Epsodic paroxysmal hemicrania with seasonal variation: case report and the EPH-cluster headache continuum hypothesis

Abstract

-Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by frequent, daily attacks of short-lived, unilateral headache with accompanying ipsilateral autonomic features. EPH has attack periods which last weeks to months separated by remission intervals lasting months to years, however, a seasonal variation has never been reported in EPH. We report a new case of EPH with a clear seasonal pattern: a 32-year-old woman with a right-sided headache for 17 years. Pain occurred with a seasonal variation, with bouts lasting one month (usually in the first months of the year) and remission periods lasting around 11 months. During these periods she had headache from three to five times per day, lasting from 15 to 30 minutes, without any particular period preference. There were no precipitating or aggravating factors. Tearing and conjunctival injection accompanied ipsilaterally the pain. Previous treatments provided no pain relief. She completely responded to indomethacin 75 mg daily. After three years, the pain recurred with longer attack duration and was just relieved with prednisone. We also propose a new hypothesis: the EPH-cluster headache continuum.KEY WORDS: episodic paroxysmal hemicrania, chronobiological dysfunction, EPH-cluster headache continuum. Hemicrânia paroxística episódica com variação sazonal: relato de caso e hipótese do continuum HPEcefaléia em salvasRESUMO -A hemicrânia paroxística episódica (HPE) é um tipo raro de cefaléia em que ocorrem breves episódios de intensa cefaléia hemicrânica, recorrendo várias vezes por dia e acompanhada de sinais autonômicos ipsilaterais. Estas dores se repetem, em geral, por períodos sintomáticos que duram de semanas a meses, separados por intervalos assintomáticos de meses a anos; no entanto, uma variação sazonal nunca foi relatada nesses casos. Descreveremos um caso de HPE com um nítido padrão sazonal. Trata-se de mulher de 32 anos, que referia dor hemicrânica à direita, mas principalmente periorbitária, em queimação, há 17 anos. A dor sempre ocorria durante 1 mês e com intervalos assintomáticos de 11 meses. Durante períodos sintomáticos a dor ocorria de 3 a 5 vezes por dia, com duração de 15 a 30 minutos, sem horário preferencial. Em 30% dos ataques apresentava lacrimejamento e congestão conjuntival. Não havia fatores desencadeantes ou de piora. No momento do exame, a paciente queixava-se da dor há 20 dias. O exame neurológico era normal. A tomografia de crânio, normal. Após a introdução de indometacina a paciente referiu desaparecimento da dor em 48 horas. Após três anos, a dor recorreu com duração mais longa e foi aliviada apenas com prednisona. Também propomos uma hipótese: o continuum EPH-cefaléia em salvas. PALAVRAS-CHAVE: hemicrania paroxística episódica, disfunção cronobiológica, continuum HPE-cefaléia em salvas. Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by frequent, daily attacks of short-lived, unilateral headache with accompanying ipsilateral autonomic features. These headaches occur, in general, for many year...

Seasonal Periodicity in SUNCT Syndrome

“…The temporal pattern usually described in episodic SUNCT is irregular, with the symptomatic periods alternating with remissions in an erratic fashion (2, 3). In contrast, it is widely acknowledged that in episodic CH bouts can occur in the same periods every year (4), particularly during spring and autumn (5), and in some PH patients seasonal variations have been described (6–8). We present a patient with episodic SUNCT syndrome with bouts that occurred twice annually in spring and autumn.…”

Seasonal Episodic Paroxysmal Hemicrania Responding to Cyclooxygenase-2 Inhibitors

“…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.…”

Coexistence of cluster headache and paroxysmal hemicrania: does it exist? A case report and literature review

“…There are just a few case reports of episodic form of PH in the literature [9]. Hence, co-occurrence of CH with CPH would be perhaps possible rather than the co-occurrence of CH with EPH, which is itself very rare.…”

“…Although response to indomethacin is not against the IHS criteria for CH, physicians hesitate in making the diagnosis of CH if a patient shows response to indomethacin, even in those people who clearly fulfil the IHS criteria for CH. There are many reports in the literature where cases of CH were wrongly diagnosed as PH because of the presence of positive response to indomethacin [9, 13, 17]. Indomethacin has never been evaluated systematically for CH prophylaxis.…”