Cluster Headache

General

Cluster headache is far less common than migraine or tension headache.  Cluster headaches occur in cyclical patterns or clusters, which gives the condition its name. Cluster headache is one of the most painful types of headache. In contrast to people with migraine headache, perhaps 5-8 times as many men as women have cluster headache. Most people get their first cluster headache at age 25 years, although they may experience their first attacks in their teens to early 50s.

As the name suggests, the cluster headache exhibits a clustering of painful attacks over a period of many weeks. The pain of a cluster headache peaks in about 5 minutes and may last for an hour. Someone with a cluster headache may get several headaches a day for weeks at a time - perhaps months - usually interrupted by a pain-free period of variable length.

Cluster headache commonly awakens you in the middle of the night with intense pain in or around one eye on one side of your head. As a result cluster headaches are sometimes referred to as “alarm clock headaches”.

Bouts of frequent attacks, known as cluster periods, may last from weeks to months, usually followed by remission periods when the headache attacks stop completely. During remission, no headaches occur for months and sometimes even years.

No one knows exactly what causes cluster headaches. As with many other headache syndromes, theories abound, many of which center on your autonomic or "automatic" nervous system or your brain's hypothalamus. These systems play a role in rhythmic or cyclical functions in your body. The involvement of either system in the syndrome would account for the periodic nature of the headache.

  • Many experts believe that cluster headache and migraine headache share a common cause that begins in the nerve that carries sensation from your head to your brain (trigeminal nerve) and ends with the blood vessels that surround your brain.
  • Others believe that the pain arises in the deep vascular channels in your head (for example, the cavernous sinus) and does not involve the trigeminal system.

Fortunately, cluster headache is rare and not life-threatening. Treatments can help make cluster headache attacks shorter and less severe. In addition, medications can help reduce the number of cluster headaches.

Symptoms

A cluster headache strikes quickly, usually without warning. Common signs and symptoms include:
  • Excruciating pain, generally located in or around one eye, but may radiate to other areas of your face, head, neck and shoulders
  • One-sided pain
  • Restlessness
  • Excessive tearing
  • Redness in your eye on the affected side
  • Stuffy or runny nasal passage in your nostril on the affected side of your face
  • Sweaty, pale skin (pallor) on your face
  • Swelling around your eye on the affected side of your face
  • Drooping eyelid

The pain of a cluster headache is often described as sharp, penetrating or burning. People with this condition say that the pain feels like a hot poker being stuck in the eye or that the eye is being pushed out of its socket.

People with cluster headache appear restless. They may pace or sit through the attack. In contrast to people with migraine, people with cluster headache usually avoid lying down during an attack because this position seems to increase the pain.

Some migraine-like symptoms — including nausea, sensitivity to light and sound, and aura — may occur with a cluster headache, though usually on one side.

Treatment

There's no cure for cluster headaches. The goal of treatment is to decrease the severity of pain, shorten the headache period and prevent the attacks. Because the pain of a cluster headache comes on suddenly and may subside within a short time, cluster headache can be difficult to evaluate and treat, as it requires fast-acting medications. Some types of acute medication can provide some pain relief quickly. Based on the latest studies, the medications and procedures listed below have proved to be most effective for acute and preventive treatment of cluster headache.

  Pharmacological Intervention: Fast-acting medication treatments available from your doctor include:
  • The injectable form of sumatriptan (Imitrex), which is commonly used to treat migraine, is also an effective treatment for acute cluster headache.

The first injection may be given while under medical observation. Some people may benefit from using sumatriptan in nasal spray form, but for most people this isn't as effective as an injection and it may take longer to work. Sumatriptan isn't recommended if you have uncontrolled high blood pressure or heart disease.

Another triptan medication, zolmitriptan (Zomig), can be taken in nasal spray or tablet form for relief of cluster headache. This medication may be an option if you can't tolerate other forms of fast-acting treatments.

  • Local anesthetics.The numbing effect of local anesthetics, such as lidocaine (Xylocaine), may be effective against cluster headache pain in some people when given through the nose (intranasal).
  • The intravenous form of dihydroergotamine (D.H.E. 45) may be an effective pain reliever for some people with cluster headache. This medication is also available in an inhaled (intranasal) form called Migranal, but this form hasn't been proved to be effective.

To have the medication administered through a vein (intravenously), you’ll need to go to a hospital or doctor’s office to have the medication administered.

Preventive Pharmacological Intervention therapy starts at the onset of the cluster episode with the goal of suppressing attacks.

Determining which medicine to use often depends on the length and regularity of your episodes. Under the guidance of your doctor, the drugs can be tapered off once the expected length of the cluster episode ends.

  • Calcium channel blockers.The calcium channel blocking agent verapamil (Calan, Verelan, others) is often the first choice for preventing cluster headache. Verapamil is often used in conjunction with other medications. Occasionally, longer term use is needed to manage chronic cluster headache.
Side effects may include constipation, nausea, fatigue, swelling of the ankles and low blood pressure.
  • Inflammation-suppressing drugs called corticosteroids, such as prednisone, are fast-acting preventive medications that may be effective for many people with cluster headaches.

Your doctor may prescribe corticosteroids if your cluster headache condition has only recently started or if you have a pattern of brief cluster periods and long remissions. Although corticosteroids may often be a good short-term option, serious side effects such as diabetes, hypertension and cataracts make them inappropriate for long-term use.

  • Lithium carbonate.Lithium carbonate, which is used to treat bipolar disorder, may be effective in preventing chronic cluster headache if other medications haven't prevented cluster headaches.

Side effects include tremor, increased thirst and diarrhea. Your doctor can adjust the dosage to minimize side effects. While you're taking this medication, your blood will be checked regularly for the developmnet of more-serious side effects, such as kidney damage.

  • Ergotamine, available as a tablet that you place under your tongue, can be taken before bed to prevent nighttime attacks.

Self-injected dihydroergotamine (D.H.E. 45) also may be helpful. Ergot medications may be effective if taken early in your cluster attacks, but they can't be combined with triptans and can only be used for brief periods of time.

  • Studies show that 10 milligrams of melatonin taken in the evening might reduce the frequency of cluster headache.

Other preventive medications used for cluster headache include anti-seizure medications such as divalproex (Depakote) and topiramate (Topamax).

Occipital Nerve Block. Injection of a numbing agent (anesthetic) and corticosteroid into the area around the occipital nerve, located at the back of your head, may help improve chronic cluster headaches. An occipital nerve block may be useful for temporary relief until long-term preventive medications take effect.

Spinal Cord Stimulators. Researchers are studying a potential treatment called occipital nerve stimulation, which may be more accurately described as Peripheral Nerve Stimulation instead of Spinal Cord Stimulation. In this procedure, your surgeon implants electrodes in the back of your head and connects them to a small pacemaker-like device (generator). The electrodes send impulses to stimulate the area of the occipital nerve, which may block or relieve your pain signals.

Several small studies of occipital nerve stimulation found that the procedure reduced pain in some people with chronic cluster headaches.

Deep Brain Stimulation. Similar research is underway with deep brain stimulation. In this procedure, doctors implant an electrode in the hypothalamus, the area of your brain associated with the timing of cluster periods. Your surgeon connects the electrode to a generator that changes your brain's electrical impulses and may help relieve your pain.

Deep brain stimulation of the hypothalamus may provide relief for people with severe, chronic cluster headaches that haven't been successfully treated with other medications. Researchers are studying other types of brain and nerve stimulation to prevent and treat cluster headaches.

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