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Migraine with aura

MedGen UID:
57822
Concept ID:
C0154723
Disease or Syndrome
Synonyms: Migraine with Aura; Migraine with Auras
SNOMED CT: Migraine with aura (4473006)
 
HPO: HP:0002077
Monarch Initiative: MONDO:0005475

Definition

A type of migraine in which there is an aura characterized by focal neurological phenomena that usually proceed, but may accompany or occur in the absence of, the headache. The symptoms of an aura may include fully reversible visual, sensory, and speech symptoms but not motor weakness. Visual symptoms may include flickering lights, spots and lines and/or loss of vision and/or unilateral sensory symptoms such as paresthesias or numbness. At least one of the symptoms of an aura develops gradually over 5 or more minutes and/or different symptoms occur in succession. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Migraine with aura

Conditions with this feature

Migraine, familial hemiplegic, 1
MedGen UID:
331388
Concept ID:
C1832884
Disease or Syndrome
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.
Migraine with or without aura, susceptibility to, 6
MedGen UID:
334829
Concept ID:
C1843765
Finding
Migraine with or without aura, susceptibility to, 5
MedGen UID:
334831
Concept ID:
C1843771
Finding
For a phenotypic description and discussion of genetic heterogeneity of migraine headaches, see MGR1 (157300).
Migraine with or without aura, susceptibility to, 3
MedGen UID:
375283
Concept ID:
C1843782
Finding
Migraine, familial typical, susceptibility to, 2
MedGen UID:
341144
Concept ID:
C1848066
Finding
Migraine with or without aura, susceptibility to, 11
MedGen UID:
387900
Concept ID:
C1857751
Finding
Migraine with or without aura, susceptibility to, 10
MedGen UID:
341839
Concept ID:
C1857752
Finding
Migraine, familial hemiplegic, 3
MedGen UID:
400655
Concept ID:
C1864987
Disease or Syndrome
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.
Migraine, familial hemiplegic, 2
MedGen UID:
355962
Concept ID:
C1865322
Disease or Syndrome
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.
Familial temporal lobe epilepsy 4
MedGen UID:
368897
Concept ID:
C1968847
Disease or Syndrome
A temporal lobe epilepsy characterized by autosomal dominant inheritance of occipitotemporal lobe epilepsy and migraine with visual aura and that has material basis in variation in the chromosome region 9q21-q22.
Migraine, with or without aura, susceptibility to, 13
MedGen UID:
462258
Concept ID:
C3150908
Finding
Any migraine disorder in which the cause of the disease is a mutation in the KCNK18 gene.
Advanced sleep phase syndrome 2
MedGen UID:
815204
Concept ID:
C3808874
Disease or Syndrome
Advanced sleep phase syndrome is characterized by very early sleep onset and offset (summary by Jones et al., 1999). For a discussion of genetic heterogeneity of advanced sleep phase syndrome, see FASPS1 (604348).
Migraine with or without aura, susceptibility to, 1
MedGen UID:
854348
Concept ID:
C3887485
Finding
Migraine is the most common type of chronic, episodic headache, as summarized by Featherstone (1985). One locus for migraine with or without aura (MGR1) has been identified on chromosome 4q24. Other loci for migraine have been identified on 6p21.1-p12.2 (MGR3; 607498), 14q21.2-q22.3 (MGR4; 607501), 19p13 (MGR5; 607508), 1q31 (MGR6; 607516), 15q11-q13 (MGR7; 609179), 5q21 (with or without aura, MGR8, 609570; with aura, MGR9, 609670), 17p13 (MGR10; 610208), 18q12 (MGR11; 610209), 10q22-q23 (MGR12; 611706), and the X chromosome (MGR2; 300125). Mutation in the KCNK18 gene (613655) on chromosome 10q25 causes migraine with aura (MGR13; 613656). See also familial hemiplegic migraine-1 (FHM1; 141500), a subtype of autosomal dominant migraine with aura (MA).
Brain small vessel disease 1 with or without ocular anomalies
MedGen UID:
1647320
Concept ID:
C4551998
Disease or Syndrome
The spectrum of COL4A1-related disorders includes: small-vessel brain disease of varying severity including porencephaly, variably associated with eye defects (retinal arterial tortuosity, Axenfeld-Rieger anomaly, cataract) and systemic findings (kidney involvement, muscle cramps, cerebral aneurysms, Raynaud phenomenon, cardiac arrhythmia, and hemolytic anemia). On imaging studies, small-vessel brain disease is manifest as diffuse periventricular leukoencephalopathy, lacunar infarcts, microhemorrhage, dilated perivascular spaces, and deep intracerebral hemorrhages. Clinically, small-vessel brain disease manifests as infantile hemiparesis, seizures, single or recurrent hemorrhagic stroke, ischemic stroke, and isolated migraine with aura. Porencephaly (fluid-filled cavities in the brain detected by CT or MRI) is typically manifest as infantile hemiparesis, seizures, and intellectual disability; however, on occasion it can be an incidental finding. HANAC (hereditary angiopathy with nephropathy, aneurysms, and muscle cramps) syndrome usually associates asymptomatic small-vessel brain disease, cerebral large vessel involvement (i.e., aneurysms), and systemic findings involving the kidney, muscle, and small vessels of the eye. Two additional phenotypes include isolated retinal artery tortuosity and nonsyndromic autosomal dominant congenital cataract.

