Cluster headaches are one of a group of trigeminal autonomic cephalalgias, also including SUNCT and paroxysmal hemicrania. Diagnostic criteria are:
- At least 5 attacks
- Attacks of severe unilateral orbital, supraorbital, or temporal pain, lasting between 15 minutes and 3 hours (however, can rarely last longer).
- At least one associated feature, which can be any of:
- Ipsilateral lacrimation
- Ipsilateral conjunctival injection
- Ipsilateral miosis
- Ipsilateral ptosis
- Ipsilateral eyelid oedema
- Ipsilateral forehead/facial sweating
- Ipsilateral nasal congestion or rhinitis
- Restlessness/agitation. (This occurs in 80% of patients and can be useful in distinguishing from migraine.)
- Attacks have a frequency between one every other day and eight daily.
- Not attributed to another disorder.
Cluster headache can be episodic (periods lasting a week to a year, with at least a month off in between), or chronic (continuing for more than a year with no remission or remissions lasting <1 month).
Frequency and duration of attacks distinguish it from a diagnosis of paroxysmal hemicrania or SUNCT, but the table given suggests some overlap with paroxysmal hemicrania – the article didn’t go into details about this but it looked as though it would be worth considering hemicrania if attacks are <30 min and occur > 5 x daily more than half the time (and at least daily).
Pain rises to maximal intensity within a few minutes and stays there for the duration of the attack, possibly with some slight waxing and waning (or with super-intense stabs of pain). Will often end as abruptly as it started. Nausea, vomiting, photophobia, phonophobia, or olfactophobia (I don’t know whether that last is a word or not) are all fairly common. Some patients get auras within the preceeding hour. Patients can have tenderness and allodynia at the site between attacks.
During clusters, red wine (and to a lesser extent other alcohols), sleep, or noxious odours can precipitate attacks. So can nitrates or sildenafil.
Cluster headache can be secondary to pituitary tumours, carotid dissections, cavernous sinus pathology, and other structural lesions, so it can be worth arranging MRI of the brain and carotids.
Oxygen treatment should be 12l/min for at least 15 minutes. The OUCH-UK site very usefully has a downloadable HOOF form already filled in with correct figures. Nasal or injectable sumatriptan 6 mg often helps, and can be used more often than in migraine – apparently rebound/tachyphylaxis are the limiting features in migraine, but this is less likely to be a problem in patients with cluster headache, who can inject sumatriptan twice daily on a long-term basis.
Prednisolone dose is 1 mg/kg up to 60 mg, for five days, then tapered by 10 mg every three days. Start a prophylactic, usually verapamil, at the same time. Verapamil dose starts at 80 mg tds and is increased by 80 mg fortnightly (unfortunately, the article didn’t clarify whether this is total dose or each dose), aiming for a total dose of between 480 and 960 mg per day. The reason for this slow escalation is the high incidence of heart block with verapamil, and therefore ECG should be performed as a baseline prior to treatment and before each dose increase,with particular attention to the PR interval.
Other possible prophylactic treatments include lithium (generally of less use and requires usual monitoring), melatonin, topiramate, sodium valproate, pizotifen, gabapentin, LA/steroid injection over greater occipital nerve, and methysergide (serious fibrotic SEs and only used under specialist supervision.
Do not bother with opiates. They don’t help.
(From the BMJ 14th April 2012)
Following this article, I had to review a woman with cluster headache, and was able to give her appropriate treatment.