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SUNCT and SUNA: Recognition and Treatment

  • HEADACHE (JR COUCH, SECTION EDITOR)
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Opinion statement

The problem of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) management remains unsolved. Despite a myriad of therapeutic trials, no convincingly effective remedy for SUNCT and SUNA is available at present. Based on open-label communications, some patients seemed to benefit from some pharmacologic, interventional, or invasive procedures. Possible effective preventive drugs are carbamazepine, lamotrigine, gabapentin, and topiramate. At present, the drug of choice for SUNCT seems to be lamotrigine whereas SUNA may better respond to gabapentin. There is no available abortive treatment for the individual attacks. During the worst periods, intravenous lidocaine may decrease the flow of SUNCT/SUNA attacks. In SUNCT, bilateral blockade of the greater occipital nerve, and superior cervical ganglion opioid blockade have been reported as temporary/partially effective in one patient each. Botulinum toxin injected around the symptomatic orbit provided sustained relief to one patient. Owing to the scarcity of reports the results of these interventions should be taken as preliminary. Invasive therapy with interventions directed to the first division of the trigeminal nerve or Gasserian ganglion, with local anesthetics or alcohol, radiofrequency thermocoagulation, microvascular decompression, and gamma-knife neurosurgery, have been tried in the treatment of refractory SUNCT. Some patients seemed to benefit from such interventions, but one should still have a critical attitude to these claims since no convincing results have been obtained as yet. The few SUNCT patients who underwent deep brain hypothalamic stimulation obtained a substantial and persistent relief.

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  1. Sjaastad O, Russell D, Horven I, Bunaes U. Multiple neuralgiform unilateral headache attacks associated with conjunctival injection and appearing in clusters. A nosological problem. Proceedings of the Scandinavian Migraine Society 1978; 31.

  2. Sjaastad O, Saunte C, Salvesen R, et al. Shortlasting, unilateral, neuralgiform headache attacks with conjuntival injection, tearing, sweating and rhinorrhea. Cephalalgia. 1989;9:147–56.

    PubMed  CAS  Google Scholar 

  3. Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review. Headache. 1997;37:195–202.

    Article  PubMed  CAS  Google Scholar 

  4. Matharu MS, Cohen AS, Boes CJ, Goadsby PJ. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome: a review. Curr Pain Headache Rep. 2003;7:308–18.

    Article  PubMed  Google Scholar 

  5. Pareja JA, Cuadrado ML. SUNCT syndrome. An update. Expert Opin Pharmacother. 2005;6:591–9.

    Article  PubMed  CAS  Google Scholar 

  6. Goadsby PJ, Cittadini E, Burns B, Cohen AS. Trigeminal autonomic cephalalgias: diagnostic and therapeutic developments. Curr Opin Neurol. 2008;21:323–30.

    Article  PubMed  Google Scholar 

  7. Goadsby PJ, Cittadini E, Cohen AS. Trigeminal autonomic cephalalgias: paroxysmal hemicrania, SUNCT/SUNA, and hemicrania continua. Semin Neurol. 2010;30:186–91.

    Article  PubMed  Google Scholar 

  8. Goadsby PJ. Trigeminal autonomic cephalalgias. Continuum (Minneap Minn). 2012;18:3–95.

    Google Scholar 

  9. Pareja JA, Pareja J, Palomo T, et al. SUNCT syndrome. Repetitive and overlapping attacks. Headache. 1994;34:114–6.

    Article  PubMed  CAS  Google Scholar 

  10. Pareja JA, Sjaastad O. SUNCT syndrome in the female. Headache. 1994;34:217–20.

    Article  PubMed  CAS  Google Scholar 

  11. Laín AH, Caminero AB, Pareja JA. SUNCT syndrome. Absence of refractory periods and modulation of attack duration by lengthening of the trigger stimuli. Cephalalgia. 2000;20:671–3.

