Newport Beach Neurologists

Cluster Headaches

by C. Philip O'Carroll, M.D.

The following is Dr. Lee Kudrow's personal account of a cluster headache, perhaps one of the most harrowing examples of pain that a human being can suffer. Few pains can incite such feelings of rage, panic and thoughts of self-destruction.

Following a period of perhaps several hours, during which I feel quite elated and energetic, I experience a fullness in my ears, somewhat more on the right side than on the left, having a character not unlike that which occurs during rapid descent in an airplane or an elevator. I next become aware of a dull discomfort and extension of ear fullness in the base of my skull - further extending over the entire head on both sides, although somewhat more on the right. At this point, two or three minutes have elapsed, seemingly short but long enough for me to know that indeed a 'cluster' has begun and will ultimately get worse. Such anticipation causes me considerable consternation regarding my decision to continue my activities or cancel plans and find a place to be alone; this gives way to a slowly increasing fear, anxiety, panic and withdrawal. I become aware of myself listening "to changes in my head." Is the cluster prematurely aborting itself, progressing further or unchanging?

A sudden stab only fleeting strikes my temple, then again - somewhere near the apex of my skull and upper molars in my face - always on the right side. It strikes me again, deep into the skull base and has quickly changed location to a small area above my eyebrow. My nose is stuffed yet runs simultaneously. If I can sneeze, I feel the attack within, but in spite of all tricks, I find myself unable to induce sneezing.

While the sharp stabs continue in this fashion, a slow crescendo of dull pain presents itself in an area of hands length and breadth over the eye and temporal area. The area of pain narrows into a smaller area but as this magnifies and enlarges in intensity, I find myself bending my head downward, though slightly, as if my head were being slowly pushed from behind. My neck, up in the base of my skull, is tight; it feels as though I am wearing a neck collar. I am compelled to remove my tie and loosen my shirt collar, even though I know that it will not offer me even a modicum of relief. In an attempt to alter this persistent discomfort, I drop my head between my legs while seated. My face and eyes seem to feel a slough, but the pain remains unchanged. In spite of my suntan, as I look into the mirror, a gaunt pale face peers back. My right eyelid is swollen and slightly drooping and the white of my eye is charted with many red vessels, giving it an overall color of pink.

Having difficulty standing in one place too long, I leave the mirror to continue my alternating pacing and sitting. As usual, I am stuck with the additional fear that the pain will never end, but I dismiss it as impossible. Even if it were the case, I would surely kill myself.

The pain, now located somewhere behind my eye and slightly above, worsens. The pain is best described as a "force", pushing with some incredible power to my eye that my head appears to be moving backward, yielding its resistance. The "force" waxes and wanes, but the duration of successive exacerbation seems to increase. The cluster is at its peak, which is celebrated by an outpouring of tears from my right eye only. I have now been in cluster for 35 minutes - 10 minutes at its peak.

My wife peeks into the room in which I hold fort. I look up and see her expression of pity, frustration and helplessness. She sees my tortured face as I have seen it in the mirror at this stage before; a drooling mouth, a gape, gray face, wet on one side and almost closed eyelid with an expression of pain and anguish. She closes the door and leaves, feeling hurt for me, anger for the stupidity of medical science and guilt - since deep within her mind is the suspicion that she is the cause of my suffering. I cry for her but more for myself. The pain is so incredible.

Suddenly I am overwhelmed by a fury. I lift a chair high above my head and crash it to the floor. With a double fist I strike the wall. The pain persists.

Waiting periods soon become longer in duration, and I allow myself to suspect that the peak is behind me - but cautiously, since I have been too often disappointed.

Indeed, the pain is ending. The descent from the mountain of pain is rapid. The "force" is gone. Only severe pain remains. My nose and eye continue to run. The road back, as with all travel, covers the same territory - but faster. Stabbing, easily tolerated pain is felt and gone. Dull, aching fullness, stiff neck - all disappearing in turn to be replaced by a welcome sensation of pins and needles over the right scalp area, not unlike after one's leg has been "asleep." Thus my head is awakened after a nightmare of torment.

Eye and nose dry, I let out a sigh. I collect my pile of what tissue has been thrown on the floor and deposit them in a wastepaper basket. The innocent chair now uprighted, I rub my slightly bruised fist. Having ended the battle and cleaned up its field, I open the door and enter my pain-free world - until tomorrow.

* Lee Kudrow, M.D., Cluster Headache, Oxford University Press, 1980.

CLUSTER

The term "cluster" was introduced in the 1950's to signify the occurrence of this form of headache and paroxysms, bursts or "clusters."

Cluster headache, as opposed to migraine, occurs primarily in males in a ratio of about 5 to 1 or 5:1. It can occur at any age, although the majority occur between the ages of 20 and 40.

TYPES OF CLUSTER

1. Episodic Cluster Headache is the most well known and common pattern. It is characterized by recurring bouts of clusters of headaches, which regularly and usually daily. Each period of cluster lasts several weeks to several months, followed by spontaneous remission lasting months to years.

2. Chronic Cluster Headache is divided into a primary or secondary chronic form. The primary chronic cluster headache pattern is characterized by recurring headache events which undergo no periods of remission.

   The secondary chronic cluster headache is characterized by a pattern of running headache attacks which have become chronic. This may have evolved from a previous episodic pattern. The absence of remission for a year or more justifies the term "chronic."

SIGNS AND SYMPTOMS

The pain of cluster headache is brief in duration. The attacks last from a few minutes to several hours, most often 30-45 minutes. Rarely is the pain present longer than four hours. The pain is extraordinarily distressing. Forceful, tortured, pressing, throbbing, burning, lancinating, screwing, piercing and stabbing are but a few of the descriptions used by patients. One patient described a red hot iron being pressed into his forehead and eye until it had burned its way through his skull.

