Cluster headache is characterised by repeated attacks of strictly unilateral
pain in the orbital region associated with local autonomic symptoms or signs. The attacks are brief but of a very severe, almost excruciating intensity. For unknown reasons males are affected more often than females. Recent studies suggest that an autosomal dominant gene has a role in some families with
cluster headache. Hormonal studies indicate a dysfunction in the central nervous system. Neuroimaging has revealed primary defects in the hypothalamic grey matter. Local homolateral dilatation in the intracranial segment of the internal carotid and ophthalmic arteries during attacks is the result of a generic neurovascular activation, probably mediated by trigeminal parasympathetic reflexes.
Sumatriptan 6mg subcutaneously is the
drug of choice in the treatment of acute attacks. Inhalation of 100%
oxygen can also be recommended. In the prophylactic treatment,
verapamil is the first option. Other drugs that can be considered are
corticosteroids, which may induce a remission of frequent, severe attacks, and
lithium. Oral
ergotamine tartrate may be sufficient for patients with night attacks and/or short, rather mild to moderately severe
cluster headache periods. Third line drugs are
serotonin inhibitors (
methysergide and
pizotifen) and
valproic acid. Patients should be encouraged to keep
headache diaries and be carefully instructed about the nature and treatment of the
headaches. Alcohol can bring on extra attacks and should not be consumed during active periods of
cluster headache.