Familial and sporadic hemiplegic migraine: Diagnosis and treatment

Current Treatment Options in Neurology

Volumn 15, Issue 1, 2013, Pages 13-27

  Pelzer, Nadine ^a   Stam, Anine H ^a   Haan, Joost ^a,b   Ferrari, Michel D ^a   Terwindt, Gisela M ^a  

^a LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

^b RIJNLAND HOSPITAL   (Netherlands)

Author keywords

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

Indexed keywords

ACETAZOLAMIDE; ADENOSINE TRIPHOSPHATASE (POTASSIUM SODIUM); AMIODARONE; ANTACID AGENT; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; CALCIUM CHANNEL P TYPE; CALCIUM CHANNEL Q TYPE; CANDESARTAN; CARBAMAZEPINE; CENTRAL DEPRESSANT AGENT; DIGOXIN; FLECAINIDE; FLUNARIZINE; LAMOTRIGINE; LITHIUM; NONSTEROID ANTIINFLAMMATORY AGENT; PARACETAMOL; PHENYTOIN; PIZOTIFEN; PLACEBO; PROPRANOLOL; PROTON PUMP INHIBITOR; REMIFENTANIL; SUFENTANIL; TOPIRAMATE; TRIPTAN DERIVATIVE; UNINDEXED DRUG; VALPROIC ACID; VERAPAMIL; VOLTAGE GATED SODIUM CHANNEL;

APATHY; ARTICLE; ATAXIA; ATP1A2 GENE; ATRIOVENTRICULAR BLOCK; BLEEDING TIME; BONE MARROW SUPPRESSION; BRADYCARDIA; BRAIN ANGIOGRAPHY; BRAIN DISEASE; CACNA1A GENE; CADASIL; CEREBROSPINAL FLUID ANALYSIS; CHANNEL GATING; CHEST TIGHTNESS; CONFUSION; DEPRESSION; DIFFERENTIAL DIAGNOSIS; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISEASE SEVERITY; DIZZINESS; DROWSINESS; DRUG BLOOD LEVEL; DRUG CONTRAINDICATION; DRUG COST; DRUG DOSE INCREASE; DRUG ERUPTION; DRUG MECHANISM; EDEMA; ELECTROENCEPHALOGRAPHY; EPILEPSY; EXTRAPYRAMIDAL SYMPTOM; FAMILIAL HEMIPLEGIC MIGRAINE; FATIGUE; FLUSHING; GASTROINTESTINAL DISEASE; GENE; GENE IDENTIFICATION; GENE MUTATION; GENETIC SCREENING; HEADACHE; HEART FAILURE; HUMAN; HYPERPROLACTINEMIA; HYPOTENSION; INCREASED APPETITE; INTERNATIONAL CLASSIFICATION OF HEADACHE DISORDERS II; LIVER INJURY; MELAS SYNDROME; MIGRAINE WITH AURA; NAUSEA; NEUROMODULATION; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; PARESTHESIA; PRESCRIPTION; SCN1A GENE; SIDE EFFECT; SLEEP DISORDER; SPORADIC HEMIPLEGIC MIGRAINE; STRESS MANAGEMENT; SYMPTOM; TERATOGENICITY; TREMOR; WEIGHT GAIN; WEIGHT REDUCTION;

EID: 84873083524     PISSN: 10928480     EISSN: 15343138     Source Type: Journal    
DOI: 10.1007/s11940-012-0208-3     Document Type: Article

References (100)

