U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Familial hemiplegic migraine

MedGen UID:
87374
Concept ID:
C0338484
Disease or Syndrome
Synonyms: Familial Hemiplegic Migraine; Familial Hemiplegic Migraines; Hemiplegic Migraine, Familial; Hemiplegic-Ophthalmoplegic Migraine
SNOMED CT: Familial hemiplegic migraine (95656000); Hemiplegic-ophthalmoplegic migraine (95656000)
 
Related genes: SCN1A, CACNA1A, ATP1A2
 
Monarch Initiative: MONDO:0000700
OMIM® Phenotypic series: PS141500

Disease characteristics

Excerpted from the GeneReview: Familial Hemiplegic Migraine
Familial hemiplegic migraine (FHM) falls within the category of migraine with aura. In migraine with aura (including FHM) the neurologic symptoms of aura are unequivocally localizable to the cerebral cortex or brain stem and include visual disturbance (most common), sensory loss (e.g., numbness or paresthesias of the face or an extremity), and dysphasia (difficulty with speech). FHM must include motor involvement, such as hemiparesis (weakness of an extremity). Hemiparesis occurs with at least one other symptom during FHM aura. Neurologic deficits with FHM attacks can be prolonged for hours to days and may outlast the associated migrainous headache. FHM is often earlier in onset than typical migraine, frequently beginning in the first or second decade; the frequency of attacks tends to decrease with age. Approximately 40%-50% of families with CACNA1A-FHM have cerebellar signs ranging from nystagmus to progressive, usually late-onset mild ataxia. [from GeneReviews]
Authors:
Joanna C Jen   view full author information

Additional description

From MedlinePlus Genetics
Familial hemiplegic migraine is a form of migraine headache that runs in families. Migraines usually cause intense, throbbing pain in one area of the head, often accompanied by nausea, vomiting, and extreme sensitivity to light and sound. These recurrent headaches typically begin in childhood or adolescence and can be triggered by certain foods, emotional stress, and minor head trauma. Each headache may last from a few hours to a few days.

Unusually severe migraine episodes have been reported in some people with familial hemiplegic migraine. These episodes have included fever, seizures, prolonged weakness, coma, and, rarely, death. Although most people with familial hemiplegic migraine recover completely between episodes, neurological symptoms such as memory loss and problems with attention can last for weeks or months. About 20 percent of people with this condition develop mild but permanent difficulty coordinating movements (ataxia), which may worsen with time, and rapid, involuntary eye movements called nystagmus.

In some types of migraine, including familial hemiplegic migraine, a pattern of neurological symptoms called an aura precedes the headache. The most common symptoms associated with an aura are temporary visual changes such as blind spots (scotomas), flashing lights, zig-zagging lines, and double vision. In people with familial hemiplegic migraine, auras are also characterized by temporary numbness or weakness, often affecting one side of the body (hemiparesis). Additional features of an aura can include difficulty with speech, confusion, and drowsiness. An aura typically develops gradually over a few minutes and lasts about an hour.  https://medlineplus.gov/genetics/condition/familial-hemiplegic-migraine

Professional guidelines

PubMed

Indelicato E, Boesch S
Handb Exp Pharmacol 2023;279:227-248. doi: 10.1007/164_2022_625. PMID: 36592223
Le Roux M, Barth M, Gueden S, Desbordes de Cepoy P, Aeby A, Vilain C, Hirsch E, de Saint Martin A, Portes VD, Lesca G, Riquet A, Chaton L, Villeneuve N, Villard L, Cances C, Valton L, Renaldo F, Vermersch AI, Altuzarra C, Nguyen-Morel MA, Van Gils J, Angelini C, Biraben A, Arnaud L, Riant F, Van Bogaert P
Eur J Paediatr Neurol 2021 Jul;33:75-85. Epub 2021 May 26 doi: 10.1016/j.ejpn.2021.05.010. PMID: 34102571
Russell MB
Expert Rev Neurother 2010 Mar;10(3):381-7. doi: 10.1586/ern.09.127. PMID: 20187861

Curated

Orphanet, Hemiplegic Migraine (HM), 2013

Recent clinical studies

Etiology

Sanchez Del Rio M, Cutrer FM
Handb Clin Neurol 2023;198:71-83. doi: 10.1016/B978-0-12-823356-6.00016-0. PMID: 38043972
Grangeon L, Lange KS, Waliszewska-Prosół M, Onan D, Marschollek K, Wiels W, Mikulenka P, Farham F, Gollion C, Ducros A; European Headache Federation School of Advanced Studies (EHF-SAS)
J Headache Pain 2023 Feb 20;24(1):12. doi: 10.1186/s10194-023-01547-8. PMID: 36800925Free PMC Article
Ducros A
Rev Neurol (Paris) 2021 Sep;177(7):801-808. Epub 2021 Jul 23 doi: 10.1016/j.neurol.2021.06.002. PMID: 34304862
Anttila V, Wessman M, Kallela M, Palotie A
Handb Clin Neurol 2018;148:493-503. doi: 10.1016/B978-0-444-64076-5.00031-4. PMID: 29478595
Thomsen LL, Olesen J
Cephalalgia 2004 Dec;24(12):1016-23. doi: 10.1111/j.1468-2982.2004.00788.x. PMID: 15566415

