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Trigeminal autonomic cephalalgias: Diagnosis and treatment

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Abstract

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral head pain that occurs in association with ipsilateral cranial autonomic features. The TACs include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and its close relative short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). These syndromes cause patients considerable disability and certainly very significant suffering. They are distinguished by the frequency of attacks of pain, the length of the attacks, and very characteristic responses to medical therapy, such that the diagnosis can usually be made clinically, which is important because it completely dictates therapy. The management of TACs can be very rewarding for physicians and highly beneficial to patients.

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References and Recommended Reading

  1. Goadsby PJ, Lipton RB: A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic features, including new cases. Brain 1997, 120:193–209.

    Article  PubMed  Google Scholar 

  2. Goadsby PJ: Trigeminal autonomic cephalagias. Pathophysiology and classification. Rev Neurol (Paris) 2005, 161:692–695.

    CAS  Google Scholar 

  3. Headache Classification Committee of The International Headache Society: The International Classification of Headache Disorders (second edition). Cephalalgia 2004, 24(Suppl 1):1–160.

    Article  Google Scholar 

  4. Bahra A, May A, Goadsby PJ: Cluster headache: a prospective clinical study in 230 patients with diagnostic implications. Neurology 2002, 58:354–361.

    PubMed  Google Scholar 

  5. Silberstein SD, Niknam R, Rozen TD, Young WB: Cluster headache with aura. Neurology 2000, 54:219–221.

    PubMed  CAS  Google Scholar 

  6. Levy M, Matharu MS, Meeran K, et al.: The clinical characteristics of headache in patients with pituitary tumours. Brain 2005, 128:1921–1930.

    Article  PubMed  CAS  Google Scholar 

  7. Goadsby PJ: Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol 2002, 1:37–43.

    Google Scholar 

  8. May A, Bahra A, Buchel C, et al.: Hypothalamic activation in cluster headache attacks. Lancet 1998, 352:275–278.

    Article  PubMed  CAS  Google Scholar 

  9. May A, Ashburner J, Buchel C, et al.: Correlation between structural and functional changes in brain in an idiopathic headache syndrome. Nat Med 1999, 5:836–838.

    Article  PubMed  CAS  Google Scholar 

  10. Ekbom K; The Sumatriptan Cluster Headache Study Group: Treatment of acute cluster headache with sumatriptan. N Engl J Med 1991, 325:322–326.

    Article  Google Scholar 

  11. Ekbom K, Waldenlind E, Cole JA, et al.: Sumatriptan in chronic cluster headache: results of continuous treatment for eleven months. Cephalalgia 1992, 12:254–256.

    Article  PubMed  CAS  Google Scholar 

  12. van Vliet JA, Bahra A, Martin V, et al.: Intranasal sumatriptan in cluster headache—randomized placebo-controlled double-blind study. Neurology 2003, 60:630–633.

    Article  PubMed  Google Scholar 

  13. Cittadini E, May A, Straube A, et al.: Effectiveness of intranasal zolmitriptan in acute cluster headache. A randomized, placebo-controlled, double-blind crossover study. Arch Neurol 2006, In press.

  14. Monstad I, Krabbe A, Micieli G, et al.: Preemptive oral treatment with sumatriptan during a cluster period. Headache 1995, 35:607–613.

    Article  PubMed  CAS  Google Scholar 

  15. Paemeleire K, Bahra A, Evers S, et al.: Medication-overuse headache in cluster headache patients. Neurology 2006, 67:109–113.

    Article  PubMed  CAS  Google Scholar 

  16. Fogan L: Treatment of cluster headache: a double blind comparison of oxygen vs air inhalation. Arch Neurol 1985, 42:362–363.

    PubMed  CAS  Google Scholar 

  17. Matharu MS, Levy MJ, Meeran K, Goadsby PJ: Subcutaneous octreotide in cluster headache—randomized placebo-controlled double-blind cross-over study. Ann Neurol 2004, 56:488–494.

    Article  PubMed  CAS  Google Scholar 

  18. Robbins L: Intranasal lidocaine for cluster headache. Headache 1995, 35:83–84.

    Article  PubMed  CAS  Google Scholar 

  19. Couch JR, Ziegler DK: Prednisone therapy for cluster headache. Headache 1978, 18:219–221.

    Article  PubMed  Google Scholar 

  20. Curran DA, Hinterberger H, Lance JW: Methysergide. Res Clin Studies Headache 1967, 1:74–122.

    CAS  Google Scholar 

  21. Ambrosini A, Vandenheede M, Rossi P, et al.: Suboccipital injection with a mixture of rapid-and long-acting steroids in cluster headache: a double-blind placebo-controlled study. Pain 2005, 118:92–96.

