Hemicrania Continua

Headache (R Halker, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Headache

Abstract

In recent years, hemicrania continua has become a well-recognized primary headache disorder known for its chronicity and resulting disability in a subset of patients with headache. The core clinical features have been well described: unilateral, side-locked headaches that are continuous (although interrupted by frequent severe exacerbations), associated with autonomic symptoms and a response to indomethacin. However, areas of relative controversy remain in its classification and diagnosis. Several relatively large case series have better delineated the associated features of this disorder, including atypical presentations. Recently, neuroimaging research has provided new insights into the underlying pathways involved in the disorder, in particular activation of the contralateral posterior hypothalamus and the ipsilateral dorsal rostral pons. Despite its well-known response to indomethacin, many patients still endure long delays in the appropriate diagnosis and treatment. There remains a need for new treatments given the morbidity associated with long-term indomethacin use.

Keywords

Hemicrania continua Unilateral headache Review Trigeminal autonomic cephalalgia Indomethacin-responsive headache 

Notes

Compliance with Ethics Guidelines

Conflict of Interest

Robert W. Charlson declares that he has no conflict of interest.

Matthew S. Robbins has received payment for manuscript preparation from MedLink Neurology and the American College of Physicians (PIER module). He has also received book royalties from Wiley (Headache) and payment for development of educational presentations from the American Headache Society and Prova Education.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