1. Thomsen LL, Kirchmann EM, Faerch RS, et al. An epidemiological survey of hemiplegic migraine. Cephalalgia. 2002;22:361-75.
2. The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;24(Suppl 1):9-160.
3. Goadsby PJ, Lipton RB, Ferrari MD. Migraine - current understanding and treatment. N Engl J Med. 2002;346:257-70.
4. Lauritzen M. Pathophysiology of the migraine aura. The spreading depression theory. Brain. 1994;117(Pt 1):199-210.
5. Olesen J, Larsen B, Lauritzen M. Focal hyperemia followed by spreading oligemia and impaired activation of rCBF in classic migraine. Ann Neurol. 1981;9:344-52.
6. Hadjikhani N, Sanchez Del Rio M, Wu O, et al. Mechanisms of migraine aura revealed by functional MRI in human visual cortex. Proc Natl Acad Sci USA. 2001;98:4687-92.
7. Bigal ME, Tepper SJ. Ergotamine and dihydroergotamine: a review. Curr Pain Headache Rep. 2003;7:55-62.
8. Ferrari MD, Saxena PR. Clinical and experimental effects of sumatriptan in humans. Trends Pharmacol Sci. 1993;14:129-33.
9. Schoonman GG, Van Der Grond J, Kortmann C, et al. Migraine headache is not associated with cerebral or meningeal vasodilatation-a 3 T magnetic resonance angiography study. Brain. 2008;131:2192-200.
10. Asghar MS, Hansen AE, Amin FM, et al. Evidence for a vascular factor in migraine. Ann Neurol. 2011;69:635-45.
11. Stam AH, Haan J, van den Maagdenberg AMJM, et al. Migraine and genetic and acquired vasculopathies. Cephalalgia. 2009;29:1006-17.
12. Burstein R, Yarnitsky D, Goor-Aryeh I, et al. An association between migraine and cutaneous allodynia. Ann Neurol. 2000;47:614-24.
13. Thomsen LL, Olesen J, Russell MB. Increased risk of migraine with typical aura in probands with familial hemiplegic migraine and their relatives. Eur J Neurol. 2003;10:421-7.
14. Eriksen MK, Thomsen LL, Olesen J. Implications of clinical subtypes of migraine with aura. Headache. 2006;46:286-97.
15. Haan J, Terwindt GM, Ophoff RA, et al. Is familial hemiplegic migraine a hereditary form of basilar migraine? Cephalalgia. 1995;15:477-81.
16. Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol. 2011;10:457-70. An excellent and extensive review article about many aspects of HM.
17. Ducros A, Denier C, Joutel A, et al. The clinical spectrum of familial hemiplegic migraine associated with mutations in a neuronal calcium channel. N Engl J Med. 2001;345:17-24.
18. Kors EE, Terwindt GM, Vermeulen FL, et al. Delayed cerebral edema and fatal coma after minor head trauma: role of the CACNA1A calcium channel subunit gene and relationship with familial hemiplegic migraine. Ann Neurol. 2001;49:753-60.
19. Jurkat-Rott K, Freilinger T, Dreier JP, et al. Variability of familial hemiplegic migraine with novel ATP1A2 Na+/K + -ATPase variants. Neurology. 2004;62:1857-61.
20. Ophoff RA, Terwindt GM, Vergouwe MN, et al. Familial hemiplegic migraine and episodic ataxia type-2 are caused by mutations in the Ca2+ channel gene CACNL1A4. Cell. 1996;87:543-52.
21. de Vries B, Frants RR, Ferrari MD, van den Maagdenberg AMJM. Molecular genetics of migraine. Hum Genet. 2009;126:115-32.
22. Kors EE, Haan J, Giffin NJ, et al. Expanding the phenotypic spectrum of the CACNA1A gene T666M mutation: a description of 5 families with familial hemiplegic migraine. Arch Neurol. 2003;60:684-8.
23. Riant F, De Fusco M, Aridon P, et al. ATP1A2 mutations in 11 families with familial hemiplegic migraine. Hum Mutat. 2005;26:281.
24. Lebas A, Guyant-Marechal L, Hannequin D, et al. Severe attacks of familial hemiplegic migraine, childhood epilepsy and ATP1A2 mutation. Cephalalgia. 2008;28:774-7.
25. Vanmolkot KR, Stroink H, Koenderink JB, et al. Severe episodic neurological deficits and permanent mental retardation in a child with a novel FHM2 ATP1A2 mutation. Ann Neurol. 2006;59:310-4.
26. Spadaro M, Ursu S, Lehmann-Horn F, et al. A G301R Na+/K + -ATPase mutation causes familial hemiplegic migraine type 2 with cerebellar signs. Neurogenetics. 2004;5:177-85.
