Skip to main content

Advertisement

SpringerLink
Log in

SUNCT and SUNA: an Update and Review

  • Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

The purpose of this review is to provide an update on the clinical features, diagnosis, pathogenesis, epidemiology, and treatment of the rare primary headache disorders short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA). Together these entities are known as short-lasting unilateral neuralgiform headache attacks (SUNHA).

Recent Findings

Recent case reports of secondary SUNCT and SUNA due to medullary infarcts support the theory that the trigeminohypothalamic pathway is involved in the pathophysiology of SUNHA. While medical therapy for SUNHA has not significantly changed, surgical therapy for refractory SUNCT and SUNA has made advancements with a recent case series demonstrating the efficacy of deep brain stimulation.

Summary

We will discuss the pathophysiology of both the pain and the autonomic symptoms experienced in SUNCT and SUNA attacks as well the medical, procedural, and surgical options for treatment with emphasis on recent advances. Specific secondary causes reported in the recent literature will be discussed in brief.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance•• Of major importance

  1. Headache Classification Committee of the International Headache Society (IHS). The international classification of headache disorders, 3rd edition (beta version). Cephalalgia. 2013;33(9):629–808. https://doi.org/10.1177/0333102413485658.

    Article  Google Scholar 

  2. Benoliel R, Sharav Y, Haviv Y, Almoznino G. Tic, triggering, and tearing: from CTN to SUNHA. Headache. 2017;57(6):997–1009. https://doi.org/10.1111/head.13040.

    Article  PubMed  CAS  Google Scholar 

  3. • Miller S, Watkins L, Matharu M. Long-term follow up of intractable chronic short lasting unilateral neuralgiform headache disorders treated with occipital nerve stimulation. Cephalalgia. 2017;38:933–42. https://doi.org/10.1177/0333102417721716. Largest reported cohort of SUNHA patients studied for effectiveness of ONS.

    Article  PubMed  Google Scholar 

  4. Zhang Y, Zhang H, Lian YJ, Ma YQ, Xie NC, Cheng X, et al. Botulinum toxin A for the treatment of a child with SUNCT syndrome. Pain Res Manag. 2016;2016:1–4. https://doi.org/10.1155/2016/8016065.

    Article  Google Scholar 

  5. Meyers SL. Cluster headache and trigeminal autonomic cephalgias. Dis Mon. 2015;61(6):236–9. https://doi.org/10.1016/j.disamonth.2015.03.007.

    Article  PubMed  Google Scholar 

  6. •• Baraldi C, Pellesi L, Guerzoni S, Cainazzo MM, Pini LA. Therapeutical approaches to paroxysmal hemicrania, hemicrania continua and short lasting unilateral neuralgiform headache attacks: a critical appraisal. J Headache Pain. 2017;18(1):71. https://doi.org/10.1186/s10194-017-0777-3. Recent comprehensive review of the treatment of TACs.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  7. Wober C. Tics in TACs: a step into an avalanche? Systematic literature review and conclusions. Headache. 2017;57(10):1635–47. https://doi.org/10.1111/head.13099.

    Article  PubMed  Google Scholar 

  8. •• Eller M, Goadsby PJ. Trigeminal autonomic cephalalgias. Oral Dis. 2016;22(1):1–8. https://doi.org/10.1111/odi.12263. Basis for the pathophysiology of TACs.

    Article  PubMed  CAS  Google Scholar 

  9. Lambru G, Matharu MS. SUNCT, SUNA and trigeminal neuralgia: different disorders or variants of the same disorder? Curr Opin Neurol. 2014;27(3):325–31. https://doi.org/10.1097/wco.0000000000000090.

    Article  PubMed  CAS  Google Scholar 

  10. Khonsary SA, Ma Q, Villablanca P, Emerson J, Malkasian D. Clinical functional anatomy of the pterygopalatine ganglion, cephalgia and related dysautonomias: a review. Surg Neurol Int. 2013;4(Suppl 6):S422–8. https://doi.org/10.4103/2152-7806.121628.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Stranding S. Gray’s anatomy. 41st ed. New York: Elsevier; 2016.

    Google Scholar 

  12. Leone M, Mea E, Genco S, Bussone G. Coexistence of TACS and trigeminal neuralgia: pathophysiological conjectures. Headache. 2006;46(10):1565–70. https://doi.org/10.1111/j.1526-4610.2006.00537.x.

    Article  PubMed  Google Scholar 

  13. • Rozen TD. Complete alleviation of treatment refractory primary SUNCT syndrome with clomiphene citrate (a medicinal deep brain hypothalamic modulator). Cephalalgia. 2014;34(12):1021–4. https://doi.org/10.1177/0333102414527647. Novel study in the pathophysiology of SUNCT.

    Article  PubMed  Google Scholar 

  14. Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol. 2002;1(4):251–7.

    Article  PubMed  Google Scholar 

  15. •• Miller S, Akram H, Lagrata S, Hariz M, Zrinzo L, Matharu M. Ventral tegmental area deep brain stimulation in refractory short-lasting unilateral neuralgiform headache attacks. Brain. 2016;139(Pt 10):2631–40. https://doi.org/10.1093/brain/aww204. First case series exploring DBS for SUNCT.

    Article  PubMed  Google Scholar 

  16. Matharu MS, Zrinzo L. Deep brain stimulation in cluster headache: hypothalamus or midbrain tegmentum? Curr Pain Headache Rep. 2010;14(2):151–9. https://doi.org/10.1007/s11916-010-0099-5.