    Article  PubMed  Google Scholar 

  12. Pareja JA, Shen JM, Kruszewski P, et al. SUNCT syndrome. Duration, frequency and temporal distribution of attacks. Headache. 1996;36:161–5.

    Article  PubMed  CAS  Google Scholar 

  13. Cohen AS, Matharu MS, 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;129:2746–60.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  15. International Headache Society. The international classification of headache disorders, 2nd edition. Cephalalgia. 2004;24 Suppl 1:1–160.

    Google Scholar 

  16. May A, Bahra A, Büchel C, et al. Functional magnetic resonance imaging in spontaneous attacks of SUNCT: short-lasting neuralgiform headache with conjunctival injection and tearing. Ann Neurol. 1999;46:791–3.

    Article  PubMed  CAS  Google Scholar 

  17. Massiou H, Launay JM, Levy C, et al. SUNCT syndrome in two patients with prolactinomas and bromocriptine-induced attacks. Neurology. 2002;58:1698–9.

    Article  PubMed  CAS  Google Scholar 

  18. Larner AJ. Headache induced by dopamine agonists prescribed for prolactinoma: think SUNCT! Int J Clin Pract. 2006;60:360–1.

    Article  PubMed  CAS  Google Scholar 

  19. Jiménez Caballero PE. SUNCT syndrome in a patient with prolactinoma and cabergoline-induced attacks. Cephalalgia. 2007;27:76–8.

    Article  PubMed  Google Scholar 

  20. Pareja JA, Kruszewski P, Sjaastad O. SUNCT syndrome: trials of drugs and anesthetic blockades. Headache. 1995;35:138–42.

    Article  PubMed  CAS  Google Scholar 

  21. Matharu MS, Boes CJ, Goadsby PJ. Management of trigeminal autonomic cephalalgias and hemicrania continua. Drugs. 2003;63:1637–77.

    Article  PubMed  CAS  Google Scholar 

  22. Pareja JA, Caminero AB, Sjaastad O. SUNCT syndrome. Diagnosis and treatment. CNS Drugs. 2002;16:373–83.

    Article  PubMed  CAS  Google Scholar 

  23. Leone M, Franzini A, D’Andrea G, et al. Deep brain stimulation to relieve drug-resistant SUNCT. Ann Neurol. 2005;57:924–7.

    Article  PubMed  Google Scholar 

  24. Cohen AS. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalalgia. 2007;27:824–32.

    Article  PubMed  CAS  Google Scholar 

  25. Dora B. SUNCT syndrome with dramatic response to oxcarbazepine. Cephalalgia. 2006;26:1171–3.

    Article  PubMed  CAS  Google Scholar 

  26. D’Andrea G, Granella F, Cadaldini M. Possible usefulness of lamotrigine in the treatment of SUNCT syndrome. Neurology. 1999;53:1609.

    Article  PubMed  Google Scholar 

  27. D’Andrea G, Granella F, Ghiotto N, Nappi G. Lamotrigine in the treatment of SUNCT syndrome. Neurology. 2001;57:1723–5.

    Article  PubMed  Google Scholar 

  28. Leone M, Rigamonti A, Usai D, et al. Two new SUNCT cases responsive to lamotrigine. Cephalalgia. 2000;20:845–7.

    Article  PubMed  CAS  Google Scholar 

  29. May A, Leone M, Afra J, et al. EFNS guidelines on the treatment of cluster headache and other trigeminal-autonomic cephalalgias. Eur J Neurol. 2006;13:1066–77.

    Article  PubMed  CAS  Google Scholar 

  30. Gutiérrez-García JM. SUNCT syndrome responsive to lamotrigine. Headache. 2002;42:823–5.

    Article  PubMed  Google Scholar 

  31. Malik K, Rizvi S, Vaillancourt PD. The SUNCT syndrome: successfully treated with lamotrigine. Pain Med. 2002;3:167–8.

