x

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.

Attention

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Orphanet doesn't provide personalised answers. To get in touch with the Orphanet team, please contact

Information provided in your contribution (including your email address) will be stocked in .CSV files that will be sent as an email to Orphanet's teams. These emails might be conserved in the teams' mailboxes, in our backoffice servers but will not be registered in our databases (for more information see our section General Data Protection Regulation and data privacy (GDPR) and Confidentiality).

Captcha image

Familial or sporadic hemiplegic migraine

Disease definition

A rare variety of migraine with aura characterized by the presence of a motor weakness during the aura. There are two main forms depending on 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).

ORPHA:569

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: G43.1
  • ICD-11: 8A80.10
  • OMIM: 141500  602481  607516  609634
  • UMLS: -
  • MeSH: -
  • GARD: 10768
  • MedDRA: -

Summary

Epidemiology

The prevalence of hemiplegic migraine (HM) is one in 10,000, with FHM and SHM being equally frequent.

Clinical description

Typical HM attacks are characterised by 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 patients. Severe attacks may occur in both FHM and 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 deficit.

Etiology

The three causative genes identified so far encode ion-transporters (CACNA1A, ATP1A2 and SCNA1). Molecular diagnosis is now possible through screening of these three genes.

Genetic counseling

FHM is transmitted in an autosomal dominant manner.

Management and treatment

Treatment involves the same approaches used for other varieties of migraine with aura, with the exception that triptans are contraindicated in FHM/SHM. Based on new pathophysiological insights, preventive treatments using various antiepileptic agents seem promising.

Prognosis

Prognosis is usually good.

Expert reviewer(s):  Dr Anne DUCROS - Last update: April 2008

  A summary on this disease is available in Deutsch (2008) Español (2008) Français (2008) Italiano (2008) Nederlands (2008) Português (2008)

Detailed information

Guidelines

Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.