27. Dichgans M, Freilinger T, Eckstein G, et al. Mutation in the neuronal voltage-gated sodium channel SCN1A in familial hemiplegic migraine. Lancet. 2005;366:371-7.
28. Riant F, Ducros A, Ploton C, et al. De novo mutations in ATP1A2 and CACNA1A are frequent in early-onset sporadic hemiplegic migraine. Neurology. 2010;75:967-72.
29. de Vries B, Freilinger T, Vanmolkot KR, et al. Systematic analysis of three FHM genes in 39 sporadic patients with hemiplegic migraine. Neurology. 2007;69:2170-6.
30. Thomsen LL, Kirchmann M, Bjornsson A, et al. The genetic spectrum of a population-based sample of familial hemiplegic migraine. Brain. 2007;130:346-56.
31. Stuart S, Roy B, Davies G, et al. Detection of a novel mutation in the CACNA1A gene. Twin Res Hum Genet. 2012;15:120-5.
32. Thomsen LL, Oestergaard E, Bjornsson A, et al. Screen for CACNA1A and ATP1A2 mutations in sporadic hemiplegic migraine patients. Cephalalgia. 2008;28:914-21.
33. Terwindt G, Kors E, Haan J, et al. Mutation analysis of the CACNA1A calcium channel subunit gene in 27 patients with sporadic hemiplegic migraine. Arch Neurol. 2002;59:1016-8.
34. Vahedi K, Denier C, Ducros A, et al. CACNA1A gene de novo mutation causing hemiplegic migraine, coma, and cerebellar atrophy. Neurology. 2000;55:1040-2.
35. Hayashi R, Tachikawa H, Watanabe R, et al. Familial hemiplegic migraine with irreversible brain damage. Intern Med. 1998;37:166-8.
36. Butteriss DJ, Ramesh V, Birchall D. Serial MRI in a case of familial hemiplegic migraine. Neuroradiology. 2003;45:300-3.
37. Spacey SD, Vanmolkot KR, Murphy C, et al. Familial hemiplegic migraine presenting as recurrent encephalopathy in a Native Indian family. Headache. 2005;45:1244-9.
38. Ohmura K, Suzuki Y, Saito Y, et al. Sporadic hemiplegic migraine presenting as acute encephalopathy. Brain Dev. 2012;34:691-5.
39. Bhatia R, Desai S, Tripathi M, et al. Sporadic hemiplegic migraine: report of a case with clinical and radiological features. J Headache Pain. 2008;9:385-8.
40. Jacob A, Mahavish K, Bowden A, et al. Imaging abnormalities in sporadic hemiplegic migraine on conventional MRI, diffusion and perfusion MRI and MRS. Cephalalgia. 2006;26:1004-9.
41. Dreier JP, Jurkat-Rott K, Petzold GC, et al. Opening of the blood-brain barrier preceding cortical edema in a severe attack of FHM type II. Neurology. 2005;64:2145-7.
42. Iizuka T, Sakai F, Suzuki K, et al. Implication of augmented vasogenic leakage in the mechanism of persistent aura in sporadic hemiplegic migraine. Cephalalgia. 2006;26:332-5.
43. Mjaset C, Russell MB. Intravenous nimodipine worsening prolonged attack of familial hemiplegic migraine. J Headache Pain. 2008;9:381-4.
44. Beauvais K, Cave-Riant F, De Barace C, et al. New CACNA1A gene mutation in a case of familial hemiplegic migraine with status epilepticus. Eur Neurol. 2004;52:58-61.
45. Prodan CI, Holland NR, Lenaerts ME, Parke JT. Magnetic resonance angiogram evidence of vasospasm in familial hemiplegic migraine. J Child Neurol. 2002;17:470-2.
46. Gonzalez-Alegre P, Tippin J. Prolonged cortical electrical depression and diffuse vasospasm without ischemia in a case of severe hemiplegic migraine during pregnancy. Headache. 2003;43:72-5.
47. Pierelli F, Grieco GS, Pauri F, et al. A novel ATP1A2 mutation in a family with FHM type II. Cephalalgia. 2006;26:324-8.
48. Motta E, Rosciszewska D, Miller K. Hemiplegic migraine with CSF abnormalities. Headache. 1995;35:368-70.
49. Gomez-Aranda F, Canadillas F, Marti-Masso JF, et al. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain. 1997;120(Pt 7):1105-13.
50. Berg MJ, Williams LS. The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology. 1995;45:1648-54.
51. Yilmaz A, Kaleagasi H, Dogu O, et al. Abnormal MRI in a patient with 'headache with neurological deficits and CSF lymphocytosis (HaNDL). Cephalalgia. 2010;30:615-9.
