Fujimoto M, Kira J, Murai H, Yoshimura T, Takizawa K, Goto I
Department of Neurology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Intern Med. 1993 Jun;32(6):510-2. doi: 10.2169/internalmedicine.32.510.
This is the first report describing hypertrophic cranial pachymeningitis which developed in association with mixed connective tissue disease (MCTD). A 56-year-old man with a two-year history of MCTD gradually developed symptoms of headache and blurred vision. Bilateral papilledema and increased opening pressure and mild pleocytosis of the cerebrospinal fluid were noted. Magnetic resonance imaging of the brain revealed a diffuse thickening and a gadolinium-enhancement of the cranial dura mater. While five previous patients, diagnosed as idiopathic or infectious hypertrophic cranial pachymeningitis in our department, had either otitis media or sinusitis and showed multiple cranial nerve involvement, the present patient had neither otitis media nor sinusitis and did not show any cranial nerve involvement other than papilledema.
这是首例描述与混合性结缔组织病（MCTD）相关的肥厚性硬脑膜炎的报告。一名患有MCTD两年病史的56岁男性逐渐出现头痛和视力模糊症状。检查发现双侧视乳头水肿、脑脊液初压升高以及轻度淋巴细胞增多。脑部磁共振成像显示硬脑膜弥漫性增厚并伴有钆增强。在我们科室之前诊断为特发性或感染性肥厚性硬脑膜炎的5例患者中，均患有中耳炎或鼻窦炎，且表现为多组颅神经受累，而本例患者既没有中耳炎也没有鼻窦炎，除视乳头水肿外未出现任何颅神经受累表现。