    Article  PubMed  Google Scholar 

  17. Leone M, Proietti CA. Short-lasting unilateral neuralgiform headache attacks: where is the headache generator? Brain. 2016;139(Pt 10):2578–80. https://doi.org/10.1093/brain/aww233.

    Article  PubMed  Google Scholar 

  18. 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(Pt 10):2746–60. https://doi.org/10.1093/brain/awl202.

    Article  PubMed  Google Scholar 

  19. De Benedittis G. SUNCT syndrome associated with cavernous angioma of the brain stem. Cephalalgia. 1996;16(7):503–6. https://doi.org/10.1046/j.1468-2982.1996.1607503.x.

    Article  PubMed  Google Scholar 

  20. Koseoglu E, Karaman Y, Kucuk S, Arman F. SUNCT syndrome associated with compression of trigeminal nerve. Cephalalgia. 2005;25(6):473–5. https://doi.org/10.1111/j.1468-2982.2005.00875.x.

    Article  PubMed  CAS  Google Scholar 

  21. Lambru G, Trimboli M, Tan SV, Al-Kaisy A. Medullary infarction causing coexistent SUNCT and trigeminal neuralgia. Cephalalgia. 2017;37(5):486–90. https://doi.org/10.1177/0333102416652093.

    Article  PubMed  Google Scholar 

  22. Jin D, Lian YJ, Zhang HF. Secondary SUNCT syndrome caused by dorsolateral medullary infarction. J Headache Pain. 2016;17:12. https://doi.org/10.1186/s10194-016-0604-2.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Martins IP, Viana P, Lobo PP. Familial SUNCT in mother and son. Cephalalgia. 2016;36(10):993–7. https://doi.org/10.1177/0333102415616879.

    Article  PubMed  Google Scholar 

  24. Gantenbein AR, Goadsby PJ. Familial SUNCT. Cephalalgia. 2005;25(6):457–9. https://doi.org/10.1111/j.1468-2982.2005.00874.x.

    Article  PubMed  CAS  Google Scholar 

  25. Cohen AS, Matharu MS, Goadsby PJ. Paroxysmal hemicrania in a family. Cephalalgia. 2006;26(4):486–8. https://doi.org/10.1111/j.1468-2982.2006.01045.x.

    Article  PubMed  CAS  Google Scholar 

  26. Zabalza RJ. Sustained response to botulinum toxin in SUNCT syndrome. Cephalalgia. 2012;32(11):869–72. https://doi.org/10.1177/0333102412452045.

    Article  PubMed  Google Scholar 

  27. Favoni V, Grimaldi D, Pierangeli G, Cortelli P, Cevoli S. SUNCT/SUNA and neurovascular compression: new cases and critical literature review. Cephalalgia. 2013;33(16):1337–48. https://doi.org/10.1177/0333102413494273.

    Article  PubMed  Google Scholar 

  28. Kitahara I, Fukuda A, Imamura Y, Ikawa M, Yokochi T. Pathogenesis, surgical treatment, and cure for SUNCT syndrome. World Neurosurg. 2015;84(4):1080–3. https://doi.org/10.1016/j.wneu.2015.05.024.

    Article  PubMed  Google Scholar 

  29. Kovac S, Wright MA, Eriksson SH, Zrinzo L, Matharu M, Walker MC. The effect of posterior hypothalamus region deep brain stimulation on sleep. Cephalalgia. 2014;34(3):219–23. https://doi.org/10.1177/0333102413505241.

    Article  PubMed  Google Scholar 

  30. Leone M, Franzini A, D'Andrea G, Broggi G, Casucci G, Bussone G. Deep brain stimulation to relieve drug-resistant SUNCT. Ann Neurol. 2005;57(6):924–7. https://doi.org/10.1002/ana.20507.

    Article  PubMed  Google Scholar 

  31. 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(2):279–81. https://doi.org/10.3171/2008.4.17493.

    Article  PubMed  Google Scholar 

  32. Bartsch T, Falk D, Knudsen K, Reese R, Raethjen J, Mehdorn HM, et al. Deep brain stimulation of the posterior hypothalamic area in intractable short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). Cephalalgia. 2011;31(13):1405–8. https://doi.org/10.1177/0333102411409070.

    Article  PubMed  CAS  Google Scholar 

  33. May A, Bahra A, Buchel C, Turner R, Goadsby PJ. Functional magnetic resonance imaging in spontaneous attacks of SUNCT: short-lasting neuralgiform headache with conjunctival injection and tearing. Ann Neurol. 1999;46(5):791–4.

    Article  PubMed  CAS  Google Scholar 

  34. Lambru G, Trimboli M, Palmisani S, Smith T, Al-Kaisy A. Safety and efficacy of cervical 10 kHz spinal cord stimulation in chronic refractory primary headaches: a retrospective case series. J Headache Pain. 2016;17(1):66. https://doi.org/10.1186/s10194-016-0657-2.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Mayo Clinic Arizona, 13400 E Shea Blvd, Scottsdale, AZ, 85259, USA

    Karissa N. Arca & Rashmi B. Halker Singh

Authors
  1. Karissa N. Arca
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rashmi B. Halker Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Karissa N. Arca.

Ethics declarations

Conflict of Interest

Karissa N. Arca and Rashmi B. Halker Singh declare no conflict of interest.

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.

Additional information

This article is part of the Topical Collection on Uncommon and/or Unusual Headaches and Syndromes

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Arca, K.N., Halker Singh, R.B. SUNCT and SUNA: an Update and Review. Curr Pain Headache Rep 22, 56 (2018). https://doi.org/10.1007/s11916-018-0707-3

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11916-018-0707-3

Keywords

Search

Navigation