Consensus Criteria for Refractory Chronic Cluster Headache

Pauline Anderson

June 23, 2015

BERLIN, Germany — The European Headache Federation (EHF) has developed a consensus statement on what constitutes the rare but often difficult-to-manage condition of refractory chronic cluster headache (rCCH).

According to the new definition, patients can be determined to have rCCH if they experience at least three severe headache attacks per week that affect quality of life and have not responded to at least three consecutive trials of preventive treatments.

Discussing the statement here at the first Congress of the European Academy of Neurology (EAN), Dimos Mitsikostas, MD, PhD, Neurology Department, Athens Naval Hospital, Greece, stressed that the three or more attacks must be severe enough to affect the patient's quality of life.

He also emphasized that patients need to have not benefitted from preventive treatments at the "maximum tolerated dose."

Cluster headaches, which are recurrent, severe headaches often involving intense pain around the eyes, occur in 1 in 1000 people, with chronic cases in only 0.16%. About 8% of CCH cases are resistant to prophylactic pharmaceutical treatments.

In such cases, it may be necessary to escalate treatments, but this is a decision made individually by the physician together with the patient, said Dr Mitsikostas.

Expert Committee

The consensus statement was developed in three stages. First, an expert committee of the EHF created a draft that was sent for review to all European National Headache Societies. A revised draft was sent to international investigators who had published studies on cluster headaches in the previous 5 years. With their additional comments, a final draft was written.

According to this final consensus, patients not only need to experience at least three headaches a week severe enough to affect quality of life but also must have had no response to consecutive prophylactic treatment trials with at least three agents that have been shown in randomized, controlled studies to be superior to placebo.

Recommended prophylactic treatments include verapamil, lithium, oral or intravenous steroids, greater occipital nerve infiltration, topiramate, methysergide, ergots, civamide, and long-acting triptans. Some of these agents, said Dr Mitsikostas, may not be available in particular jurisdictions.

The final consensus statement, which was published in The Journal of Headache and Pain, "explains the evidence and the strength of the evidence for each drug," said Dr Mitsikostas. "It also makes it clearer that verapamil should be the drug of first choice."

Dr Mitsikostas commented that in his personal practice, his first choice for patients with rCCH is verapamil, but if the highest tolerated dose of that drug doesn't do the trick, his second choice is often occipital nerve infiltration.

Physicians need to monitor patients taking not only verapamil but also lithium and methysergide, said Dr Mitsikostas. He added that to offer greater occipital nerve infiltration, physicians need appropriate clinical experience. "It's a simple technique, but physicians have to have particular expertise."

Drug Combinations

The committee recommended consideration of drug combinations, but at the discretion of the physician, said Dr Mitsikostas. Responding later to a query from a delegate about whether he combined verapamil with a steroid, Dr Mitsikostas said his center does this "sometimes, but not often."

He added that he doesn't often combine drugs except when there are comorbidities, in which case he offers a "soup" of drugs, each targeted to a specific condition.

Session co-chair Rigmor Jensen, MD, PhD, professor, Department of Neurology, University of Copenhagen, Denmark, stressed the need for caution in using steroids long term, given the risks for osteoporosis and other adverse effects. "We should be careful with the steroids and use them only for a short time."

According to Dr Mitsikostas, patients need to try prophylactic drugs at the maximum tolerated dose over a sufficient period. The committee did not specify what the "sufficient" time should be, he said.

"Some members believed that 3 months was enough; some thought 4 or 6 months was enough, so we decided not to define what period of time it should be. It's up to the physician's judgment."

However, he said, "we insist that the maximum tolerated dose of this preventive treatment should be tested; not just the recommended dose, but the maximum tolerated by the patient."

The committee recommended that to rule out symptomatic CCH, physicians should order brain MRI and magnetic resonance angiography. If they're still "not comfortable," they could supplement this with further investigations, including carotid computed tomographic angiography or triplex carotid ultrasonography.

Committee members also suggested that physicians rule out other primary headache conditions, including persistent idiopathic facial pain, short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, cluster-tic syndrome, or paroxysmal hemicranias.

Polysomnography might be a useful test, at least to rule out sleep issues, said Dr Mitsikostas. "If the headaches coexist with obstructive sleep apnea, it makes it very difficult to treat."

He added that medication-overuse headaches only rarely accompany rCCH.

Physicians should manage comorbidities, including such things as depression and "nocebo behaviors," he said. Some patients are so reluctant to try a new drug because of what they're convinced were previous bad experiences that they "continue to suffer," said Dr Mitsikostas.

Dr Mitsikostas has disclosed no relevant financial relationships.

Congress of the European Academy of Neurology (EAN). Abstract 02210. Presented June 21, 2015.

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