恶性萎缩性丘疹(Degos病)的视神经和脊髓表现
Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)作者机构：Department of Neurology Niigata City General Hospital 2-6-1 Shichikuyama Niigata 950-8739 Japan Dr.
出 版 物：《世界核心医学期刊文摘（神经病学分册）》 (Digest of the World Core Medical Journals:Clinical Neurology)
年 卷 期：2006年第2卷第6期
页 面：36-36页
学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 100206[医学-皮肤病与性病学] 10[医学]
主 题：恶性萎缩性丘疹 Degos病 视神经 脊髓 T1加权成像 T2加权成像 髓鞘神经纤维 动脉血栓栓塞 血管内皮增殖 检查结果
摘 要：A fatal case of malignant atrophic papulosis (Degos disease)with optic nerve and spinal cord involvement is described. Magnetic resonance imaging (MRI) of the optic nerve showed abnormal signal enhancement on fat suppressed T1 weighted images after intravenous meglumine gadopentetate infusion. On T2 weighted sagittal images, a sawtooth pattern was observed over seven vertebral segments of the spinal cord. On necropsy, a severe loss of myelinated nerve fibres in the left optic nerve was seen, with thrombotic obstruction of the central retinal artery. Spongy degeneration was observed in all levels of the spinal cord, with patchy and motheaten patterns caused by thromboses and endothelial proliferation in subarachnoid vessels. Findings on MRI were consistent with findings on pathological ex amination.
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