瘤块型脱髓鞘病变的临床及病理特点

A clinicopathological study of demyelination pseudotumors of the brain

目的 观察瘤块型脱髓鞘病变的临床表现和病理特点 ,探讨其病理特征及发生原因。方法 对 3例瘤块型脱髓鞘病变的临床特点 ,CT、MRI所见 ,治疗效果及随访材料进行分析 ,并用组织学 (HE、髓鞘和轴索染色法 )及免疫组织化学SP法观察其病理学改变。结果 瘤块型脱髓鞘病变以急性起病为主 ,临床表现为头痛、恶心呕吐及神志恍惚、语言迟钝、瘫痪、病理征阳性等脑实质受损和脑局灶性定位的症状和体征。CT、MRI扫描为脑实质占位性病变。对激素治疗均有效。随访 6～ 31个月均未发现病情进展或复发。病理特点主要侵犯两侧大脑半球 ,病灶区域脑组织脱髓鞘 ,但轴索仍保留 ,星形胶质细胞增生 ,大量淋巴细胞在血管周围呈套袖样浸润 ,并可见单核巨噬细胞反应。结论瘤块型脱髓鞘病变是具有独特临床表现和病理形态特点的炎性疾病 ;

Objective To study the clinicopathological characteristics diagnosis, differential diagnosis and etiology of demyelination pseudotumors of the brain Methods The clinical features,CT,MRI scan findings,corticosteroid therapeutic effects and follow up data of 3 cases of demyelination pseudotumors of the brain were analysed,and pathological changes were observed by histologic (HE, Luxol fast blue and Bodian) and immunohistochemical (S P method) techniques Results The acute onset of demyelination pseudotumors appeared to be more predominant in our data Clinical manifestations included headache,vomiting,a depressed conscious level,dysphasia,and paresis CT,MRI scans showed solitary or multiple lesions in cerebral hemisphere All the patients presented excellent response to steroid treatment Follow up for a period of 6 to 31 months, revealed the absence of progression or recurrence The pathological changes were mainly located in both cerebral hemispheres, in which there were relative axonal preservation in foci loss of myelin, reactive gliosis, profuse perivascular lymphocytic infiltration and mixtures of foamy macrophages Conclusion Demyelination pseudotumor is a distinct clinicopathologic encephalitic entity The findings of this study suggest that the cause of tumefactive demyelination may be related to an allergic reaction triggered by viral infection