CLUSTER
HEADACHE
THE MOST SEVERE HEADACHE
George Urban, MD
Co-Director Diamond Headache Clinic
Diamond Inpatient Headache Unit
Presence-Saint Joseph Hospital
Chicago, Illinois
Cluster Headache has been described as the most painful
type of headache one can endure. Fortunately, it is not
very common. Cluster headache is one of the primary
headaches that is not caused by other disorders. Although
this headache was recognized for at least a century, the
term, Cluster Headache, was coined relatively recently,
in 1952 by Doctor E. Charles Kunkle and his colleagues.
Prior to this title, cluster headache was known under
a variety of names including: Migrainous neuralgia;
Horton’s headache; Harris-Horton’s disease; Histaminic
Cephalalgia; Ciliary neuralgia; and, Erythromelalgia of
the head.
In 1745, Gerhard van Swieten presented a case of a
“healthy, robust man of middle age [who] was suffering
from troublesome pain which came on every day at the
same hour at the same spot above the orbit of left eye…”
This description fulfills the current diagnostic criteria of
Cluster Headache.
How common is this disorder?
Cluster headache is a relatively uncommon headache
disorder. The prevalence is about 10 per 100,000 in male
patients, which is about 19 to 25 times less than the
prevalence of migraine headache in men. Most cluster
headache sufferers are males, with the gender ratio varying
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from 3.5:1 to 6.7:1 in favor of males. No racial, ethnic,
or genetic factor has been documented. Cluster headache
can begin at any age, but the average mean age of onset is
during the late 20s and early 30s.
What is characteristic for
this condition?
The acute attack of cluster headache occurs suddenly,
without any warning. It peaks in intensity within 10 to 15
minutes, and lasts usually from 40 to 90 minutes. The pain
is excruciating and described most commonly as boring,
burning, piercing, sharp, knife-like, or “a hot poker in
the eye.” A soreness or lingering dull pain may persist for
hours after the main attack. The pain is strictly one-sided,
and occurs on the same side at the same location during
a cycle. It may change sides in the next cycle. The typical
location is at the temple, above the eye, or behind the
eye. The area of pain is small, and often the patient can
point to the pain’s location with the tip of a finger. The
headache has been described as the most severe form of
pain a human can endure. It is not unusual for women
who experience cluster headache to describe the pain as
worse than the pain associated with labor and delivery. The
cluster headache has been nicknamed by some sufferers as
the “killer” or “suicide” headache.
The headache episodes occur in peculiar periodicity. The
term “cluster” derives from attacks occurring repeatedly
within a relatively limited time span, called the period,
cycle, bout, or cluster. On average, a cluster cycle lasts
6 to 12 weeks. Many patients experience one cycle
every year, usually at the same time each year. Typically,
cluster headaches manifest during spring or autumn, a
phenomenon called circannual rhythm. During the cycle,
the attacks appear daily, usually once a day at the same
time–circadian periodicity. In between the cycles, the
patient is completely headache-free.
More than 50 percent of cluster patients report that the
attacks occur at night, most commonly about 2 to 3 hours
after falling asleep. Many patients will have their attacks at
a predictable rate. The number of attacks usually do not
exceed three in 24 hours. However, some patients with
chronic cluster headache may experience more than five
attacks a day.
The typical symptoms, which must be present for
the correct diagnosis, are autonomic responses. These
responses include eye tearing and redness, runny nose and
congestion, sweating of half of the face, and sometimes
a droopy eyelid. These symptoms occur on the same side
of the headache, and continue throughout the entire
attack. Nasal congestion and runny nose may lead to an
incorrect diagnosis of sinus headache or sinus infection.
Some patients may also experience nausea, vertigo, neck
muscle stiffness or tenderness, and possibly fluctuations in
the heart rate and blood pressure.
Another distinguishing feature of cluster headache is
the behavior of patients during the attacks. As opposed
to migraine sufferers,