Abstract
Severe shortlasting headaches are rare but very disabling conditions with a major impact on the patients’ quality of life. Following the IHS criteria, these headaches broadly divide themselves into those associated with autonomic symptoms, so called trigeminal autonomic cephalgias (TACs), and those with few autonomic symptoms. The trigeminal-autonomic cephalgias include cluster headache, paroxysmal hemicranias, and a syndrome called SUNCT (short lasting unilateral neuralgic cephalgias with conjunctival injection and tearing). In all of these syndromes, hemispheric head pain and cranial autonomic symptoms are prominent. The paroxysmal hemicranias have, unlike cluster headaches, a very robust response to indomethacin, leading to a notion of indomethacin-sensitive headaches. Although TACs are, in comparison with migraine, quite rare, it is nevertheless very important to consider the clinical factor that they are easy to diagnose and the treatment is very effective in most patients.
Similar content being viewed by others
References
Bahra A, Gawel MJ, Hardebo JE, Millson D, Breen SA, Goadsby PJ (2000) Oral zolmitriptan is effective in the acute treatment of cluster headache. Neurology 54:1832–1839
Black D, Dodick DW (2002) Two cases of medically and surgically intractable SUNCT: a reason for caution and an argument for a central mechanism. Cephalalgia 3:201–204
Chazot G, Claustrat B, Brun J, Jordan D, Sassolas G, Schott B (1984) A chronobiological study of melatonin, cortisol, growth hormone and prolactin secretion in cluster headache. Cephalalgia 4:213–220
Cohen AS, Matharu MS, Kalisch R, Friston K, Goadsby PJ (2004) Functional MRI in SUNCT shows differential hypothalamic activation with increasing pain. Cephalalgia 24:1098–1099
Dodick D, Rozen T, Goadsby P, Silberstein S (2000) Cluster headache. Cephalalgia 20:787–803
Dodick DW (2004) Indomethacin-responsive headache syndromes. Curr Pain Headache Rep 8:19–26
Donnet A, Valade D, Regis J (2005) Gamma knife treatment for refractory cluster headache: prospective open trial. J Neurol Neurosurg Psychiatry 76:218–221
Ekbom K (1968) Nitroglycerin as a provocative agent in cluster headache. Arch Neurol 19:487–493
Ekbom K (1970) Patterns of cluster headache with a note on the relations to angina pectoris and peptide ulcer. Acta Neurol Scand 46:225–237
Ferrari E, Canepari C, Bossolo PA, Vailati A, Martignoni E, Micieli G, Nappi G (1983) Changes of biological rhythms in primary headache syndromes. Cephalalgia 3:58–68
Franzini A, Ferroli P, Leone M, Broggi G (2003) Stimulation of the posterior hypothalamus for treatment of chronic intractable cluster headaches: first reported series. Neurosurgery 52:1095–1101
Goadsby PJ (2002) Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol 1:251–257
Goadsby PJ, Edvinsson L (1994) Human in vivo evidence for trigeminovascular activation in cluster headache. Neuropeptide changes and effects of acute attacks therapies. Brain 117:427–434
Goadsby PJ, Lipton RB (1997) A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 120:193–209
Headache Classification Committee of the International Headache Society (1988) Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia 8:1–96
Headache Classification Committee of the International Headache Society (2004) The International Classification of Headache Disorders, 2nd edn. Cephalalgia 24:1–160
Isler H (1993) Episodic cluster headache from a textbook of 1745: Van Swieten’s classic description. Cephalalgia 13:172–174
Jarrar RG, Black DF, Dodick DW, Davis DH (2003) Outcome of trigeminal nerve section in the treatment of chronic cluster headache. Neurology 60:1360–1362
Kudrow L (1987) The cylic relationship of natural illumination to cluster period frequency. Cephalalgia 7:76–78
Kunkle EC, Pfieffer J, Wilhoit WM, Hamrick J (1952) Recurrent brief headache in cluster pattern. Trans Am Neurol Assoc 27:240–243
Leone M (2004) Chronic cluster headache: new and emerging treatment options. Curr Pain Headache Rep 8:347–352
Leone M, Attanasio A, Croci D, D’Amico D, Grazzi L, Ferraris A, Nespolo A, Bussone G (1999) Neuroendocrinology of cluster headache. Ital J Neurol Sci 20:S18–S20
Leone M, Franzini A, D’Amico D, Grazzi L, Rigamonti A, Mea E, Broggi G, Bussone G (2006) Long-term follow-up of hypothalamic stimulation to relieve intractable chronic cluster headache. Neurology:in press
Leone M, May A, Franzini A, Broggi G, Dodick D, Rapoport A, Goadsby P, Schoenen J, Bonavita V, Bussone G (2004) Deep brain stimulation for intractable chronic cluster headache: proposals for patient selection. Cephalalgia 24:934–937
Leone M, Patruno G, Vescovi A, Bussone G (1990) Neuroendocrine dysfunction in cluster headache. Cephalalgia 10:235–239
Matharu MS, Boes CJ, Goadsby PJ (2003) Management of trigeminal autonomic cephalgias and hemicrania continua. Drugs 63:1637–1677
