More than just a few good men: The males-only myth of cluster headache
If you’re a woman who lives with the burden of severe headaches, or if you care about a woman who does, you know the incredible burden it places on daily living. The pain is debilitating and can impact virtually every aspect of your life.
But what can significantly add to the burden is if your struggle is not just with your condition but with the diagnosis you’ve received, or maybe the diagnosis you’ve not received. It’s possible the doctors you’ve seen haven’t been able to diagnose your condition—they know you’re having headaches, but they haven’t been able to pinpoint what type. Or maybe you’ve received a diagnosis, but the treatment you’ve been prescribed does not seem effective. You’re wondering if the diagnosis you received is not on target and if you should see a specialist.
For some women, an obstacle to quickly getting the right diagnosis could have something to do with your gender.
Cluster headache and its traditional “gender identity”
Cluster headache, which occurs in clusters of attacks, is one of the most severe, painful and disabling types of headache. It’s characterized by intense stabbing pain around one eye accompanied by tearing or redness, nasal congestion or runny nose, swelling or drooping of the eyelid, and all on the same side of the face, which is why it’s known as a unilateral or one-sided headache.
While cluster headache is rare, affecting just one in 1,000 adults, it’s described as being 10 out of 10 in severity,1 and repeated cluster attacks leave many sufferers unable to fully participate in life, work, or activities with family and friends.
While both men and women can suffer from cluster headache, it has long been regarded by many as a males-only disorder. Traditionally, 8 of 10 cluster headache patients were reported to be male. In fact, across 18 studies published between 1952 and 1983, males represented 84 percent of the more than 3,000 patients involved.2
Misdiagnosis and delays in diagnosis, which are a significant concern in cluster headache, can also contribute to the perception that cluster headache is a male disorder. A study of 351 patients published this year by a team at the University of Copenhagen, Denmark, led by Dr. Nunu Lund, found that more women than men were not typically diagnosed with cluster headache until they were examined in a specialized headache center. Also, women were misdiagnosed or not diagnosed more often than men were in primary care settings and even general neurology clinics. This is not surprising considering the rarity of the disease and how infrequently many frontline healthcare professionals encounter patients with cluster headache.3
Nevertheless, “the failure to diagnose women with cluster headache,” said Dr. Lund, “implies that cluster headache is perceived as a males-only disorder.”
Of course, when a disease is not gender-specific but the weight of statistics strongly identifies a disease with one gender, members of the other gender can often suffer not only from the disease itself but also from delayed or mistaken diagnosis. However, thanks to more recent research looking at the male-to-female ratio, that trend may be changing for women with cluster headache.
A declining gender ratio & the eroding myth
According to a number of studies, the male-to-female ratio of cluster headache patients has been decreasing since the 1960s. Two studies in particular documented this decline, based on the patients’ year of onset for cluster headache.
In one Italian study, Dr. Gian Manzoni at the University of Parma looked at the records of 482 cluster headache patients that had been referred to the university’s Headache Center. He found that the ratio had steadily dropped from 6.2 males for every female before 1960 to 2.1 males per female in the first half of the 1990s.4
Demonstrating a relatively parallel decline, a team led by Dr. Karl Ekbom found in a study of 554 cluster headache patients seen at the three Swedish hospitals that the same ratio had declined from 5.9 males per female before 1960 to 3.1 in the 1990s.2
While the evidence wasn’t conclusive as to whether this change was due to a decrease in males with cluster headache or a rise in the number of females, Manzoni noted that “it seems much more likely it is due to an increase in the number of female sufferers.” And while support of his team’s data for the same conclusion was only slight, Ekbom also mentioned that since the late 1970s, the three Swedish hospitals recorded more diagnosed female patients while at the same time experiencing no significant change in referrals of men with cluster headache.
What’s Driving the Male-Only Myth in Cluster Headache?
Considering the substantial decrease in cluster headache’s male-to-female ratio and weakening of the myth that it’s a males-only disorder, it might be helpful for cluster headache patients—and yet-to-be-diagnosed patients—to understand some potential reasons behind the trend.
Growing awareness of cluster headache
While the issues of misdiagnosis and delays in diagnosing cluster headache are still significant and need to be addressed, cluster headache researchers have suggested that one of the reasons for the declining gender ratio may be related to increasing awareness among physicians of the occurrence of cluster headache in women.3
“During recent decades patients suffering from cluster headaches have been increasingly recognized,” Ekbom said. “The literature on cluster headache has expanded considerably since the 1960s and we have got an increased knowledge of the clinical features, pathophysiology and treatment of the disease.”2
Improving diagnosis/Less misdiagnosis of women patients
While women with cluster headache are often misdiagnosed at a higher rate than men, the misdiagnosis of cluster headache in women happens most often with migraine.2, 7 This tendency is potentially driven by several factors:
- First, it’s well known that migraine is more common in females while, as we’ve already noted, cluster headache is known to be especially common in males.
