Familial Cluster Headache Research Articles

The clinical characteristics of familial cluster headache.

BackgroundA 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.ObjectivesTo 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.MethodsA 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.ResultsFamilial 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).ConclusionThese 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.

Prevalence of familial cluster headache: a systematic review and meta-analysis

IntroductionThe population rate of familial cluster headache (CH) has been reported to be as high as 20% however this varies considerably across studies. To obtain a true estimate of family history in CH, we conducted a systematic review and meta-analysis of previously published data.MethodsOur systematic review involved a search of electronic databases (Medline, EMBASE, PubMed, CINAHL) to identify and appraise studies of interest utilising the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) guidelines. To further ameliorate the accuracy of our analysis we included an additional unpublished cohort of CH patients recruited at a tertiary referral centre for headache, who underwent detailed family history with diagnostic verification in relatives. Data was extracted and meta-analysis conducted to provide a true estimation of family history.ResultsIn total, we identified 7 studies which fulfilled our inclusion criteria. The estimated true prevalence of CH patients with a positive family history was 6.27% (95% CI:4.65–8.40%) with an overall I2 of 73%. Fitted models for gender subgroups showed higher estimates 9.26% (95% CI: 6.29–13.43%) in females. However the I2 for the female model was 58.42% and significant (p = 0.047).ConclusionOur findings estimate a rate of family history in CH to be approximately 6.27% (95% CI: 4.65–8.40%). While estimates were larger for female probands, we demonstrated high heterogeneity in this subgroup. These findings further support a genetic role in the aetiology of CH.

Familial cluster headache in an Italian case series.

To investigate the familial occurrence of cluster headache (CH) in a series of Italian patients, we focused on possible differences in the mean age of onset between familial and non-familial CH cases. We considered all consecutive patients referred to the Parma Headache Centre between 1975 and 2013 affected by CH; we subsequently reviewed these cases applying the ICHD3-beta criteria (785 probands, 569 men and 216 women). We identified those cases who reported at least a first-degree relative with a diagnosis of CH, which was confirmed by the clinical documentation they provided. Each one of the "familial cases" was matched by sex and age (±2 years) at the first visit to three consecutive CH patients who did not report any first-degree relative affected by CH. A positive family history of CH was found in 40 probands (5.1 %), 28 men (4.9 %), and 12 women (5.6 %). The male:female ratio was 2.3:1 among the 40 CH familial cases, while it was 2.7:1 among all the CH non-familial cases (745 patients). The mean age of onset was significantly (p < 0.01) lower in women with familial CH (28.5 years, SD 17.7 years, range 10-63 years) than in women with non-familial CH (46.7 years, SD 13.7 years, range 11-74 years); we did not find a significant age difference among men (the mean age of onset for the familial cases was 29.6 years, SD 13.6 years, range 6-63 years; while for the non-familial cases, it was 29.3 years, SD 13.2 years, range 13-59 years). Our study suggests that genetic factors may play a role in the female gender causing an earlier age of onset and a lower male-to-female sex ratio in familial cases.

Familial Cluster Headache: Demographic Patterns in Affected and Nonaffected

Smoking has been claimed to be more common in cluster headache (CH) sufferers than in nonaffected subjects. Other demographic information such as handedness, body mass index, eye color, education, occupation, and alcohol use has been described as being different in CH patients compared with a control population. The aim of this study was to get more detailed information in CH patients with a positive family history and their nonaffected relatives, assuming that there would be demographic differences between the groups. Affected CH patients and nonaffected first- and second-degree relatives (n = 114) received a posted questionnaire. Information from 83 subjects (42 affected and 41 first- and/or second-degree relatives) from 23 families was received. P < .05 was chosen to be significant. Parental cigarette smoking during childhood and adolescence of patients and controls and current or former smoking was significantly more common in CH patients. Frequent alcohol intake (2-3 times/week or more) was significantly more common in the affected group of CH patients. There were significant differences as regards the life history of head trauma, but some of the affected had had the trauma after the age of onset of CH. Interestingly, CH patients worked more full-time than nonaffected. Formerly described demographic relationships in CH regarding cigarette smoking, alcohol consumption, and head trauma were also seen in our CH patients and their nonaffected relatives. These findings might represent a gene environment interaction in affected CH patients or it could be personality-lifestyle-related phenomena or a combination of these mechanisms.

Familial Cluster Headache. Is Atypical Cluster Headache in Family Members Part of the Clinical Spectrum?

Familial cluster headache (CH) was analysed in 21 Swedish families. Diagnosis was made according to The International Classification of Headache Disorders 2004. We identified 55 affected, of whom 42 had episodic or chronic CH, one had probable CH and 12 had atypical symptoms. The atypical cases did not fulfil the diagnostic criteria for CH, but had clinical symptoms with more resemblance to CH than to migraine or other trigeminal autonomic cephalgia syndromes. The overall male : female ratio was 1.8:1. The overall mean age at onset was significantly lower in the second/third generation than in the first generation (mean age at onset 22 vs. 31 years, SD +/- 7 vs. 13 years; P < 0.01). This may be anticipation or selection bias, since individuals with late age at onset from the second/third generation may not yet have symptoms. The prevalence of migraine was 24% (13/55), i.e. similar to the prevalence in the general population. The high incidence of atypical CH cases in the Swedish families with other members affected with CH may suggest that the spectrum of CH is broader than previously thought. We suggest that atypical CH in CH families may represent an expanded spectrum of the disease with a common aetiology, i.e. a common genetic background.