February 5, 2010
Dear Mayo Clinic:
I have had severe headaches for five years and have been going to a neurologist for three years. The neurologist has been treating me for cluster headaches. He has prescribed a number of medications for pain, but nothing helps. During these attacks I can't do anything but walk the floor and cry. Would I be a good candidate for deep brain stimulation?
Deep brain stimulation can significantly reduce the frequency of cluster headaches for some people. Because it's a very invasive surgical procedure, though, deep brain stimulation is used only for a small minority of patients whose cluster headaches don't respond to medical treatment. You should try other treatment options before considering deep brain stimulation.
First, you should confirm that you are actually having cluster headaches. These headaches typically occur during what's called a cluster period. That period can last weeks to months and is typically followed by a remission that may last months or years.
Diagnosing a headache disorder isn't always straightforward. Some of my patients come with a previous diagnosis of cluster headache, when, in fact, they have migraine headache or another headache disorder. Conditions that may cause cluster-like headaches need to be ruled out, too, such as intracranial tumors, sinusitis and cerebrovascular disease, among others.
If cluster headache is the correct diagnosis, a review of the treatments you've already received is in order. You mention that pain medications haven't worked. Before you dismiss the usefulness of medications, I would recommend that you discuss your medication history with a headache specialist. That specialist can determine if you've received the appropriate medications in the appropriate doses.
Preventing cluster headache is the priority. Preventives should be considered when headache attacks are frequent, severe, begin rapidly and are too short-lived for medications that are designed to stop the individual headache or when those medications don't work.
For short-term prevention, I typically prescribe an 18-day course of prednisone, an inflammation-suppressing drug. It acts quickly in most people and is the most effective intervention to break the cluster period, inducing remission. Because of potential adverse side effects, though, long-term use of prednisone isn't recommended. For longer-term prevention, verapamil frequently helps many but not all patients. Other less commonly used preventive medications are lithium carbonate, topiramate and divalproex sodium.
Preventive treatment is best started early in the cluster period and should continue until you are headache-free for at least several weeks. Depending on your circumstances, you may need to keep taking preventives until you are headache-free for several months. Then, the maintenance preventive can sometimes be tapered off. Therapy should restart when the next cluster period begins.
A minority of those who have cluster headaches don't respond to treatment with medication. In those situations, more invasive treatment may be appropriate to reduce the frequency of headaches. Although deep brain stimulation is one option, I usually first recommend occipital nerve stimulation. Although its effectiveness is less than deep brain stimulation, occipital nerve stimulation doesn't require invasive brain surgery. The occipital nerve is stimulated beneath the skin at the back of the head on the side of pain or sometimes on both sides. Its potential complications also are minor compared to deep brain stimulation. Occipital nerve stimulation can significantly improve quality of life for some people.
If occipital nerve stimulation is ineffective, then deep brain stimulation may be appropriate. In this procedure, a wire lead is surgically implanted in the hypothalamus, the area of the brain that controls sleep and the body's biological clock. This area is believed to be involved in the genesis of cluster headaches. The lead is connected to an implanted pacemaker-like device programmed to send electrical pulses that prevent cluster attacks from occurring.
An important final factor to consider is the frequency and length of time you have cluster headaches. The distinction between episodic cluster headache versus chronic cluster headache is crucial in determining appropriate treatment. Cluster headaches are considered episodic when a person has experienced cluster periods for less than one year, and those cluster periods are separated by pain-free periods that last for at least one month. In the chronic form, cluster headache attacks recur for longer than one year without remission or with remission periods lasting less than one month. Invasive treatment options are largely reserved for chronic cluster headache sufferers who have very frequent, severe attacks that fail to respond to appropriate medical therapy.
I recommend that you consult a neurologist with headache expertise to confirm your diagnosis, review your medical and treatment history, and develop a plan to reduce and manage your headaches.
—Ivan Garza, M.D., Neurology, Mayo Clinic, Rochester, Minn.