    Article  PubMed  Google Scholar 

  32. Chakravarty A, Mukherjee A. SUNCT syndrome responsive to lamotrigine: documentation of the first Indian case. Cephalalgia. 2003;23:474–5.

    Article  PubMed  CAS  Google Scholar 

  33. Piovesan EJ, Siow C, Kowacs PA, Werneck LC. Influence of lamotrigine over the SUNCT syndrome: one patient follow-up for two years. Arq Neuropsiquiatr. 2003;61:691–4.

    PubMed  Google Scholar 

  34. Rosselli JL, Karpinski JP. The role of lamotrigine in the treatment of short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome. Ann Pharmacother. 2011;45:108–13. A thorough review article dealing with the treatment of SUNCT with lamotrigine that provided a resolution of symptoms or a substantial improvement in the frequency of attacks.

    Article  PubMed  CAS  Google Scholar 

  35. Graff-Radford SB. SUNCT syndrome responsive to gabapentin (Neurontin). Cephalalgia. 2000;20:515–7.

    Article  PubMed  CAS  Google Scholar 

  36. Porta-Etessam J, Benito-Leon J, Martinez-Salio A, Berbel A. Gabapentin in the treatment of SUNCT syndrome. Headache. 2002;42:523–4.

    Article  PubMed  CAS  Google Scholar 

  37. Rocha Filho PA, Rabello GD, Galvão AC, et al. Gabapentin in the treatment of SUNCT syndrome. Arq Neuropsiquiatr. 2007;65:503–5.

    Article  PubMed  Google Scholar 

  38. Hunt CH, Dodick DW, Bosch EP. SUNCT responsive to gabapentin. Headache. 2002;42:525–6.

    Article  PubMed  Google Scholar 

  39. Volcy M, Tepper SJ, Rapoport A, et al. Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)—a case report. Cephalalgia. 2005;25:470–2.

    Article  PubMed  CAS  Google Scholar 

  40. Etemadifar M, Maghzi AH, Ghasemi M, et al. Efficacy of gabapentin in the treatment of SUNCT syndrome. Cephalalgia. 2008;28:1339–42.

    Article  PubMed  CAS  Google Scholar 

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

    Google Scholar 

  42. 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 

  43. Kuhn J, Vosskaemper M, Berwemeyer H. SUNCT syndrome: a possible bilateral case responding to topiramate. Neurology. 2005;64:2159.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  45. Ikawa M, Imai N, Manaka S. A case of SUNCT syndrome responsive to zonisamide. Cephalalgia. 2011;31:501–3.

    Article  PubMed  Google Scholar 

  46. Matharu MS, Cohen AS, Goadsby PJ. SUNCT syndrome responsive to intravenous lidocaine. Cephalalgia. 2004;24:985–92.

    Article  PubMed  CAS  Google Scholar 

  47. Williams MH, Broadley SA. SUNCT and SUNA: Clinical features and medical treatment. J Clin Neurosci. 2008;15:526–34.

    Article  PubMed  Google Scholar 

  48. Arroyo AM, Durán XR, Beldarrain MG, et al. Response to intravenous lidocaine in a patient with SUNCT syndrome. Cephalalgia. 2010;30:110–2.

    PubMed  Google Scholar 

  49. Marmura MJ. Intravenous lidocaine and mexiletine in the management of trigeminal autonomic cephalalgias. Curr Pain Headache Rep. 2010;14:145–50.

    Article  PubMed  Google Scholar 

  50. Porta-Etessam J, Cuadrado ML, Galán L, et al. Temporal response to bupivacaine great occipital block in a patient with SUNCT syndrome. J Headache Pain. 2010;11:179.

    Article  PubMed  Google Scholar 

  51. Sabatowski R, Huber M, Meuser T, Radbruch L. SUNCT syndrome: a treatment option with local opioid blockade of the superior cervical ganglion? A case report. Cephalalgia. 2001;21:154–6.