52. Raets I. Diffusion restriction in the splenium of the corpus callosum in a patient with the syndrome of transient headache with neurological deficits and CSF lymphocytosis (HaNDL): a challenge to the diagnostic criteria? Acta Neurol Belg. 2012;112:67-9.
53. Chapman KM, Szczygielski BI, Toth C, et al. Pseudomigraine with lymphocytic pleocytosis: a calcium channelopathy? Clinical description of 10 cases and genetic analysis of the familial hemiplegic migraine gene CACNA1A. Headache. 2003;43:892-5.
54. Heinzen EL, Swoboda KJ, Hitomi Y, et al. De novo mutations in ATP1A3 cause alternating hemiplegia of childhood. Nat Genet. 2012;44:1030-4.
55. Hutchinson M, O'Riordan J, Javed M, et al. Familial hemiplegic migraine and autosomal dominant arteriopathy with leukoencephalopathy (CADASIL). Ann Neurol. 1995;38:817-24.
56. Montagna P, Gallassi R, Medori R, et al. MELAS syndrome: characteristic migrainous and epileptic features and maternal transmission. Neurology. 1988;38:751-4.
57. Hansen JM, Hauge AW, Ashina M, Olesen J. Trigger factors for familial hemiplegic migraine. Cephalalgia. 2011;31:1274-81.
58. Schoonman GG, Evers DJ, Ballieux BE, et al. Is stress a trigger factor for migraine? Psychoneuroendocrinology. 2007;32:532-8.
59. Ye Q, Yan LY, Xue LJ, et al. Flunarizine blocks voltage-gated Na(+) and Ca(2+) currents in cultured rat cortical neurons: a possible locus of action in the prevention of migraine. Neurosci Lett. 2011;487:394-9.
60. Tobita M, Hino M, Ichikawa N, et al. A case of hemiplegic migraine treated with flunarizine. Headache. 1987;27:487-8.
61. De Cunto A, Bensa M, Tonelli A. A case of familial hemiplegic migraine associated with a novel ATP1A2 gene mutation. Pediatr Neurol. 2012;47:133-6.
62. Mohamed BP, Goadsby PJ, Prabhakar P. Safety and efficacy of flunarizine in childhood migraine: 11 years' experience, with emphasis on its effect in hemiplegic migraine. Dev Med Child Neurol. 2012;54:274-7.
63. Dobrev D, Milde AS, Andreas K, Ravens U. The effects of verapamil and diltiazem on N-, P- and Q-type calcium channels mediating dopamine release in rat striatum. Br J Pharmacol. 1999;127:576-82.
64. Yu W, Horowitz SH. Familial hemiplegic migraine and its abortive therapy with intravenous verapamil. Neurology. 2001;57:1732-3.
65. Yu W, Horowitz SH. Treatment of sporadic hemiplegic migraine with calcium-channel blocker verapamil. Neurology. 2003;60:120-1.
66. Razavi M, Razavi B, Fattal D, et al. Hemiplegic migraine induced by exertion. Arch Neurol. 2000;57:1363-5.
67. Cutrer FM, Limmroth V, Moskowitz MA. Possible mechanisms of valproate in migraine prophylaxis. Cephalalgia. 1997;17:93-100.
68. Evers S, Afra J, Frese A, et al. EFNS guideline on the drug treatment of migraine-revised report of an EFNS task force. Eur J Neurol. 2009;16:968-81.
69. Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-2.
70. Stefani A, Spadoni F, Siniscalchi A, Bernardi G. Lamotrigine inhibits Ca2+ currents in cortical neurons: functional implications. Eur J Pharmacol. 1996;307:113-6.
71. Steiner TJ, Findley LJ, Yuen AW. Lamotrigine versus placebo in the prophylaxis of migraine with and without aura. Cephalalgia. 1997;17:109-12.
72. Chen WT, Fuh JL, Lu SR, Wang SJ. Persistent migrainous visual phenomena might be responsive to lamotrigine. Headache. 2001;41:823-5.
73. Lampl C, Buzath A, Klinger D, Neumann K. Lamotrigine in the prophylactic treatment of migraine aura-a pilot study. Cephalalgia. 1999;19:58-63.
74. D'Andrea G, Granella F, Cadaldini M, Manzoni GC. Effectiveness of lamotrigine in the prophylaxis of migraine with aura: an open pilot study. Cephalalgia. 1999;19:64-6.
75. Lampl C, Katsarava Z, Diener HC, Limmroth V. Lamotrigine reduces migraine aura and migraine attacks in patients with migraine with aura. J Neurol Neurosurg Psychiatry. 2005;76:1730-2.
76. Pascual J, Caminero AB, Mateos V, et al. Preventing disturbing migraine aura with lamotrigine: an open study. Headache. 2004;44:1024-8.
