Arens Taga1,2, Gian Camillo Manzoni3, Marco Russo2, Maria Vittoria Paglia2, Paola Torelli2. 1. Department of Neurology, Johns Hopkins University, Baltimore, MD, USA. 2. Dipartimento di Medicina e Chirurgia, Centro Cefalee, Università degli Studi di Parma, Parma, Italy. 3. Poliambulatorio Dalla Rosa Prati, Centro Diagnostico Europeo, Parma, Italy.
Abstract
BACKGROUND/ OBJECTIVES: Puberty seems to be a turning point in cluster headache (CH) onset. To verify its influence on CH phenotype, we focused on cases with onset ≤13 years. A review of the literature follows. METHOD: We considered CH cases with age-of-onset ≤13 years evaluated at our center between 1975 and 2015; these cases were matched by sex to two consecutive patients with age-of-onset as close as possible (±2 years) to the median age-of-onset of the overall CH population. RESULTS: Of the overall 808 cases (585 men and 223 women, M:F ratio = 2.6), 38 patients (20 men and 18 women, M:F ratio = 1.1) had pediatric onset (PO). The diagnostic delay was significantly higher among cases with PO (21.2 ± 12.4 years, P < .0001). In this group, females had more frequently a chronic course and a familiarity for CH. Men with PO had some significant distinctive features, including higher frequency and longer duration of headache attacks, and higher proportion of various cranial autonomic and migraine-like symptoms. CONCLUSIONS: We confirmed that CH with childhood onset does not show a male predominance, which was actually inverted for chronic cases. Furthermore, males with PO seem to have a specific clinical phenotype.
BACKGROUND/ OBJECTIVES: Puberty seems to be a turning point in cluster headache (CH) onset. To verify its influence on CH phenotype, we focused on cases with onset ≤13 years. A review of the literature follows. METHOD: We considered CH cases with age-of-onset ≤13 years evaluated at our center between 1975 and 2015; these cases were matched by sex to two consecutive patients with age-of-onset as close as possible (±2 years) to the median age-of-onset of the overall CH population. RESULTS: Of the overall 808 cases (585 men and 223 women, M:F ratio = 2.6), 38 patients (20 men and 18 women, M:F ratio = 1.1) had pediatric onset (PO). The diagnostic delay was significantly higher among cases with PO (21.2 ± 12.4 years, P < .0001). In this group, females had more frequently a chronic course and a familiarity for CH. Men with PO had some significant distinctive features, including higher frequency and longer duration of headache attacks, and higher proportion of various cranial autonomic and migraine-like symptoms. CONCLUSIONS: We confirmed that CH with childhood onset does not show a male predominance, which was actually inverted for chronic cases. Furthermore, males with PO seem to have a specific clinical phenotype.