Professional guidelines

PubMed

Lai J, Dilli E
Curr Neurol Neurosci Rep 2020 May 19;20(6):17. doi: 10.1007/s11910-020-01037-3. PMID: 32430657
Fraser CL, Hepschke JL, Jenkins B, Prasad S
Semin Neurol 2019 Dec;39(6):739-748. Epub 2019 Dec 17 doi: 10.1055/s-0039-1700525. PMID: 31847045
Burch R
Med Clin North Am 2019 Mar;103(2):215-233. Epub 2018 Dec 3 doi: 10.1016/j.mcna.2018.10.003. PMID: 30704678

Recent clinical studies

Etiology

Lucas C
Rev Neurol (Paris) 2021 Sep;177(7):779-784. Epub 2021 Aug 9 doi: 10.1016/j.neurol.2021.07.010. PMID: 34384631
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
Viana M, Tronvik EA, Do TP, Zecca C, Hougaard A
J Headache Pain 2019 May 30;20(1):64. doi: 10.1186/s10194-019-1008-x. PMID: 31146673Free PMC Article
Zhang Y, Parikh A, Qian S
Stroke Vasc Neurol 2017 Sep;2(3):160-167. Epub 2017 May 29 doi: 10.1136/svn-2017-000077. PMID: 28989805Free PMC Article
Calhoun AH
Headache 2017 Feb;57(2):184-193. Epub 2016 Oct 24 doi: 10.1111/head.12960. PMID: 27774589

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
Lucas C
Rev Neurol (Paris) 2021 Sep;177(7):779-784. Epub 2021 Aug 9 doi: 10.1016/j.neurol.2021.07.010. PMID: 34384631
Lai J, Dilli E
Curr Neurol Neurosci Rep 2020 May 19;20(6):17. doi: 10.1007/s11910-020-01037-3. PMID: 32430657
Fraser CL, Hepschke JL, Jenkins B, Prasad S
Semin Neurol 2019 Dec;39(6):739-748. Epub 2019 Dec 17 doi: 10.1055/s-0039-1700525. PMID: 31847045
Zhang Y, Parikh A, Qian S
Stroke Vasc Neurol 2017 Sep;2(3):160-167. Epub 2017 May 29 doi: 10.1136/svn-2017-000077. PMID: 28989805Free PMC Article

Therapy

Lucas C
Rev Neurol (Paris) 2021 Sep;177(7):779-784. Epub 2021 Aug 9 doi: 10.1016/j.neurol.2021.07.010. PMID: 34384631
Di Stefano V, Rispoli MG, Pellegrino N, Graziosi A, Rotondo E, Napoli C, Pietrobon D, Brighina F, Parisi P
J Neurol Neurosurg Psychiatry 2020 Jul;91(7):764-771. Epub 2020 May 19 doi: 10.1136/jnnp-2020-322850. PMID: 32430436Free PMC Article
Burch R
Med Clin North Am 2019 Mar;103(2):215-233. Epub 2018 Dec 3 doi: 10.1016/j.mcna.2018.10.003. PMID: 30704678
Calhoun AH
Headache 2017 Feb;57(2):184-193. Epub 2016 Oct 24 doi: 10.1111/head.12960. PMID: 27774589
Chabriat H, Joutel A, Dichgans M, Tournier-Lasserve E, Bousser MG
Lancet Neurol 2009 Jul;8(7):643-53. doi: 10.1016/S1474-4422(09)70127-9. PMID: 19539236

