Chronic inflammatory polyradiculoneuropathy.
Chronic inflammatory polyradiculoneuropathy.
复制标题
慢性炎症性多发性神经根神经病。
作者：
P. Dyck;A. Lais;M. Ohta;J. A. Bastron;H. Okazaki;R. Groover
对53例慢性炎症性多神经根神经病变（CIP）患者的诊断标准、自然病史、神经传导特征、病理、实验室特征及皮质类固醇治疗的疗效进行了个人评价。这些患者包括单相神经功能障碍在6个月后未达到顶峰的患者、复发患者和稳定或逐步进展的患者。CIP的典型特征包括无相关疾病，既往经常感染或接受外源蛋白，倾向于累及头颅、躯干、近端和远端肢体结构，周围神经传导速度弥漫性减慢。最明显的减速通常发生在近端。病理特征包括浆液性水肿、单个核细胞浸润（特别是在血管周围区域，但没有血管炎的证据）、巨噬细胞诱导的节段性脱髓鞘和肥厚性神经炎。如果我们的病人是有代表性的，完全康复的情况很少发生；大约60%的患者能够走动和工作，25%的患者只能坐在轮椅上或卧床不起，大约10%的患者死于这种疾病。虽然大部分病理改变影响脊髓根和近端神经，但也可能累及脑和脊髓。髓鞘卵泡变性是踝关节腓肠神经髓鞘纤维变性的主要类型。
The diagnostic criteria, natural history, nerve conduction characteristics, pathology, laboratory features, and efficacy of corticosteroid treatment have been evaluated personally in 53 patients with chronic inflammatory polyradiculoneuropathy (CIP) who were followed up for an average of about 7.5 years. These were patients whose monophasic neurologic deficit had not crested by 6 months, patients with recurrences, and patients with a steady or stepwise progression. The typical features of CIP include absence of an associated disease, frequent history of preceding infection or receipt of foreign protein, and tendency to involve cranial, truncal, and proximal as well as distal limb structures and to have diffusely slow conduction velocity of peripheral nerves. The most marked slowing is often very proximal. The pathologic features include serous edema, mononuclear cell infiltrates (especially in perivascular areas, but without evidence of vasculitis), macrophage-induced segmental demyelination, and hypertrophic neuritis. If our patients are representative, complete recovery occurs only infrequently; about 60% of patients are able to be ambulatory and work, 25% become confined to a wheelchair or become bedridden, and approximately 10% die from their disease. Although the bulk of the pathologic changes affect spinal roots and proximal nerves, the brain and spinal cord may be involved also. Degeneration into linear rows of myelin ovoids is the predominant type of myelinated fiber degeneration of the sural nerve at the ankle.