SUNCT and SUNA: an Update and Review
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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 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.
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.
KeywordsSUNCT SUNA SUNHA Trigeminal autonomic cephalalgia TAC
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.
Papers of particular interest, published recently, have been highlighted as: • Of importance•• Of major importance
- 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
- 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
- 11.Stranding S. Gray’s anatomy. 41st ed. New York: Elsevier; 2016.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. CrossRefPubMedGoogle 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. CrossRefPubMedGoogle 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.CrossRefPubMedGoogle 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. CrossRefPubMedGoogle 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. CrossRefPubMedGoogle 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.CrossRefPubMedPubMedCentralGoogle Scholar