Blackwell Science, LtdOxford,
UKCHACephalalgia1468-2982Blackwell Publishing, 2004247608609Brief report Chronic paroxysmal hemicrania in childhoodD Benzecry de Almeida et al.
doi:10.1111/j.1468-2982.2004.00732.x
CLINICAL CORRESPONDENCE
Chronic paroxysmal hemicrania in early childhood: case report D Benzecry de Almeida1,3, P Afonso Cunali2, H Larocca Santos1, M Brioschi & M Prandini3 1
Instituto de Neurologia de Curitiba, Curitiba, 2Universidade Federal do Paraná, Curitiba and 3Universidade Federal de São Paulo, São Paulo, Brazil
Daniel Benzecry de Almeida, Abílio César Borges St. 79/61, Curitiba, Brazil, 80.730–060. Tel. +55 41 3361540, fax +55 41 2231843, e-mail
[email protected] Received 15 September 2003, accepted 27 November 2003
Chronic paroxysmal hemicrania (CPH) is an unusual cause of headache, affecting predominantly women in the third to fourth decade. It is characterized by multiple attacks of unilateral pain, mostly located in fronto-orbito-temporal region, often associated with autonomic symptoms, such as lacrymation, conjunctival hyperaemia and nasal obstruction or rhinorrhea. Each attack usually lasts from 2 to 45 min and as diagnostic criteria it should be absolutely responsive to indomethacin in varying doses (1). Since the first description and definition of diagnostic criteria, few cases of CPH have been described in children. We report a 10 years-old girl with symptoms beginning since early childhood.
Case report A 10-year-old girl complained of attacks of left unilateral headache. Their parents explain that since she was a baby (1 year old), she had sudden periods of apparently unmotivated crying lasting about five minutes, approximately once a week, when she took her hands to the left ear region. As she started communication, on numerous occasions she said that she had pain or cold sensation in the periauricular region. At age 4, these attacks became more severe, lasting about 40 min, many times a day. She had a normal neurological, clinical and otological examination and CT scan, head MRI (three times since then), skull X-ray, auditory evoked-potentials, audiometry and blood tests were all normal. Thermography showed a cold-patch image in left frontal area, characteristic of hemiparoxystic headaches. Since the first attacks, she tried different medication, including NSAIDs, amitryptilin, imipramine, propranolol and carbamazepine, all without pain relief. The only effective medication was indomethacin, with a good efficacy since the first use at age 6, at doses of 25 mg 2–4 times a day it kept her free of headache if taken at least twice per day or with partial relief in lower doses. A psychological evaluation 608
did not show any major affective disorders except coping strategies to avoid pain. At the time of consultation, the attacks varied in intensity and frequency. When she refrained from taking indomethacin, the attacks lasted from 15 to 40 min, followed by a brief relief of symptoms and posterior return after about 40 min. During severe attacks, lacrymation and conjunctival hyperaemia in the left eye could be seen. Rarely, her parents observed ipsilateral eyelid eodema but no nasal congestion or rhinorrhea. Some attacks have been videotaped and her behaviour was stereotyped in all of them, when she remained seated and crying, with her hand over the left side of her head and face, especially in left fronto-temporo-orbital region, sometimes including periauricular region. She markedly closed her left eye. Most of the time she remained seated but with occasional standing. After the diagnosis of possible chronic paroxysmal hemicrania was made, she was prescribed verapamil at increasing doses up to 240 mg, with a partial relief of symptoms, but still required indomethacin at lower doses (about 25 mg) 1–2 times a day.
Discussion Chronic paroxysmal hemicrania is a rare and welldefined disease. Since the first paper by Sjaastad and Dale (2), in 1974, describing two women in their late fifties with a unilateral headache only responsive to indomethacin, few cases have been reported, mostly in women 25–40 years old (3). In the beginning, CPH was considered a variation of cluster headache, but actually it is classified as a specific entity (4, 5), with some differences: CPH is predominant in women (ratio 3 : 1), the duration of each attack is shorter (2–45 min), and higher frequency of attacks per day (4–38 times a day). Sixtytwo percent of CPH patients have lacrymation on the affected side during headache, and one third have conjunctival hyperaemia, nasal congestion and rhin© Blackwell Publishing Ltd Cephalalgia, 2004, 24, 608–609
Chronic paroxysmal hemicrania in childhood orrhoea. Some patients describe myosis and eyelid ptosis. In some cases, complete differentiation between these two diseases is difficult (6). The aetiology remains unknown. In the case of this child, the location of pain, duration of symptoms, daily frequency and indomethacin-responsiveness are all highly suggestive of CPH. Few cases have been described in children (7–9) and, based on a Medline review, this is the youngest case ever described. The authors think that although rare, CPH may occur in children and an educational program to health professionals treating a paediatric population concerning causes and treatment of headaches is mandatory.
References 1 Vincent. MB. Hemicrania paroxística crônica: aspectos clínicos. Rev Bras Neurol 1989; 25:69–75. 2 Sjaastad O, Dale I. Evidence for a new (?) treatable headache entity. Headache 1974; 14:195–08.
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3 Sjaastad O, Apfelbaum R, Caskey W, Christoffersen B, Diamond S, Graham J, et al. Chronic paroxysmal hemicrania (CPH): The clinical manifestations: a review. Ups J Med Sci 1980; 31:27–35. 4 Merskey H, Bond MR, Bonica JJ, Boyd DB, Carmon A, Deathe AB, et al. Classification of chronic pain. Description of chronic pain syndromes and definition of pain terms. Pain Supplement 1986; 3:1–226. 5 Headache Classification Subcommittee of the International Headache Society. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia 1988; 8 Suppl 7:1–96. 6 Solomon S, Newman LC. Chronic paroxysmal hemicrania in a child? Headache 1995; 35:234. 7 Gladstein J, Holden EW, Peralta L. Chronic paroxistic hemicrania in a child. Headache 1994; 34:519–20. 8 Klassen BD, Dooley JM. Chronic paroxysmal hemicranialike headaches in a child: response to a headache diary. Headache 2000; 40:853–5. 9 Kudrow DB, Kudrow L. Successful aspirin prophylaxis in a child with chronic paroxysmal hemicrania. Headache 1989; 29:280–1.
