EFNS guidelines on the treatment of cluster headache and other trigeminal‐autonomic cephalalgias

Abstract

Cluster headache and the other trigeminal-autonomic cephalalgias [paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome] are rare but very disabling conditions with a major impact on the patient's quality of life. The objective of this study was to give evidence-based recommendations for the treatment of these headache disorders based on a literature search and consensus amongst a panel of experts. All available medical reference systems were screened for any kind of studies on cluster headache, paroxysmal hemicrania and SUNCT syndrome. The findings in these studies were evaluated according to the recommendations of the European Federation of Neurological Societies resulting in level A, B or C recommendations and good practice points. For the acute treatment of cluster headache attacks, oxygen (100%) with a flow of at least 7 l/min over 15 min and 6 mg subcutaneous sumatriptan are drugs of first choice. Prophylaxis of cluster headache should be performed with verapamil at a daily dose of at least 240 mg (maximum dose depends on efficacy or tolerability). Although no class I or II trials are available, steroids are clearly effective in cluster headache. Therefore, the use of at least 100 mg methylprednisone (or equivalent corticosteroid) given orally or at up to 500 mg i.v. per day over 5 days (then tapering down) is recommended. Methysergide, lithium and topiramate are recommended as alternative treatments. Surgical procedures, although in part promising, require further scientific evaluation. For paroxysmal hemicranias, indomethacin at a daily dose of up to 225 mg is the drug of choice. For treatment of SUNCT syndrome, large series suggest that lamotrigine is the most effective preventive agent, with topiramate and gabapentin also being useful. Intravenous lidocaine may also be helpful as an acute therapy when patients are extremely distressed and disabled by frequent attacks. ObjectivesThese guidelines give evidence-based recommendations for the treatment of cluster headache, paroxysmal hemicranias and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. A brief clinical description of the headache disorders is included. The headache disorders are defined based on the diagnostic criteria of the International Headache Society (IHS) [1].

Non–Invasive Vagus Nerve Stimulation for the ACute Treatment of Cluster Headache: Findings From the Randomized, Double‐Blind, Sham‐Controlled ACT1 Study

“…According to evidence‐based recommendations, the primary symptomatic treatments for CH attacks are subcutaneous sumatriptan and inhaled oxygen 7, 8. Alternative acute CH treatments include intranasal triptans and intravenous dihydroergotamine (DHE) 7, 8, 9.…”

“…Alternative acute CH treatments include intranasal triptans and intravenous dihydroergotamine (DHE) 7, 8, 9. Only subcutaneous sumatriptan and intravenous DHE are approved in the United States for the acute pharmacologic treatment of CH 10, 11.…”

General Issues Arising from the Use of Drugs for Headache Disorders

“…Even when a correct diagnosis has been made, undertreatment and mismanagement have been observed despite the existence of guidelines for clinical practice (May et al 2006;Steiner et al 2007;Evers et al 2009). Patients may receive acute treatment, but no prevention (when indicated) and vice versa.…”

“…There was a clear underuse of migraine prevention in the American Migraine Prevalence and Prevention study (Diamond et al 2007). A significant fraction of cluster headache patients never have had access to SC sumatriptan or high-flow oxygen, both first line acute treatments according to current recommendations (May et al 2006;Van Alboom et al 2009). Cluster headache patients may receive ineffective prophylactic drugs such as propranolol, carbamazepine or amitriptyline (Van Alboom et al 2009).…”

Deep Brain Stimulation in Trigeminal Autonomic Cephalalgias

“…Agitation, insomnia, and even frank psychosis may also be effects of chronic steroid use. 29 For these reasons, we have proposed that patients be designated as completely drug-resistant and considered for DBS only when all indicated drugs in the guidelines 29 or in the wider literature have been tried (unless specifically contraindicated in a given case) at adequate dose and for a sufficient period but have nonetheless proved to be ineffective or associated with intolerable adverse effects. 24,26 Outcome measures for surgical purposes…”