急性多发性硬化的临床与磁共振表现及病理变化

Clinical,Pathological and MRI Feature of Acute Multiple Sclerosis

【目的】分析急性多发性硬化(AMS)临床、MRI和病理特点,初步探讨其发病机制。【方法】收集我院AMS 4例,分别从临床、头颅MRI和病理进行分析。治疗上予3个疗程甲基强的松龙(MPS)冲击,并辅以人体丙种球蛋白(IVIG)治疗。治疗前和治疗后进行功能残缺评定量表(EDSS)评分。【结果】4例AMS中,2例为Marburg型,2例为Balò型。临床共同特点是男性、起病急、亚急性病程、进展快、病情重及有不同程度的意识障碍;头颅MRI Marburg型以大脑半球白质和脑干大块的高信号为主,Balò型DWI病灶呈高、低交替的环形病灶。1例Balò型病理改变与经典MS(CMS)不同,病灶呈同心圆分布,病灶内未见CD4+或CD8+T细胞浸润,可见大量吞噬髓鞘的巨噬细胞,伴血脑屏障(BBB)破坏。3例患者经过3个疗程MPS冲击治疗,辅以IVIG冲击治疗有效,EDSS平均减少5±1.5分。【结论】与CMS相比,AMS无论在临床、病理,还是在头颅MRI上有明显的异质性;Balò发病机制可能与BBB破坏和巨噬细胞毒性有关;治疗上联合应用大剂量、多疗程MPS与IVIG有效。

[ Objective] To investigate the clinical, pathological, pathogenetic and brain MRI features of the patients with acute multiple sclerosis （AMS）. [Methods] To collect AMS cases that were definitely diagnosed by our two neurologists and analyze the characters of their clinic, pathology, and MRI. However, one patient of them, who was suspected as multiple metastatic tumor, was performed with biopsy. All patients were treated with 3-day high-dose methylprednisolone （MPS）, assisted by 3-day high-dose intravenous immunoglobulin （IVIG）. Finally, on one day before and 2 months after treatment, disability scale scores （DSS） of multiple sclerosis was evaluated. [Results] Two of d patients are Marburg＇s type, the others were Balo＇s type. Almost all patients were male, and they had abrupt onset of illness, subacute course and fast swift deterioration. Their MRI showed multiple T1 hypotensive and T2 hypertensive lesions in cerebral white matter and brain stem. Pathological distribution of AMS was concentric of myelinating area alternating with demyelinating area. The ultrastructure showed amount macrophages infiltrating and demyelination accompanied by obvious BBB damage; All patients were effective to MPS and IVIG treatment. [ Conclusion ] AMS is heterogenous to classical MS in clinic, pathology and MRI; the combined therapy with high-dose of MPS and IVIG in AMS is effective.