慢性炎症性脱髓鞘性多发性神经病的电生理及病理学特征

慢性炎症性脱髓鞘性多发性神经病(CIDP)是周围神经的慢性复发性疾病,也称慢性格林-巴利综合征,其发病率远低于急性炎症性脱髓性多发性神经病(AIDP),诊断上有一定的困难。收集近5年来收治6例CIDP病例,总结其临床和实验室特     

免疫球蛋白联合甲基泼尼松龙治疗慢性格林一巴利综合征疗效观察

慢性格林一巴利综合征即慢性炎症性脱髓鞘性多发性神经根神经炎（CIDP），是一种慢性一获得性脱髓鞘神经病，病因不明，目前认为与急性炎症性脱髓鞘性多发性神经根炎一样属于免疫介导的脱髓鞘疾病。作者自1998年6月至2005年6月收治经临床、脑脊液、肌电图确诊CIDP患者，分别采用静脉滴注免疫球蛋白联合甲基泼尼松龙冲击治疗（Ⅷ硼？）或单独使用MPPT治疗，现将临床疗效及随访情况报告如下。     

1例神经束蛋白抗体阳性的慢性炎性脱髓鞘性多发性神经根神经病病人的护理

正慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)是一种免疫介导性的周围神经病,同时累及感觉以及运动系统,多表现为慢性进行性的四肢无力以及末梢型感觉减退,静脉丙种球蛋白及激素治疗通常有效。CIDP发病机制尚未完全明确,可能与多种自身免疫反应有关。最新研究表明,CIDP病人常有周围神经郎飞结受     

慢性炎症性脱髓鞘性多发性神经病的电生理及病理学特征

慢性炎症性脱髓鞘性多发性神经病(CIDP)是周围神经的慢性复发性疾病，也称慢性格林—巴利综合征，其发病率远低于急性炎症性脱髓性多发性神经病(AIDP)，诊断上有一定的困难。收集近5年来收治6例CIDP病例，总结其临床和实验室特点，以提高对该病的认识。     

氯乙稀中毒性周围神经病23例临床分析

氯乙烯中毒性周围神经病 ,是氯乙烯慢性中毒常见疾病类型之一。主要以慢性多发性周围神经病的症状与体征为临床表现。我院近 8年来共收治因聚氯乙稀生产过程造成慢性氯乙稀中毒性周围神经病病人 2 3例。现分析报告如下。1　临床资料1)　一般资料 :本组病人均是聚氯乙烯车间合成     

静脉注射免疫球蛋白对慢性炎性脱髓鞘性多发性神经根神经病作用机制的研究进展

慢性炎性脱髓鞘性多发性神经根神经病(CIDP)是一类由免疫介导的主要累及神经根和周围神经的慢性脱髓鞘性神经病.目前CIDP的发病机制尚不明确,免疫治疗效果明显,特别是静脉注射免疫球蛋白(IVIg)较其他治疗相比,具有短期和长期疗效显著、副作用小等优势,临床上常作为CIDP的首选治疗.IVIg对CIDP的作用机制较复杂....     

HIV相关性周围神经病诊治进展

周围神经病是人类免疫缺陷病毒（human immunodeficiency virus, HIV）感染者最常见的神经系统疾病,临床表现以远端对称性周围神经病、急性炎症性脱髓鞘性多发性神经病或慢性炎症性脱髓鞘性多发性神经病及多发性单神经病最为常见,严重影响患者的生活质量。本文旨在通过分析HIV相关性周围神经病（HIV peripheral neuropathy,HIV PN）的病理生理变化及发病机制,深入了解HIV PN的临床表现,总结其诊治经验,从而指导临床医师正确认识、合理预防、规范诊治该病。     

1例慢性炎症性脱髓鞘性多发性神经病患者的护理

慢性炎症性脱髓鞘性多发性神经病（CIDP），为一组免疫介导的、慢性、复发性周围神经病。近年来随着对本病临床研究的深入，发病有日益增加的趋势。我科于2005年3月收治1例此病患者，经免疫球蛋白、免疫抑制剂、营养神经治疗及生理、心理上的整体护理，促进了患者的康复,现将护理体会报道如下。     