    Article  PubMed  CAS  Google Scholar 

  22. Anthony M: Arrest of attacks of cluster headache by local steroid injection of the occipital nerve. In Migraine: Clinical and Research Advances. Edited by Rose FC. London: Karger; 1985:169–173.

    Google Scholar 

  23. Afridi SK, Shields KG, Bhola R, Goadsby PJ: Greater occipital nerve injection in primary headache syndromes—prolonged effects from a single injection. Pain 2006, 122:126–129.

    Article  PubMed  CAS  Google Scholar 

  24. Shields KG, Levy MJ, Goadsby PJ: Alopecia and cutaneous atrophy following greater occipital nerve infiltration. Neurology 2004, 63:2193–2194.

    PubMed  CAS  Google Scholar 

  25. Gabai IJ, Spierings EL: Prophylactic treatment of cluster headache with verapamil. Headache 1989, 29:167–168.

    Article  PubMed  CAS  Google Scholar 

  26. Bussone G, Leone M, Peccarisi C, et al.: Double blind comparison of lithium and verapamil in cluster headache prophylaxis. Headache 1990, 30:411–417.

    Article  PubMed  CAS  Google Scholar 

  27. Leone M, D’Amico D, Frediani F, et al.: Verapamil in the prophylaxis of episodic cluster headache: a double-blind study versus placebo. Neurology 2000, 54:1382–1385.

    PubMed  CAS  Google Scholar 

  28. Cohen AS, Matharu MS, Goadsby PJ: ECG abnormalities on verapamil in cluster headache. Cephalalgia 2005, 25:1200.

    Google Scholar 

  29. Ekbom K: Lithium for cluster headache: review of the literature and preliminary results of long-term treatment. Headache 1981, 21:132–139.

    Article  PubMed  CAS  Google Scholar 

  30. Goadsby PJ, Schoenen J, Ferrari MD, et al.: Towards a definition of intractable headache for use in clinical practice and trials. Cephalalgia 2006, 26:1168–1170.

    Article  PubMed  CAS  Google Scholar 

  31. Leone M, Franzini A, Broggi G, Bussone G: Hypothalamic stimulation for intractable cluster headache: long-term experience. Neurology 2006, 67:150–152.

    Article  PubMed  Google Scholar 

  32. Dodick DW, Trentman T, Zimmerman R, Eross EJ: Occipital nerve stimulation for intractable chronic primary headache disorders. Cephalalgia 2003, 23:701.

    Google Scholar 

  33. Antonaci F, Costa A, Ghirmai S, et al.: Parenteral indomethacin (the INDOTEST) in cluster headache. Cephalalgia 2003, 23:193–196.

    Article  PubMed  CAS  Google Scholar 

  34. Matharu MS, Goadsby PJ: Functional brain imaging in hemicrania continua: implications for nosology and pathophysiology. Curr Pain Headache Rep 2005, 9:281–288.

    Article  PubMed  CAS  Google Scholar 

  35. Cohen AS, Goadsby PJ: Short-lasting Unilateral Neuralgiform Headache Attacks with Conjunctival injection and Tearing (SUNCT) or Cranial Autonomic Features (SUNA). A prospective clinical study of SUNCT and SUNA. Brain 2006, 129:2746–2760.

    Article  PubMed  Google Scholar 

  36. Antnaci F, Sjaastad O: Chronic paroxysmal hemicrania (CPH): a review of the clinical manifestations. Headache 1989, 29:648–656.

    Article  Google Scholar 

  37. Cittadini E, Goadsby PJ: Revisiting the International Headache Society criteria for paroxysmal hemicrania: a case series of 21 patients. Cephalalgia 2006, 26:1401–1402.

    Google Scholar 

  38. Russell D: Paroxysmal hemicrania. In Cluster Headache & Related Conditions. Edited by Olesen J, Goadsby PJ. Oxford: Oxford University Press; 1999:27–36.

    Google Scholar 

  39. Boes CJ, Swanson JW, Dodick DW: Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis. Headache 1998, 38:787–791.

    Article  PubMed  CAS  Google Scholar 

  40. Irimia P, Cittadini E, Paemeleire K, Cohen A, et al.: Unilateral phonophobia and photophobia: a helpful symptom for the diagnosis of trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC). Cephalalgia 2005, 25:856.

