The clinical characteristics of familial cluster headache

Cephalalgia. 2022 Jul;42(8):715-721. doi: 10.1177/03331024221076478. Epub 2022 Feb 15.


Background: A positive family history predisposes to the development of cluster headache. The distinct characteristics of familial cluster headache have yet to be confirmed, however, evidence suggests a younger age of onset and higher proportion of females in this subgroup.

Objectives: To assess the rate and mode of inheritance of familial cluster headache in a tertiary referral centre for headache. To describe the clinical features of familial cluster headache.

Methods: A retrospective study conducted between 2007 and 2017. Cluster headache was confirmed in probands and affected relatives. Differences in demographics, clinical characteristics, and response-to-treatment in familial cluster headache were delineated through multivariate analysis using a control cohort of 597 patients with sporadic cluster headache.

Results: Familial cluster headache was confirmed in 48 (7.44%) patients and predominantly reflected an autosomal dominant mode of inheritance with reduced penetrance. Familial cases were more likely to report nasal blockage (OR 4.06, 95% CI; 2.600-6.494, p < 0.001) during an attack and a higher rate of concurrent short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (OR 3.76, 95% CI; 1.572-9.953, p = 0.004).

Conclusion: These findings add to evidence suggesting a genetic component to cluster headache. Here, we demonstrated prominent nasal blockage, and a higher occurrence of concomitant short-lasting unilateral neuralgiform headache with conjunctival injection and tearing in this subgroup, further delineating the phenotype.

Keywords: Cluster headache; familial; family history; genetic.

MeSH terms

  • Cluster Headache* / complications
  • Cluster Headache* / epidemiology
  • Cluster Headache* / genetics
  • Female
  • Headache / complications
  • Humans
  • Nasal Obstruction* / complications
  • Neuralgia*
  • Retrospective Studies

Supplementary concepts

  • Cluster Headache, Familial