Hemicrania continua is an uncommon form of chronic daily headache. The name basically signifies that half (hemi) of the head (crania) hurts all the time (continua). It is in fact a continuous, one-sided headache of moderate severity, with episodes of severe pain and with associated migraine-like features (nausea, sensitivity to light and sound), as well as some cluster headache-like features (tearing, redness of the eye, droopy eyelid).
Hemicrania continua is generally considered to be a rare form of headache, although a recent report from our center described 34 new cases diagnosed over a 3-year period (out of a total of 3,000-4,000 headache patients seen over that time period). Correctly distinguishing hemicrania continua from other forms of chronic daily headache is very important because hemicrania continua almost always responds rapidly to indomethacin (Indocin), a nonsteroidal anti-inflammatory (NSAID) medication.
Hemicrania continua exists in two forms–continuous and remitting (comes and goes). The daily headache bouts of the remitting form last from 1 to 6 months, separated by pain-free periods of 2 weeks to 6 months. The continuous variety can be continuous from the start or evolve from the remitting pattern.
In addition to the pain, there are three main categories of symptoms: the cluster headache-like symptoms, “jabs and jolts,” and migraine-like features. Cluster headache-like symptoms consist of eye redness, tearing, runny nose, nasal stuffiness, eyelid swelling, and forehead sweating. These symptoms are not as prominent in hemicrania continua as they are in cluster headache. Symptoms of eye discomfort, in particular the sensation of sand in the eye, have been described by patients with hemicrania continua.
The jabs and jolts syndrome consists of sharp pains that lasts less than 1 minute (usually seconds). They can occur in headache-free individuals or people with tension-type headache, migraine or those with cluster headache. They also respond to indomethacin. Jabs and jolts occur in hemicrania continua, more frequently in the episodes of worsened headache. Migraine-like features (nausea, vomiting, light and sound sensitivity) are common in hemicrania continua, again, particularly during the episodes of worsened headache.
The cause or mechanism of hemicrania continua is unknown. It has been speculated that it might be a migraine variant, since many patients have migraine-like features. There are more females than males with hemicrania continua, as is the case with migraine. The mechanisms explaining the peculiar, often almost magical response to the medicine indomethacin are still unknown. Hemicrania continua is only one of several “indomethacin-responsive headaches” that have this special sensitivity to indomethacin. Because hemicrania continua can have features of both migraine and cluster headache, the diagnosis may not be clear until indomethacin is tried.
Most patients I see who have chronic daily headaches that never ever change sides get an indomethacin trial, even if I think the likelihood that the patient has hemicrania continua is low. The reason for this is that headache preventives generally take 4 to 8 weeks to work, while an indomethacin trial takes less than 1 week. Since nothing else works like indomethacin, and the diagnosis of hemicrania continua requires a trial of indomethacin, it makes sense to try the indomethacin early on. There is no single formula for an indomethacin trial, but the general concept is to start at a low dose which increases over about 1 week to the highest safe dose. If the “miraculous” indomethacin effect occurs, of course, the dose is not increased further.
The dose of indomethacin required to treat hemicrania continua varies greatly. I have a patient who takes a very low dose (25 mg) which works for about 72 hours, at which time a slight headache develops and he takes his next dose. Other patients require between 200 and 250 mg of indomethacin each day in divided doses.
In the long run many patients cannot tolerate indomethacin because of its irritating effects on the stomach. There is also a higher risk of kidney damage with this medicine than with other medicines in its class. Occasionally indomethacin makes people feel tired or ill in some other way. For these reasons we sometimes have to settle for less effective NSAID medications with fewer side effects and potential risks. Some of our patients have responded to the newest class of NSAIDs (Cox-2 inhibitors), particularly rofecoxib (Vioxx).
–William B. Young, MD. Assistant Professor, Department of Neurology, and Jefferson Headache Center, Thomas Jefferson University. Philadelphia, PA
Hemicrania continua is suspected if …
· Pain is continuous but only on one side of the head
· Pain is moderate with occasional episodes of severe pain
· During the more severe pain episodes, many patients will have additional symptoms, such as:
– Red or tearing eyes or drooping eyelid
– Nasal congestion or runny nose
– Nausea
– Light or sound sensitivity
· Brief stabbing pains may occur in addition to the daily continuous headache
If you have continuous daily headaches that are always one-sided, ask your doctor whether you might have hemicrania continua and should try indomethacin.
From Headache, The Newsletter of ACHE. Spring 2002, vol. 13, no. 1.