Onset of cluster headache triggered by emotional effect: a case report

Cluster headache is a strictly unilateral headache that occurs in association with autonomic symptoms. Stress is a recognised precipitant of migraines, but not of cluster attacks. We describe the case of a patient having migraine for years, in whom extreme emotional stress triggered cluster headache attacks.

Background

Neuroimaging has contributed considerably to the knowledge about the neurobiology of cluster headache. Activation in the region of the posterior hypothalamus is observed,¹ coinciding with subtle structural abnormalities in the same region.²

Genetic studies indicate an autosomal dominant inheritance with low penetrance and an increase in risk of 4–18 times for cluster headache in first‐degree relatives when compared with the general population.³

Although the triggers for individual episodes are well recognised, the most important ones being nitroglycerin,⁴ alcohol,⁵ increased body heat and exertion,⁶ this is not the case for the triggers for bouts.

Case report

A man, about 50 years old, had been treated by us for a decade. He had experienced migraine without aura from his early teens. He had a positive family history of headache, with both his parents and two siblings having migraine. A typical migraine episode consists of pain that is mostly unilateral but with the side changing, periorbital or temporal, and sometimes bifrontal, accompanied by nausea, phonophobia, photophobia and often osmophobia. Our patient never had any autonomic signs accompanying his migraine episodes. The headache would typically be severe, with a throbbing character and exacerbated by movement. The episode lasted from 1 hour, when treated, to several days and prevented him from carrying out his daily activities.

The episodes responded best to subcutaneous sumatriptan 6 mg and only partially to combined over‐the‐counter painkillers or non‐steroidal anti‐inflammatory drugs. The frequency of episodes varied between twice a month and twice weekly over the 10 years of review. Overuse of drugs for acute migraine had been a recurring problem and attempts to reduce the number of days of taking drugs for acute headache on an outpatient basis proved successful and were followed by periods of prophylactic treatment. During phases of increased frequency of episodes and owing to overuse of drugs for acute headache, the headache had less features and was less severe, but returned to its original characteristics and frequency after withdrawal of treatment. In the past, the patient had used several β‐adrenoreceptor blockers and tricyclic antidepressants, as well as valproate and gabapentin, to prevent migraine.

The patient is a non‐smoker and does not drink alcohol. A few years ago, he had severe mouth ulcers and was examined to exclude possible Behçet disease. He has hypercholesterolaemia and arterial hypertension, which is treated with pravastatin (20 mg/day) and ramipril (10 mg/day). He is an academic and has held several responsible positions. After 10 years of marriage, he had gone through a difficult divorce and subsequently struggled to maintain contact with his three children. His private life has become quieter only in the past few years. He had remarried and his 21‐year‐old son had been living in his house for a few years.

He came to see us via an emergency appointment in autumn 2004, because of a new, previously unknown type of headache that had started 2 weeks before the appointment. At this time, he was not taking any migraine prophylactics, had not changed his regular drugs and did not use any psychotropic substance, including illicit drugs.

“Totally unexpectedly”, his son had announced that he was moving back in with his mother, the patient's ex‐wife. The patient described this as the worst moment of the past years, of having had the feeling that “everything is taken away”; he was tearful while describing it. He said, “... during the afternoon I tried to rationalize this ... but it did not work and I had to cry several times”. In the early evening of the same day, after an afternoon of intense worrying, he started to have a type of headache, which he had not had previously. The new headache occurred regularly thereafter, with a frequency of one to two attacks every 24 h, mostly occurring during the night and waking him up. This pain, starting abruptly, peaked in minutes, was strictly left temporoparietal and was accompanied by lacrimation and conjunctival injection. Whereas the previous headaches were worsened by movement and he preferred to lie down and rest, the new headaches were not exacerbated by movement, and he was restless and paced around. The intensity of the new episodes was higher than those previously experienced, with 10 out of 10 on a verbal rating scale. The pain was stabbing, not pulsating. As the pain was so unbearably severe, he always treated it with subcutaneous sumatriptan 6 mg, with resolution in 15–30 min. A magnetic resonance image with gadolinium showed no abnormality and particularly no hypothalamic or cavernous sinus or pituitary lesions.

Cluster headache was diagnosed, and we started him on a course of steroids with prednisolone 60 mg over 5 days, tapering it down over 5 days. We introduced methysergide, which was increased to 5 mg/day, when no further headaches occurred. Eventually the dose was titrated down to 2 mg/day, with control of the headache and bearable, mild side effects. When the dose of methysergide was reduced to below 2 mg after 1 and 4 months, the new type of headache recurred twice daily, and was controlled when the patient increased the methysergide back to 2 mg daily. During the 2 weeks between the onset of the new headache and the appointment, and while he was on methysergide, no episodes of migraine occurred.

Discussion

This patient presented with a new type of headache fulfilling the International Headache Society criteria⁷ for cluster headache after having been treated in our specialised headache clinic for migraine without aura for 10 years and headache caused by the overuse of drugs. A careful study of the clinical notes and correspondence over the past 10 years did not suggest any previous occurrence of cluster headache.

Several cases of cluster headache triggered by, or secondary to, lesions localised in the trigeminal territory, such as those caused by head injury,⁸ dental extraction,⁹ impacted superior wisdom tooth¹⁰ and ocular enucleation,¹¹ have been published.

Emotional triggers are well recognised for cardiac death,¹² stroke¹³ and also for migraine.¹⁴ For cluster headache, the only reported emotional trigger for a first‐time cluster headache is orgasm.¹⁵ The emotional stress temporally associated with the onset of cluster headaches was extreme in our patient and he became tearful, even when talking about it 4 months later.

Cluster headache is very rare, with a prevalence of about 0.2%,³ and an incidence of 1.6 per million people per person‐year.¹⁶ Against this background, we propose a causal relationship between the extreme emotional stress in our patient and the onset of his cluster headaches. Clinical or imaging hints for a secondary origin of the new type of headache were absent.

In conclusion, we describe a patient who had migraine for years and in whom extreme emotional stress triggered a new type of headache. Placebo effects in the treatment of cluster headache are mediated by information and induce neurobiological changes. In a recent review, it was described as comparable in magnitude to migraine.¹⁷ Emotional stress as a trigger, also mediated by information, may be another example that the heritage in research from Descartes, of trying to conceptually separate the brain from the results of its function, is outdated.