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Chronic Paroxysmal Hemicrania (CPH) - Excerpt from Book
Excerpted from Dr. Robbins
Posted April 2000
Excerpt from 2nd Edition of Mangagement of Headache and Headache Medications

Chronic Paroxysmal Hemicrania (CPH)
CPH may be a variation on chronic cluster headache, but this remains unclear. CPH has a female-to-male ratio of 3:1, with a typical onset from age 25 to 35. The pain is usually about the ophthalmic division of the trigeminal nerve, concentrated around the eye, temple, and forehead. However, pain may occur in the occiput or periaural areas. While almost exclusively unilateral, there have been rare instances of bilateral pain. The attacks last from 2 to 45 minutes, with a frequency of 5 to 20 or more per day. The usual attack lasts from 10 to 15 minutes. Occasionally, head movement or mechanical stimulation may precipitate the pain. The pain is associated with at least one of the following: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, ptosis, or eyelid edema. Except for the ptosis, these are mediated by parasympathetic nerves. The pain tends to be very severe, and is usually stabbing or severely aching. However, at the beginning of the headache, it may be throbbing. There is no clear increase at one particular time of day, and CPH may awaken patients from sleep. In the unusual situation where there is a break of at least a few months (or possibly years), we would term it episodic paroxysmal hemicrania.

Since CPH is rare, a workup to exclude secondary causes may be appropriate. These would include tumors, collagen vascular disease, cerebrovascular disease, and an aneurysm. MRI and MRA, along with routine lab tests including the sedimentation rate, would be prudent. If extremely high doses of medication are required, we think about secondary causes of CPH. With bilateral CPH, intracranial hypertension may need to be excluded by a lumbar puncture (LP). In very rare circumstances, a chest x-ray may be necessary to discover a Pancoast tumor as the cause of the CPH. Arteriovenous malformations (AVMs) have been reported to be a secondary cause of CPH.

Medication Treatment of CPH
CPH is almost always relieved by indomethacin (Indocin). If indomethacin does not help, the diagnosis of CPH is in doubt (although it still could be CPH). The dose of indomethacin varies greatly with some patients requiring as little as 25mg per day and others needing 250mg or more. Although the Indocin SR 75mg renders dosing more convenient, the 25 or 50mg capsules, taken throughout the day, may be more effective. Patients may titrate their own dose, for at times the attacks may decrease in severity. Usually, when Indocin is tapered or stopped, the attacks resume, but long term remissions may occur. Indomethacin should be taken with food, as GI upset is very common. Although headache may occur as a side effect of indomethacin, it is not common in patients with preexisting headaches. Cognitive side effects, such as fatigue, lightheadedness, and mood swings, may be a problem with indomethacin. Retinal or corneal problems have been reported with long term us of indomethacin. As with all of the anti-inflammatories, renal and hepatic functions need to be monitored through blood tests. Tachyphylaxis does not usually occur with indomethacin.

Corticosteroids, naproxen, and calcium blockers (verapamil) may provide some benefit, but these have limited usefulness in CPH. Acetazolamide may be of benefit in some patients. The triptans do not appear to be particularly effective for CPH.