Short-lasting unilateral neuralgiform headache attacks: Treatment
- Manjit S Matharu, MD
Manjit S Matharu, MD
- Consultant Neurologist
- The National Hospital for Neurology and Neurosurgery, London
- Anna S Cohen, MD
Anna S Cohen, MD
- Consultant Neurologist
- Royal Free Hospital, London
The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features [1,2]. The TACs include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua . There are two subtypes of short-lasting unilateral neuralgiform headache attacks :
●Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)
●Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)
Although SUNCT and SUNA were once considered to be refractory to therapy, beneficial responses with several categories of medications have been reported in small numbers of patients. However, the evidence consists mainly of case reports and small case series.
This topic will review the treatment and prognosis of SUNCT and SUNA. The clinical features and diagnosis of these headaches are reviewed elsewhere. (See "Short-lasting unilateral neuralgiform headache attacks: Clinical features and diagnosis".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- 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 ( Pt 1):193.
- Goadsby PJ. Trigeminal autonomic cephalalgias. Pathophysiology and classification. Rev Neurol (Paris) 2005; 161:692.
- Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.
- 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.
- Matharu MS, Cohen AS, Goadsby PJ. SUNCT syndrome responsive to intravenous lidocaine. Cephalalgia 2004; 24:985.
- Prakash KM, Lo YL. SUNCT syndrome in association with persistent Horner syndrome in a Chinese patient. Headache 2004; 44:256.
- Köseoglu E, Karaman Y, Kücük S, Arman F. SUNCT syndrome associated with compression of trigeminal nerve. Cephalalgia 2005; 25:473.
- Rossi P, Cesarino F, Faroni J, et al. SUNCT syndrome successfully treated with topiramate: case reports. Cephalalgia 2003; 23:998.
- Hunt CH, Dodick DW, Bosch EP. SUNCT responsive to gabapentin. Headache 2002; 42:525.
- Gardella L, Viruega A, Rojas H, Nagel J. A case of a patient with SUNCT syndrome treated with Jannetta procedure. Cephalalgia 2001; 21:996.
- Volcy M, Tepper SJ, Rapoport AM, et al. Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)--a case report. Cephalalgia 2005; 25:470.
- Narbone MC, Gangemi S, Abbate M. A case of SUNCT syndrome responsive to verapamil. Cephalalgia 2005; 25:476.
- Malik K, Rizvi S, Vaillancourt PD. The SUNCT syndrome: successfully treated with lamotrigine. Pain Med 2002; 3:167.
- Putzki N, Nirkko A, Diener HC. Trigeminal autonomic cephalalgias: a case of post-traumatic SUNCT syndrome? Cephalalgia 2005; 25:395.
- Cohen AS. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalalgia 2007; 27:824.
- Williams MH, Broadley SA. SUNCT and SUNA: clinical features and medical treatment. J Clin Neurosci 2008; 15:526.
- Pareja JA, Caballero V, Sjaastad O. SUNCT syndrome. Statuslike pattern. Headache 1996; 36:622.
- Montes E, Alberca R, Lozano P, et al. Statuslike SUNCT in two young women. Headache 2001; 41:826.
- Rowbotham MC, Reisner-Keller LA, Fields HL. Both intravenous lidocaine and morphine reduce the pain of postherpetic neuralgia. Neurology 1991; 41:1024.
- Ferrante FM, Paggioli J, Cherukuri S, Arthur GR. The analgesic response to intravenous lidocaine in the treatment of neuropathic pain. Anesth Analg 1996; 82:91.
- Wallace MS, Dyck JB, Rossi SS, Yaksh TL. Computer-controlled lidocaine infusion for the evaluation of neuropathic pain after peripheral nerve injury. Pain 1996; 66:69.
- Gil-Gouveia R, Goadsby PJ. Neuropsychiatric side-effects of lidocaine: examples from the treatment of headache and a review. Cephalalgia 2009; 29:496.
- D'Andrea G, Granella F, Cadaldini M. Possible usefulness of lamotrigine in the treatment of SUNCT syndrome. Neurology 1999; 53:1609.
- Leone M, Rigamonti A, Usai S, et al. Two new SUNCT cases responsive to lamotrigine. Cephalalgia 2000; 20:845.
- D'Andrea G, Granella F, Ghiotto N, Nappi G. Lamotrigine in the treatment of SUNCT syndrome. Neurology 2001; 57:1723.
- Gutierrez-Garcia JM. SUNCT syndrome responsive to lamotrigine. Headache 2002; 42:823.
- 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.
- 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.
- Cohen AS, Matharu MS, Goadsby PJ. Double-blind placebo-controlled trial of topiramate in SUNCT. Cephalalgia 2007; 27:758.
