Herbert G. Markley, M.D.
New England Regional Headache Center
Of all the different types of headaches, a special
category causes unusual symptoms caused by activation
of the autonomic nervous system of the trigeminal
nerve in the face, hence the name trigeminal
autonomic cephalalgia (abbreviated TACs). These
autonomic features include redness and watering of
the eye (conjunctival injection and lacrimation),
one-sided nasal congestion or discharge (rhinorrhea),
forehead and facial sweating, constriction of a pupil,
as well as drooping and swelling of an eyelid (miosis,
ptosis and eyelid edema).
Cluster headache -
Patients have recurrent attacks of severe one-sided
pain in and around one eye or in the temple. The pain
is steady, knifelike and burning, one of the most
intense pains known to man. The pain lasts from 15
to 180 minutes, but occurs up to 8 times per day.
The patients have some or all of the autonomic symptoms
on the side of the headache. In the episodic form
of cluster headache there may be daily attacks for
a few weeks (clustering), followed by complete remission
for weeks to years. The secondary chronic form, daily
attacks without remission, eventually evolves from
the episodic form in about 10%. However, the very
rare primary chronic form begins with daily typical
headaches that never remit. Cluster headache is fairly
rare, estimated to afflict about 0.5% of the population,
10-40 times less common than migraine. It is predominantly
a male disorder, with a male/female ratio of 5.6 to
The mechanisms of the
pain of cluster headache, as with all the TACs, are
unknown - despite abnormalities demonstrated in the
deep brain, in functional imaging and in a host of
trigeminal autonomic functions. The attack probably
is initiated by rapid firing of neurons in the hypothalamus,
near the neurons responsible for setting autonomic
rhythms such as sleep-wake cycle and body temperature.
There is typical behavior
associated with the cluster pain. Patients pace about
and cannot lie still. They carry out irrational, violent,
Acute therapy for each
attack is limited by the rapid onset and short, repetitive
nature of the attacks. Analgesics are effective only
if they have a very rapid onset, such as injectable
narcotics. Inhalation of pure oxygen is effective
in 75%. The best acute therapy is injectable sumatriptan,
having been proven effective in a European multi-center
trial, in which every patient responded. Zolmitriptan
nasal spray may also be effective.
therapy is the best treatment for cluster headache.
Drugs of choice at present are the calcium-channel
blockers, which offer the safest, most effective treatment.
In the US, verapamil is the first choice, but the
rest of the world relies on another calcium channel
blocker, flunarizine. These drugs may provide up to
100% relief, and are effective in both episodic and
chronic cluster. Other effective prophylactics, divalproex
and lithium carbonate, can be effective as well. Newer
drugs, such as topiramate, recently approved for migraine,
may also help cluster, with less adverse effects.
Corticosteroids may provide marked temporary improvement,
but their benefits must be weighed against substantial
risks, many of which are severe. Indomethacin, so
useful in other TACs, is only occasionally helpful
in cluster headache.
Drugs which have proven to be NOT helpful include
antidepressants and beta blockers.
Deep brain stimulation
may be very effective in selective intractable cases
of chronic cluster, but significant adverse effects,
including neurological damage or worsened pain, may
Chronic paroxysmal hemicrania
(CPH) is a disorder
with attacks with the same characteristics of pain,
associated symptoms and signs as cluster headache,
but the attacks are much shorter and more frequent.
Pain is side-locked, as in cluster headache, but occurs
mostly in females, unlike cluster headache. The headaches
are absolutely responsive to indomethacin.
Most attacks last 2 to
20 minutes, never more than 45 minutes, while frequency
may be as high as 30 attacks in 24 hours. There is
at least one autonomic sign or symptom on the painful
side, including conjunctival injection, lacrimation
or nasal congestion. Long lasting remission (clustering)
is not seen, but the frequency, duration and severity
of attacks are variable. Migraine features such as
nausea/vomiting may rarely accompany attacks. The
major diagnostic criterion is absolute effectiveness
of indomethacin. The continuous, chronic stage may
be preceded by an episodic stage similar to that seen
in cluster headache.
Unilateral Neuralgiform headache attacks with Conjunctival
injection, Tearing, sweating, and rhinorrhea).
SUNCT is a trigeminal autonomic cephalalgia which
usually presents with a long clinical history of unilateral
headache and eye pain, side-locked, with neuralgic
pain paroxysms of short duration (15-60 sec), and
high frequency (5-30 times per hour). The cause is
unknown. Trigeminal autonomic symptoms and signs are
severe, with intense conjunctival injection for the
duration of the pains, massive lacrimation, forehead
sweating and rhinorrhea, all on the symptomatic side.
The attacks can be precipitated by jaw movements such
as chewing, or eating or movements of the head and
neck. The headache is completely refractory to drug
therapy, including indomethacin.
symptoms with hemicrania (LASH)
is the rarest of the TACs, and has been only recently
described. Patients suffer attacks of autonomic symptoms
such as ptosis, eyelid edema, lacrimation and nasal
congestion which precede headache by 3 - 4 hours.
This is then followed by severe side-locked headache,
with continued autonomic symptoms. Unlike the other
TACs, there are migrainous features like nausea, vomiting
and photosonophobia. These attacks last much longer
(1-3 days), and may occur at a frequency of once per
week. Unlike migraine, these attacks are completely
responsive to indomethacin, but show no response to
verapamil, oxygen, sumatriptan or opiates.
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