Cluster Headaches

Frequently Asked Questions

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Cluster Headaches (by Joel R. Saper, MD, & MHNI Staff)

• What are cluster headaches?
• What clinical features distinguish cluster from migraine headache?
• What is the prevalence of cluster headache?
• What is the proposed mechanism of cluster headache?
• What specific exams or tests are performed for determining cluster headaches?
• What treatment is available for cluster headaches?
• What is cluster tic syndrome?

What are cluster headaches?

Cluster headache is a devastating, painful affliction. Unlike migraine and daily chronic headache (DCH), this condition primarily affects men. Cluster headache is a periodic (30-90 minute) attack of severe pain, primarily localized to the eye, temple, forehead, or cheek region. Up to 50% of patients may have tenderness at the base of the skull and neck on the same side as the headache pain.

The term "cluster headache" was originally used to describe the clustering or sequence of bouts of painful attacks that occur from weeks to months at a time and then spontaneously terminate. The headache cycle may return weeks or months later. The interval between cycles is called the interim. It is now recognized that a chronic form of cluster headache exists in which recurring attacks, without interim, occur for years at a time.

Key clinical features

As stated above, cluster headache occurs predominantly in males, compared with the female to male ratio (3:1) of migraine. The frequency of attacks is 1-6 times per day. Alcohol typically provokes an attack. Many cluster headache patients are heavy smokers and alcohol drinkers. Attacks frequently occur during sleep or napping times. Each attack of cluster headache is usually accompanied by same side eye watering and nasal drainage, eye lid drooping, pupillary change, and eye congestion/redness.

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Feature	Cluster	Migraine
Number of attacks	1-6 per day	1-10 per month
Duration of pain	30-90 minutes	4-24 hours
Location of pain	Always one-sided, around the eyes; sometimes referred to the back of the head	One-sided or both sides
Occurrence of attacks	Multiple daily attacks for several weeks	Usually 2-5 times per month
Time of day of attacks	Frequently at night, often at the same time each day	Any time
Typical age at onset	Onset 20 years or older	10-50 years (can be younger or older)
Sex	Majority male	Majority female
Warning signs	None	Often present
Nausea and vomiting	2-5%	85%
Blurring of vision	Infrequent	Frequent
Eye watering	Frequent	Infrequent
Nasal congestion	70%	Uncommon
Eyelid drooping	30%	1-2%
Family history	7%	90%
Contraction of the pupil	50%	Absent
Behavior during attack	Paces, pounds fist	Rests in quiet, dark room

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What is the prevalence of cluster headache?

The prevalence of cluster headache is reported to be approximately 0.1-0.3% of the population, though some estimates are higher. The attacks can begin at any age, although they usually occur between the ages of 20 and 40 years. A family history of cluster headache is occasionally present (as well as an increased prevalence of migraine).

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Currently, the exact mechanism of cluster headache remains uncertain. The sphenopalatine ganglion and cavernous sinus are among the sites previously considered potentially important for cluster headache pathogenesis. Most recently, PET scanning techniques have revealed the hypothalamic region as important in cluster headache pathogenesis (May, 1998). Critical serotonergic function, which regulates the biological clock, may also be altered (Leone, 1997).

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The neurological examination of cluster headache is normal, except for changes during the headache that might include eyelid drooping, pupillary change, etc. There are no specific diagnostic tests for cluster headache, although provocation with alcohol is considered a key and reliable feature.

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The primary treatment strategy is prevention of the attacks. Because of the frequency and brevity of the attacks, symptomatic treatment is not generally the mainstay of therapy and is considered supplemental. Due to the devastating nature of the condition, patients must be seen by their physicians, whenever appropriate. Visits cannot be postponed when recurring, untreated attacks are occurring.

Patients must be provided effective and aggressive preventive and symptomatic relief measures. Although steroids (to be discussed) are reliably effective, the risks must be weighed against the benefits. Other preventive agents are often more appropriate first-line treatments.

Specific treatment approaches:

A.	Symptomatic treatment includes:
		1.	Oxygen inhalation*;
		2.	Dihydroergotamine (nasal spray or parenteral);
		3.	Sumatriptan (s.c. and nasal spray) or the other "triptans";
		4.	Sphenopalatine blockade;
		5.	Intranasal lidocaine;
		6.	Intranasal capsaicin;
		7.	Indomethacin (rectal suppositories, occasionally effective); and
		8.	Opioids (rectal/Stadol nasal spray; avoid frequent use). *Oxygen (100%) inhalation should be administered via a face mask at 7 liters/min for 10-15 minutes at a time, preferably given at the onset of the attack.
B.	Preventive Treatment The following agents are most appropriate for the prevention of cluster headache:
		1.	Verapamil is a first-line treatment for prevention of cluster headache, although weeks of therapy may be required before control is established. Verapamil must be administered at relatively high dosages to be effective (120-160 mg t.i.d.-q.i.d). Short-acting forms of verapamil are generally more reliable than long-acting forms due to variations in bioavailability. Thus, long-acting forms often require upward adjustment of dosage.
		2.	Steroids are reliably effective (80-90%) in preventing attacks during active therapy. Though not appropriate for prolonged preventive therapy, steroids can be used for:
			a.	Difficult-to-treat exacerbations
			b.	At the onset of a cycle to allow time for other medications to take effect
			c.	As an available "insurance treatment" for breakthrough attacks while traveling or otherwise away from medical care
			The risks of steroids must be carefully reviewed by the prescribing physician. Continuous steroid treatment should not be used. Repetitive, interval administration should be considered only in truly resistant cases.
		3.	Lithium
		4.	Methysergide/methylergonovine
		5.	Divalproex sodium
		6.	Maintenance neuroleptics, such as chlorpromazine, may have a value in rare instances.
		7.	Transdermal or oral clonidine (possible benefits have recently been reported by D'Andrea, 1995)
		8.	Daily ergotamine tartrate, daily dihydroergotamine, sumatriptan, or other triptans. (The risks of daily use of these agents for prolonged clusters makes this use unacceptable except in the most extreme and debilitating cases. The risks must be weighed against the value, since cluster headache patients, whether they smoke or not, may be at increased risk for cardiovascular disease, and alternate treatments, including hospitalization, are generally effective.)
		9.	Daily opioids. (This must be reserved for extreme cases where all other reasonable treatments have failed or are unacceptable alternatives.)
C.	Neural Blockade and Surgery Sphenopalatine ganglion (SPG) blockade is reported effective in some patients (Saunders, 1997). Though control of an acute attack may be achieved with local application of anesthetic agents, repetitive SPG blockade has not generally achieved acceptance in neurological circles. Various surgical procedures are available, the most popular of which is percutaneous SPG radiofrequency rhizotomy (a technique that uses microwave heat to inactivate a nerve). Taha and Tew (1995) reported long-term results of radiofrequency rhizotomy in seven patients with cluster headache. All patients reported relief immediately after surgery. Two patients remained pain-free 5 and 20 years later, respectively. Three patients experienced mild pain recurrence 6-12 months after surgery, and two of these patients were able to control the pain with prescribed medication. The third patient controlled the pain with simple analgesics. Two patients had poor results. Major recurrence was noted in one patient 4 days after surgery and in the other, 2 months after surgery. According to Mathew (1990), approximately 65-75% of patients had excellent, very good, or good results in his extensive series. Poor results are infrequent, often the consequence of post-surgical difficulties. Repeated surgery is sometimes necessary. Despite these reports, some authorities believe that surgical success is 50% or less, with significant complications in many. The authors of this text are reluctant to recommend surgery, except in the most extreme cases and when all other options have been explored. Several personally encountered patients have done poorly after surgery. Headaches have recurred, or persistent deafferentation syndromes have emerged. Recently, Ford and colleagues (1998) reported that gamma knife radiosurgery of the trigeminal nerve provided benefit to five of the six patients treated. The authors suggested that the technique carries negligible short- and long-term risk. The ultimate value of this intervention will await further studies.
		Matthew suggests the following criteria for surgery:
		1.	Chronic cluster headache without pain remission for at least one year in patients who are totally resistant to aggressive medical management for a "reasonable" period of time;
		2.	Strictly unilateral pain; and
		3.	Patients who are physiologically stable, not prone to medication overusage, and otherwise medically and mentally healthy.
D.	Hospitalization
	Hospitalization for cluster headache patients may be essential during resistant, severe episodes or when patients become desperate. The use of IV fluids, sedation, parenteral DHE, and other parenteral therapies may be required. Patients with cluster headache should generally avoid alcohol, particularly during cluster cycles. Discontinuing smoking may be very important as well, but is quite difficult to achieve, particularly on an outpatient basis. Hospitalization often allows confronting these and other factors, which sometimes are critical. The physician must be firm on these matters. In one author's experience (Saper) and in the published outcome series (Lake, 1993), cluster headache appears to respond better than any of the other primary disorders to the aggressive interventions in a hospital setting.

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What is cluster tic syndrome?

The cluster tic syndrome features the primary symptoms of cluster headache but with the added component of stabbing, ice-pick neuralgic-like components involving the eye, face, and jaw. The syndrome is found in 10-20% of patients but is often undiagnosed. True trigeminal neuralgia may coexist with cluster headache.

Alberca and Ochoa (1994) reviewed 37 reported cases of cluster tic syndrome. They noted equal gender representation and found that trigeminal neuralgia usually appeared first. Some attacks appeared to blend both neuralgia and cluster headache symptomatology and could be triggered by touching of the upper lip on the involved side. Medical treatment was often not effective, although a combination of cluster headache therapy with that for trigeminal neuralgia was sometimes useful.

Several antineuralgic agents are available. These include carbamazepine, phenytoin, baclofen valproate, and clonazepam. Most recently, gabapentin has shown promising results in neuropathic pain disorders. As for surgical interventions, suboccipital surgery (Solomon, 1985) revealed compression of the trigeminal nerve by aberrant vasculature, and following treatment, the neuralgic component resolved.

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