Abstract
Chronic paroxysmal hemicrania (CPH) was first described by Sjaastad who also described a remitting form of this condition (1, 2). This new entity was named episodic paroxysmal hemicrania (EPH) in 1987 by Kudrow (3). It is characterized by brief, frequent attacks of unilateral orbital or temporal pain with associated autonomic symptoms. Most cases respond to indomethacin. A seasonal variant of EPH has been described (4), but never a response to treatment with cyclooxygenase (COX)-2 inhibitors.
Chronic paroxysmal hemicrania (CPH) was first described by Sjaastad who also described a remitting form of this condition (1, 2). This new entity was named episodic paroxysmal hemicrania (EPH) in 1987 by Kudrow (3). It is characterized by brief, frequent attacks of unilateral orbital or temporal pain with associated autonomic symptoms. Most cases respond to indomethacin. A seasonal variant of EPH has been described (4), but never a response to treatment with cyclooxygenase (COX)-2 inhibitors.
Case history
A 66-year-old woman presents with a 30-year history of headaches. She describes the pain as severe, occurring three to four times daily over the right temporal, frontal, and parietal region, and lasting 20 min There is associated nasal congestion and lacrimation of the right eye. Two years ago, her headache became seasonal, with cycles in autumn (October) and spring (March). The cycles last two to three months, after which she is pain-free until the next cycle begins. She has hypertension and her son has cluster headaches. Her physical and neurological examinations and a head computed tomography were normal. Various preventive medications, including amitriptyline, propranolol, atenolol, nifedipine, valproic acid and topiramate were of no benefit. The combination analgesic of aspirin, caffeine and butalbital brings minimal relief.
Indomethacin was started at 25 mg daily and she became pain free. Routine blood work performed after indomethacin initiation revealed an elevated creatinine level. The medication was discontinued. A nephrologist was consulted and it was agreed that the COX-2 inhibitors could be tried. She was then put on valdecoxib, 10 mg twice a day for nine days with no relief. This was then changed to celecoxib, 200 mg twice a day, from which she obtained substantial but not total relief. She was then switched to rofecoxib and at 50 mg once a day, she became completely pain free. She took rofecoxib for 4 weeks and remained pain free during this time. She then had a repeat creatinine level checked which was again elevated. On advice of her nephrologist, she was taken off rofecoxib and her headaches returned almost immediately. She continued to get headaches which gradually tapered off in intensity and frequency until she went out of cycle two months later.
Comment
EPH is a very rare headache condition with only 20 well documented cases (4–6). There is no gender preference and the age of onset is anywhere from 12 to 51 years of age (7, 8). This is the second reported case of EPH with a seasonal variation (4). A family history of cluster headache has been reported in chronic paroxysmal hemicrania (CPH) but not EPH (8). This patient has many cluster headache features except for the complete response to indomethacin. This may suggest a dysfunction of the suprachiasmatic nucleus that regulates the rhythmic secretion of melatonin. The normal nocturnal peak in melatonin secretion and the 24-h production of melatonin is reduced in cluster headache patients. with an extension of the time from midnight to peak melatonin level (9). Seasonal EPH may have similar melatonin abnormalities. Melatonin supplementation may play a role in treating EPH as it has in cluster headaches (10).
Response to indomethacin is almost diagnostic of this condition. Treatment with indomethacin at 25 mg three times a day brings prompt relief within 24–48 h (11). Other medications that have been shown to be somewhat efficacious include the calcium channel blockers (12). A case of CPH responding to celecoxib has been reported (13). Hemicrania continua, another indomethacin responsive headache, has also been reported to be effectively treated by the COX-2 inhibitors (14). This is the first case, however, of EPH responding to COX-2 inhibitors. The patient had no response to valdecoxib, a newer COX-2 inhibitor, partial response to celecoxib, and total response to rofecoxib. Therefore, response to one COX-2 inhibitor does not predict response to another, and a trial of the various types of COX-2 inhibitors should be initiated in patients diagnosed with EPH who are intolerant of indomethacin for whatever reason.