OCTOBER  1999 MEETING

TOPIC:  “CLUSTER HEADACHES”

Michael Stein, M.D., gave a presentation on Cluster Headaches for the October 12th meeting of the East Bay Headache Support Group.  The meeting was held in the Ball Auditorium at John Muir Medical Center with 17 people in attendance.

Dr. Stein is a neurologist in Walnut Creek who specializes in the treatment of headache patients, and is co-founder and medical advisor of the East Bay Headache Support Group. 

He began his talk by passing out an outline which listed the various topics he would cover during the evening.  He explained that the “epidemiology” of cluster headaches is how the phenomenon occurs in large populations.  To help non-sufferers understand how painful this type of headache can be, Dr. Stein said that some of the comparatively few women who suffer from cluster headaches have described the pain as being worse than childbirth. 

Dr. Stein also said that cluster headache has been given more names than other types of headache.

What is cluster headache and how is it diagnosed?

The International Headache Society has established criteria to help identify whether a patient is suffering from cluster headaches, or some other condition.  The criteria are:

1.)   At least five attacks.

2.)   Severe, one-sided pain in the region in and around one eye and/or the temporal region.

3.)        Associated with: 

            Eye bloodshot and tearing on side of head where pain is felt.
            Pupil may be smaller on affected side than unaffected side.
            Swelling or drooping of eyelid on side of headache.
            Runny nose on side of headache.
            Sweating of forehead or face on side of headache.

 4.)   Attacks occurring from 1 every day to 8 per day.

 What is cluster headache?

 •   Cluster “periods” last a week or longer; the usual length is a month; remissions can last months.  Dr. Stein added that sometimes patients can go 5 to 6 years between cluster episodes.

•   The side of the head where the pain is felt usually remains the same throughout a “period;” in 10% to 15% of individuals, pain may switch to the opposite side.

•   No other cause can be found, such as sinusitis or an abnormality within the brain (a tumor, unusual collection of blood vessels or aneurysms, inflammation of arteries in the scalp), or abnormalities in the teeth, etc.

Other features of cluster headache:

•   Attacks last 15 minutes to 3 hours; usual length is 45 minutes.  This is a peculiar headache in that it occurs regularly.

 •  Attacks may occur with a clock-like regularity within 24 hours; often awake individual from deep sleep.

•   Cluster periods seem to occur around the times of year when amount of sunlight is either at its longest or shortest:  January and July.

 •  During cluster period, alcohol will “trigger” attack; the rest of the time it has no effect.

 •   About 10% of individuals have a form of “Chronic Cluster Headache” where there is no period of remission.  This may develop after years of having typical “Episodic Cluster Headache.”

•   Individuals typically can’t remain still during a headache; cluster headache sufferers pace, whereas migraine sufferers tend to lie still.

How does cluster headache differ from other headaches?

                    Severity              Duration             Location                
                    of Pain                 of Pain                of Pain          Frequency

Cluster        Severe;                 Minutes, to a        Always          Regularly
Headache    said to be              few hours            one-sided      one or times
                    worst headache      a day

Migraine     Moderate              Hours,                  Can be            Sporadic;
                     to severe               sometimes days    one-sided         usually no
                                                                              or both sides    set time

Tension-      Mild or                 Hours,                  Both                Sporadic;
Type            moderate             sometimes days     sides                usually no
Headache                                                                                    set time

Epidemiology of Cluster Headache

•   Relatively rare; occurs in about 1 per 1,000 adults (migraine by comparison occurs in about 14 per 1,000 adults).

 •   Seen predominantly in men; male to female ratio is about 5 to 1 (in migraine the ratio of female to male is 3 to 1.  Dr. Stein stressed that this is a man’s disease, whereas migraine is more a woman’s disease.

•   No racial prevalence.

•   Usual age of onset is 27 to 31 (10 years later than migraine); can begin at any age.