    Article  Google Scholar 

  41. Boes CJ, Dodick DW: Refining the clinical spectrum of chronic paroxysmal hemicrania: a review of 74 patients. Headache 2002, 42:699–708.

    Article  PubMed  Google Scholar 

  42. Goadsby PJ, Edvinsson L: Neuropeptide changes in a case of chronic paroxysmal hemicrania—evidence for trigeminoparasympathetic activation. Cephalalgia 1996, 16:448–450.

    Article  PubMed  CAS  Google Scholar 

  43. Goadsby PJ, Edvinsson L: Human in vivo evidence for trigeminovascular activation in cluster headache. Brain 1994, 117:427–434.

    Article  PubMed  Google Scholar 

  44. May A, Goadsby PJ: The trigeminovascular system in humans: pathophysiological implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab 1999, 19:115–127.

    Article  PubMed  CAS  Google Scholar 

  45. Antonaci F, Sandrini G, Danilov A, Sand T: Neurophysiological studies in chronic paroxysmal hemicrania and hemicrania continua. Headache 1994, 34:479–483.

    Article  PubMed  CAS  Google Scholar 

  46. Micieli G, Cavallini A, Facchinetti F, et al.: Chronic paroxysmal hemicrania: a chronobiological study (case report). Cephalalgia 1989, 9:281–286.

    Article  PubMed  CAS  Google Scholar 

  47. Matharu MS, Cohen AS, Frackowiak RS, Goadsby PJ: Posterior hypothalamic activation in paroxysmal hemicrania. Ann Neurol 2006, 59:535–545.

    Article  PubMed  Google Scholar 

  48. Cohen AS, Goadsby PJ: Functional neuroimaging of primary headache disorders. Exp Rev Neurotherapeut 2006, 6:1159–1172.

    Article  Google Scholar 

  49. Sjaastad O: Chronic paroxysmal hemicrania. In Handbook of Clinical Neurology. Edited by Vinken PJ, Bruyn GW, Klawans HL, Rose FC. Amsterdam: Elsevier Science; 1986:257–266.

    Google Scholar 

  50. Sjaastad O, Apfelbaum R, Caskey W, et al.: Chronic paroxysmal hemicrania (CPH). The clinical manifestations. A review. Uppsala J Med Sci 1980, 31(Suppl):27–33.

    CAS  Google Scholar 

  51. Lisotto C, Maggioni F, Mainardi F, Zanchin G: Rofecoxib for the treatment of chronic paroxysmal hemicrania. Cephalalgia 2003, 23:318–320.

    Article  PubMed  CAS  Google Scholar 

  52. Chakravarty A, Mukherjee A, Roy D: Trigeminal autonomic cephalgias and variants: clinical profile in Indian patients. Cephalalgia 2004, 24:859–866.

    Article  PubMed  CAS  Google Scholar 

  53. Siow HC: Seasonal episodic paroxysmal hemicrania responding to cyclooxygenase-2 inhibitors. Cephalalgia 2004, 24:414–415.

    Article  PubMed  CAS  Google Scholar 

  54. Mathew NT, Kailasam J, Fischer A: Responsiveness to celecoxib in chronic paroxysmal hemicrania. Neurology 2000, 55:316.

    PubMed  CAS  Google Scholar 

  55. Lenzer J: FDA advisers warn: COX 2 inhibitors increase risk of heart attack and stroke. BMJ 2005, 330:440.

    Article  PubMed  Google Scholar 

  56. Evers S, Husstedt IW: Alternatives in drug treatment of chronic paroxysmal hemicrania. Headache 1996, 36:429–432.

    Article  PubMed  CAS  Google Scholar 

  57. Coria F, Claveria LE, Jimenez-Jimenez FJ, Seijas EV: Episodic paroxysmal hemicrania responsive to calcium channel blockers. J Neurol Neurosurg Psychiatry 1992, 55:166.

    Article  PubMed  CAS  Google Scholar 

  58. Cohen AS, Goadsby PJ: Paroxysmal hemicrania responding to topiramate. J Neurol Neurosurg Psychiatry 2007, 78:96–97.

    Article  PubMed  CAS  Google Scholar 

  59. Antonaci F, Pareja JA, Caminero AB, Sjaastad O: Chronic paroxysmal hemicrania and hemicrania continua: anaesthetic blockades of pericranial nerves. Funct Neurol 1997, 12:11–15.

