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Don’t fight Familial hemiplegic migraine alone.
Find your community on the free RareGuru App.Familial hemiplegic migraine (FHM) is an inherited form of hemiplegic migraine. Hemiplegic migraine is a type of migraine with aura that causes motor impairment (such as weakness) in addition to at least one visual, sensory, or speech disturbance (aura) that occurs before the migraine headache begins. FHM commonly begins during childhood or adolescence. The symptoms of FHM can be scary and distressing.
FHM is currently classified into 4 subtypes, distinguished by their genetic cause:
Inheritance of FHM is autosomal dominant, but not everyone who inherits a mutation responsible for FHM will have symptoms (a phenomenon called reduced penetrance). The diagnosis of FHM requires that at least one first- or second- degree relative has also been diagnosed with hemiplegic migraine. Tests such as a CT scan or MRI of the brain, cerebrospinal fluid analysis, and EEG may be needed to rule out other potential causes of headache and neurological symptoms. Genetic testing may confirm the subtype of FHM in a family. Treatment of hemiplegic migraine involves medications to alleviate pain, stop the migraines, and prevent future migraines. Severe headache attacks may require hospitalization. In most people with hemiplegic migraines, aura symptoms completely go away in between migraines and the migraines become less frequent with age. Rarely, hemiplegic migraines may cause permanent neurological symptoms, cognitive impairment, stroke, coma, or death.
Source: GARD Last updated on 05-01-20
Familial hemiplegic migraine (FHM) commonly begins during childhood or adolescence. It is characterized by migraine with aura along with motor impairment (such as weakness on one side of the body, known as hemiparesis), in addition to “classic” aura which is marked by visual, sensory, and/or speech disturbances. The aura usually occurs before the migraine headache begins, developing over minutes and lasting less than an hour. Motor symptoms such as weakness may switch sides between or during attacks, and rarely may affect both sides of the body. While a headache doesn’t always follow, when it does, it causes throbbing pain, nausea, and/or sensitivity to light. The headache can last several hours to a few days The symptoms of FHM can be scary and distressing.
Last updated on 05-01-20
The benefits and/or limitations of Topamax in treating individuals with FHM have not been well studied. We were not able to find information in the medical literature specifically noting an effect on memory retention. More research is needed to determine whether this medication has a positive impact on this symptom.
Last updated on 05-01-20
Topamax associated side effects are reported to occur in approximately 70% of individuals prescribed the medication for prevention (prophylaxis) of migraines. The most commonly reported side effects are a burning or prickling sensation in the hands, legs or feet (paresthesia), fatigue, nausea, difficulties with concentration, taste disturbance, and weight loss. Topiramate may cause other side effects as well. For a full list of side effects, visit the page on Topiramate provided by MedlinePlus, a resources through the National Library of Medicine.
These side effects occur most frequently during times of medication adjustment and lead to discontinuation of use in about 30% of cases. We unfortunately were not able to locate information in the medical literature describing the amount of time these symptoms are typically present while using this medication. As with any side effects caused by medications, we recommend you discuss your concerns with your prescribing physician.
If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (http://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).
Last updated on 05-01-20
Although most people with familial hemiplegic migraine (FHM) recover completely between episodes, neurological symptoms such as memory loss and problems with attention can last for weeks or months. The occurrence of FHM attacks tends to decrease with age. The eventual neurologic outcome is often benign; however, about 20 percent of individuals with FHM develop mild but permanent difficulty coordinating movements (ataxia), which may worsen with time, and nystagmus (rapid, involuntary eye movements). Unusually severe migraine episodes have been reported in some people with FHM. These episodes may include fever, seizures, prolonged weakness, coma, and, rarely, death.
Last updated on 05-01-20
There are no official treatment guidelines for familial hemiplegic migraine (FHM), and no randomized, controlled trials regarding treatment for hemiplegic migraine have been conducted. People with hemiplegic migraine typically are treated with the same medicines used for typical migraine with aura (for pain, and to stop or prevent migraines). However, medicines that constrict the blood vessels (vasoconstrictors) should be avoided because they may increase the risk for stroke. Examples of vasoconstrictors that may be used to treat typical migraine with aura include triptans and ergotamines. Treatment may depend on the severity and frequency of attacks as well as specific symptoms, and therefore may vary from person to person. Multiple therapies, either alone or in combination, may need to be tried before finding a treatment regime that is helpful.
Treatment of sudden hemiplegic migraine “attacks” aims to ease symptoms and may include the use of non-steroidal anti-inflammatory drugs (NSAIDs), anti- nausea medicines (antiemetics), and/or prescription pain medicines. A medicine called ketamine, ingested through the nose (intranasal), may shorten the duration of aura symptoms if taken as soon as symptoms begin.
Examples of medicines that may be used to prevent attacks (prophylactic medicines) may include verapamil, flunarizine, ketamine, lamotrigine, and naloxone. Non-randomized studies have suggested acetazolamide may be effective in preventing attacks in some people with FHM. Other medicines that may be used to prevent attacks include amitriptyline, topiramate, and valproic acid. Individualized anti-seizure therapy may additionally be needed for people with FHM who have seizures (particularly those with FHM type 2).
Last updated on 05-01-20
The Migraine Trust, a support organization, offers information on Familial hemiplegic migraine
Last updated on 04-27-20
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