  1. 1.
    Peres M, Valenca M, Goncalves A. Misdiagnosis of hemicrania continua. Expert Rev Neurother. 2009;9:1371–8.PubMedCrossRefGoogle Scholar
  2. 2.
    Medina JL, Diamond S. Cluster headache variant: spectrum of a new headache syndrome. Arch Neurol. 1981;35:705–9.CrossRefGoogle Scholar
  3. 3.
    Sjaastad O, Spierings EL. Hemicrania continua: another headache absolutely responsive to indomethacin. Cephalalgia. 1984;4:65–70.PubMedCrossRefGoogle Scholar
  4. 4.
    Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders. 3rd edition (beta version). Cephalalgia. 2013;33:629–808.CrossRefGoogle Scholar
  5. 5.
    Spierings EL. Hemicrania continua should not be classified as a trigeminal autonomic cephalalgia. Headache. 2013;53(5):869–70.PubMedCrossRefGoogle Scholar
  6. 6.
    Vincent MB. Hemicrania continua. Unquestionably a trigeminal autonomic cephalalgia. Headache. 2013;53(5):836–68.CrossRefGoogle Scholar
  7. 7.
    Wheeler SD, Allen KF, Pusey T. Is hemicrania continua a migraine variant? Cephalalgia. 2001;21:508.Google Scholar
  8. 8.
    Prakash S, Shah ND, Marmura MJ. Hemicrania continua unresponsive to indomethacin do exist. Cephalalgia. 2010;30(1):123–5.PubMedGoogle Scholar
  9. 9.
    Newman LC, Spears RC, Lay CL. Hemicrania continua: a third case in which attacks alternate sides. Cephalalgia. 2004;44(8):821–3.Google Scholar
  10. 10.
    Pasquier F, Leys D, Petit H. Hemicrania continua: the first bilateral case? Cephalalgia. 1987;7(3):169–70.PubMedCrossRefGoogle Scholar
  11. 11.
    Peres M, Slow HC, Rozen TD. Hemicrania continua with aura. Cephalalgia. 2002;22(3):246–8.PubMedCrossRefGoogle Scholar
  12. 12.
    Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders. 2nd edition. Cephalalgia. 2004;24:1–160.Google Scholar
  13. 13.
    Cittadini E, Goadsby PJ. Hemicrania continua: a clinical study of 39 patients with diagnostic implications. Brain. 2010;133:1973–86.PubMedCrossRefGoogle Scholar
  14. 14.
    Moura LM, Bezerra JM, Fleming NR. Treatment of hemicrania continua: case series and literature review. Rev Bras Anestesiol. 2012;62(2):173–87.PubMedCrossRefGoogle Scholar
  15. 15.
    Prakash S, Golwala P. A proposal for revision of hemicrania continua diagnostic criteria based on critical analysis of 62 patients. Cephalalgia. 2012;32(11):860–8.PubMedCrossRefGoogle Scholar
  16. 16.
    Goadsby PJ, Cittadini E, Burns B, Cohen A. Trigeminal autonomic cephalalgias: diagnostic and therapeutic developments. Curr Opin Neurol. 2008;21(3):323–30.PubMedCrossRefGoogle Scholar
  17. 17.
    Kuritzky A. Indomethacin resistant hemicranias continua. Cephalalgia. 1992;12(1):57–9.PubMedCrossRefGoogle Scholar
  18. 18.
    Prakash S, Shah ND, Bhanvadia RJ. Hemicrania continua unresponsive or partially responsive to indomethacin: does it exist? A diagnostic and therapeutic dilemma. J Headache Pain. 2009;10(1):59–63.PubMedCentralPubMedCrossRefGoogle Scholar
  19. 19.
    Southerland AM, Login IS. Rigorously defined hemicrania continua presenting bilaterally. Cephalalgia. 2011;31(14):1490–2.PubMedCrossRefGoogle Scholar
  20. 20.
    Goadsby PJ, Lipton RB. The hemicrania continua diagnosis. Cephalalgia. 2002;22:563.PubMedCrossRefGoogle Scholar
  21. 21.
    Prakash S. Hemicrania continua: Disease or syndrome? Cephalalgia. 2012;32:942–3.PubMedCrossRefGoogle Scholar
  22. 22.
    Sjaastad O, Tjorstad K. Hemicrania continua: a third Norwegian case. Cephalalgia. 1987;7(3):175–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Zukerman E, Hannuch SN, Carvalho Dde S, et al. Hemicrania continua: a case report. Cephalalgia. 1987;7(3):171–3.PubMedCrossRefGoogle Scholar
  24. 24.
    Iordanidis T, Sjaastad O. Hemicrania continua: a case report. Cephalalgia. 1989;9(4):301–3.PubMedCrossRefGoogle Scholar
  25. 25.
    Pareja JA, Palomo T, Gorriti MA, et al. Hemicrania continua. The first Spanish case: a case report. Cephalalgia. 1990;10:143–5.PubMedCrossRefGoogle Scholar
  26. 26.
    Bigal ME, Lipton RB, Tepper SJ, et al. Primary chronic daily headache and its subtypes in adolescents and adults. Neurology. 2004;63:843–7.PubMedCrossRefGoogle Scholar
