Kitamura Eiji, Imai Noboru, Konisi Takashi, Suzuki Youji, Serizawa Masahiro, Okabe Takashi
Department of Neurology, Shizuoka Red Cross Hospital, 8-2 Otemachi, Aoi-ku, Shizuoka City 420-0853, Japan.
No To Shinkei. 2006 Jun;58(6):514-7.
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 symptons was gradually getting better and finally he felt no pain. This case demonstrated occurence mechanism of cluster headache and preventive effect of verapamil associated with cavernous sinus.
马富西综合征是一种罕见的先天性、非遗传性中胚层发育异常,其特征为软组织血管瘤和多发性内生软骨瘤。一名52岁男性在童年时被诊断为马富西综合征。他主诉轻度感觉异常,逐渐发展为持续一到两小时的难以忍受的疼痛并伴有鼻漏(丛集性头痛)。磁共振成像显示垂体腺瘤侵犯右侧海绵窦。血清激素水平基本正常,因此我们诊断为无功能垂体腺瘤。用于治疗头痛的药物预防性治疗,如洛索洛芬、舒马曲坦、氯苯苄嗪均无效。另一方面,维拉帕米使他的头痛有中度改善,随后症状逐渐好转,最终他不再感到疼痛。该病例证明了丛集性头痛的发生机制以及维拉帕米对海绵窦相关疾病的预防作用。