Abstract
We describe a man with chronic paroxysmal hemicrania, who remained free of headaches on indomethacin, 25 mg once or twice daily. However, in this case, in contrast to typical cases of paroxysmal hemicrania, the pain of the headaches was nonlateralized and was located in the centre of the forehead. The headaches were not associated with local autonomic symptoms or signs involving the eyes or nose. Initially, the pain of the headaches lasted for seconds only and was brought on by coughing.
Introduction
Paroxysmal hemicrania resembles cluster headache but with a shorter duration (10–30 min) and higher frequency of the attacks (5 to 15 per day), and with an absolute response to preventive treatment with indomethacin. Similar to cluster headache, the location of the pain is unilateral, often with fixed lateralization to one side of the head, although alternating lateralization also occurs (1, 2). However, bilateral cases of cluster headache and of paroxysmal hemicrania have been described as well (3, 4), to which we add a case of chronic paroxysmal hemicrania with nonlateralized, central location of the pain, fully relieved with indomethacin, 25 or 50 mg per day.
Case study
A 42-year-old-man experienced a head cold in 1995, followed by severe stabbing pain in the centre of his forehead, just above the bridge of the nose. The pain was brought on by coughing, lasted for seconds, and occurred for 1 or 2 weeks. In 1996, he got the flu with recurrence of the stabbing pain but with radiation of the pain bilaterally into the forehead. It was brought on by coughing, sneezing, squatting or bending over and lasted from a few minutes to 30 min, with an average of 10–15 min. The pain occurred daily for 4 months, 4–20 times per 24 h but during the day only, and sometimes associated with restlessness and pacing.
In 1997, not triggered by anything in particular, three episodes of pain lasted for 2 months, 4 months and 2 weeks, respectively. During the episodes, the pain occurred daily, again 4–20 times per 24 h but now also at night, waking him up out of sleep at 04.45. The pain usually lasted for 10–15 min and sometimes radiated up out of the back of the neck. It was most intense in the centre of the forehead and was not associated with local autonomic symptoms or signs involving the eyes or nose. The pain was also not associated with symptoms such as nausea, vomiting, photophobia or phonophobia or preceded by symptoms reminiscent of migraine aura. It was no longer triggered by coughing, sneezing etc., or aggravated by movement or activity. The pain was more consistently associated with an urge to move, sometimes along with stamping of the feet or pounding of the hands. He did not use alcohol and therefore was unaware of alcohol as a trigger. Sumatriptan 6 mg subcutaneously, tried in 1997 on two occasions, did not relieve the pain but aspirin, taken in doses of 500 mg for 1 or 2 weeks, mitigated it somewhat.
At the end of 1997, he experienced another episode of pain in the centre of his forehead. The pain occurred daily, on average four times per day, lasting for 10–15 min, until consultation in February 1998. The physical and neurological examinations were unremarkable and routine haematology and blood chemistry were normal. Cranial and sinus computerized tomography and cranial magnetic resonance imaging (T1, T2, echo T2 and flair) were unremarkable as well. He was treated with indomethacin, 25 mg three times daily, resulting in immediate and complete resolution of the pain. He has been able to remain free of pain since, taking 25 mg once or twice per day, with recurrence of the pain within 4 or 5 days whenever he discontinued the medication, which he tried at least half a dozen times. He never tried any preventive headache medications belonging to the categories of beta-blockers, tricyclic antidepressants or calcium-entry blockers. Mechanical stimulation of the neck to bring on the pain of the headaches was never attempted, with or without indomethacin treatment.
Discussion
The previously reported case of bilateral paroxysmal hemicrania concerned a 67-year-old woman, who experienced sudden occipital headaches (4). The pain, bilaterally from the start, had an intense, piercing quality and radiated to the temporal area. The attacks lasted for several seconds and initially occurred 2–5 times during the day, with symptoms appearing also at night after 10 months. In the course of some months, the attack frequency increased to 5–10 per 24 h, with an average duration of 5–8 min. Indomethacin, 25 or 50 mg per day rectally, brought about a complete cessation of the attacks within 24 h.
The headache history of our patient, a man, is remarkably similar to that of the patient described above. The pain was initially of very short duration, lasting only for seconds (stabbing headache), but subsequently acquired the duration typical of paroxysmal hemicrania, lasting for 10–15 min on average. The attacks occurred daily, 4–20 times per day, initially only during the day but later also at night. In our patient, the condition only secondarily became chronic while in the reported case, it was chronic from the onset. In both cases, the pain was nonlateralized and bilateral or central, in our patient frontal and in the reported case occipital, and local autonomic symptoms or signs were absent. In both cases, the attacks responded immediately to treatment with indomethacin in the low dosages of 25 or 50 mg per day with complete cessation, confirming the proper diagnosis.
The initial pain in our patient was an exertional headache, brought on by coughing, sneezing, squatting, and bending over, which is unusual for stabbing headache or paroxysmal hemicrania. Initially, it was short lasting and only seconds in duration but lasting up to 30 min the second time it occurred. The first episode developed after a head cold and the second one during flu, a not uncommon sequence of events with ‘cough headache’. The patient was not examined neurologically at those times and did not undergo any testing, including lumbar puncture. The subsequent episodes were not triggered by anything in particular and the individual attacks no longer occurred related to exertion.
In the two cases, the problem is more semantic than it is clinical in the sense that a nonlateralized, bilateral or central headache cannot be referred to as ‘hemicrania’. A semantic problem, even to a greater extent, exists with the term migraine, where an estimated one-third of headaches is bilateral. Migraine, a word of French origin, is a mediaeval corruption of the Greek, ηµιχρανια, hemicrania, referring to one-sided pain in the head (5). Unless we call bilateral migraine, ολοχρανια, holocrania, there is actually no need to refer to bilateral paroxysmal hemicrania as paroxysmal holocrania. However, both cases also suggest that a diagnosis of paroxysmal hemicrania may be made on the temporal pattern of the headaches alone. This would mean that the frequency and duration of the headaches are more important in determining the response to indomethacin than the unilateral location of the pain or the presence of local autonomic symptoms or signs.