The levels of evidence (class I-IV) supporting the recommendations and ratings of recommendations (A-C, good practice point) are defined at the end of the "Major Recommendations" field.
Table 5. Treatment Recommendations for Cluster Headache, Paroxysmal Hemicrania and Short-lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing (SUNCT) Syndrome
| |
Treatment of Choice |
| Therapy |
Cluster Headache |
Paroxysmal Hemicrania |
SUNCT Syndrome |
| Acute |
100% oxygen, 15 l/min (A)
Sumatriptan 6 mg, subcutaneous (A)
Sumatriptan 20 mg nasal (A)
Zolmitriptan 5 mg nasal (A/B)
Zolmitriptan 10 mg nasal (A/B)
Zolmitriptan 10 mg oral (B)
Zolmitriptan 5 mg oral (B)
Lidocaine intranasal (B)
Octreotide (B)
|
None |
None |
| Preventative |
Verapamil (A)
Steroids (A)
Lithium carbonate (B)
Methysergide (B)
Topiramate (B)
Ergotamine tartrate (B)
Valproic acid (C)
Melatonin (C)
Baclofen (C)
|
Indomethacin (A)
Verapamil (C)
Non-steroidal anti-inflammatory drugs (NSAIDs) (C)
|
Lamotrigine (C) |
For exact doses see original guideline document (A denotes effective, B denotes probably effective, C denotes possibly effective).
Treatment of Cluster Headache
Level A Recommendation
The first option for the treatment of acute attacks of cluster headache should be the inhalation of 100% oxygen with at least 7 l/min over 15 min (class II trials) or with the subcutaneous injection of 6 mg sumatriptan (class I trials). An alternative would be sumatriptan 20 mg nasal spray or zolmitriptan 5 mg nasal spray (one class I trial each), with the disadvantage of a slower onset and the advantage of being able to treat more attacks in 24 hours than with injected sumatriptan.
Prophylaxis of cluster headache should be tried first with verapamil at a daily dose of at least 240 mg (maximum dose depends on efficacy or tolerability, electrocardiogram [ECG] controls are obligatory with increasing doses). Although no class I or II trials are available, steroids are clearly effective for treating cluster headache. Therefore, the use of at least 100 mg methylprednisone (or equivalent corticosteroid) given orally or up to 500 mg intravenously (i.v.) per day over 5 days (then tapering down) is recommended.
Level B Recommendation
Intranasal lidocaine (4%) and subcutaneous octreotide (100 micrograms) can be tried for treating acute cluster headache attacks if level A medication is ineffective or contraindicated. Oral administration of zolmitriptan at 5 to 10 mg is effective in some patients (class I trial) but high doses produce more side effects and limit practical use.
Methysergide and lithium are drugs of second choice if verapamil is ineffective or contraindicated. Corticosteroids can be used for short periods where bouts are short or to help establish another medication. Topiramate is promising, but only open trials exist at this point. Melatonin is useful in some patients. Except for lithium, the maximum dose depends on efficacy and tolerability. Ergotamine tartrate is recommended for short-term prophylaxis (class III studies). Despite positive class II studies, pizotifen and intranasal capsaicin should only be used in rare cases because of side effects.
Level C Recommendation
Baclofen 15 to 30 mg and valproic acid showed possible efficacy and can be tried as drugs of third choice.
Good Practice Point
Surgical procedures are not indicated in most of the patients with cluster headache. Patients with intractable chronic cluster headache should be referred to centres with expertise in both destructive and neuromodulatory procedures to be offered all reasonable alternatives before a definitive procedure is conducted.
Treatment of Paroxysmal Hemicrania
Paroxysmal hemicrania is to be treated with indomethacin up to 200 mg (level A recommendation). Alternatively, verapamil and other NSAIDs can be tried (level C recommendation).
Treatment of Short-lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing (SUNCT) Syndrome
Recent large case series outcomes suggest that lamotrigine is the treatment of choice in SUNCT, followed by topiramate and gabapentin.
Definitions:
Evidence Classification Scheme for a Therapeutic Intervention
Class I: An adequately powered prospective, randomized, controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:
- Randomization concealment
- Primary outcome(s) is/are clearly defined
- Exclusion/inclusion criteria are clearly defined
- Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
- Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized, controlled trial in a representative population that lacks one criteria a–e
Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment
Class IV: Evidence from uncontrolled studies, case series, case reports, or expert opinion
Rating of Recommendations
Level A rating (established as effective, ineffective, or harmful) requires at least one convincing class I study or at least two consistent, convincing class II studies.
Level B rating (probably effective, ineffective, or harmful) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (possibly effective, ineffective, or harmful) requires at least two convincing class III studies.
Good practice point Where there was a lack of evidence but consensus was clear, the Task Force has stated their opinion as good practice points (GPPs).
