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Cluster Headache

The pain associated with cluster headache is said to be the worst known to mankind and it requires prompt diagnosis and management. Diagnostic criteria are shown in Box 160-5. The pain uniquely makes the patient move about in his or her distress. The basic mechanism of cluster headache is unknown.

Each episode of pain is manifest by sudden, very severe pain with a relatively short duration of an hour or two, one or more times per day or night, but usually with the first REM sleep period (Fig. 160-3). Marked autonomic features (ipsilateral sweating, flushing, tearing, enophthalmos, miosis, nasal blocking, and rhinorrhea) accompany the pain. Attacks frequently occur with clocklike regularity during or immediately after REM sleep. During the attacks the patients are up and about, pacing, stamping, locking themselves into rooms, violently resisting help, at times screaming, begging for relief, occasionally going into a trancelike state for short periods. Sometimes patients threaten suicide during an attack, but they rarely if ever perform it. Many patients explain that the pain is worse than that experienced with a fracture, surgery, or childbirth, or that associated with passing a kidney stone. Although the attacks of pain usually remain limited to a given side of the head and face during a given cluster, in some patients the attacks may switch to the other side before the cluster is over and, in a few rare instances, may involve both sides at once during one or several individual attacks.

During the clusters, which may last weeks or months, the patient is extremely vulnerable to vasodilating stimuli (e.g., alcohol, nitrites, histamine) and great changes in pace (e.g., weather, time, and activity), such as occur during naps or REM sleep, travel, seasonal changes, and work. Once the cluster has passed, these stimuli are no longer active until the next cluster supervenes.

Cluster headache patients, mostly men, are said to have an athletic build, including increased height, a leonine facies characterized by thick "orange peel" skin; deep furrows in forehead, cheeks, and chin, and telangiectasia, especially on the cheeks and bridge of the nose (Fig. 160-4 and Box 160-6). Clusters may begin during childhood or more often in young adulthood, or they may not occur until middle and late middle age. They may persist over a lifetime into the 70s and 80s, but more often they tend to decrease during the 60s. Intervals between clusters may range from a few weeks to a few months or a few years. The headaches recur occasionally after 10, 15, or even 35 years of freedom.

Chronic Cluster Headache

A few unfortunate individuals may remain in a constant or chronic cluster state almost daily for more than a year or even for several years. Usually, chronic clusters result from the gradual compression of periodic clusters closer together until a continuous or chronic state results. Rarely, clusters are chronic or classified as primary chronic cluster from the beginning.

Differential Diagnosis

Cluster headache is most often confused with tic douloureux or trigeminal neuralgia. However, unless secondary to multiple sclerosis or tumor, tic first appears in patients over 60 years of age, whereas cluster headache begins even during childhood and most frequently during the second, third, and fourth decades of life. Tic pain is described in. Rarely, cluster pain is accompanied by short shocks or jolts of pain similar to that of tic superimposed on the constant steady pain, creating the so-called cluster-tic syndrome.

Keratitis, corneal abrasions, and glaucoma cause pain and redness of the eye, but the eye examination should confirm the diagnosis. Raeder and Tolosa-Hunt syndromes sometimes present with recurring steady pain in the eye and face lasting days, weeks, or occasionally months. Malignant growths of the nose and throat may also mimic the pain of cluster headache, even presenting at first with intermittent bouts of pain associated with Horner's syndrome, pain in the cheek and eye, and nasal blockage. Radiographs and CT scans of the nasofacial bones and sinuses may reveal organic lesions in the nasopharynx and sinus areas, with destructive lesions in the hard palate and floor of the skull (Fig. 160-5).

Treatment

Treatment for the pain of the individual acute attack of cluster headache must be immediately available and quick in its action. Patients should always have their treatment with them or within easy reach because the pain develops rapidly. Although it does not last long, the pain is devastating, giving rise to the term suicide headaches. Strenuous exercise for 5 to 10 minutes at onset and ice-cold or very hot applications to the affected area are of questionable value. Acute abortive therapy today comprises breathing 100% oxygen by nasal mask at 7 to 10 L/minute for 10 to 15 minutes at onset of attacks; the intranasal instillation of 4% lidocaine into the nostril on the affected side, with the head dependent; or subcutaneous sumatriptan, 6 mg, or DHE, 1 mg.

The best prophylactic agent is prednisone (or equivalents), 40 to 60 mg/day, reduced by 5 mg/day every 2 or 3 days. The total period of therapy should be no longer than 6 to 8 weeks—preferably 4 weeks—because of possible chronic steroid side effects. Lithium and verapamil are appropriate long-term therapies. One of these drugs should be started at the same time as the steroids are given. The usual dose is 900 mg of lithium carbonate/day in three doses, to achieve a blood lithium level of 0.5 to 1.0 mEq/L. Thyroid function, blood counts, and renal function are monitored as they are in other patients taking lithium. As the patient improves, the dose may be gradually reduced. In chronic cluster headache, it may be wise to continue one dose of lithium per day for many months or even permanently. When headache cycles break through, the dosage may be raised appropriately. Ergot or steroids may be added to the lithium regimen if recurrence takes place.

The calcium channel blocker verapamil (240 mg/day) must be taken for at least a month to obtain full effect. Other drugs that can be used include cyproheptadine (Periactin), 4 mg orally one to four times daily; tricyclics such as amitriptyline (Elavil) and doxepin (Sinequan); and methysergide, 2-mg tablets one to four times daily. This last drug is an effective preventative agent but its side-effect profile includes potentially lethal complications (it must be stopped at 3-month intervals for at least 2 weeks), which lead us to consider it a drug of last resort.

Finally, surgery may be necessary if cluster headache has failed to respond to medical treatment and is seriously affecting the patient's life. Sphenopalatine ganglion block or excision, greater superficial petrosal neurectomy, and nervus intermedius neurectomy by radiofrequency, surgical transection, and/or glycerol injection are other techniques to be considered.

Chronic Paroxysmal Hemicrania

Chronic paroxysmal hemicrania is a rare variant of cluster headache characterized by its occurrence most often in women (in 90% of cases), the short duration of the attacks (minutes), and their frequent occurrence (perhaps 15 attacks or more per day). The pain resembles that of cluster headache in its unilaterality; its involvement of the eye, cheek, and temples; and the associated autonomic dysfunction. A remarkable feature of this condition is its specific rapid response to indomethacin, 150 to 200 mg daily. Within a few hours of the first dose, attacks usually cease completely. Some patients with this syndrome have multiple daily attacks for several years without relief from any of the ordinary cluster headache remedies.