77. Strupp M, Zwergal A, Brandt T. Episodic ataxia type 2. Neurotherapeutics. 2007;4:267-73.
78. Battistini S, Stenirri S, Piatti M, et al. A new CACNA1A gene mutation in acetazolamide-responsive familial hemiplegic migraine and ataxia. Neurology. 1999;53:38-43.
79. Athwal BS, Lennox GG. Acetazolamide responsiveness in familial hemiplegic migraine. Ann Neurol. 1996;40:820-1.
80. Koch HJ, Sykora A, Hausn P. Off-label use of acetazolamide in a patient with familial hemiplegic migraine and concomitant psychotic episodes. Psychiatr Prax. 2010;37:148-51.
81. De Simone R, Marano E, Di Stasio E, et al. Acetazolamide efficacy and tolerability in migraine with aura: a pilot study. Headache. 2005;45:385-6.
82. Vahedi K, Taupin P, Djomby R, et al. Efficacy and tolerability of acetazolamide in migraine prophylaxis: a randomized placebo-controlled trial. J Neurol. 2002;249:206-11.
83. Silberstein SD, Goadsby PJ. Migraine: preventive treatment. Cephalalgia. 2002;22:491-512.
84. Striano P, Zara F, Santorelli FM, Striano S. Topiramate-associated worsening symptoms in a patient with familial hemiplegic migraine. J Neurol Sci. 2008;272:194-5.
85. Bardwell A, Trott JA. Stroke in migraine as a consequence of propranolol. Headache. 1987;27:381-3.
86. Olesen J, Hougard K, Hertz M. Isoproterenol and propranolol: ability to cross the blood-brain barrier and effects on cerebral circulation in man. Stroke. 1978;9:344-9.
87. Zifkin B, Andermann E, Andermann F, Kirkham T. An autosomal dominant syndrome of hemiplegic migraine, nystagmus, and tremor. Ann Neurol. 1980;8:329-32.
88. Lai CW, Ziegler DK, Lansky LL, Torres F. Hemiplegic migraine in childhood: diagnostic and therapeutic aspects. J Pediatr. 1982;101:696-9.
89. Hsu DA, Stafstrom CE, Rowley HA, et al. Hemiplegic migraine: hyperperfusion and abortive therapy with intravenous verapamil. Brain Dev. 2008;30:86-90.
90. Omata T, Takanashi J, Wada T, et al. Genetic diagnosis and acetazolamide treatment of familial hemiplegic migraine. Brain Dev. 2011;33:332-4.
91. Tfelt-Hansen P, Saxena PR, Dahlof C, et al. Ergotamine in the acute treatment of migraine: a review and European consensus. Brain. 2000;123(Pt 1):9-18.
92. Maassen van den Brink A, Reekers M, Bax WA, et al. Coronary side-effect potential of current and prospective antimigraine drugs. Circulation. 1998;98:25-30.
93. Klapper J, Mathew N, Nett R. Triptans in the treatment of basilar migraine and migraine with prolonged aura. Headache. 2001;41:981-84.
94. Artto V, Nissila M, Wessman M, et al. Treatment of hemiplegic migraine with triptans. Eur J Neurol. 2007;14:1053-6.
95. Hirota K, Lambert DG. Ketamine: its mechanism(s) of action and unusual clinical uses. Br J Anaesth. 1996;77:441-4.
96. Kaube H, Herzog J, Kaufer T, et al. Aura in some patients with familial hemiplegic migraine can be stopped by intranasal ketamine. Neurology. 2000;55:139-41.
97. Connor KM, Shapiro RE, Diener HC, et al. Randomized, controlled trial of telcagepant for the acute treatment of migraine. Neurology. 2009;73:970-7.
98. Ho TW, Ferrari MD, Dodick DW, et al. Efficacy and tolerability of MK-0974 (telcagepant), a new oral antagonist of calcitonin gene-related peptide receptor, compared with zolmitriptan for acute migraine: a randomized, placebo-controlled, parallel-treatment trial. Lancet. 2008;372:2115-23.
99. Hoffmann J, Goadsby PJ. New agents for acute treatment of migraine: CGRP Receptor Antagonists, iNOS Inhibitors. Curr Treat Options Neurol. 2012;14:50-9. A clear and recent review article about future pharmacological treatment options in migraine.
100. Jenkins B, Tepper SJ. Neurostimulation for primary headache disorders, part 1: pathophysiology and anatomy, history of neuromodulation in headache treatment, and review of peripheral neuromodulation in primary headaches. Headache. 2011;51:1254-66.