Prognosis

Chiang CC, Chen SP
Handb Clin Neurol 2024;199:465-474. doi: 10.1016/B978-0-12-823357-3.00021-5. PMID: 38307663
Kaniecki R
Handb Clin Neurol 2024;199:367-379. doi: 10.1016/B978-0-12-823357-3.00019-7. PMID: 38307657
de Boer I, Hansen JM, Terwindt GM
Handb Clin Neurol 2024;199:353-365. doi: 10.1016/B978-0-12-823357-3.00015-X. PMID: 38307656
Sacco S, Harriott AM, Ayata C, Ornello R, Bagur R, Jimenez-Ruiz A, Sposato LA
Neurology 2023 Apr 11;100(15):716-726. Epub 2022 Dec 15 doi: 10.1212/WNL.0000000000201699. PMID: 36522158Free PMC Article
Zhang Y, Parikh A, Qian S
Stroke Vasc Neurol 2017 Sep;2(3):160-167. Epub 2017 May 29 doi: 10.1136/svn-2017-000077. PMID: 28989805Free PMC Article

Clinical prediction guides

de Boer I, Hansen JM, Terwindt GM
Handb Clin Neurol 2024;199:353-365. doi: 10.1016/B978-0-12-823357-3.00015-X. PMID: 38307656
Coppola G, Di Lorenzo C, Parisi V, Lisicki M, Serrao M, Pierelli F
J Headache Pain 2019 Apr 29;20(1):42. doi: 10.1186/s10194-019-0997-9. PMID: 31035929Free PMC Article
Hadjikhani N, Vincent M
J Headache Pain 2019 Apr 3;20(1):32. doi: 10.1186/s10194-019-0983-2. PMID: 30943894Free PMC Article
Capo A, Affaitati G, Giamberardino MA, Amerio P
J Eur Acad Dermatol Venereol 2018 Jan;32(1):57-61. Epub 2017 Aug 2 doi: 10.1111/jdv.14472. PMID: 28707753
Vincent MB
Headache 2015 Apr;55(4):595-9. Epub 2015 Mar 11 doi: 10.1111/head.12531. PMID: 25758366

Recent systematic reviews

Onofri A, Pensato U, Rosignoli C, Wells-Gatnik W, Stanyer E, Ornello R, Chen HZ, De Santis F, Torrente A, Mikulenka P, Monte G, Marschollek K, Waliszewska-Prosół M, Wiels W, Boucherie DM, Onan D, Farham F, Al-Hassany L, Sacco S; European Headache Federation School of Advanced Studies (EHF-SAS)
J Headache Pain 2023 Feb 14;24(1):8. doi: 10.1186/s10194-023-01541-0. PMID: 36782182Free PMC Article
Christensen RH, Gollion C, Amin FM, Moskowitz MA, Hadjikhani N, Ashina M
J Headache Pain 2022 Jun 1;23(1):60. doi: 10.1186/s10194-022-01430-y. PMID: 35650524Free PMC Article
Scutelnic A, Mattle HP, Branca M, Jung S, Reichlin T, Fischer U, Schankin CJ
Eur J Neurol 2022 Mar;29(3):910-920. Epub 2021 Dec 8 doi: 10.1111/ene.15198. PMID: 34826198
Viana M, Tronvik EA, Do TP, Zecca C, Hougaard A
J Headache Pain 2019 May 30;20(1):64. doi: 10.1186/s10194-019-1008-x. PMID: 31146673Free PMC Article
Linde K, Allais G, Brinkhaus B, Fei Y, Mehring M, Vertosick EA, Vickers A, White AR
Cochrane Database Syst Rev 2016 Jun 28;2016(6):CD001218. doi: 10.1002/14651858.CD001218.pub3. PMID: 27351677Free PMC Article

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