Miyazaki Hiromichi, Tabuse Masanao, Ishiyama Naomi, Kikuchi Ryogo, Ogihara Toru, Nanki Kousaku
Department of Neurosurgery, Hiratsuka City Hospital, Kanagawa Japan.
Brain Nerve. 2011 Jul;63(7):795-9.
Multifocal fibrosclerosis(MFS) is a rare disorder of unknown etiology, characterized by chronic inflammation with dense fibrosis and lymphoplasmacytic infiltration into the connective tissue of various organs. Recently, MFS was classified as IgG4-related systemic disease. In this paper, we report a 60-year-old man with no history of head injury presenting with chronic subdural hematoma(CSDH). After surgery, he complained of severe, continuous headache and persistent high-grade fever. Extensive evaluation, including ⁶⁷Ga scintigraphy suggesting inflammations in various organs, liver needle biopsy showing sclerosing cholangitis, and blood examination showing elevated serum IgG4 levels, led to the diagnosis of MFS. To our knowledge this is the first report of MFS causing CSDH. The mechanism of the formation of CSDH is presumed to involve reactive granular membrane together with exudative subdural collection caused by MFS, which gives rise to minor and repeated bleeding. In this case, oral corticosteroid therapy was dramatically effective in the treatment of the condition.
多灶性纤维硬化症（MFS）是一种病因不明的罕见疾病，其特征为慢性炎症伴致密纤维化以及淋巴细胞和浆细胞浸润至各器官的结缔组织。最近，MFS被归类为IgG4相关性全身性疾病。在本文中，我们报告了一名60岁男性，无头部受伤史，表现为慢性硬膜下血肿（CSDH）。手术后，他抱怨有严重的持续性头痛和持续高热。广泛评估包括⁶⁷Ga闪烁扫描提示各器官有炎症、肝脏穿刺活检显示硬化性胆管炎以及血液检查显示血清IgG4水平升高，最终诊断为MFS。据我们所知，这是首例MFS导致CSDH的报告。CSDH形成的机制推测涉及反应性颗粒膜以及由MFS引起的渗出性硬膜下积液，进而导致轻微反复出血。在此病例中，口服糖皮质激素治疗对该病症有显著疗效。