  27. 27.
    Ramon C, Mauri G, Vega J, et al. Diagnostic distribution of 100 unilateral, side-locked headaches consulting a specialized clinic. Eur Neurol. 2013;69(5):289–91.PubMedCrossRefGoogle Scholar
  28. 28.
    Sjaastad O, Bakketeig LS. The rare, unilateral headaches. Vaga study of headache epidemiology. J Headache Pain. 2007;8:19–27.PubMedCentralPubMedCrossRefGoogle Scholar
  29. 29.
    Weatherall MW, Bahra A. Familial hemicrania continua. Cephalalgia. 2011;31(2):245–9.PubMedCrossRefGoogle Scholar
  30. 30.
    Newman LC, Lipton RB, Solomon S. Hemicrania continua: ten new cases and a review of the literature. Neurology. 1994;44:2111–4.PubMedCrossRefGoogle Scholar
  31. 31.
    Peres M, Silberstein SD, Nahmias S. Hemicrania continua is not that rare. Neurology. 2001;57:948–51.PubMedCrossRefGoogle Scholar
  32. 32.
    Marmura MJ, Silberstein SD, Gupta M. Hemicrania continua: who responds to indomethacin? Cephalalgia. 2009;29(3):300–7.PubMedCrossRefGoogle Scholar
  33. 33.
    Newman LC, Lipton RB, Russell M, et al. Hemicrania continua: attacks may alternate sides. Headache. 1992;32(5):237–8.PubMedCrossRefGoogle Scholar
  34. 34.
    Marano E, Giampiero V, Gennaro DR, et al. Hemicrania continua: a possible case with alternating sides. Cephalalgia. 1994;14(4):307–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Baldacci F, Nuti A, Cafforio G, et al. “Indotest” in atypical hemicrania continua. Cephalalgia. 2008;28(3):300–1.PubMedCrossRefGoogle Scholar
  36. 36.
    Bordini C, Antonaci F, Stovner LJ, et al. “Hemicrania continua”: a clinical review. Headache. 1991;31(1):20–6.PubMedCrossRefGoogle Scholar
  37. 37.
    Peres M, Masruha MR, Young MB. Side shifting hemicranias continua with aura (migraine with aura with autonomic symptoms responsive to indomethacin?). Cephalalgia. 2006;26(8):917–9.PubMedCrossRefGoogle Scholar
  38. 38.
    Matharu MS, Bradbury P, Swash M. Hemicrania continua: side alteration and response to topiramate. Cephalalgia. 2006;26(3):341–4.PubMedCrossRefGoogle Scholar
  39. 39.
    Sjaastad O, Vincent M, Stovner LJ. Side alteration of pain in hemicrania continua. Headache. 1993;33(1):43–5.PubMedCrossRefGoogle Scholar
  40. 40.
    Peres M, Stiles MA, Oshinsky M, Rozen TD. Remitting form of hemicrania continua with seasonal pattern. Headache. 2001;41(6):592–4.PubMedCrossRefGoogle Scholar
  41. 41.
    Rozen TD. Can indomethacin act as a disease modifying agent in hemicrania continua? A supportive clinical case. Headache. 2009;49(5):759–61.PubMedCrossRefGoogle Scholar
  42. 42.
    Summ O, Andreou AP, Akerman S, Goadsby PJ. A potential nitregic mechanism of action for indomethacin, but not of other COX inhibitors: relevance to indomethacin-responsive headaches. J Headache Pain. 2010;11(6):477–83.PubMedCentralPubMedCrossRefGoogle Scholar
  43. 43.
    Summ O, Evers S. Mechanism of action of indomethacin in indomethacin-responsive headaches. Curr Pain Headache Rep. 2013;17(4):327.PubMedCrossRefGoogle Scholar
  44. 44.
    Pareja JA, Caminero AB, Franco E, et al. Dose, efficacy, and tolerability of long-term indomethacin treatment for chronic paroxysmal hemicrania and hemicrania continua. Cephalalgia. 2001;21:906–10.PubMedCrossRefGoogle Scholar
  45. 45.
    Matharu MS, Cohen AS, McGonigle DJ, et al. Posterior hypothalamic and brainstem activation in hemicrania continua. Headache. 2004;44:747–61.PubMedCrossRefGoogle Scholar
  46. 46.
    May A, Bahra A, Buchel C, et al. Hypothalamic activation in cluster headache attacks. Lancet. 1998;352:275–8.PubMedCrossRefGoogle Scholar
  47. 47.
    Sprenger T, Boecker H, Tolle TR, et al. Specific hypothalamic activation during a spontaneous cluster headache attack. Neurology. 2004;62:516–7.PubMedCrossRefGoogle Scholar
  48. 48.
    Mathuru MS, Goadsby PJ. Functional brain imaging in hemicrania continua: Implications for nosology and pathophysiology. Curr Pain Headache Rep. 2005;9(4):281–8.CrossRefGoogle Scholar
  49. 49.
    Irimia P, Arbizu J, Prieto E, et al. Activation of the brainstem but not of the hypothalamus in hemicranias continua without autonomic symptoms. Cephalalgia. 2009;29(9):974–9.PubMedCrossRefGoogle Scholar