    Article  PubMed  CAS  Google Scholar 

  52. Morales-Asín F, Espada F, López-Obarrio LA, et al. A SUNCT case with response to surgical treatment. Cephalalgia. 2000;20:67–8.

    Article  PubMed  Google Scholar 

  53. Baabor M, Rosas CS, Pérez-Limonte L. SUNCT responsive to percutaneous balloon compression of the gasserian ganglion–10-year follow-up. Headache. 2010;50:143–5.

    Article  PubMed  Google Scholar 

  54. Ertsey C, Bozsik G, Afra J, Jelencsik I. A case of SUNCT syndrome with neurovascular compression. Cephalalgia. 2000;20:325.

    Article  Google Scholar 

  55. Hannerz J, Linderoth B. Neurosurgical treatment of short-lasting, unilateral, neuralgiform hemicrania with conjunctival injection and tearing. Br J Neurosurg. 2002;16:55–8.

    Article  PubMed  CAS  Google Scholar 

  56. Effendi K, Jarjoura S, Mathieu D. SUNCT syndrome successfully treated by gamma knife radiosurgery: case report. Cephalalgia. 2011;31:870–3.

    Article  PubMed  Google Scholar 

  57. Mathew T, Srinivas D, Aroor S, et al. SUNCT syndrome treated with gamma knife targeting the trigeminal nerve and sphenopalatine ganglion. J Headache Pain. 2012;13:491–2.

    Article  PubMed  Google Scholar 

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

    Google Scholar 

  59. Gardella L, Viruega A, Nagel J. A case of a patient with SUNCT syndrome treated with Jannetta procedure. Cephalalgia. 2001;21:996–9.

    Article  PubMed  CAS  Google Scholar 

  60. Lagares A, Gómez PA, Pérez-Núñez A, et al. Short-lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing Syndrome treated with microvascular decompression of the trigeminal nerve: case report. Neurosurgery. 2005;56:E413.

    Article  PubMed  Google Scholar 

  61. Sprenger T, Valet M, Platzer S, et al. SUNCT: Bilateral hypothalamic activation during headache attacks and resolving of symptoms after trigeminal decompression. Pain. 2005;113:422–6.

    Article  PubMed  Google Scholar 

  62. Irimia P, González-Redondo R, Dominguez PD, et al. Microvascular decompression may be effective for refractory SUNCT regardless of symptom duration. Cephalalgia. 2010;30:626–30.

    PubMed  CAS  Google Scholar 

  63. Williams M, Bazina R, Tan L, Rice H, Boroadley SA. Microvascular decompression of the trigeminal nerve in the treatment of SUNCT and SUNA. J Neurol Neurosurg Psychiatry. 2010;81:998–6. According to the data presented in this report a substantial number of SUNCT and SUNA patients improved after decompression of the trigeminal nerve.

    Google Scholar 

  64. Leone M, Franzini A, D’Andrea G, et al. Deep brain stimulation to relieve drug-resistant SUNCT. Ann Neurol. 2005;57:924–7.

    Article  PubMed  Google Scholar 

  65. Lyons MK, Dodick DW, Evidente VG. Responsiveness of short lasting unilateral neuralgiform headache with conjunctival injection and tearing to hypothalamic deep brain stimulation. J Neurosurg. 2009;110:279–81.

    Article  PubMed  Google Scholar 

  66. Bartsch T, Falk D, Knudsen K, Reese R, Raethjen J, Mehdorn HM, Volkmann J, Deuschl G. Deep brain stimulation of the posterior hypothalamic area in intractable short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. Cephalalgia. 2011;31:1405–8. Present and previous communications by Leone and Lyons indicate that deep brain stimulation of the posterior hypothalamus can be effective in the treatment of chronic refractory SUNCT.

    Article  PubMed  CAS  Google Scholar 

  67. Leone M, Franzini A, Proietti Cecchini A, et al. Deep brain stimulation in trigeminal autonomic cephalalgias. Neurotherapeutics. 2010;7:220–8.