    PubMed  CAS  Google Scholar 

  60. Matharu MS, Cohen AS, Boes CJ, Goadsby PJ: SUNCT syndrome: a review. Curr Pain Headache Rep 2003, 7:308–318.

    Article  PubMed  Google Scholar 

  61. Cohen AS, Matharu MS, Goadsby PJ: Suggested guidelines for treating SUNCT and SUNA. Cephalalgia 2005, 25:1200.

    Google Scholar 

  62. May A, Bahra A, Buchel C, et al.: Functional MRI in spontaneous attacks of SUNCT: short-lasting neuralgiform headache with conjunctival injection and tearing. Ann Neurol 1999, 46:791–793.

    Article  PubMed  CAS  Google Scholar 

  63. Cohen AS, Matharu MS, Kalisch R, et al.: Functional MRI in SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing) and SUNA (Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms) shows differential hypothalamic activation with increasing pain. Cephalalgia 2006, 26:1402–1403.

    Google Scholar 

  64. Matharu MS, Cohen AS, Goadsby PJ: SUNCT syndrome responsive to intravenous lidocaine. Cephalalgia 2004, 24:985–992.

    Article  PubMed  CAS  Google Scholar 

  65. Matharu MS, Boes CJ, Goadsby PJ: Management of trigeminal autonomic cephalalgias and hemicrania continua. Drugs 2003, 63:1637–1677.

    Article  PubMed  CAS  Google Scholar 

  66. Black DF, Dodick DW: Two cases of medically and surgically intractable SUNCT: a reason for caution and an argument for a central mechanism. Cephalalgia 2002, 22:201–204.

    Article  PubMed  CAS  Google Scholar 

  67. Sprenger T, Valet M, Platzer S, et al.: SUNCT: bilateral hypothalamic activation during headache attacks and resolving of symptoms after trigeminal decompression. Pain 2005, 113:422–426.

    Article  PubMed  Google Scholar 

  68. Rossi P, Cesarino F, Faroni J, et al.: SUNCT syndrome successfully treated with topiramate: case reports. Cephalalgia 2003, 23:998–1000.

    Article  PubMed  CAS  Google Scholar 

  69. Matharu MS, Boes CJ, Goadsby PJ: SUNCT syndrome: prolonged attacks, refractoriness and response to topiramate. Neurology 2002, 58:1307.

    PubMed  CAS  Google Scholar 

  70. Koseoglu E, Karaman Y, Kucuk S, Arman F: SUNCT syndrome associated with compression of trigeminal nerve. Cephalalgia 2005, 25:473–475.

    Article  PubMed  CAS  Google Scholar 

  71. Volcy M, Tepper S, Rapoport A, et al.: Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)—a case report. Cephalalgia 2005, 25:470–472.

    Article  PubMed  CAS  Google Scholar 

  72. Cohen AS: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalalgia 2006, In press.

  73. Lenaerts M, Diederich N, Phuoe D: A patient with SUNCT cured by the Jannetta procedure. Cephalalgia 1997, 17:460.

    Google Scholar 

  74. Gardella L, Viruega A, Rojas H, Nagel J: A case of a patient with SUNCT syndrome treated with Jannetta procedure. Cephalalgia 2001, 21:996–999.

    Article  PubMed  CAS  Google Scholar 

  75. Pareja JA, Kruszewski P, Sjaastad O: SUNCT syndrome: trials of drugs and anesthetic blockades. Headache 1995, 35:138–142.

    Article  PubMed  CAS  Google Scholar 

  76. Peres MF, Stiles MA, Siow HC, et al.: Greater occipital nerve blockade for cluster headache. Cephalalgia 2002, 22:520–522.

    Article  PubMed  CAS  Google Scholar 

  77. Cohen AS, Matharu MS, Kalisch R, et al.: Functional MRI in SUNCT shows differential hypothalamic activation with increasing pain. Cephalalgia 2004, 24:1098–1099.

    Article  Google Scholar 

  78. Leone M, Franzini A, D’Andrea G, et al.: Deep brain stimulation to relieve drug-resistant SUNCT. Ann Neurol 2005, 57:924–927.

    Article  PubMed  Google Scholar 

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  1. Institute of Neurology, Queen Square, London, WC1N 3BG, UK

    Peter J. Goadsby MD, PhD, DSc

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  1. Peter J. Goadsby MD, PhD, DSc
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  2. Anna S. Cohen MRCP, PhD
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  3. Manjit S. Matharu MRCP
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Correspondence to Peter J. Goadsby MD, PhD, DSc.

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Goadsby, P.J., Cohen, A.S. & Matharu, M.S. Trigeminal autonomic cephalalgias: Diagnosis and treatment. Curr Neurol Neurosci Rep 7, 117–125 (2007). https://doi.org/10.1007/s11910-007-0006-6

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