1例慢性炎症性脱髓鞘性多发性神经病患者的护理

慢性炎症性脱髓鞘性多发性神经病(CIDP),为一组免疫介导的、慢性、复发性周围神经病.近年来随着对本病临床研究的深入,发病有日益增加的趋势[1].我科于2005年3月收治1例此病患者,经免疫球蛋白、免疫抑制剂、营养神经治疗及生理、心理上的整体护理,促进了患者的康复,现将护理体会报道如下.     

慢性炎性脱髓鞘性多发性神经根神经病的临床治疗进展

慢性炎性脱髓鞘性多发性神经根神经病（chronic inflammatory demyelinating polyradiculoneuropathy, CIDP）是一类由免疫介导的运动感觉周围神经病,大部分患者均是缓慢起病,多伴有脑脊液蛋白一细胞分离,病理显示有髓纤维多灶性脱髓鞘、神经内膜水肿、炎细胞浸润等特点。     

接触性热痛诱发电位在慢性炎性脱髓鞘性多发性周围神经病中的应用

目的:应用接触性热痛诱发电位(CHEPs)技术研究慢性炎性脱髓鞘性多发性周围神经病(CIDP)患者的痛觉传导通路病变特点,探讨CHEPs在CIDP诊断中的应用价值。方法:选取确诊为CIDP的患者21例及对照组32例,应用CHEP刺激器进行刺激,记录Cz/N的潜伏期,分析比较外周神经Aδ纤维及N波峰潜伏期,同时进行神经传导速度的测定并比较其阳性率。结果:(1)对照组CHEPs波形引出率高于患者组。(2)患者组中VAS评分明显低于对照组(P<0.05)。(3)患者组Aδ纤维传导速度明显低于对照组(P<0.05)。(4)患者组上肢Aδ纤维传导速度减慢率较下肢低(P<0.05)。(5)患者组上下肢近端N波峰潜伏期较对照组延长(P>0.05);但患者组上下肢远端N波峰潜伏期较对照组延长(P<0.05)。(6)患者组Aδ纤维CV异常率高于MCV及SCV(P<0.05)。结论:CHEPs能反应CIDP患者的痛觉传导通路的异常部位及损伤程度,且较传统的神经传导速度更敏感,可作为临床诊断CIDP的一项电生理参考指标。     

History,Diagnosis, and Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is probably the best recognized progressive immune-mediated peripheral neuropathy. It is characterized by a symmetrical, motor-predominant peripheral neuropathy that produces both distal and proximal weakness. Large-fiber abnormalities (weakness and ataxia) predominate, whereas small-fiber abnormalities (autonomic and pain) are less common. The pathophysiology of CIDP is inflammatory demyelination that manifests as slowed conduction velocities, temporal dispersion, and conduction block on nerve conduction studies and as segmental demyelination, onion-bulb formation, and endoneurial inflammatory infiltrates on nerve biopsies. Although spinal fluid protein levels are generally elevated, this finding is not specific for the diagnosis of ClDP. Other neuropathies can resemble CIDP, and it is important to identify these to ensure correct treatment of these various conditions. Consequently, metastatic bone surveys (for osteosclerotic myeloma), serum electrophoresis with immunofixation (for monoclonal gammopathies), and human immunodeficiency virus testing should be considered for testing in patients with suspected CIDP. Chronic inflammatory demyelinating polyradiculoneuropathy can present as various subtypes, the most common being the classical symmetrical polyradiculoneuropathy and the next most common being a localized asymmetrical form, multifocal CIDP. There are 3 well-established, first-line treatments of CIDP—corticosteroids, plasma exchange, and intravenous immunoglobulin—with most experts using intravenous immunoglobulin as first-line therapy. Newer immune-modulating drugs can be used in refractory cases. Treatment response in CIDP should be judged by objective measures (improvement in the neurological or electrophysiological examination), and treatment needs to be individualized to each patient.     

