particularly as abortion drugs can lose their effectiveness over time.




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