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Cluster headache
is classified as headache type 3.1 in the IHS Classification (Table 1).
The features of cluster headache are outlined in Table 5: Table 5. Cluster headache
The phrase "cluster headache" denotes a characteristic type of cephalgia defined as a severe unilateral head or facial pain, which lasts minutes to hours; it is often associated with ipsilateral lacrimation, nasal congestion, and facial flushing. The headaches tend to occur in separate bouts or clusters in one or more attacks daily for periods of weeks to months.{134-139} Often during an attack, and sometimes persisting following the episode, there is ipsilateral miosis and ptosis, ie, and oculosympathetic paresis (Horner syndrome). Table 6. Cluster Headache and Variants
(Modified from Bickerstaff EB: Cluster headaches. In Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology, Vol. 5, Headache and Cranial Neuralgias. New York, American Elsevier, 1968) Romberg in 1840 described "ciliary neuralgia" as recurrent pain in the eye with injection and pupillary constriction.140 Harris described the same syndrome in 1926 as "periodic migrainous neuralgia"{141} and later (1928) as ciliary (migrainous) neuralgia.{142} Multiple redescriptions and rediscoveries of the syndrome have resulted in a host of synonymous terms as well as confusion with other entities not related to this particular headache syndrome. (Table 6 ) The pain in cluster headache is very severe, being described as "boring," "sharp," "unbearable," and "the worst pain I have ever felt." The headache usually appears in or around one eye or on the cheek and then can spread to the temple, frontal region, occiput, or the ipsilateral neck. It rapidly builds to a peak within a few minutes, with an intensity greater than most other headache varieties. The usual duration is from 30 minutes to a few hours. Rather than lying down in seclusion, as preferred by most migraine sufferers, the cluster victim often paces about, holds onto his face, or applies very hot or cold water to the affected region, even to the point of injuring himself. The attacks usually occur once in a 24-hour period, often at a specific time, and frequently in the early morning hours awakening the patient from sleep. Men are affected much more commonly than women, in a ratio of approximately 5:1. Usually the episodes begin in the second or third decade and, according to Bickerstaff,135 frequently are ipsilateral to previous head trauma. The headache tends to remain on the same side in a given cluster but rarely may alternate in subsequent clusters. Typical migraine headache is frequently found in family members. While most patients have clusters occurring over weeks or months (often at the sameseason of year), other patients have sporadic attacks or irregular episodes for indeterminate periods of time. Typical migraine headaches may occur in the same individual with cluster, sometimes waning as the cluster commences. In the cluster attacks as described, it is extremely rare that any organic pathology is ever demonstrated; however, the variants of the syndrome require more detailed analysis. When the pain becomes persistent, becomes bilateral, or additional neurologic abnormalities are present (such as fifth nerve or optic nerve involvement), other conditions must be ruled out. Regarding the mechanism of cluster headache, few specifics are known. However, Ekbom and Greitz{143} have demonstrated a localized narrowing of the extradural portion of the internal carotid artery distal to its exit from the carotid canal in a patient during an attack of cluster headache. They speculated that, in addition to the headache, the partial Horner syndrome might be due to repeated dilatation and edema of the internal carotid artery resulting in damage to the sympathetic nerves surrounding the vessel. Bickerstaff{135} has reviewed the variety of terms used to describe cluster headache and the relationship to other types of facial and cranial neuralgias. Vidian neuralgia as described by Vail{144} referred to recurrent aching pain affecting the nose, eye, face, neck, and shoulder on one side, but he also included recurrent episodes which would clearly conform to the cluster headache syndrome. The facial pain described originally by Sluder included clinical variants but primarily referred to a constant pain affecting eye, upper jaw, hard palate, and teeth on one side, usually in menopausal women.{145} In 1924, Raeder described 5 patients with Horner syndrome but without facial anhydrosis (oculosympathetic paresis), with pain or numbness in the distribution of the ophthalmic branch of the trigeminal nerve, which he designated as "paratrigeminal neuralgia";{146} however, some of his patients had other cranial nerve signs. Additional findings suggested specific intracranial lesions. Boniuk and Schlezinger{147} divided Raeder syndrome into two groups: 1) characterized by hemicrania, ipsilateral oculosympathetic paresis, and parasellar cranial nerve (III, IV, V, and VI) involvement, the additional cranial nerve signs suggesting disease in the middle cranial fossa and indicating appropriate diagnostic studies, and 2) hemicrania and group the most common cause is a cluster headache variant,{148-150} and further diagnostic studies including arteriography are unwarranted. However, in Raeder type 2 neuralgia, aneurysm,{151,152} and fibromuscular dysplasia of the carotid artery{153} have been reported. In the patients with such demonstrable lesions, unilateral facial pain was persistent rather than the episodic excruciating variety described in the classic cluster headache syndrome. Therapy recommended for cluster headache has been quite variable and often was tailored to the suspected condition. Harris' method was to inject the gasserian ganglion.{142} Horton tried to desensitize his patients to histamine,{154} and others have recommended sphenopalatine ganglionectomy.{155} Histamine is indeed elevated in serum during a cluster attack, with minimal change in serotonin (as contrasted to depressed levels of serotonin in typical migrainous episodes).{156} Ergotamine preparations have been widely used, parenterally in the acute attack and orally as a prophylactic agent.{156} The prophylactic use of methysergide (Sansert) has been employed successfully,{157} and currently propranolol (Inderal), 20 to 40 mg b.i.d. to q.i.d, may serve as an effective preventive.{158} Calcium channel blocking agents may also be effective in cluster headache, particularly nimodipine.159 Therapy of cluster headaches and their relationship to other forms of vascular headache has been reviewed by Lance.{160}.
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