Diagnosis

Nandyala A, Shah T, Ailani J
Curr Neurol Neurosci Rep 2023 Jul;23(7):381-387. Epub 2023 May 29 doi: 10.1007/s11910-023-01277-z. PMID: 37247170
Indelicato E, Boesch S
Handb Exp Pharmacol 2023;279:227-248. doi: 10.1007/164_2022_625. PMID: 36592223
Brunklaus A, Brünger T, Feng T, Fons C, Lehikoinen A, Panagiotakaki E, Vintan MA, Symonds J, Andrew J, Arzimanoglou A, Delima S, Gallois J, Hanrahan D, Lesca G, MacLeod S, Marjanovic D, McTague A, Nuñez-Enamorado N, Perez-Palma E, Scott Perry M, Pysden K, Russ-Hall SJ, Scheffer IE, Sully K, Syrbe S, Vaher U, Velayutham M, Vogt J, Weiss S, Wirrell E, Zuberi SM, Lal D, Møller RS, Mantegazza M, Cestèle S
Brain 2022 Nov 21;145(11):3816-3831. doi: 10.1093/brain/awac210. PMID: 35696452Free PMC Article
Hommersom MP, van Prooije TH, Pennings M, Schouten MI, van Bokhoven H, Kamsteeg EJ, van de Warrenburg BPC
J Neurol 2022 Jun;269(6):3094-3108. Epub 2021 Nov 22 doi: 10.1007/s00415-021-10897-9. PMID: 34806130
Kors E, Haan J, Ferrari M
Curr Pain Headache Rep 2003 Jun;7(3):212-7. doi: 10.1007/s11916-003-0075-4. PMID: 12720601

Therapy

Gotra P, Bhardwaj N, Ludhiadch A, Singh G, Munshi A
Mol Neurobiol 2021 Aug;58(8):3874-3883. Epub 2021 Apr 15 doi: 10.1007/s12035-021-02386-x. PMID: 33856647
Hansen JM, Hauge AW, Ashina M, Olesen J
Cephalalgia 2011 Sep;31(12):1274-81. Epub 2011 Jul 22 doi: 10.1177/0333102411415878. PMID: 21784774
Russell MB
Expert Rev Neurother 2010 Mar;10(3):381-7. doi: 10.1586/ern.09.127. PMID: 20187861
Gordon N
Brain Dev 1998 Jan;20(1):9-13. doi: 10.1016/s0387-7604(97)00086-7. PMID: 9533553
Glista GG, Mellinger JF, Rooke ED
Mayo Clin Proc 1975 Jun;50(6):307-11. PMID: 1127995

Prognosis

Mangano GD, Capizzi MR, Mantuano E, Veneziano L, Santangelo G, Quatrosi G, Nardello R, Raieli V
Headache 2023 Jul-Aug;63(7):889-898. Epub 2023 Jun 16 doi: 10.1111/head.14582. PMID: 37326332
Lipman AR, Fan X, Shen Y, Chung WK
Clin Genet 2022 Oct;102(4):288-295. Epub 2022 Jun 26 doi: 10.1111/cge.14180. PMID: 35722745Free PMC Article
Vetro A, Nielsen HN, Holm R, Hevner RF, Parrini E, Powis Z, Møller RS, Bellan C, Simonati A, Lesca G, Helbig KL, Palmer EE, Mei D, Ballardini E, Van Haeringen A, Syrbe S, Leuzzi V, Cioni G, Curry CJ, Costain G, Santucci M, Chong K, Mancini GMS, Clayton-Smith J, Bigoni S, Scheffer IE, Dobyns WB, Vilsen B, Guerrini R; ATP1A2/A3-collaborators
Brain 2021 Jun 22;144(5):1435-1450. doi: 10.1093/brain/awab052. PMID: 33880529
Hasırcı Bayır BR, Tutkavul K, Eser M, Baykan B
Seizure 2021 May;88:87-94. Epub 2021 Mar 31 doi: 10.1016/j.seizure.2021.03.028. PMID: 33839563
Haan J, Kors EE, Vanmolkot KR, van den Maagdenberg AM, Frants RR, Ferrari MD
Curr Pain Headache Rep 2005 Jun;9(3):213-20. doi: 10.1007/s11916-005-0065-9. PMID: 15907261

Clinical prediction guides

Lipman AR, Fan X, Shen Y, Chung WK
Clin Genet 2022 Oct;102(4):288-295. Epub 2022 Jun 26 doi: 10.1111/cge.14180. PMID: 35722745Free PMC Article
Vetro A, Nielsen HN, Holm R, Hevner RF, Parrini E, Powis Z, Møller RS, Bellan C, Simonati A, Lesca G, Helbig KL, Palmer EE, Mei D, Ballardini E, Van Haeringen A, Syrbe S, Leuzzi V, Cioni G, Curry CJ, Costain G, Santucci M, Chong K, Mancini GMS, Clayton-Smith J, Bigoni S, Scheffer IE, Dobyns WB, Vilsen B, Guerrini R; ATP1A2/A3-collaborators
Brain 2021 Jun 22;144(5):1435-1450. doi: 10.1093/brain/awab052. PMID: 33880529
Anttila V, Wessman M, Kallela M, Palotie A
Handb Clin Neurol 2018;148:493-503. doi: 10.1016/B978-0-444-64076-5.00031-4. PMID: 29478595
Vincent M, Hadjikhani N
Headache 2007 Jun;47(6):820-33. doi: 10.1111/j.1526-4610.2006.00715.x. PMID: 17578530Free PMC Article
Haan J, Kors EE, Vanmolkot KR, van den Maagdenberg AM, Frants RR, Ferrari MD
Curr Pain Headache Rep 2005 Jun;9(3):213-20. doi: 10.1007/s11916-005-0065-9. PMID: 15907261

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...