Advertisement

SUNCT and SUNA: an Update and Review

  • Karissa N. Arca
  • Rashmi B. Halker Singh
Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
  • 84 Downloads
Part of the following topical collections:
  1. Topical Collection on Uncommon and/or Unusual Headaches and Syndromes

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.

Keywords

SUNCT SUNA SUNHA Trigeminal autonomic cephalalgia TAC 

Notes

Compliance with Ethical Standards

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.

References

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

  1. 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. CrossRefGoogle Scholar
  2. 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.CrossRefPubMedGoogle Scholar
  3. 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. CrossRefPubMedGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 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.CrossRefPubMedGoogle Scholar
  6. 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. CrossRefPubMedPubMedCentralGoogle Scholar
  7. 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.CrossRefPubMedGoogle Scholar
  8. 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. CrossRefPubMedGoogle Scholar
  9. 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.CrossRefPubMedGoogle Scholar
  10. 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. CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Stranding S. Gray’s anatomy. 41st ed. New York: Elsevier; 2016.Google Scholar
  12. 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.CrossRefPubMedGoogle Scholar
  13. 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. CrossRefPubMedGoogle Scholar
  14. 14.
    Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol. 2002;1(4):251–7.CrossRefPubMedGoogle Scholar
  15. 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. CrossRefPubMedGoogle Scholar
  16. 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.CrossRefPubMedGoogle Scholar
  17. 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.CrossRefPubMedGoogle Scholar
  18. 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.CrossRefPubMedGoogle Scholar
  19. 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. CrossRefPubMedGoogle Scholar
  20. 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. CrossRefPubMedGoogle Scholar
  21. 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. CrossRefPubMedGoogle Scholar
  22. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 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. CrossRefPubMedGoogle Scholar
  24. 24.
    Gantenbein AR, Goadsby PJ. Familial SUNCT. Cephalalgia. 2005;25(6):457–9.  https://doi.org/10.1111/j.1468-2982.2005.00874.x.CrossRefPubMedGoogle Scholar
  25. 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. CrossRefPubMedGoogle Scholar
  26. 26.
    Zabalza RJ. Sustained response to botulinum toxin in SUNCT syndrome. Cephalalgia. 2012;32(11):869–72.  https://doi.org/10.1177/0333102412452045. CrossRefPubMedGoogle Scholar
  27. 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. CrossRefPubMedGoogle Scholar
  28. 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.CrossRefPubMedGoogle Scholar
  29. 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. CrossRefPubMedGoogle Scholar
  30. 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.CrossRefPubMedGoogle Scholar
  31. 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.CrossRefPubMedGoogle Scholar
  32. 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. CrossRefPubMedGoogle Scholar
  33. 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.CrossRefPubMedGoogle Scholar
  34. 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.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Mayo Clinic ArizonaScottsdaleUSA

Personalised recommendations