Electrophysiological Evaluation of Chronic Inflammatory Demyelinating Polyneuropathy and Charcot-Marie-Tooth Type 1: Dispersion and Correlation Analysis

[Purpose] The purpose of this study was to analyze and compare electrophysiological characteristics observed in nerve conduction studies (NCS) of chronic inflammatory demyelinating polyneuropathy (CIDP) and Charcot-Marie-Tooth disease type 1 (CMT 1). [Subjects] A differential diagnosis of acquired and congenital demyelinating neuropathies was based on a study of 35 patients with NCS-confirmed CIDP and 30 patients with CMT 1 genetically proven by peripheral myelin protein-22 (PMP-22) gene analysis, pulsed-field gel electrophoresis (PFGE), and Southern blot analysis. [Methods] We analyzed values collected in motor nerve conduction studies. We conducted dispersion analysis of the amplitudes of the compound muscle action potential (CMAP) of various nerve types and correlation coefficient analysis of the motor nerve conduction velocity (MNCV). [Results] We found that CIDP and CMT 1 were clearly attributable to severe polyneuropathy. In dispersion analysis, CIDP showed greater differences in proximal-to-distal amplitude ratios. Moreover, CMT 1 showed relatively high correlations compared to CIDP based on correlation coefficient analysis of MNCV. [Conclusion] The results of this study suggest that CIDP showed greater asymmetry than CMT 1 in MNCV and CMAP amplitudes.Key words: Chronic inflammatory demyelinating polyneuropathy, Charcot-Marie-Tooth disease type 1, Dispersion and correlation analysis     

慢性炎症性脱髓鞘性多发性神经病的误诊分析

目的：探讨慢性炎症脱髓鞘性多发性神经病（CIDP）的临床特点，误诊原因及对策。方法：对误诊的12例CIDP病例进行回顾性分析。结果：本组患的临床表现主要有：首发症状：双下肢无力及足底感觉异常（68％），共有症状：不同程度的对称性四肢无力及腱反射减弱或消失（100％），特殊症状，深感觉障碍性共济失调2例，治疗中出现缓解，复发5例次；单纯运动障碍1例，12例中先后误诊为：未梢神经炎，共济失调，多发性硬化，肌病，脊髓病变，急性炎症性脱髓鞘性多发性神经病（AIDP），结论：对CIDP认识不足是误诊的主要原因，加强对本病的了解及认识，详尽的神经系检查，尤其是腱反射的检查，腰穿查有无脑脊液蛋白－细胞分离现象以及电生理检查，必要时行神经活检是提高CIDP检出率的关键。     

A pilot study to compare the use of the Excorim staphylococcal protein immunoadsorption system and IVIG in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated neuropathy responding to immunomodulation with IVIG or plasma exchange (PE). We tested the efficacy and safety of selective immunoglobulin removal by Excorim immunoadsorption (IA) in a pilot trial in CIDP patients randomized to monthly IA or IVIG treatments for 6 months. Response rates at 2 and 6 months were greater with IA due to longer disease duration and greater disability at baseline in the patients receiving IVIG. IA appears to be a safe and efficacious therapy for patients with CIDP, but an appropriately powered clinical trial with stratification for disease duration is required.     

Long‐term plasma exchange in pediatric CIDP

Therapeutic plasma exchange (TPE) is not frequently used in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) because it usually gives only a short‐term benefit. We report on a 16‐year‐old boy with renal insufficiency undergoing hemodialysis who developed CIDP and underwent TPE with dramatic long‐term response to therapy. Nerve ultrasound and MRI findings are also reported. In our patient TPE was chosen because he was already undergoing hemodialysis. Though it is not considered a first‐line therapy in pediatric CIDP, TPE may be a good therapeutic choice also in long‐term period. J. Clin. Apheresis 30:364–366, 2015. © 2015 Wiley Periodicals, Inc.     