  50. 50.
    Valenca MM, Andrade-Valenca LP, da Silva WF, et al. Hemicrania continua secondary to an ipsilateral brainstem lesion. Headache. 2007;47(3):438–41.PubMedCrossRefGoogle Scholar
  51. 51.
    Friedman D. Unilateral headache associated with pontine infarction. Cephalalgia. 2010;30(12):1524–6.PubMedCrossRefGoogle Scholar
  52. 52.
    Rossi P, Faroni J, Tassorelli C, et al. Diagnostic delay and suboptimal management in a referral population with hemicrania continua. Headache. 2009;49(2):227–34.PubMedCrossRefGoogle Scholar
  53. 53.
    Cortijo E, Guerrero AL, Herrero S, et al. Hemicrania continua in a headache clinic: referral source and diagnostic delay in a series of 22 patients. J Headache Pain. 2012;13(7):567–9.PubMedCentralPubMedCrossRefGoogle Scholar
  54. 54.
    Viana M, Tassorelli C, Allena M, et al. Diagnostic and therapeutic errors in trigeminal autonomic cephalalgias and hemicrania continua: a systematic review. J Headache Pain. 2013;14(1):14.PubMedCentralPubMedCrossRefGoogle Scholar
  55. 55.
    Prakash S, Dholakia SY. Hemicrania continua-like headache with leprosy: casual or causal connection? Headache. 2008;48(7):1132–4.PubMedCrossRefGoogle Scholar
  56. 56.
    Ferreira KS, Freitas DJ, Speciali JG. Is there a relation between hemicrania continua and leprosy? Indian J Lepr. 2012;84(4):317–20.PubMedGoogle Scholar
  57. 57.
    Robbins MS, Grosberg BM. Hemicrania continua-like headache from metastatic lung cancer. Headache. 2010;50(6):1055–6.PubMedCrossRefGoogle Scholar
  58. 58.
    Wang SJ, Hung CW, Fuh JL, et al. Cranial autonomic symptoms in patient with pituitary adenoma presenting with headaches. Acta Neurol Taiwan. 2009;18(2):104–12.PubMedGoogle Scholar
  59. 59.
    Kim K, Yang HS. A possible case of symptomatic hemicrania continua from an osteoid osteoma of the ethmoid sinus. Cephalalgia. 2010;30:242–8.PubMedGoogle Scholar
  60. 60.
    Evans RW. Hemicrania continua-like headache due to nonmetastatic lung cancer – a vagal cephalalgia. Headache. 2007;47(9):1349–51.PubMedCrossRefGoogle Scholar
  61. 61.
    Vikelis M, Xifaras M, Magoufis G, et al. Headache attributed to unruptured saccular aneurysm, mimicking hemicrania continua. J Headache Pain. 2005;6(3):156–8.PubMedCentralPubMedCrossRefGoogle Scholar
  62. 62.
    Rogalewski A, Evers S. Symptomatic hemicrania continua after internal carotid artery dissection. Headache. 2005;45(2):167–9.PubMedCrossRefGoogle Scholar
  63. 63.
    Lay CL, Newman LC. Postraumatic hemicrania continua. Headache. 1999;39(4):275–9.PubMedCrossRefGoogle Scholar
  64. 64.
    Peres MF, Zukerman E, Porto PP, et al. Headache and pineal cyst: a (more than) coincidental relationship? Headache. 2004;44(9):929–30.PubMedCrossRefGoogle Scholar
  65. 65.
    Spitz M, Peres MF. Hemicrania continua postpartum. Cephalalgia. 2004;24(7):603–4.PubMedCrossRefGoogle Scholar
  66. 66.
    Wilbrink LA, Ferrari MD, Kruit MC, et al. Neuroimaging in trigeminal autonomic cephalalgias: who, how, and of what? Curr Opin Neurol. 2009;22(3):247–53.PubMedCrossRefGoogle Scholar
  67. 67.
    Prakash S, Shah ND, Soni RK. Secondary hemicrania continua: case reports and a literature review. J Neurol Sci. 2009;280(1–2):29–34.PubMedCrossRefGoogle Scholar
  68. 68.
    Sjaastad O, Stovner LJ, Stolt-Nielsen A, et al. CPH and hemicrania continua: Requirements of high indomethacin dosages – an ominous sign? Headache. 1995;35:363–7.PubMedCrossRefGoogle Scholar
  69. 69.
    Antonaci F, Pareja JA, Caminero AB, et al. Chronic paroxysmal hemicranias and hemicrania continua. Parental indomethacin: the “Indotest”. Headache. 1998;38(2):122–8.PubMedCrossRefGoogle Scholar
  70. 70.
    Prakash S, Husain M, Sureka DS, et al. Is there a need to search for alternatives to indomethacin for hemicrania continua? Case reports and a review. J Neurol Sci. 2009;277(1):187–90.PubMedCrossRefGoogle Scholar
  71. 71.
    Jurgens TP, Schulte LH, May A. Indomethacin-induced de novo headache in hemicrania continua – fighting fire with fire? Cephalalgia. 2013;33(14):1203–5.PubMedCrossRefGoogle Scholar
