In the simplest of terms, paroxysmal hemicrania
(PH) is a rare form of headache that usually begins in
Characteristics of the pain of PH
- severe throbbing
- claw-like, or
- usually on one side of the face; in, around, or behind
- occasionally reaching to the back of the neck
The pain of PH may be accompanied by:
- red and tearing eyes (lacrimation)
- a drooping or
swollen eyelid on the affected side of the face
- dull pain, soreness, or
tenderness between attacks
Episodes of paroxysmal hemicrania
typically occur from 5 to 40 times per day and last 2 to 45 minutes.
The disorder has two forms:
- Chronic PH: patients experience
attacks on a daily basis for a year or more
- Episodic PH: the headaches may remit for months or years
Certain movements of
the head or neck or external pressure to the neck may trigger these
headaches in some patients. The disorder is more common in women
than in men.
Is there any treatment?
The nonsteroidal anti-inflammatory drug (NSAID) indomethacin often provides complete
relief from symptoms. Other less effective NSAIDs including
celecoxib (Celebrex), calcium-channel
blocking drugs (such as
verapamil), and corticosteroids may be used
to treat the disorder. Patients with both paroxysmal hemicrania and
trigeminal neuralgia (a condition of the 5th cranial nerve that
causes sudden, severe pain typically felt on one side of the jaw or
cheek) should receive treatment for each disorder.
What is the prognosis?
Many patients experience complete to near-complete relief of symptoms following
physician-supervised medical treatment. Paroxysmal hemicrania may
last indefinitely but has been known to go into remission or stop
Information from the International Headache
Society's International Classification of Headache Disorders, 2nd Edition, is
the best description of CP available:
3.2 Paroxysmal hemicrania
Attacks with similar characteristics of pain and associated
symptoms and signs to those of cluster
headache, but they are shorter-lasting, more frequent,
occur more commonly in females and
respond absolutely to indomethacin.
- At least 20 attacks fulfilling criteria B–D
- Attacks of severe unilateral orbital, supraorbital
or temporal pain lasting 2–30 minutes
- Headache is accompanied by at least one of the
- ipsilateral (on the same side as
the headache) conjunctival
injection (The forcing of a fluid into the conjuctiva, the mucous membrane that
lines the eyelids.) and/or
- ipsilateral nasal congestion and/or rhinorrhoea
- ipsilateral eyelid oedema
- ipsilateral forehead and facial sweating
- ipsilateral miosis (abnormal
contraction of the pupils) and/or ptosis (drooping of the eyelid)
- Attacks have a frequency above 5 per day for
more than half of the time, although periods with
lower frequency may occur
- Attacks are prevented completely by therapeutic
doses of indomethacin (Note 1)
- Not attributed to another disorder
- In order to rule out incomplete response,
indomethacin should be used in a dose of 150mg or more
daily orally or rectally, or 100 mg or by injection,
but for maintenance smaller doses are often
- History and physical and neurological examinations
do not suggest any of the disorders listed in
groups 5–12, or history and/or physical and/or
neurological examinations do suggest such disorder
but it is ruled out by appropriate investigations,
or such disorder is present but attacks do
not occur for the first time in close temporal relation
to the disorder.
There is no male predominance. Onset is usually in
adulthood, although childhood cases are reported.
In the first edition all paroxysmal hemicranias
were referred to as chronic paroxysmal hemicrania. Sufficient
clinical evidence for the episodic subtype has
accumulated to separate it in a manner analogous to
Paroxysmal hemicrania with coexistent trigeminal
neuralgia (CPH-tic syndrome):
Patients who fulfill criteria for both 3.2 Paroxysmal
hemicrania and 13.1 Trigeminal neuralgia should
receive both diagnoses. The importance of this
observation is that both conditions require treatment.
The pathophysiological significance of the
association is not yet clear.
Summary and comments:
Paroxysmal Hemicrania (PH) is rare
and difficult to treat. In most cases, patients with PH should
seek care from a headache and Migraine specialist. Some of the
symptoms, to an untrained practitioner could be mistaken for cluster headache. The response to indomethacin is one
confirmation of a PH diagnosis. However, indomethacin is not
well tolerated by some patients. If that occurs, other less
effective NSAIDs including celecoxib (Celebrex), calcium-channel
blocking drugs (such as
verapamil), and corticosteroids may be
The International Headache Society.
"International Classification of Headache Disorders, 2nd Edition." Cephalalgia, Volume 24 Issue s1. May, 2004.
Silberstein, Stephen D.; Lipton, Richard B.;
Goadsby, Peter J.; Smith, Robert T. "Headache in Primary Care." Isis Medical
Young, William B.; Silberstein, Stephen D.
"Migraine and Other Headaches." AAN Press. 2004.
Goadsby, Peter J.; Silberstein, Stephen D.;
Dodick, David W. "Chronic Daily Headache for Clinicians." BC Decker Inc. 2005.
Teri Robert, 2006 - Present
Last updated November 23, 2011.
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