群発頭痛様の疼痛を呈したMaffucci's syndromeの1例

Cluster Like Headache in a Patient with the Maffucci's Syndrome

　要旨　Maffucci症候群は血管腫と多発性内軟骨腫からなる稀な非遺伝性の中胚葉形成異常疾患である。症例は52歳男性，幼少期より皮膚に弾性軟の多発性海綿状血管腫と内軟骨腫を認めMaffucci症候群と診断されていた。鼻漏を伴う1～2時間続く右眼窩部の激痛が約2カ月間ほぼ毎日出現したため（群発頭痛様疼痛），来院。頭部MRIでは右海綿静脈洞に浸潤する下垂体腺腫を認め，ホルモンの異常はなく，非機能性下垂体腺腫と診断した。ロキソプロフェンナトリウム，スマトリプタン，ロメリジンを投与するも，いずれも症状の改善を認めなかったが，ベラパミルを投与したところ次第に症状軽快し，疼痛は消失した。群発頭痛の発生機序および，ベラパミルの予防効果には海綿静脈洞部が関与していることが推測された。

　Maffucci's syndrome is a rare congenital, nonhereditary mesodermal dysplasia characterized by soft tissue hemangiomas and multiple enchondromas. A 52 years old man was diagnosised as Maffucci's syndrome in his childhood. He complained of mild paraesthesia which gradually progressed to intolerable pain lasting one to two houres with rhinorrhea（cluster like headache）. Magnetic resonance imaging showed a pituitary adenoma which invaded into right cavernous sinus. Serum hormone level was almost normal, therefore we diagnosed nonfunctional pituitary adenoma. The pharmacological preventive treatments for headache, such as loxoprofen, sumatriptan, lomerizine had no effect.On the other hand, verapamil showed moderate improvement of his headache, then the symptoms was gradually getting better and finally he felt no pain. This case demonstrated occurrence mechanism of cluster headache and preventive effect of verapamil associated with cavernous sinus.