Preventive Therapies for Cluster Headaches
Cluster headache is among the most severe pains known to mankind. It is characterized by excruciating, debilitating pain lasting from 15 minutes to 3 hours, and occasionally longer. The pain is usually located around or through one eye, or on the temple. The series of cluster headaches lasts several weeks to several months, and can occur once or twice per year, every other year, or even less frequently. Cluster headaches tend to occur more in the spring and fall.
Cluster headaches occur more frequently in men than women (2.5:1 ratio). Approximately 1 out of every 250 men have cluster headaches. The age of onset of cluster headaches is usually between age 20 and 45, but there are cases of cluster headaches occurring in teenagers, and occasionally the headaches begin after age 50, though rarely after 70 years of age. Women tend to have an older age of onset than men. There is usually no family history of cluster headaches, but occasionally cluster headaches do run in families.
The pain of the cluster attack is extreme and starts very quickly, usually without an aura or a warning. Within minutes, it becomes very severe. Although the pain is usually located about the eye or temple, it may be more intense in the neck or facial areas. Usually unilateral, the pain does change sides in 10% to 15% of patients, either during a cluster cycle, or at the next cycle. The pain itself is excruciating, described as sharp, stabbing, and even “like my eye is being pulled out.”
The length of headache attacks varies, but 45 minutes is the average. Cluster headache patients usually experience 1 or 2 headaches per day, but this may increase to as many as 7 headaches occurring in 24 hours, or decrease to as little as 1 or 2 attacks per week. The attacks usually occur around the same time each day, with the time period between 9 PM and 10 AM being most frequent. Approximately 50% of patients report being woken by the headaches. Several of the following symptoms are usually present at the time of the attack: lacrimation, nasal congestion, rhinorrhea, conjunctival injection, ptosis, miosis of the pupil, or forehead and facial sweating. Nausea, bradycardia, and general perspiration also occur in many patients.
Richard is a 40-year-old man with a history of 4-weeklong cycles of cluster headaches, occurring once a year in the fall. The headaches began when he was 35. The cluster period begins slowly, increasing over one week’s time and reaching a peak where Richard has 2 or 3 severe attacks per day. These occur during the night from 10 pm to 3 am. Each cluster attack lasts from 40 to 90 minutes, and the pain is severe. The headache is always on the right side, and is accompanied by eye tearing and nasal congestion.
Examination and Treatment Plan
Richard visits our office during the first week into his 4-week headache series. The headaches are increasing in intensity and he is miserable from the pain. At this point, we want to put Richard on a prophylactic regimen, and give him an abortive to help ease the acute attack. We decide to use prednisone, one 20-mg tablet in the morning and another with dinner (40 mg/day) for 4 days. We will reduce this to 20 mg/day after the first 4 days, and then to 10 mg/day after another 6 days. We will then taper off the prednisone entirely over the next 4 to 6 days.
Limiting the amount of corticosteroids is important for two reasons: 1) serious side effects are decreased, and 2) if necessary, we may want to utilize additional prednisone later in the cluster series. If the patient has been on a high-dose of steroids for 3 weeks, we cannot use more corticosteroid. In contrast, by keeping the amount to a minimum, we are able to use steroids later in the cluster period. Cluster sufferers may be more prone to femoral head necrosis with the use of corticosteroids.
With the prednisone, we begin a slow release form of verapamil. This is started at 240 mg/day; we may eventually increase to 2 doses per day, which is generally the maximum (480 mg/day). As the prednisone dose is decreased, and the patient is weaned off the medication, it is hoped that the verapamil will have taken effect.
The use of oxygen as an abortive is discussed with Richard, but he prefers to wait. We give him sumatriptan tablets, 100 mg, as he is reluctant to self-inject sumatriptan. Richard is also instructed to apply ice to the areas of pain. (See Table 2 for list of the most common preventative agents prescribed for cluster headaches.)
Six days later, Richard calls the office. He has had 5 very good days, but as the prednisone is being decreased, the headaches are becoming more severe. Sumatriptan tablets do not help; last night, he had 90 minutes of extremely intense pain. At this point, we convince Richard to try oxygen, at 10 to 13 L/min, as needed, and he rents a tank. Richard also is given sumatriptan injections, 4 mg.
We continue the original plan of decreasing prednisone, and we increase the dose of verapamil to 480 mg/d. We will monitor Richard’s blood pressure. He now has oxygen and sumatriptan injections available as abortive agents; adding lithium or valproate are considerations, as is indomethacin.
I see Richard 4 days later. He is now in his third week of clusters, and by his previous pattern, has 1 to 2 weeks left in the cycle. However, at times a cluster period may exceed the previous one in length, and extended cluster periods of up to several months do occur. Richard states that the oxygen does help his headaches.
The clusters are less severe, but still occur regularly twice a night. Sumatriptan injections stop the attacks within 10 minutes of administration. The verapamil may be having some effect as well. He is down to 20 mg/day of prednisone, and we decide to taper off the dose over the next 4 days. If the headaches increase dramatically, he could return to the prednisone.
Six days later, the headaches are gone, and after a week without headaches, Richard is tapered off the verapamil over the course of 6 days. If the headaches were to return during those 6 days, we would immediately increase the dose of verapamil to the maximum of 480 mg, and consider using prednisone again.
It is important to chart which medications are most effective for treating a patient’s cluster headaches, so as to be ready to use them for the next cluster series. I usually write the plan for the next series in the patient’s chart, and inform the patient of the plan.