Other features of cluster headache:

•   Men often have a ruddy appearance; face is wrinkled.

•   Typical individual is a heavy cigarette smoker; often said to be “hard driving,” aggressive.

•   Frequently seen in association with a history of high blood pressure, high cholesterol, ulcers.  Dr. Stein added that these individuals are typically classified as Type A personalities, but cluster headaches can bring them to their knees.  He also mentioned there is a doctor in Chicago who has said that the eyes of cluster headache sufferers tend to be hazel in color.

How is cluster headache diagnosed?

Dr. Stein said that typically patients suffer for many years before they’re diagnosed with cluster headaches.  As classical as it is, this condition is very often missed.  It’s a rare condition, plus men may go to their dentist first, then the eye doctor, then the ear, nose, and throat specialist, before finally seeing a neurologist about their headaches.

•   History is usually very characteristic; few other entities like it.

•   Physical exam between attacks and in “non-cluster” period normal.

•   There are no blood tests or scans of the brain to diagnose cluster headaches; other tests are normal.

•   No test diagnostic of condition; response to treatment often diagnostic.  Dr. Stein explained that the test for this condition is actually therapy for the condition, which means that the patient is diagnosed as a result of how he responds to particular therapies.

There are two types of therapy for cluster headache:

Abortive Therapy (treating the headache after it starts):

•   Over-the-counter medications

•   Butalbitol Compound (Fiorinal)

•   Midrin (this is usually given for migraine)

•   Oxygen (inhale 100% pure oxygen--can usually stop a cluster headache)

•   Ergotamine (found in Cafergot) (works fairly quickly if taken sublingually)

•   DHE (an injection)

•   Triptans (Imitrex, Maxalt, Zomig) (injection works best) (triptans have superceded ergots, DHE)

•   Courses of steroids (Prednisone)

•   Narcotics (Vicodin, Demerol, Tylenol with Codeine)

•   Nasal Lidocaine (taking it is tedious, but it is cheaper than the triptans)

Preventive Medicines

•   Verapamil (originally developed for high blood pressure)

•   Lithium (used for manic depressive illness)

•   Steroids

•   Sansert (derivative of ergotamine) (but a side effect is that it forms gristle inside the stomach, so it’s not used as much anymore)

•   Newer medications (anti-epileptic medicines which are now also used in psychiatry) include:

     •   Depakote

     •   Neurontin

     •   Topamax

Dr. Stein added that he would prescribe the above preventive medications in the sequence listed, meaning he would start a patient out on Verapamil first, and if that didn’t work, he would try Lithium, and on down the line. 

When are preventive medications used?

•   When attacks are frequent, severe, of rapid onset and often too short-lived for abortive medications to take effect.

•    When use of abortive medications is excessive (for instance, taking 2-3 injections of Imitrex daily).

•   When abortive medications only postpone the attack.

•   Some believe that early treatment of a cluster episode with preventive medications may shorten it and/or prevent appearance of chronic cluster headache.

Principles of preventive therapy in cluster headache:

•   Start medications early.

•     If attacks persist while on one medication, add a 2nd and 3rd medication, if necessary.

 •   Initial course of tapering steroids may be necessary to get headaches under control.

 •   Medications should be continued until the patient is headache-free at least 2 weeks; then gradually taper.

 •   Restart medications as soon as possible in next episode.

 Treatment of refractory cluster headache:

 •     Desensitization injections of histamine; results are mixed.

 •   Surgical procedure to ablate the upper branch of the Trigeminal Nerve; variably helpful; associated with some complications; done only when all pharmacological medications fail.

Genetics of cluster headache:

•   No clear cut pattern of inheritance; some evidence that children of cluster headache sufferers, especially boys, may be at higher risk of developing cluster headache.

•   Often family history will reveal members with cluster headache and/or migraine headache, suggesting a common underlying genetic susceptibility.  Dr. Stein added that migraine and cluster headache may be somehow linked.  He also said that currently no one can predict whether the children of a cluster headache sufferer will also be afflicted with the condition.

