Case Report

Ipsilateral cluster headache and chronic paroxysmal hemicrania: two casereports

Alain Djacoba Tehindrazanarivelo1, Jean Marc Visy2, Marie-Germaine Bousser1

Service de NeuroIogie, Hôpital Saint Antoine1, Paris, France; Service de Neurologie, Hôpital Lariboisière2, Paris, France

Cephalalgia Tehindrazanarivelo AD, Visy JM, Bousser MG. Ipsilateral cluster headache and chronic paroxysmalhemicrania: two case reports. Cephalalgia 1992;12:318-20. Oslo. ISSN 0333-1024

We report two patients with ipsilateral attacks of cluster headache and chronic paroxysmalhemicrania. The first patient, a 33-year-old man, started having attacks of chronic cluster headacheat the age of 27. At 33, they were replaced by typical attacks of ipsilateral chronic paroxysmalhemicrania which showed a dramatic improvement with indomethacin 150 mg daily. After two daysof complete remission, cluster headache attacks reappeared and persisted until verapamil, 360 mga day, was added to indomethacin. The second patient, a 45-year-old man, first developed attacksof episodic cluster headache at the age of 35. At 44, he experienced ipsilateral typical attacks ofchronic paroxysmal hemicrania, and two months later attacks of cluster headache. Under verapamil240 mg daily, attacks of cluster headache disappeared, but those of chronic paroxysmal hemicraniaincreased in frequency until indomethacin 150 mg daily was added. These observations suggest aclose relationship but not a similarity between cluster headache and chronic paroxysmal hemicrania,and show the practical therapeutic interest of maintaining this distinction. Cluster headache, chronicparoxysmal hemicrania, indomethacin, verapamil

Alain Djacoba TehindrazanariveIo, Service de Neurologie. Hôpital Saint Antoine, 184, rue duFaubourg Saint Antoine, 75012 Paris, France. Received 12 June 1992, accepted 17 June 1992

Cluster headache (CH) occurs in attacks of 15 to 180 min duration, characterized by severe, unilateral, orbital,supraorbital or temporal pain associated with signs of autonomic dysfunction such as conjunctival injection,lacrimation, nasal stuffiness, among others (1). Chronic paroxysmal hemicrania (CPH) is a rare condition (less than100 published cases), also characterized by attacks of unilateral pain with signs of autonomic dysfunction, but itdiffers from CH by its female preponderance, by the higher frequency ( > 5 a day) and shorter duration ( 2 to 45min) of attacks, and by its dramatic response to indomethacin (2). The association of ipsilateral CH and CPH hasso far been reported in only two patients (3, 4), to which we now add two.

Case reports (Fig. 1)

Patient I

A 33-year-old man presented to our clinic in February 1991 for recurrent attacks of headache. Except for a headtrauma with coma at the age of 22, he had no past medical history. At the age of 27 he started to regularly suffer,two to three times a week, attacks of headache which lasted 30 min to 2 h. The pain was always extremely severe.It started around the left eye, spread to the left temple, and was associated with redness of the left eye, ptosis,lacrimation, rhinorrhea and nasal stuffiness. Analgesics, aspirin and methyser-gide were ineffective. In February1991, attacks became shorter (lasting from 10 to 20 min) but increased in frequency (up to 20 to 30 per day). Allother characteristics remained unchanged. He received ergotamine tartrate 2 mg daily but was unresponsive to thistreatment and was first seen in our clinic 10 days later. General and neurological examinations were normal, exceptfor a left Horner's syndrome. Routine blood tests, chest X-rays, electrocardiogram and computerized tomogram(CT) of the head with and without contrast injection, carotid ultrasonography, transcranial Doppler and cerebral MRIwere also normal. He received indomethacin 150 mg daily and within 36 h headache attacks completelydisappeared. But, three days later, ipsilateral long-lasting attacks (2 h) recurred at a frequency of two per day.Verapamil 360 mg daily was then added to indomethacin and long attacks disappeared within five days. After sixweeks without attacks, indomethacin was slowly tapered off, then stopped without recurrence of shorts attacks.Verapamil was stopped six months later. The patient has been headache-free since then, with a follow-up of 14months.

Patient 2

A 45-year-old man first developed attacks of cluster headache at the age of 35. The pain was located in theright temple behind the eye. It was an extremely severe, pressure-like pain, associated with ipsilateral lacrimationand injection of the eye, nasal stuffiness and rhinorrhea. Attacks lasted from I to 2 h and occurred daily (1 per 24 h)during four months. The patient was headache-free until the age of 42 when a second episode occurred, with dailyleft-sided attacks for six months. Two years later, at the age of 44, right-sided attacks recurred but they

Table 1. Association of ipsilateral cluster headache and chronic paroxysmal hemicrania: clinical andtherapeutic profile.

