Most patients arise from bed during an attack and sit in a chair and rock or pace the floor, holding a hand to the side of the head. The pain of a given attack may leave as rapidly as it began or may fade away gradually. Almost always the same orbit is involved during a cluster of headaches as well as in recurring bouts. During the period of freedom from pain, alcohol, which commonly precipitates headaches during a cluster, no longer has the capacity to do so. The picture of cluster headache, including the patient’s nocturnal behavior in response to it, is usually so characteristic that it cannot be confused with any other disease, although those unfamiliar with it may entertain a diagnosis of migraine, trigeminal neuralgia, carotid aneurysm, or temporal arteritis.
A somewhat similar syndrome is produced by the Tolosa-Hunt syndrome of eye pain and ocular motor paralysis caused by dural granuloma at the orbital apex (see further on) and the paratrigeminal syndrome of Raeder, which consists of paroxysms of pain somewhat like that of tic douloureux in the distribution of the ophthalmic and maxillary divisions of the fifth nerve, in association with unilateral Horner syndrome (ptosis and miosis but with preservation of facial sweating). Loss of sensation in a trigeminal nerve distribution and mild weakness of muscles innervated by the fifth nerve are often added. Raeder syndrome is now recognized as a heterogeneous syndrome, some cases being cluster and others caused by a structural lesion in or near the carotid siphon.
Cases of paroxysmal pain behind the eye or nose or in the upper jaw or temple—associated with blocking of the nostril or lacrimation and described in the past under the titles of sphenopalatine (referred to as Sluder’s sphenopalatine neuralgia), petrosal, vidian, and ciliary neuralgia—probably represent variants of cluster headache. A similar head pain may occasionally be confined to the lower facial, postauricular, or occipital areas. Ekbom distinguished yet another “lower cluster headache” syndrome with infraorbital radiation of the pain, an ipsilateral partial Horner syndrome, and ipsilateral hyperhidrosis. There is no evidence to support the separation of these neuralgias as distinct entities, and they have collectively been called trigeminal autonomic cephalgias. They are important, however, because of the frequency of underlying intracranial lesions. In other words, these are not always primary headache disorders. Favier and colleagues collected 4 of their own cases and 27 from the literature to emphasize the range of underlying diseases, including intracranial aneurysms, peritentorial or parasellar meningiomas, or other tumors and nasopharyngeal cancers surrounding the carotid artery. We have encountered a case of Wegener granulomatosis of the soft palate that presented as a paroxysmal trigeminal autonomic neuralgia. The headache syndrome disappeared with cyclophosphamide treatment of the underlying granulomatous disorder.
Chronic paroxysmal hemicrania was the name given by Sjaastad and Dale to a primary headache consisting of rapidly repetitive unilateral form of headache that resembles cluster headache in many respects but has several distinctive features. These are of much shorter duration (2 to 45 min) than cluster and usually affect the temporoorbital region of one side, accompanied by conjunctival hyperemia, rhinorrhea, and in some cases a partial Horner syndrome. Even periorbital ecchymosis may accompany a severe attack. Unlike cluster headache, however, the paroxysms occur many times each day, recur daily for long periods (the patient of Price and Posner had an average of 16 attacks daily for more than 40 years), and, most important, respond dramatically to the administration of indomethacin, 25 to 50 mg tid. Unlike cluster headache, chronic paroxysmal hemicrania is more common in women than in men (ratio of 3:1).