Sudoh S, Kohriyama T, Tohji H, Nakamura S
Third Department of Internal Medicine, Hiroshima University School of Medicine.
Rinsho Shinkeigaku. 1997 Jul;37(7):626-30.
We report a 57-year-old woman with optic-spinal form of active multiple sclerosis, who developed a large lobar type hemorrhage of the brain. She initially suffered from left visual loss, and three month later, she was hospitalized with paraplegia and total sensory loss up to the fourth thoracic level accompanied by sphincteric disturbance. Diagnosis of clinically probable multiple sclerosis was based on the relapsing-remitting clinical course and laboratory findings. Five months after admission, she developed sudden consciousness loss. Brain CT scan showed a massive hemorrhage in the right frontal to parietal lobe. The patient had no risk factors for cerebral hemorrhage including hypertension. Histopathological study of brain tissues obtained at surgical evacuation of hematoma did not reveal any malignancy, and congo-red staining of this specimen was negative. We analyzed coagulation, fibrinolytic, and endothelial parameters during the follow-up period. von Willebrand factor (vWF) as a marker for endothelial damages was elevated persistently. Moreover, thrombin-antithrombin III complex (TAT) as a marker for activation of coagulation was also elevated constantly throughout the clinical course. The findings suggest that fragility of the vascular walls and permeability changes associated with immunological mechanisms might have resulted in the cerebral hemorrhage. Although there are few reports of cerebral hemorrhage in patients with multiple sclerosis, it has been reported that vascular wall damage is an important aspect of the pathology of multiple sclerosis and acute cerebral vascular damage may sometimes occur in multiple sclerosis. We propose that coagulation studies including the endothelial marker such as vWF would provide a useful information regarding the risk of cerebrovascular complication in multiple sclerosis.
我们报告了一名57岁患有视神经脊髓型活动性多发性硬化症的女性，她发生了大面积脑叶型脑出血。她最初出现左眼视力丧失，三个月后因截瘫和直至胸4水平的完全感觉丧失伴括约肌功能障碍入院。临床可能的多发性硬化症诊断基于复发-缓解型临床病程和实验室检查结果。入院五个月后，她突然意识丧失。脑部CT扫描显示右额叶至顶叶有大量出血。该患者没有包括高血压在内的脑出血危险因素。在手术清除血肿时获取的脑组织的组织病理学研究未发现任何恶性肿瘤，该标本的刚果红染色为阴性。我们在随访期间分析了凝血、纤溶和内皮参数。作为内皮损伤标志物的血管性血友病因子（vWF）持续升高。此外，作为凝血激活标志物的凝血酶-抗凝血酶III复合物（TAT）在整个临床过程中也持续升高。这些发现表明，与免疫机制相关的血管壁脆性和通透性变化可能导致了脑出血。虽然关于多发性硬化症患者脑出血的报道很少，但据报道血管壁损伤是多发性硬化症病理学的一个重要方面，并且在多发性硬化症中有时可能发生急性脑血管损伤。我们建议包括vWF等内皮标志物在内的凝血研究将为多发性硬化症脑血管并发症的风险提供有用信息。