Matharu MS, Cohen AS, Boes CJ, Goadsby PJ (2003) Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome: a review. Curr Pain Headache Rep 7:308–318
Matharu MS, Cohen AS, McGonigle DJ, Ward N, Frackowiak RS, Goadsby PJ (2004) Posterior hypothalamic and brainstem activation in hemicrania continua. Headache 44:747–761
Matharu MS, Goadsby PJ (2002) Persistence of attacks of cluster headache after trigeminal nerve root section. Brain 125:976–984
Matharu MS, Good CD, May A, Bahra A, Goadsby PJ (2003) No change in the structure of the brain in migraine: a voxel-based morphometric study. Eur J Neurol 10:53–57
May A (2005) Cluster headache: pathogenesis, diagnosis, and management. Lancet 366:843–855
May A (2003) Headaches with (ipsilateral) autonomic symptoms. J Neurol 250:1273–1278
May A, Ashburner J, Buchel C, McGonigle DJ, Friston KJ, Frackowiak RS, Goadsby PJ (1999) Correlation between structural and functional changes in brain in an idiopathic headache syndrome. Nat Med 5:836–838
May A, Bahra A, Buchel C, Turner R, Goadsby PJ (1999) Functional magnetic resonance imaging in spontaneous attacks of SUNCT: short-lasting neuralgiform headache with conjunctival injection and tearing. Ann Neurol 46:791–794
May A, Leone M, Áfra J, Linde M, Sándor P, Evers S, Goadsby P (2006) EFNS guideline on the treatment of cluster headache and other trigemino-autonomic cephalgias. Eur J Neurol:in press
May A, Leone M, Boecker H, Sprenger T, Juergens T, Bussone G, Tolle T (2006) Hypothalamic Deep Brain Stimulation in PET. J Neurosci:in press
Nilsson Remahl AI, Laudon Meyer E, Cordonnier C, Goadsby PJ (2003) Placebo response in cluster headache trials: a review. Cephalalgia 23:504–510
Olesen J, Tfelt-Hansen P, Welch K (1999) The Headaches. Lippincott Williams & Wilkins, Philadelphia
Pareja JA, Caminero AB, Sjaastad O (2002) SUNCT Syndrome: diagnosis and treatment. CNS Drugs 16:373–383
Schoenen J, Di Clemente L, Vandenheede M, Fumal A, De Pasqua V, Mouchamps M, Remacle JM, de Noordhout AM (2005) Hypothalamic stimulation in chronic cluster headache: a pilot study of efficacy and mode of action. Brain 128:940–947
Schuh-Hofer S, Reuter U, Kinze S, Einhaupl KM, Arnold G (2002) Treatment of acute cluster headache with 20 mg sumatriptan nasal spray– an open pilot study. J Neurol 249:94–99
Sjaastad O (ed) (1992) Cluster Headache Syndrome. W B Saunders Company Ltd, London
Sjaastad O, Apfelbaum R, Caskey W, Christoffersen B, Diamond S, Graham J, Green M, Horven I, Lund-Roland L, Medina J, Rogado S, Stein H (1980) Chronic paroxysmal hemicrania (CPH). The clinical manifestations. A review. Ups J Med Sci Suppl 31:27–33
Sjaastad O, Dale I (1974) Evidence for a new (?), treatable headache entity. Headache 14:105–108
Sprenger T, Boecker H, Tolle TR, Bussone G, May A, Leone M (2004) Specific hypothalamic activation during a spontaneous cluster headache attack. Neurology 62:516–517
Sprenger T, Valet M, Hammes M, Erhard P, Berthele A, Conrad B, Tolle T (2004) Hypothalamic activation in trigeminal autonomic cephalgia: functional imaging of an atypical case. Cephalalgia 24:753–757
Sprenger T, Valet M, Platzer S, Pfaffenrath V, Steude U, Tolle TR (2005) SUNCT: bilateral hypothalamic activation during headache attacks and resolving of symptoms after trigeminal decompression. Pain 113:422–426
Strittmatter M, Hamann GF, Grauer M, Fischer C, Blaes F, Hoffmann KH, Schimrigk K (1996) Altered activity of the sympathetic nervous system and changes in the balance of hypophyseal, pituitary and adrenal hormones in patients with cluster headache. Neuroreport 7:1229–1234
Van Vliet JA, Bahra A, Martin V, Ramadan N, Aurora SK, Mathew NT, Ferrari MD, Goadsby PJ (2003) Intranasal sumatriptan in cluster headache: Randomized placebo-controlled double-blind study. Neurology 60:630–633
Waldenlind E, Drummond PD (1999) Synthesis of cluster headache pathophysiology. In: Olesen J, Tfelt Hansen P, Welch KM (eds) The Headaches. Lippincott Williams & Wilkins, Philadelphia
Waldenlind E, Gustafsson SA, Ekbom K, Wetterberg L (1987) Circadian secretion of cortisol and melatonin in cluster headache during active cluster periods and remission. J Neurol Neurosurg Psychiatry 50:207–213
Acknowledgments
The author wish to thank E. Schoell for editing the manuscript. AM is supported by a grant of the Deutsche Forschungsgemeinschaft (MA 1862/2) and by a grant of the BMBF (project no. 371 57 01).
Author information
Authors and Affiliations
Corresponding author
Additional information
Received in revised form: 11 May 2006
Rights and permissions
About this article
Cite this article
May, A. Update on the diagnosis and management of Trigemino-Autonomic Headaches. J Neurol 253, 1525–1532 (2006). https://doi.org/10.1007/s00415-006-0303-2
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/s00415-006-0303-2