急性炎性脱髓鞘性多根神经病变的研究进展

Gabriel Palombo, Blake Hoppe
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摘要

平均而言,在北美和欧洲诊断为格林-巴勒综合征的患者中有97%患有一种称为急性炎症性脱髓鞘性多根神经病变(AIDP)的特定亚型。AIDP被怀疑是促炎细胞因子引起脱髓鞘的结果,特别是在周围神经系统中,作为对应激的反应。AIDP发病前的事件包括胃肠道或呼吸道感染、手术和创伤。AIDP主要表现为急性上升性麻痹和反射性松弛。AIDP的诊断通过神经传导检查(NCSs)、肌电图(EMG)和腰椎穿刺脑脊液(CSF)分析得到证实。NCS/EMG用于评估周围神经功能,通常需要进行系列检查。脑脊液分析正在寻找白蛋白-细胞学分离。最常见的是静脉注射免疫球蛋白治疗AIDP,血浆置换是第二常用的治疗方法。预后取决于髓鞘再生过程,但总体而言相对较好。超过80%的患者在康复一年内可以独立行走。
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Review of Acute Inflammatory Demyelinating Polyradiculoneuropathy
On average, 97% of patients in North America and Europe diagnosed with Guillain-Barré syndrome have a specific subtype known as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). AIDP is suspected to be a result of proinflammatory cytokines causing demyelination, specifically within the peripheral nervous system, in response to stress. Events that precede the onset of AIDP include gastrointestinal or respiratory infections, surgeries, and trauma. AIDP primarily presents with acute ascending paralysis and areflexia. Diagnosis of AIDP is confirmed by nerve conduction studies (NCSs) with electromyography (EMG) and lumbar puncture for cerebral spinal fluid (CSF) analysis. NCS/EMG is used to assess the peripheral nerve function, and serial examinations are often needed. CSF analysis is looking to identify albumino-cytological dissociation. Most commonly, intravenous immunoglobulin is used to treat AIDP with plasmapheresis being the second most common treatment. Prognosis is dependent on the remyelination process but overall is relatively good. More than 80% of patients can walk independently within one year of recovery.
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