Tajti J, Stefani E, Appel S H
Department of Neurology, Baylor College of Medicine, Houston 77030.
J Neuroimmunol. 1991 Nov;34(2-3):143-51. doi: 10.1016/0165-5728(91)90123-o.
Guinea pigs inoculated with bovine spinal cord ventral horn homogenate develop a syndrome termed experimental autoimmune gray matter disease (EAGMD) characterized by extremity weakness, bulbar signs, and a loss of lower and upper motoneurons. To provide evidence for the role of autoimmune mechanisms, we have administered the immunosuppressant cyclophosphamide prior to and after gray matter immunization. Pretreatment with cyclophosphamide prevented the appearance of clinical signs of disease and decreased the loss of spinal cord motoneurons, the appearance of damaged motoneurons, and the antibody titer to motoneurons. Treatment 7 days after immunization attenuated the expression of disease. Treatment immediately after signs also improved the clinical and pathological findings. In all cyclophosphamide-treated animals there was less IgG within motoneurons and less inflammation. These results support the role for autoimmune mechanisms in motoneuron loss and degeneration in EAGMD.
接种牛脊髓腹角匀浆的豚鼠会出现一种称为实验性自身免疫性灰质疾病（EAGMD）的综合征，其特征为肢体无力、延髓体征以及上下运动神经元丧失。为了提供自身免疫机制作用的证据，我们在灰质免疫之前和之后给予了免疫抑制剂环磷酰胺。环磷酰胺预处理可预防疾病临床症状的出现，并减少脊髓运动神经元的丧失、受损运动神经元的出现以及运动神经元抗体滴度。免疫后7天进行治疗可减轻疾病的表达。出现症状后立即治疗也改善了临床和病理结果。在所有接受环磷酰胺治疗的动物中，运动神经元内的IgG较少，炎症也较少。这些结果支持自身免疫机制在EAGMD运动神经元丧失和变性中的作用。
