Cluster headache (CH) is one of the worst primary headaches that remain underdiagnosed and inappropriately treated. There are recent advances in the understanding of this disease and available treatments. This paper aims to review CH's recent clinical and pathophysiological findings, diagnosis, and treatment. We performed a narrative literature review on the socio-demographics, clinical presentations, pathophysiological findings, and diagnosis and treatment of CH. CH affects 0.1% of the population with an incidence of 2.07-9.8/100,00 person-years-habitants, a mean prevalence of 53/100,000 inhabitants (3-150/100,000 inhabitants). The male-to-female ratio remains inconclusive, as the ratio of 4.3:1 has recently been modified to 1.3-2.6, possibly due to previous misdiagnosis in women. Episodic presentation is the most frequent (80%). It is a polygenetic and multifactorial entity that involves dysfunction of the trigeminovascular system, the trigeminal autonomic reflex, and the hypothalamic networks. An MRI of the brain is mandatory to exclude secondary etiologies. There are effective and safe pharmacological treatments oxygen, sphenopalatine, and great occipital nerve block, with the heterogeneity of clinical trial designs for patients with CH divided into acute, transitional, or bridge treatment (prednisone) and preventive interventions. In conclusion, CH remains underdiagnosed, mainly due to a lack of awareness within the medical community, frequently causing a long delay in reaching a final diagnosis. Recent advances in understanding the principal risk factors and underlying pathophysiology exist. There are new therapeutic possibilities that are effective for CH. Indeed, a better understanding of this challenging pathology will continue to be a subject of research, study, and discoveries in its diagnostic and therapeutic approach.
Competing Interests: DS-J received honoraria for consulting activities, serving on advisory boards, and as a speaker from Pfizer. KV-J received honoraria from Allergan-AbbVie, Pfizer, Eli Lilly, Abbot, and Carnot de México. JH received honoraria for consulting activities and serving on advisory boards and as a speaker from Allergan, Abbvie, Autonomic Technologies Inc., Cannovex BV, Chordate Medical AB, Eli Lilly, Hormosan Pharma, Lundbeck, MD-Horizonte, Novartis, Pfizer, Sanofi, and Teva. He received personal fees for Medico-Legal work and from NEJM Journal Watch, Oxford University Press, Quintessence Publishing, Sage Publishing, and Springer Healthcare. He holds stock options from Chordate Medical AB. He also reports a research grant from Bristol Myers Squibb. JH serves as Associate Editor for Cephalalgia, Cephalalgia Reports, Journal of Headache and Facial Pain as well as for Frontiers in Pain Research. He is an elected member of the Board of Trustees of the International Headache Society (IHS). He serves as a Council Member and Treasurer of the British Association for the Study of Headache (BASH). None of the reported activities is directly related to the submitted work. AM-M received honoraria for consulting activities, serving on advisory boards, and as a speaker from Pfizer. IR-L received honoraria for consulting activities, serving on advisory boards, and as a speaker from Allergan-AbbVie, Pfizer, Eli Lilly, and Carnot de México. SG received honoraria for consulting activities, serving on advisory boards, and as a speaker from Eli Lilly. JN-K received honoraria for consulting activities, serving on advisory boards, and as a speaker from Eli Lilly and Pfizer. EC received honoraria for consulting activities and serving on advisory boards and as a speaker from Novartis, Sanofi, Ferrer, Pfizer, Boehringer-Ingelheim, Merk, Silanes, ApoPharma, Asofarma, MG-C received honoraria for consulting activities, serving on advisory boards, and as a speaker from Allergan-AbbVie, Grünenthal, Eli Lilly, and Pfizer. HJ-J received honoraria for consulting activities, serving on advisory boards, and as a speaker from Allergan-AbbVie. MM-G received honoraria for consulting activities, serving on advisory boards, and as a speaker from Pfizer. AM received honoraria for consulting activities, serving on advisory boards, and as a speaker from Pfizer and Eli Lilly. RD-V received honoraria for consulting activities, serving on advisory boards, and as a speaker from Pfizer. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.
(© 2024 San-Juan, Velez-Jimenez, Hoffmann, Martínez-Mayorga, Melo-Carrillo, Rodríguez-Leyva, García, Collado-Ortiz, Chiquete, Gudiño-Castelazo, Juárez-Jimenez, Martínez-Gurrola, Marfil, Nader-Kawachi, Uribe-Jaimes, Darío-Vargas and Villareal-Careaga.)