Jotkowitz (1978) Pearce et al. (1987) Patient 1 Patient 2Age at 45 58 33 45diagnosis(years)Sex Male Male Male MalePast history Pulmonary infection Migraine with aura Head trauma at 22 None

CH CPH CH CPH CH CPH CH CPHAge at 1st 41 45 48 58 27 33 35 45attack (years)Attack 45-60 20-30 30-120 5-10 30-120 10-20 60-120 5-15duration (min)Attack 1-3 8 1 10-I4 Once other 20-30 1 15-30frequency (per a dayday)Periodicity Episodic Chronic Chronic Chronic Chronic Episodic Episodic EpisodicSevere orbital + + + + + + + +painSide R R L L L L R, L then R

RLacrimation, + + + + + + +rhinorrhea,nasal stuffinessVerapamil ? 7 7 ? + ? +efficacyIndomethacin ? + ? + - + ?efficacy+ = yes: - = no; ? = not indicated or unknown; CH = cluster headache: CPH = chronic paroxysmalhemicrania; L = left; R = right,

were much shorter, lasting from 2 to 4 min. Attacks increased in frequency and duration over the next two months so that, inJanuary 1991, 15 to 30 attacks occurred per day, each lasting from 5 to 30 min. The patient then started to experience onceor twice a week ipsilateral long-lasting (2 h) attacks which occurred in bouts of two to three per day and were similar to hisprevious cluster attacks. General and neurological examinations were normal except for a right Horner's syndrome. Routineblood test, CT of the head with and without contrast injection and cerebral angiography were normal. Verapamil was firstadministered at a daily dose of 240 mg. Long-lasting attacks disappeared within a week, but short attacks persistedunchanged. Indomethacin 150 mg daily was added to verapamil a month later. Within 24 h short attacks decreased infrequency (one to three per day) and completely disappeared on the fourth day. He was seen a month later because of therecurrence of one to two short attacks two to three times a week. Indomethacin was increased to 200 mg daily and attacksdisappeared in three days. The patient has been, with a follow-up of one year, headache-free since then, although verapamilwas stopped and indomethacin was reduced to 50 mg on June 1991, then stopped on December 1991.

Discussion

We report two patients with attacks of CH and ipsilateral CPH fulfilling the IHS diagnostic criteria. This association has beenpreviously reported in two cases published by Jotkowitz in 1978 (3) and by Pearce et al. in 1987 (4). On considering thesefour cases (Table 1) we note that the association of CH and ipsilateral CPH concerns male patients, with a mean age of onsetof 38, and starts with attacks of CH followed 4 to 10 years later by ipsilateral attacks of CPH. The two varieties of attacksoccurred separately in three of four cases, but their simultaneous occurrence was observed in one of our patients. Theassociation of these two varieties of facial pain suggests a close relationship between these two conditions.

The interesting point made by our two observations is the dramatic and selective response of each variety of headache todifferent drugs. Long attacks (CH) totally disappeared with verapamil .but were unresponsive to indomethacin, whereas shortfrequent attacks (CPH) totally disappeared with indomethacin but were not modified by verapamil. Although the mode ofaction of these drugs as well as the pathophysiology of CH and CPH remain essentially unknown (5-7) this selective efficacyof one drug in one condition and its inefficacy in the other does suggest a pathophysiological difference in the mechanism ofpain in these two conditions and indicates the practical therapeutic interest of maintaining a distinction between them.

References

1. Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria forheadache disorders, cranial neuralgias and facial pain. Cephalalgia 1988;8(suppl 7):35-8

2. Antonaci F, Sjaastad O. Chronic paroxysmal hemicrania (CPH): a review of the clinical manifestations. Headache1989;29:648-56

3. Jotkowitz S. Chronic paroxysmal hemicrania and cluster. Ann Neurol 1978;4:389

4. Pearce SHS, Cox JGC, Pearce JMS. Chronic paroxysmal hemicrania, episodic cluster headache and classic migrainein one patient. J Neurol Neurosurg Psychiatry 1987;50:1599-70

5. Kudrow L. Cluster headaches. In: Blau JN ed Migraine. Clinical, therapeutic, conceptual and research aspects.London: Chapman and Hall, 1987:113-33

6. Sjaastad O. Chronic paroxysmal hemicrania: clinical aspects and controversies. In: Blau JN ed Migraine. Clinical,therapeutic, conceptual and research aspects. London: Chapman and Hall, 1987:135-52

7. Gabai IJ, Spierings ELH. Prophylactic treatment of cluster headache with verapamil. Headache 1989;29:167-8