[Familial and sporadic hemiplegic migraine]
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Published on: June 2, 2014
1Centre d'urgences céphalées, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France. anne.ducros@lrb.aphp.fr
Abstract:
Hemiplegic migraine (HM) is a rare variety of migraine with aura characterized by the presence of a motor weakness during the aura. Hemiplegic migraine has two main forms according to the familial history: patients with at least one first- or second-degree relative who has aura including motor weakness have familial hemiplegic migraine (FHM); patients without such familial history have sporadic hemiplegic migraine (SHM). The prevalence of HM is one in 10,000 with FHM and SHM being equally frequent. Typical HM attacks include a motor weakness that is always associated with other aura symptoms, the most frequent being sensory, visual and speech disorders. In addition, basilar-type symptoms occur in up to 70% of the patients. Severe attacks may occur in FHM as well as in SHM with prolonged hemiplegia, confusion, coma, fever and seizures. The clinical spectrum also includes permanent cerebellar signs (nystagmus, ataxia, dysarthria) and less frequently various types of seizures and intellectual deficiency. FHM is the only variety of the autosomal dominant migraine and all three know genes encode ion-transporters. A genetic diagnosis is now possible by screening the three known genes involved in FHM (CACNA1A, ATP1A2 and SCNA1). Prognosis is usually good. Treatment is similar to approaches used in other varieties of migraine with aura, excepted for triptans that are contraindicated in MHF/MHS. Based on new pathophysiological insight, preventive treatments by various antiepileptic agents seem promising.
Insights
Hemiplegic migraine (HM) is a rare neurological disorder causing temporary motor weakness. Genetic testing is now available for familial hemiplegic migraine (FHM), with promising new preventive treatments.
Area of Science:
- Neurology
- Genetics
- Migraine Disorders
Context:
- Hemiplegic migraine (HM) is a rare subtype of migraine with aura.
- Characterized by transient motor weakness during the aura phase.
- Two forms exist: familial (FHM) and sporadic (SHM), with equal frequency.
Purpose:
- To describe the characteristics, genetics, and treatment of hemiplegic migraine.
- To highlight the availability of genetic diagnosis for FHM.
- To discuss current and emerging treatment strategies.
Summary:
- HM attacks involve motor weakness alongside other aura symptoms like sensory, visual, and speech disturbances.
- Severe attacks can include prolonged hemiplegia, confusion, coma, fever, and seizures.
- FHM is an autosomal dominant condition linked to ion-transporter genes (CACNA1A, ATP1A2, SCNA1).
Impact:
- Genetic diagnosis for FHM is now feasible through gene screening.
- Prognosis for HM is generally good.
- While triptans are contraindicated, preventive treatments with antiepileptic agents show promise.
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