What treatment is available for cluster headaches?
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:
Symptomatic treatment includes:
- Oxygen inhalation*
- Dihydroergotamine (nasal spray, injections, or inhalant)
- Sumatriptan (s.c. and nasal spray) or the other "triptans"
- Sphenopalatine blockade
- Intranasal lidocaine
- Intranasal capsaicin
- Indomethacin (rectal suppositories, occasionally effective)
- Opioids (rectal/Stadol nasal spray; avoid frequent use)
*Oxygen (100%) inhalation should be administered via a face mask at 7 liters/min or higher for 10-15 minutes at a time, preferably given at the onset of the attack.
The following agents are most appropriate for the prevention of cluster headache:
- 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.
- Steroids are reliably effective (80-90%) in preventing attacks during active therapy. Though not appropriate for prolonged preventive therapy, steroids can be used for:
- Difficult-to-treat exacerbations
- At the onset of a cycle to allow time for other medications to take effect
- 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.
- Divalproex sodium
- Maintenance neuroleptics, such as chlorpromazine, may have a value in rare instances.
- Transdermal or oral clonidine (possible benefits have recently been reported by D'Andrea, 1995)
- 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)
- Daily opioids (This must be reserved for extreme cases where all other reasonable treatments have failed or are unacceptable alternatives)
Neural Blockade, Neurostimulation 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.
Neurostimulation is the insertion of an active electrode near a nerve in the back of the head and neck area to stimulate a nerve, which in many cases turns off the pain. Recent advances in neurostimulation have had a dramatic impact on a number of patients with cluster headache. The electrode has been placed high in the neck area and has dramatically turned off or limited the number of attacks (Dodick, et al., 2007)
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:
- 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;
- Strictly unilateral pain; and
- Patients who are physiologically stable, not prone to medication overusage, and otherwise medically and mentally healthy.
In some very advanced cases, a more drastic surgery has taken place in which an electrode is placed deep in the brain in the area of the hypothalamus.
Hospitalization for cluster headache patients may be essential during resistant or particularly severe episodes or when patients become desperate from recurring attacks. The use of IV meds and fluids, sedation, and other injection therapies may be required and can be dramatically helpful. 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. 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.