- Etemadifar M, Maghzi AH, Ghasemi M, et al. Efficacy of gabapentin in the treatment of SUNCT syndrome. Cephalalgia 2008; 28:1339.
- Graff-Radford SB. SUNCT syndrome responsive to gabapentin (Neurontin). Cephalalgia 2000; 20:515.
- Porta-Etessam J, Martinez-Salio A, Berbel A, Benito-Leon J. Gabapentin (neuronetin) in the treatment of SUNCT syndrome. Cephalalgia 2002; 22:249; author reply 249.
- Cohen AS, Matharu MS, Goadsby PJ. SUNCT syndrome in the elderly. Cephalalgia 2004; 24:508.
- Gantenbein AR, Goadsby PJ. Familial SUNCT. Cephalalgia 2005; 25:457.
- Marziniak M, Breyer R, Evers S. SUNCT syndrome successfully treated with the combination of oxcarbazepine and gabapentin. Pain Med 2009; 10:1497.
- Dora B. SUNCT syndrome with dramatic response to oxcarbazepine. Cephalalgia 2006; 26:1171.
- Rozen TD, Saper JR, Sheftell FD, Dodick DW. Clomiphene citrate as a new treatment for SUNCT: hormonal manipulation for hypothalamic-influenced trigeminal autonomic cephalalgias. Headache 2005; 45:754.
- Rozen TD. Complete alleviation of treatment refractory primary SUNCT syndrome with clomiphene citrate (a medicinal deep brain hypothalamic modulator). Cephalalgia 2014; 34:1021.
- Sesso RM. SUNCT syndrome or trigeminal neuralgia with lacrimation and conjunctival injection? Cephalalgia 2001; 21:151.
- Pareja JA, Kruszewski P, Sjaastad O. SUNCT syndrome: trials of drugs and anesthetic blockades. Headache 1995; 35:138.
- 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.
- 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.
- Ikawa M, Imai N, Manaka S. A case of SUNCT syndrome responsive to zonisamide. Cephalalgia 2011; 31:501.
- Zabalza RJ. Sustained response to botulinum toxin in SUNCT syndrome. Cephalalgia 2012; 32:869.
- Calvo JF, Bruera OC, de Lourdes Figuerola M, et al. SUNCT syndrome: clinical and 12-year follow-up case report. Cephalalgia 2004; 24:900.
- Trauninger A, Alkonyi B, Kovács N, et al. Methylprednisolone therapy for short-term prevention of SUNCT syndrome. Cephalalgia 2010; 30:735.
- Goadsby PJ. Migraine pathophysiology. Headache 2005; 45 Suppl 1:S14.
- Goadsby PJ, Bartsch T, Dodick DW. Occipital nerve stimulation for headache: mechanisms and efficacy. Headache 2008; 48:313.
- Porta-Etessam J, Cuadrado ML, Galán L, et al. Temporal response to bupivacaine bilateral great occipital block in a patient with SUNCT syndrome. J Headache Pain 2010; 11:179.
- Matharu M, Watkins L, Shanahan P. POH04 Treatment of medically intractable SUNCT and SUNA with occipital nerve stimulation. J Neurol Neurosurg Psychiatry 2010; 81:e51.
- 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.
- Hannerz J, Linderoth B. Neurosurgical treatment of short-lasting, unilateral, neuralgiform hemicrania with conjunctival injection and tearing. Br J Neurosurg 2002; 16:55.
- Sebastian S, Schweitzer D, Tan L, Broadley SA. Role of trigeminal microvascular decompression in the treatment of SUNCT and SUNA. Curr Pain Headache Rep 2013; 17:332.
- Williams M, Bazina R, Tan L, et al. Microvascular decompression of the trigeminal nerve in the treatment of SUNCT and SUNA. J Neurol Neurosurg Psychiatry 2010; 81:992.
- May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiologic implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab 1999; 19:115.
- Leone M, Franzini A, D'Andrea G, et al. Deep brain stimulation to relieve drug-resistant SUNCT. Ann Neurol 2005; 57:924.
- 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.
- Matharu MS, Zrinzo L. Deep brain stimulation in cluster headache: hypothalamus or midbrain tegmentum? Curr Pain Headache Rep 2010; 14:151.
- Miller S, Akram H, Lagrata S, et al. Ventral tegmental area deep brain stimulation in refractory short-lasting unilateral neuralgiform headache attacks. Brain 2016; 139:2631.
- ACUTE MEDICATIONS
- - Administration and side effects
- PREVENTIVE MEDICATIONS
- - Dose and side effects
- - Dose and side effects
- - Dose and side effects
- Treatment choice
- Greater occipital nerve blockade and stimulation
- Other local blockades
- Trigeminal microvascular decompression
- Destructive trigeminal nerve procedures
- Deep brain stimulation
- SUMMARY AND RECOMMENDATIONS