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Classification and external resources
Hemicrania continua (HC) is a persistent unilateral headache that responds to indomethacin. It is usually unremitting,
but rare cases of remission have been documented. Hemicrania
continua is considered a primary headache disorder, meaning that
it's not caused by another condition.
International Headache Society's International Classification of
Headache Disorders classifies hemicrania continua as a primary
The following diagnostic criteria are given for hemicrania
- Headache for more than 3 months fulfilling other 3
- All of the following characteristics:
- Unilateral pain without side-shift
- Daily and continuous, without pain-free periods
- Moderate intensity, but with exacerbations of severe pain
- At least one of the following autonomic features occurs during
exacerbations and ipsilateral to the side of pain:
- Complete response to therapeutic doses of indomethacin
A variant on hemicrania continua has also been described, in
which the attacks may shift sides, although meeting the above
criteria in all other respects.
Hemicrania continua was first described in 1981,
at that time around 130 cases were described in the literature.
However, rising awareness of the condition has led to increasingly
frequent diagnosis in headache clinics, and it seems that it is not
as rare as these figures would imply. The condition occurs more
often in women than men and tends to first present in adulthood,
although it has also been reported in children as young as 5 years
The cause of hemicrania continua is unknown. There is no
definitive diagnostic test for hemicrania continua. Diagnostic
tests such as imaging studies may be ordered to rule out other
causes for the headache. When the symptoms of hemicrania continua
are present, it's considered "diagnostic" if they respond
completely to indomethacin.
The factor that allows hemicrania continua and its exacerbations
to be differentiated from migraine and cluster headache is that hemicrania
continua is completely responsive to indomethacin. Triptans
and other abortive medications do not affect hemicrania
In addition to persistent daily headache of HC, which is usually
mild to moderate, HC can present other symptoms.
These additional symptoms of HC can be divided into three main
- Autonomic symptoms:
- conjunctival injection
- nasal stuffiness
- eyelid edema
- forehead sweating
- Stabbing headaches:
- Short, "jabbing" headaches superimposed over the persistent
- Usually lasting less than one minute.
- Migrainous features:
Hemicrania continua generally responds only to indomethacin 25-300 mg daily, which
must be continued long term. Unfortunately, gastrointestinal side
effects are a common problem with indomethacin, which may require
additional acid-suppression therapy to control.
In patients who are unable to tolerate indomethacin, the use of
400-800 mg per day (Celebrex) and rofecoxib 50 mg per day (Vioxx - no
longer available) have both been shown to be effective and are
likely to be associated with fewer GI side effects. There
have also been reports of two patients who were successfully
managed with topiramate 100-200 mg per day (Topamax)
although side effects with this treatment can also prove
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