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Chronic paroxysmal hemicrania was first described by Sjaastad and Dale in 1974.1 It is characterised by attacks of excruciating unilateral oculotemporal pain associated with autonomic changes, such as lacrimation, rhinorrhea, ptosis, miosis, and conjunctival suffusion. It differs from cluster headache for its female predominance, brevity (5–45 minutes), and frequency (5–20/day) of pain attacks, as well as for its response to indomethacin in doses of up to 150 mg/day.2 Experience with subcutaneous sumatriptan in chronic paroxysmal hemicrania is scarce. Here we report on the effect of subcutaneous sumatriptan in a patient with chronic paroxysmal hemicrania.
In May 1994, when 34 years old, this previously healthy woman started to have attacks of severe temporal and periorbital pain usually associated with conjunctival injection, lacrimation, ptosis, eyelid oedema, and nasal congestion. Frequency and duration of attacks were variable. She experienced at least five pain attacks a day from the beginning of her clinical presentation, but every few months the frequency increased up to more than 20 episodes each day in bouts lasting 20–30 days. The duration of attacks ranged from several minutes to 1 hour. A diagnosis of cluster headache was made. She was treated with the maximum doses that she could tolerate of verapamil (40 mg/6 h), and methysergide (1 mg/ 6 h), without success. Ergotamine (2 mg at night) was added with doubtful symptomatic improvement, so domiciliary 100% oxygen (7 l/min) and/or subcutaneous sumatriptan (6 mg) were prescribed. She thought that oxygen inhalation during the attacks did not stop the pain but alleviated it if the episode was mild and if the oxygen was taken during the earliest part of the attack. Subcutaneous sumatriptan stopped the attacks in less than 15 minutes. In addition, during the bouts, when she experienced multiple consecutive pain attacks, the patient noticed that after subcutaneous sumatriptan she was free of pain for a maximum of 8 hours and a minimum of 6 hours. She was sent to us in March 1996. Systemic and neurological examinations, as well as high resolution brain CT (with and without contrast) were unremarkable. Indomethacin (75 mg daily), was started, with absolute effectiveness after 4 days. Indomethacin has been withdrawn twice, with immediate pain reappearance. During more than one year of follow up, she has twice needed to increase indomethacin up to 125 mg daily for several weeks because of further pain episodes, which promptly stopped with subcutaneous sumatriptan on the four occasions that this was injected. She is now taking 75 mg indomethacin daily and ranitidine, with very infrequent attacks of only slight pain.
The differential diagnosis between chronic paroxysmal hemicrania and chronic cluster headache still poses some problems. With that in mind, this woman clearly meets the criteria of the International Headache Society classification for diagnosis of chronic paroxysmal hemicrania.2 Subcutaneous sumatriptan consistently relieved her chronic paroxysmal hemicrania attacks in less than 15 minutes and protected her from further pain attacks for 6 to 8 hours. Subcutaneous sumatriptan is a very effective symptomatic treatment for cluster headache pain attacks, this confirming that trigemino-vascular activation with cranial vasodilatation or plasma protein extravasation are the pathophysiological explanation for cluster headache attacks.3 The aetiology and pathogenesis of chronic paroxysmal hemicrania are still unknown. Goadsby and Edvinsson have recently reported a patient with chronic paroxysmal hemicrania in whom concentrations of calcitonin gene related peptide and vasoactive intestinal polypeptide, two markers of trigemino-vascular activation, were raised in the cranial circulation during attacks. Moreover, the concentrations of both peptides became normal when indomethacin was given, all this suggesting a shared pathophysiology between chronic paroxysmal hemicrania and cluster headache.4 The finding of the effect of subcutaneous sumatriptan in chronic paroxysmal hemicrania clarifies this point further. To the best of our knowledge, the effect of subcutaneous sumatriptan has been reported only in three patients with chronic paroxysmal hemicrania and gives discordant results.5-7 One patient, a male but with very typical chronic paroxysmal hemicrania attacks, did not respond to subcutaneous sumatriptan on two different occasions, although the duration of this patient’s attacks was not mentioned.5 Two other patients had a clearly positive response.6 7 These two patients, however, were atypical, one also having intracranial hypertension and the other both chronic paroxysmal hemicrania and trigeminal neuralgia. Although more experience is necessary, our findings suggest that, in parallel with migraine and cluster headache, trigemino-vascular activation also occurs in chronic paroxysmal hemicrania.
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