A 45-year-old man presents with a severe headache involving his right eye and
orbit. He notes that his headaches usually occur at night and often awaken him from
sleep. At times his eyelid droops (see photo below). He also notes concomitant nasal
congestion and injection of the eye. His headaches have not improved when he has taken
aspirin or nonsteroidal antiinflammatory drugs.
What is your diagnosis, what test(s) would you order to confirm your suspicions, and what treatment, if any, do you recommend?
DIAGNOSIS: Cluster headache (CH). A comparatively rare cause of headache, CHs are
three times more common in men than women and often nocturnal in onset. The pain in
CHs is retroorbital, excruciating in intensity, nonfluctuating, and explosive in onset.
Patients often pace or rock back and forth in an attempt to alleviate the pain (in
contrast to migraine headaches where patients prefer to lie still). A core feature
of a CH is periodicity, with attacks occurring in clusters 8-10 weeks a year. Sometimes
referred to as histamine headaches, CHs may be accompanied by parasympathetic nervous
system activation with ipsilateral conjunctival injection, lacrimation, rhinorrhea,
and nasal congestion. Ptosis may occur, reflecting secondary involvement of sympathetic
fibers. Patients may also experience ipsilateral photophobia and phonophobia. The
disorder is likely due to involvement of central pacemaker neurons and neurons in
the posterior hypothalamus.
Administration of 100% oxygen at 10-12 L/min for 15-20 minutes is the most effective treatment of a CH. Sumatriptan 6 mg SC or 20 mg given by nasal sprays will usually shorten an attack, whereas oral administration of sumatriptan is generally ineffective. Preventitive treatments include prednisone 1mg/kg/d up to 60 mg/day for 21 days, verapamil 160-960 mg/d (verapamil can cause serious cardiac side effects, including heart block and should be started in low doses (e.g., 60 mg/d) with EKG monitoring), melatonin 9-12 mg/day, gabapentin 1200-3600 mg/d, and lithium 400-800 mg/d. In recalcitrant cases, neuromodulation may be attempted (Sphenopalatine ganglion or occipital nerve stimulation).