  72. 72.
    Seaton RA, France AJ. Recurrent aseptic meningitis following non-steroidal anti-inflammatory drugs – a reminder. Postgrad Med J. 1999;75:771–2.PubMedCentralPubMedGoogle Scholar
  73. 73.
    Lambru G, Manzoni GC, Torelli P, et al. Reversible cerebral vasoconstriction phenomena following indomethacin administration. Headache. 2011;51(5):813–8.PubMedCrossRefGoogle Scholar
  74. 74.
    Hoppmann RA, Peden JG, Ober SK. Central nervous system side effects of nonsteroidal anti-inflammatory drugs. Aseptic meningitis, psychosis, and cognitive dysfunction. Arch Intern Med. 1991;151(7):1309–13.PubMedCrossRefGoogle Scholar
  75. 75.
    Porta-Etessam J, Cuadrado M, Rodriguez-Gomez O, et al. Are COX-2 drugs the second-line option in indomethacin responsive headaches? J Headache Pain. 2010;11(5):405–7.PubMedCentralPubMedCrossRefGoogle Scholar
  76. 76.
    Peres MF, Zukerman E. Hemicrania continua responsive to rofecoxib. Cephalalgia. 2000;20(2):130–1.PubMedCrossRefGoogle Scholar
  77. 77.
    Sjaastad O, Antonaci F. A piroxicam derivative partly effective in chronic paroxysmal hemicrania continua. Headache. 1995;35(9):549–50.PubMedCrossRefGoogle Scholar
  78. 78.
    Trucco M, Antonaci F, Sandrini G. Hemicrania continua: a case responsive to piroxicam-beta-cyclodextrin. Headache. 1992;32(1):39–40.PubMedCrossRefGoogle Scholar
  79. 79.
    Camarda C, Camarda R, Monastero R. Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two reports. Clin Neurol Neurosurg. 2008;110(1):88–91.PubMedCrossRefGoogle Scholar
  80. 80.
    Brighina F, Palermo A, Consentino G, Fierro B. Prophylaxis of hemicrania continua: two new cases effectively treated with topiramate. Headache. 2007;47(3):441–3.PubMedCrossRefGoogle Scholar
  81. 81.
    Prakash S, Brahmbhatt KJ, Chwda NT, Tandon N. Hemicrania continua responsive to intravenous methylprednisolone. Headache. 2009;49(4):604–7.PubMedCrossRefGoogle Scholar
  82. 82.
    Khalil M, Ahmed F. Hemicrania continua responsive to botulinum toxin type A: a case report. Headache. 2013;53(5):831–3.PubMedCrossRefGoogle Scholar
  83. 83.
    Garza I, Cutrer F. Pain relief and persistence of dysautonomic features in a patient with hemicrania continua responsive to botulinum toxin type A. Cephalalgia. 2010;30(4):500–3.PubMedGoogle Scholar
  84. 84.
    Spears RC. Is gabapentin an effective treatment for hemicrania continua? J Headache Pain. 2009;10(4):271–5.PubMedCentralPubMedCrossRefGoogle Scholar
  85. 85.
    Rozen TD. Melatonin responsive hemicrania continua. Headache. 2006;46(7):1203–4.PubMedCrossRefGoogle Scholar
  86. 86.
    Spears RC. Hemicrania continua: a case in which a patient experienced complete relief on melatonin. Headache. 2006;46(3):524–7.PubMedCrossRefGoogle Scholar
  87. 87.
    Rajabally YA, Jacob S. Hemicrania continua responsive to verapamil. Headache. 2005;45(8):1082–3.PubMedCrossRefGoogle Scholar
  88. 88.
    Rozen TD. Verapamil-responsive hemicrania continua in a patient with episodic cluster headache. Cephalalgia. 2006;26(3):351–3.PubMedGoogle Scholar
  89. 89.
    Guerrero AL, Herrero-Velazquez S, Penas ML, et al. Peripheral nerve blocks: a therapeutic alternative for hemicrania continua. Cephalalgia. 2012;32(6):505–8.PubMedCrossRefGoogle Scholar
  90. 90.
    Schwedt TJ, Dodick DW, Trentman TL, et al. Occipital nerve stimulation for chronic cluster headache and hemicrania continua: pain relief and persistence of autonomic features. Cephalalgia. 2006;26(8):1025–7.PubMedCrossRefGoogle Scholar
  91. 91.
    Burns B, Watkins L, Goadsby PJ. Treatment of hemicrania continua by occipital nerve stimulation with a bion device: long-term follow-up of a crossover study. Lancet Neurol. 2008;7(11):1001–12.PubMedCrossRefGoogle Scholar

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© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of Neurology, Montefiore Medical CenterAlbert Einstein College of MedicineBronxUSA
  2. 2.Montefiore Headache Center, Department of NeurologyAlbert Einstein College of MedicineBronxUSA

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