脱髓鞘疾病进修课件.ppt

急性播散性脑脊髓炎ADEM 蛋白含量正常或轻度升高，若有明显升高又排除椎管梗阻，则提示有脊神经根受累。20％至25％CSF中的IgG升高，并可有寡克隆带Significant increase without obstruction suggest the lesion of spinal nerve root. 20-25％of IgG raised , oligoclone zone may appear. 1000mg/dl,10% no abnormity. 在AHLE患者脑脊髓中，通常有中性粒细胞和红细胞，蛋白浓度也升高。25%病人大于200mg/dl，最高至1000mg/dl。10%患者CSF完全正常。 In CSF of AHLE,there is always neutrophil and erythrocyte with improved protein. 25% 急性播散性脑脊髓炎ADEM ADEM和MS 鉴别关键在于MRI表现及病程的发展。 ADEM组MRI显示皮层下白质病灶，两组无差别。ADEM组中90％患者部分或全部病灶消失，而无新病灶出现。 The key point of differentiation of ADEM and MS lies MRI and the development of the disease。The lesion of subcortex white matter in MRI shows no difference between ADEM and MDEM. The lesions of 90％ of ADEM/EDEM patients completely or partial disappeared without new lesion. 1.免疫抑制剂 硫唑嘌呤(2-3 mg/Kg/d) 单用或联合口服强的松 (1 mg/Kg/d)治疗NMO，有效超过18个月，对于NMO-IgG血清阳性患者应长期应用免疫抑制剂，以防复发(B级推荐)。 环孢素A、环磷酰胺、麦考酚酯、莱氟米特、FK506、米托蒽醌 2.利妥昔单抗(Rituximab) 利妥昔单抗是一种针对B细胞表面CD20的单克隆抗体，国内常用于B细胞淋巴瘤的靶向治疗，该药对类风湿关节炎等免疫疾病同样有效。 3.糖皮质激素 有部分NMO患者对糖皮质激素有一定依赖性，对于这部分患者激素减量要比MS慢，有报道小剂量强的松维持治疗能减少NMO复发，也有报道定期激素冲击，如每3月冲击1次，能减少NMO复发，但尚无大样本多中心随机对照试验，目前尚无充分循证医学证据表明糖皮质激素能预防NMO复发。 视神经脊髓炎 Neuromyelitis optica 临床表现clinical manifestation 20~40岁起病， 女性多见 急性或亚急性起病 前驱症状 视神经脊髓炎 Neuromyelitis optica 先后出现双眼视力障碍，可完全失明 可伴眼球胀痛或头痛 眼底早期为炎性改变，晚期可出现萎缩 脊髓表现为不同程度的横贯性损害 视神经脊髓炎 Neuromyelitis optica 辅助检查 Laboratory Findings 常规 CSF 诱发电位 MRI 视神经脊髓炎 Neuromyelitis optica 诊断diagnose 病史history CSF 改变 the change of CSF MRI 与ADEM 、MS 及球后视神经炎鉴别diffretiation Devics disease affects only the optic nerves and spinal cord, whereas MS affects the brain as well. Attacks of Devics disease tend to be more frequent and severe than in MS.. An MRI of the brain is typically normal in Devics disease. An MRI of the spinal cord shows large extensive areas of inflammation of the spinal cord whereas in MS typically the areas are much smaller. Spinal fluid studies tend not to show the typical elevation of antibodies detected in patients with