    Article  PubMed  Google Scholar 

  68. 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;22:201–4.

    Article  PubMed  CAS  Google Scholar 

  69. Hannerz J, Greitz D, Hansson P, Ericson K. SUNCT may be another manifestation of orbital venous vasculitis. Headache. 1992;32:384–9.

    Article  PubMed  CAS  Google Scholar 

  70. Raimondi E, Gardella L. SUNCT syndrome. Two cases in Argentina. Headache. 1998;38:369–71.

    Article  PubMed  CAS  Google Scholar 

  71. Salazar JA, Tamayo JA, Fernandez O. SUNCT type headache. A report of a new case. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Neurologia. 1998;13:148–50.

    PubMed  CAS  Google Scholar 

  72. Narbone MC, Gangemi S, Abbate M. A case of SUNCT syndrome responsive to verapamil. Cephalalgia. 2005;25:476–8.

    Article  PubMed  CAS  Google Scholar 

  73. Jiménez-Huete A, Franch O, Pareja JA. SUNCT syndrome: Priming of symptomatic periods and worsening of symptoms by treatment with calcium channel blockers. Cephalalgia. 2002;22:812–4.

    Article  PubMed  Google Scholar 

  74. Trauninger A, Alkonyi B, Kovács N, Komoly S. Methylprednisolone therapy for short-term prevention of SUNCT syndrome. Cephalalgia. 2010;30:735–9.

    PubMed  CAS  Google Scholar 

  75. De Lourdes Figuerola M, Bruera O, Pozzo MJ, Leston J. SUNCT syndrome responding absolutely to steroids in two cases with different etiologies. J Headache Pain. 2009;10:55–7.

    Article  PubMed  Google Scholar 

  76. Rozen TD, Saper JL, Sheftell FD, Dodick DW. Clomiphene citrate as a new treatment for SUNCT: hormonal manipulation for hypothalamic-influenced trigeminal autonomic cephalalgias. Headache. 2005;45:754–6.

    Article  PubMed  Google Scholar 

  77. Schwaag S, Frese A, Husstedt IW, Evers S. SUNCT syndrome: the first German case series. Cephalalgia. 2003;23:398–400.

    Article  PubMed  CAS  Google Scholar 

  78. Zabalza RJ. Sustained response to botulinum toxin in SUNCT syndrome. Cephalalgia. 2012;32:869–72.

    Article  PubMed  Google Scholar 

  79. Sékhara T, Pelc K, Mewasingh LD, Boucquey D, Dan B. Pediatric SUNCT syndrome. Pediatr Neurol. 2005;33:206–7.

    Article  PubMed  Google Scholar 

  80. Unalp A, Ozturk AA. SUNCT syndrome in a child: a rare cause of paroxysmal headache. Ann Saudi Med. 2008;28:386–7.

    Article  PubMed  Google Scholar 

  81. Blättler T, Capone Mori A, Boltshauser E, Bassetti C. Symptomatic SUNCT in an eleven-year-old girl. Neurology. 2003;60:2012–3.

    Article  PubMed  Google Scholar 

  82. Sciruicchio V, Sardaro M, Gagliardi D, et al. A case of early onset and monophasic trigeminal autonomic cephalalgia: could it be a SUNCT? J Headache Pain. 2010;11:363–5.

    Article  PubMed  Google Scholar 

  83. Martino GH. SUNCT syndrome (Short lasting unilateral neuralgiform headache with conjunctival injection and tearing): a rare case in a pediatric patient with favourable response to topiramato. Arch Argent Pediatr. 2008;106:40–6.

    PubMed  Google Scholar 

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Pareja, J.A., Álvarez, M. & Montojo, T. SUNCT and SUNA: Recognition and Treatment. Curr Treat Options Neurol 15, 28–39 (2013). https://doi.org/10.1007/s11940-012-0211-8

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