Clinical and electrophysiological study of chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP)is a set of chronic,immune－ mediated polyneuropathy.The incidence of this disorder is increasing in recent years, but so far its mechanism is not known well. In order to delineate its clinical and electrophysiological features,mechanism and treatment,11patients with CIDP were assessed clinically and electrophsiologically in present study. 2 Clinical data 2.1 General data We collected 52 patients with Guillain－ Barre Syndrome(GBS)f…     

血浆置换在神经系统疾病中的应用

目的回顾分析血浆置换（plasma exchange,PE）治疗神经免疫性疾病患者的资料,了解其疗效和不良反应。方法对78 例应用PE 换治疗过的患者资料回顾性分析,其中包括73 例重症肌无力（myasthenia gravis,MG）,3 例吉兰巴雷综合征（Guillain-Barre′syndrome,GBS）,1例慢性炎性脱髓鞘性多神经根神经病（chronic inflammatory demyelinating polyneuropathy, CIDP）和1 例多发性硬化（multiple sclerosis,MS）。PE 头3 次为隔日1 次,之后为隔周1 次,偶有1周2 次。应用临床评分观察疗效,根据临床记录了解有关副反应。结果 73 例MG 患者PE 后第3d、第14d和第30d临床绝对评分明显降低,差异具有统计学意义。PE对3例GBS和1例CIDP患者疗效肯定,1例MS患者行双重置换（double filtration plasmapheresis,therapy, DFPP）后临床症状改善不明显,以上患者在PE 治疗过程中未见严重不良反应。结论 PE 在多数神经系统免疫疾病如MG、GBS 和CIDP 疗效肯定,不良反应较少。在治疗中枢神经系统脱髓鞘病（例如MS等）的情况有待进一步观察。     

Disability and health status in patients with chronic inflammatory demyelinating polyneuropathy

Purpose.?Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder affecting the peripheral nerves. The purpose of this study was to describe disability and health status in patients with CIDP in Sweden.

Methods.?All 22 adult patients with CIDP at the Karolinska University Hospital, Huddinge were invited to participate. Twenty-one patients performed all measures. Their mean age was 54 years. The following measures were used: vibration perception threshold (VPT); the Fatigue Severity Scale (FSS); the Berg Balance Scale; finger dexterity using the Nine-Hole Peg Test (NHPT); functional mobility using the Timed ‘Up and Go’ Test; health status with the SF-36 questionnaire.

Results.?Fifty-seven per cent of the patients had a higher thumb and ankle VPT than published normative data. The FSS showed that 38% scored over 5, indicating severe fatigue. The majority of the patients had reduced functional balance. Sixty-two per cent had a subnormal result on the NHPT. Results of the SF-36 showed lower scores than the Swedish norm on the sub-scales describing physical health.

Conclusions.?The majority of the patients with CIDP had disabilities and decreased physical health status. The presence of fatigue may be taken into consideration in immunomodulatory treatments and during physical rehabilitation.     

Treatment of chronic dysimmune polyneuropathies

Chronic dysimmune neuropathies are less common than Guillain-Barré syndrome (GBS). They mainly comprise chronic idiopathic demyelinating polyneuropathy (CIDP), multifocal motor neuropathy with persistent conduction blocks (MMNCB) and polyneuropathy associated with monoclonal gammopathy. However, as GBS, they are considered to be immune-mediated disorders and they may respond to various immunosuppressive treatments, such as corticosteroids, plasma exchanges (PE) or intravenous immunoglobulins (IVIg). The first step is to characterize these neuropathies on clinical, electrophysiological and sometimes immunochemical criteria, because the response to treatment may be different according to the type of neuropathy. For example, polyneuropathy associated with IgM monoclonal gammopathy does not respond to steroids and MMNCB may worsen under PE, while CIDP may respond either to steroids, PE or IVIg. The second step is to choose the type and the regimen of the treatment. Some neurological conditions may require only short-term therapy, for example in relapsing CIDP, whereas other conditions may require long-term therapy.