Guideline sources The following summarized guidelines for the management of cluster headache are prepared by our editorial team based on guidelines from the American Headache Society (AHS 2016). 1 2 2 2 2 Definition Cluster headache is a primary headache disorder characterized by severe unilateral pain mainly in the first division of the trigeminal nerve accompanied by ipsilateral cranial autonomic symptoms and a sense of agitation and restlessness during attacks. 2 Epidemiology Cluster headache is caused by the activation of trigeminovascular complex and trigeminal- autonomic reflex. 2 Disease course Clinical manifestations include excruciating, unilateral headache over the peri- and retro-orbital regions and the temple accompanied by ipsilateral lacrimation, eye redness, eye discomfort, ptosis, nasal congestion, rhinorrhea, aural fullness, throat swelling, flushing, sense of restlessness, and agitation. Episodic headaches may subsequently become chronic cluster headaches or may remit with age. 2 Prognosis and risk of recurrence Cluster headaches are not associated with increased mortality. 2 https://web.pathway.md/diseases/recthDt7ZOLzG3jsh 1/3 6/23/23, 3:25 AM Cluster headache Pathway Guidelines 1. Medical management 100% oxygen: administer 100% oxygen at flow rates of 6 to 12 L/min to improve headache response. A Abortive agents: sumatriptan is potent in improving headache response. A Preventative agents: suboccipital single injection or injection series with corticosteroids is effective in reducing attack frequency. A Show 3 more Preventative agents with no evidence for benefit: insufficient evidence that frovatriptan is effective in reducing attack frequency. I 2. Therapeutic procedures Sphenopalalatine ganglion stimulation: sphenopalatine ganglion stimulation is effective in improving headache response. B Deep brain stimulation: avoid unilateral hypothalamic deep brain stimulation, as it is not effective in reducing attack frequency. D References 1. Robbins MS, Starling AJ, Pringsheim TM et al. Treatment of Cluster Headache: The American Headache Society Evidence-Based Guidelines. Headache. 2016 Jul;56 7 1093 106. Open 2. Diana Yi-Ting Wei, Jonathan Jia Yuan Ong, and Peter James Goadsby. Cluster Headache: Epidemiology, Pathophysiology, Clinical Features, and Diagnosis. Ann Indian Acad Neurol. 2018 Apr; 21 Suppl 1 S3 S8. Open 3. I. Favier, J. Haan, and M. D. Ferrari. Chronic cluster headache: a review. J Headache Pain. 2005 Feb; 6 1 3 9. Open 4. Diana Yi-Ting Wei, Jonathan Jia Yuan Ong, Peter James Goadsby. Cluster Headache: Epidemiology, Pathophysiology, Clinical Features, and Diagnosis. Ann Indian Acad Neurol. 2018 Apr;21 Suppl 1 S3 S8. Open 5. Fayyaz Ahmed. Headache disorders: differentiating and managing the common subtypes. Br J Pain. 2012 Aug;6 3 124 32. Open 6. Shivang Joshi, Paul Rizzoli, Elizabeth Loder. The comorbidity burden of patients with cluster headache: a population-based study. J Headache Pain. 2017 Dec;18 1 76. Open 7. Zhao Dong, Hai Di, Wei Dai et al. Clinical profile of cluster headaches in China - a clinic-based study. J Headache Pain. 2013 Mar 21;14 1 27. Open https://web.pathway.md/diseases/recthDt7ZOLzG3jsh 2/3 6/23/23, 3:25 AM Cluster headache Pathway 8. Elizabeth Leroux, Anne Ducros. Cluster headache. Orphanet J Rare Dis. 2008 Jul 23;3 20. Open 9. Agneta Snoer, Nunu Lund, Rasmus Beske et al. Pre-attack signs and symptoms in cluster headache: Characteristics and time profile. Cephalalgia. 2018 May;38 6 1128 1137. Open 10. Jong-Hee Sohn, Yun-Ju Choi, Byung-Kun Kim et al. Clinical Features of Probable Cluster Headache: A Prospective, Cross-Sectional Multicenter Study. Front Neurol. 2018 Oct 26;9 908. Open 11. Mi Ji Lee, Soo-Jin Cho, Jeong Wook Park et al. Increased suicidality in patients with cluster headache. Cephalalgia. 2019 Sep;39 10 1249 1256. Open 12. Rubesh Gooriah, Alina Buture, Fayyaz Ahmed. Evidence-based treatments for cluster headache. Ther Clin Risk Manag. 2015 Nov 9;11 1687 96. Open 13. William S Kingston, David W Dodick. Treatment of Cluster Headache. Ann Indian Acad Neurol. 2018 Apr;21 Suppl 1 S9 S15. Open 14. Srdjan Ljubisavljevic, Jasna Zidverc Trajkovic. Cluster headache: pathophysiology, diagnosis and treatment. J Neurol. 2019 May;266 5 1059 1066. Open 15. Sharon A. Kandel, Pujyitha Mandiga. Cluster Headache. In: StatPearls Internet]. Treasure Island FL StatPearls Publishing; 2021 Jan. 2020 Jun 30. Open https://web.pathway.md/diseases/recthDt7ZOLzG3jsh 3/3