- Cluster headache also shares a number of symptoms in common with migraine, including nausea, vomiting, photophobia (light sensitivity), phonophobia (sound sensitivity), and even aura.7
- Another complicating factor is that cluster headache was regarded as a variant of migraine for many years.2
Clearly, misdiagnosing female cluster headache patients with migraine falsely lowers the prevalence rates for cluster headache in women, which would increase the gender ratio. But in light of the gender ratio’s actual decline in recent decades, the increasing recognition of cluster headache in women, and the growing body of literature on the subject, Ekbom asked whether there may have actually been a change (read “decrease”) in the likelihood of misdiagnosis that has had a positive “impact on detecting afflicted female subjects.”2
In the Swedish study by Ekbom et al,2 noted earlier, and in a study published in 2016 of 808 patients seen at Manzoni’s headache clinic in Italy,8 both noted from their data the impact that hormonal factors may play on the changing male-to-female ratio of cluster headache patients. Manzoni observed that, based on the decade when patients experienced the onset of cluster headache, the preponderance of males developing cluster headache compared to the number of females really occurred during four “in-between” decades (ages 10 to 49), and that before adolescence and after the age of 50, the likelihood that a woman would develop cluster headache was shown to be the same as that of a man.
Ekbom suggested that sex hormone regulation, possibly originating in the hypothalamus, could be influencing these differences in the male-to-female ratio, a conclusion that was mirrored by Manzoni.
Tracking your target, closing in: The reward of accurate diagnosis
Dr. Tim Jurgens of the University Medical Center Rostock, Germany, points out that healthcare providers know from research “that diagnosing cluster headache is easier than diagnosing migraine and the patient’s sex should not prevent us from doing so rapidly.”9
That being the case, women with recurring severe headache who are struggling to get a diagnosis or who suspect the potential of misdiagnosis may want to ask their doctor if they should see a specialist. And considering the high rate of misdiagnosis, this may be even more important to consider if symptoms and/or response to treatment are leading you or someone you care about to suspect the possibility of cluster headache.
For both women and men, and there are treatments available for cluster headache. Faster-acting treatments for acute relief of cluster attacks include various injectables and inhalants, and sometimes, these are even taken in chorus with preventive treatments in an effort to head off attacks upon signs of a potential episode. A recent area of research that is considered promising by headache experts not only for its effectiveness but also because it lacks the side effects of medication10 is neurostimulation, such as the direct stimulation of the SPG nerve bundle (sphenopalatine ganglion), which has been approved for relieving the acute pain of cluster headache in the EU and is under investigation in a clinical study the US.
To find a headache center to further explore potential treatments, please click here.
Enjoyed this article? Don’t forget to share.
- Mitsokostas, et al. Refractory chronic cluster headache: a consensus statement on clinical definition from the European Headache Federation. The Journal of Headache and Pain. 2014; 15:79
- Ekbom, et al. Age at onset and sex ratio in cluster headache: observations over three decades. Cephalalgia 2002;22:94–100.
- Lund, et al. Chronobiology differs between men and women with cluster headache, clinical phenotype does not. Neurology. 2017 Mar 14;88(11):1069-1076.
- Manzoni GC. Male preponderance of cluster headache is progressively decreasing over the years. Headache 1997;37:588–589.
- Voiticovshi-losob, et al. Diagnostic and therapeutic errors in cluster headache: a hospital-based study. The Journal of Headache and Pain 2014, 15:56
- Sánchez del Rio, et al. Errors in Recognition and Management are Still Frequent in Patients with Cluster Headache. Eur Neurol 2014;72:209–212
- Rozen TD, Fishman RS. Cluster headache in the United States of America: demographics, clinical characteristics, triggers, suicidality, and personal burden. Headache 2012;52:99–113.
- Manzoni, et al. Age of onset of episodic and chronic clusterheadache – a review of a large case series from a single headache centre. J Headache Pain. 2016;17:44
- Jurgens TP. Comment: Sex-specific differences in cluster headache: Not a males-only disorder. Neurology. 2017 Mar 14;88(11):1074
- Jürgens, et al. Long-term effectiveness of sphenopalatine ganglion stimulation for cluster headache. Cephalalgia, 2016; 0:1-12.