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Familial and Sporadic Hemiplegic Migraine: Diagnosis and Treatment

Opinion statement

Hemiplegic migraine (HM) is a rare subtype of migraine with aura, characterized by transient hemiparesis during attacks. Diagnosis is based on the International Classification of Headache Disorders criteria (ICHD-II). Two types of HM are recognized: familial (FHM) and sporadic hemiplegic migraine (SHM). HM is genetically heterogeneous. Three genes have been identified (CACNA1A, ATP1A2, and SCN1A) but more, so far unknown genes, are involved. Clinically, attacks of the 3 subtypes cannot be distinguished. The diagnosis can be confirmed but not ruled out by genetic testing, because in some HM patients other, not yet identified, genes are involved. The presence of additional symptoms (such as chronic ataxia or epilepsy) may increase the likelihood of identifying a mutation. Additional diagnostics like imaging, CSF analysis, or an EEG are mainly performed to exclude other causes of focal neurological symptoms associated with headache. Conventional cerebral angiography is contraindicated in HM because this may provoke an attack. Because HM is a rare condition, no clinical treatment trials are available in this specific subgroup of migraine patients. Thus, the treatment of HM is based on empirical data, personal experience of the treating neurologist, and involves a trial-and-error strategy. Acetaminophen and NSAIDs often are the first choice in acute treatment. Although controversial in HM, triptans can be prescribed when headaches are not relieved sufficiently with common analgesics. An effective treatment for the severe and often prolonged aura symptoms is more warranted, but currently no such acute treatment is available. Prophylactic treatment can be considered when attack frequency exceeds 2 attacks per month, or when severe attacks pose a great burden that requires reduction of severity and frequency. In no strictly preferred order, flunarizine, sodium valproate, lamotrigine, verapamil, and acetazolamide can be tried. While less evidence is available for prophylactic treatment with topiramate, candesartan, and pizotifen, these drugs can also be considered. The use of propranolol in HM is more controversial, but evidence of adverse effects is insufficient to contraindicate beta-blockers.

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Acknowledgments

This work was supported by grants of the Netherlands Organization for Scientific Research (NWO) (VICI 918.56.602, M.D.F.; 907-00-217, G.M.T.; 920-03-473, A.H.S.; VIDI 91711319, G.M.T.)

Disclosure

Dr. Pelzer received support for conference visits from Menarini and Allergan UK. Dr. Ferrari received grant and consultancy or industry support from Almirall, Coherex, Colucid, Eisai, GlaxoSmithKline, Linde, MAP, Medtronic, Menarini, Merck & Co., Minster, Pfizer, Allergan, and St. Jude and independent support from NWO, NIH, European Community FP6, Biomed EC, and the Dutch Heart Foundation. Dr. Terwindt received grant and consultancy/industry support from Merck & Co., Janssen-Cilag, Allergan, and Menarini, and independent support from NWO. Dr. Stam and Dr. Haan reported no potential conflicts of interest relevant to this article.

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Correspondence to Gisela M. Terwindt MD PhD.

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Pelzer, N., Stam, A.H., Haan, J. et al. Familial and Sporadic Hemiplegic Migraine: Diagnosis and Treatment. Curr Treat Options Neurol 15, 13–27 (2013). https://doi.org/10.1007/s11940-012-0208-3

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  • DOI: https://doi.org/10.1007/s11940-012-0208-3

Keywords

  • Migraine
  • Familial hemiplegic migraine
  • Sporadic hemiplegic migraine
  • Cortical spreading depression
  • Channelopathies
  • CACNA1A gene
  • ATP1A2 gene
  • SCN1A gene
  • Imaging
  • CSF analysis
  • Electroencephalography
  • Prophylactic treatment
  • Acute treatment
  • Antiepileptic drugs
  • Calcium antagonists
  • Triptans
  • Ergot alkaloids
  • CGRP receptor antagonists
  • Beta-blockers