The attacks are usually one-sided, around an eye, temple or forehead. Few people have experienced their pain primarily in the neck with minor radiation to temples or eye sockets. A substantial number of patients experience pain in the lower half of the face. Radiation of the pain into the jaw, nose, chin and teeth is also common.

ADDITIONAL SIGNS AND SYMPTOMS

Usually the pain is accompanied by other findings. Most common is tearing of the eye on the same side as the head pain associated with redness of the eyes and nasal stuffiness with drainage. Drooping of the eyelid on the effected side, pupil changes, unilateral or bilateral facial swelling, and facial flushing are frequently seen. Another associated finding is the tendency of those experiencing an attack to pace, rock or otherwise act out in anguish.

OTHER DIAGNOSTIC CLUES

In the 1960s, Dr. John Graham first identified a particular facial appearance which occurred in many patients with cluster headache. He describes this appearance as "lionized" or lion-like. These men had very prominent skin lines, a thick-skinned appearance, a square thickly-upholstered chin, with a sharp crease between it and a well chiseled lower lip. They usually have thick furrows in their foreheads. Similar facial characteristics are also present in women (tending to a more "masculine" appearance). The patients are almost always heavy drinkers and smokers.

TIMING

During a cluster period, one or more attacks occur daily. Although the average frequency is one to three attacks per day, up to six attacks during a 24 hour period can occur. During the early or late stages in the cluster period, headaches may take place a few days per week. A headache may begin at any time of the day or night. Repetitive nightly awakening is very common and often occurs around the same time. The adverse effect of sleep is shown by provocation of daytime napping.

CAUSES OF CLUSTER HEADACHES

The cause of cluster headaches is not known, but there is speculation regarding a disturbance in the hypothalamus.

The hypothalamus is the part of the brain which regulates body cycles, appetite and hormone secretion. The idea of a hypothalamic origin for cluster headaches is reported by the cyclical pattern of the disorder.

TRIGGERS

Like migraine, cluster headaches may also be provoked by external stimuli, especially alcohol. The majority of cluster sufferers are sensitive to alcohol during the cluster period. During their remission, alcohol can be consumed without triggering headaches. Additional precipitating influences include 1) cold wind or heat blown into the face, 2) blood vessel expanding substances in food in medication (especially nitrites), 3) let down from work or excitement and sleep.

TREATMENT

1. (Non Drug Treatment): Patients are strongly advised to discontinue all alcohol and avoid daytime napping. If necessary, drug therapy is used to normalize sleep cycles. Patients are also urged to reduce or discontinue smoking.

   Inhalation of 100% oxygen is an effective means of relieving the pain in many patients. Oxygen therapy must begin very early in the attack along with a mask to administer it.

2. (Drug Treatment): Ergotamine products are usually the most effective in aborting the attack. They may reverse the attack within minutes, either in spray, suppositories or injectable form. However, they need to be used daily, exposing the person to the problems accompanying the use of daily Ergot alkaloids.

   MB: Injectable narcotic pain killers are NOT advisable for the symptomatic treatment of cluster headache because of the tendency for overuse. Oral pain killers are usually without value, although over the counter sinus preparations have been reported to help some patients.

3. Preventive Treatment: It is preferable to prevent cluster headaches instead of treating the symptoms. However, it must be stated that NO medication can be consistently relied upon to provide effective preventive therapy.
   1. Calcium Channel Blockers - In The Headache Institute, we use these drugs regularly, especially Verapamil (Isoptin or Calan). Procardia and Cardizem may also be used. The dosage must be individualized.

   2. Sansert - This must be used for short periods and for patients whom safety considerations have been taken into account.

   3. Prednisone - This is a form of steroid (Cortisone like drug) and can be dramatically effective for short term use. It must be used early in the cluster cycle to be effective. The drug is used in high doses (60 mg.) for 7-10 days and then tapered slowly. Because of its multiple side effects, including reduction of immunity against infection, weight gain and ulcers, aggravation of high blood pressure and diabetes, this drug can be used only for short periods.

   4. Lithium Carbonate: (Eskalith, Lithobid) - This is useful in over 60% of patients. Lithium was first useful in the treatment of manic depressive disease, a psychiatric disorder characterized by severe mood swings. The mechanism which Lithium helps cluster headaches is not known but may be related to its effect on biologic cycles controlled by the hypothalamus.

      Lithium usage must be individualized. Lab tests should be done regularly to measure blood levels and various consequences of usage, including the effects on the kidneys and thyroid.

      Side effects may include nausea, vomiting, diarrhea, tremor, blurred vision, unsteadiness and aggravation of certain skin conditions such as psoriasis.

      Patients using Lithium must not avoid salt or take diuretics (water pills) because this can make Lithium more toxic. Salt replacement or reduction in dosage is sometimes necessary during hot summer months when salt depletion is common, or during periods of active perspiration. Lithium can be combined with calcium channel blockers and several other medications useful for cluster headaches.

   5. Histamine Desensitization - Is an old therapy but one which still has value. This process requires hospitalization to desensitize the individual to Histamine, requiring intravenous injections.

   Recently, certain surgical procedures have been found to help some patients with cluster headache. This treatment should be reserved for only the most difficult and hard to treat cases. Local infiltration of the sphenopalatine ganglion with cocaine can be attempted two to three times weekly as long as symptoms persists. Though this therapy consistently relieves the patient's complaints, on occasional patient may benefit from cryotherapy of the sphenopalatine ganglion.

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