Cause of cluster headache:

•   No actual cause known; all theories.

•   Clock-like regularity suggests involvement of “biological clock,” the hypothalamus.  Dr. Stein said that your eye has a nerve branch that goes to the hypothalamus which tells the time of day, etc.

•   The area of the brain called Cavernous Sinus has been suggested.

•   Cluster headaches rarely begin after some sort of trauma, accident.

•   Recent observations on hypothalamic dysfunction:  PET Scan data.

Future treatment of cluster headache:

•     Phototherapy; use of lights to try to “retrain” the biological clock.  Dr. Stein said you can get “photoboxes” which emit light like sunlight.  Using these photoboxes can trick your brain into thinking it’s springtime instead of wintertime.

•    Chronotherapy; alteration of the normal circadian rhythm.  This is shifting your internal clock--move to different parts of the world.

•   Identification of genetic marker and “gene therapy.”

•   Better abortive and preventive medications.

•   Botox?  Dr. Stein said that people with cluster headaches are desperate and will try any medication.  Botox, derived from the organism causing botulism, has been injected into the skin on the face to deaden nerves, which relieves wrinkles for about 3 months.  Recently it has been tried on migraine patients, and now cluster headache patients, although Dr. Stein said it makes no sense why it might work. 

At the conclusion of Dr. Stein’s presentation, he asked for questions from the audience.

Questions and Answers, and Comments

Q.  Would using sleeping medications make a difference if cluster headaches wake a person up?

 A.  Dr. Stein’s experience is that this doesn’t work.

Dr. Stein said that it is important to be knowledgeable, to get to the doctor and be treated.  Frequently people, especially men, go for years without being diagnosed. 

One woman in the audience whose husband suffers from cluster headaches commented that frequently she can tell the day before that her husband will be getting a cluster headache, but that he doesn’t notice the changes.  Dr. Stein said that cluster headache patients typically don’t have an aura, but one male in the group said that he gets very restless before the onset of a cluster headache.

Ellen Place, a biofeedback therapist in the audience, mentioned that she has known some patients who’ve had success taking Atavan, a muscle relaxant. 

Dr. Stein made the comment that men have been known to commit suicide because the pain of their cluster headaches was just too much to bear.  He said using counterpain might help.  This somehow blocks the pain of migraine and cluster headache.  One male in the group said that he put furniture on the back of his neck to produce counterpain, and it helped for a week.

Dr. Stein said that he doesn’t think cluster headache is brought on by stress, however it may be one minor factor.

Dr. Stein said that Inderal, a channel blocker used for heart patients and sometimes used as a preventive medication for migraine patients, doesn’t work in preventing cluster headaches.  And Lithium doesn’t work with migraines, but it does help to prevent cluster headaches in some individuals.

Q.  One female participant said she has been taking Imitrex since it first came out.  Should she change to a different abortive medication?

 A.  Dr. Stein answered that if a person suffers from 2 or more migraine headaches per month, or their headaches are disabling, that person should be using preventive medications.  Elavil was discussed as a preventive medication.  Dr. Stein said that, in his experience, low doses of Elavil don’t work as well as Inderal as a preventive.  The dose needs to be built up.  One participant commented that amitryptilene (Elavil) was sedating, and Dr. Stein said that nortryptilene (Pamelor) is a little less so.

Q.  Can diuretics trigger migraines?

 A.  Typically “no.”  Oftentimes a woman with menstrual migraine will take a diuretic and the migraine will be relieved.  Some people think water retention causes headaches.

Q.  Are there any long-term side effects of Imitrex?

A.  Dr. Stein has seen patients who take 2 Imitrex per day, and it doesn’t seem to harm them.  He added, though, that it is best not to use it that often.

The intention of the East Bay Headache Support Group is to provide information and resources.  It does not provide medical advice, which should be obtained directly from a physician.