影响慢性炎症性脱髓鞘诊断的因素

Amer Avdagic, Tejas Mehta, Krista Schoff, Raghav Govindarajan
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摘要

背景:慢性炎症性脱髓鞘多神经病变(CIDP)是一种神经系统疾病,导致周围神经脱髓鞘表现为一系列症状。CIDP的症状包括但不限于感觉丧失、反射丧失、刺痛和疼痛以及虚弱。欧洲联邦神经学会(EFNS)已经制定了诊断这种疾病的指南。本研究的目的是观察EFNS诊断标准与诊断为CIDP的患者是否符合该标准之间的关系。完成对诊断为CIDP的患者的数据收集,然后对诊断但不符合标准的患者进行分析,以查看是否存在导致诊断的共同异常值。结果:共纳入20例患者,其中男性13例,女性7例。使用EFNS/PNS指南正确诊断的患者中有83%在诊断时表现出反射不足。绝大多数使用EFNS/PNS指南正确诊断的患者(83%)在诊断时表现出远端无力。在诊断时,肌电图显示,大多数不符合EFNS/PNS标准的患者没有表现出潜伏期增加的神经。58%的符合EFNS/PNS指南标准的患者有两个或更多的神经表现出增加的潜伏期。测试患者的速度显示,所有不符合EFNS/PNS标准的患者都没有出现神经速度降低。结论:CIDP误诊仍然是导致这些患者管理不善的一个问题。本研究表明,即使不符合电生理标准,临床成分也优先用于诊断CIDP。
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Factors Influencing the Diagnosis of Chronic Inflammatory Demyelinating
BACKGROUND: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a neurological disorder that leads to demyelination of peripheral nerves presenting with an array of symptoms. Symptoms of CIDP include but are not limited to loss of sensation, loss of reflexes, tingling and pain, and weakness. European Federation Neurological Society (EFNS) has developed guidelines for the diagnosis of this disorder. The objective of this study is to look at the relationship between the EFNS diagnostic criteria and whether patients that have the diagnosis of CIDP met this criteria. Data collection was completed on the patients diagnosed with CIDP and then the patients that were diagnosed but did not meet the criteria were analyzed to see what common outliers exist that led to the diagnosis.   RESULTS: A total of 20 patients (13 males and 7 females) were included in the study. Eighty-three percent of patients that were correctly diagnosed using the EFNS/PNS guidelines displayed hyporeflexia at the time of their diagnosis. A large majority of the patients (83%) correctly diagnosed using the EFNS/PNS guidelines displayed distal weakness at the time of their diagnosis. At the time of their diagnosis, EMG showed that majority of those who did not meet the EFNS/PNS criteria had no nerves that displayed increased latency. Fifty-eight percent of those who did meet the criteria outlined by the EFNS/PNS guidelines had two or more nerves that presented with increased latency. Testing the velocity of patients displayed that all of the patients that did not meet the EFNS/PNS criteria did not present with nerves that had diminished velocity.   CONCLUSION: CIDP misdiagnosis continues to be an issue leading to mismanagement of these patients. This study showed a preference of the clinical component for diagnosis of CIDP even if electrophysiological criteria was not met.
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