GM1-IgM抗体阳性慢性炎性脱髓鞘性多神经病临床、电生理与病理特点

目的探讨GM1-IgM抗体阳性的慢性炎性脱髓鞘性多神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)的临床、电生理和病理改变特点。方法纳入研究的4例患者,分别为2例男性和2例女性,发病年龄在12~64岁之间,发病到就诊时间在6 m~2 y之间,3例出现非对称性下肢无力,1例表现为单侧上肢和对侧下肢无力。对4例患者进行血和脑脊液的GM1-IgM和IgG抗体检查,进行神经电生理检查和腓肠神经活检。结果 4例均出现血清GM1-IgM抗体阳性,电生理检查提示多个感觉神经和运动神经传导速度减慢和诱发电位波幅降低,1例出现神经传导阻滞现象。病理检查发现腓肠神经的轻度有髓神经纤维轴索变性和髓鞘脱失。结论GM1-IgM抗体阳性CIDP多表现为以下肢无力为主的非对称性神经病。     

慢性炎性脱髓鞘性多发性神经病的治疗进展

慢性炎性脱髓鞘性多发性神经病（chronic inflammatory demyelinating polyradiculopathy,CIDP）是一种获得性的免疫介导的周围神经病.临床特征包括进展性或复发性的肢体无力、感觉缺失和腱反射消失等.     

儿童慢性炎症性脱鞘性多神经病的临床和病理改变特点

目的探讨儿童慢性炎症性脱鞘性多神经病(chronic inflammatory demyelinating polyneuropathy,CI-DP)的临床及病理改变特点。方法根据欧洲神经肌肉病中心修订的儿童CIDP诊断标准诊断的10例17岁以下患者,收集其临床资料,进行周围神经电生理以及腓肠神经的病理检查。结果所有患者主要表现为肢体无力,分别有4例和3例出现四肢感觉减退和颅神经损害。9例有脑脊液蛋白细胞分离现象。10例均出现运动或感觉神经传导速度减慢及远端潜伏期延长,9例患者的动作电位波幅降低。所有患者的有髓神经纤维出现轻-重度减少,其中3例患者的纤维脱失程度在不同束间存在差异,6例患者以脱髓鞘为主;3例以轴索损害为主。1例患者仅出现轻微改变。9例患者存在炎细胞浸润。结论儿童CIDP以肢体无力为主。部分患者以轴索损害为主,神经纤维脱失程度可以存在束间差异。     

Chronic inflammatory demyelinating polyneuropathy associated with dysglobulinemia: a peripheral nerve biopsy study in 18 cases

The possible occurrence of chronic inflammatory demyelinating polyneuropathy (CIDP) in association with an identified dysglobulinemic status is recognized and a causal relationship between the two has been suggested. We had the opportunity to study 18 patients presenting with CIDP and dysglobulinemia. This was an IgG monoclonal gammopathy (IgG MG) in 8 cases, an IgM monoclonal gammopathy (IgM MG) in 8, an IgG-IgM biclonal gammopathy in 1 case and an IgM monoclonal cryoglobulinemia in another. A peripheral nerve biopsy specimen was available for all patients and the morphological findings in these specimens in the cases of CIDP with IgG MG or cryoglobulin did not differ from those without, whereas characteristic features were observed in the cases of CIDP with IgM MG and anti-myelin associated glycoprotein activity. Received: 7 April 1999 / Revised, accepted: 6 October 1999     

Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy

We describe a patient with the previously unreported association of chronic inflammatory demyelinating polyneuropathy (CIDP) and myasthenia gravis (MG). Immunosuppressive treatment with azathioprine and prednisone achieved clinical and electrophysiological remission of MG and improvement of CIDP. As ophthalmoplegia occurs infrequently in CIDP, the possibility of MG should be considered when this sign is present in a patient with CIDP. Since current therapy with corticosteroids, plasma exchange and other immunomodulating agents is effective against both diseases, their association may be undereported.     

High-dose intravenous gammaglobulin for chronic inflammatory demyelinating polyneuropathy

We report our preliminary experience of high-dose intravenous gammaglobulin in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) selected for inefficacy or severe side effects of steroid and immunosuppressive treatment. Our treatment proved safe and effective, reversing the disability of CIDP, the improvement being temporally related to the commencement of intravenous high-dose gammaglobulin. The possible mechanisms of action are discussed.

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Riassunto Gli AA. riportano i risultati emersi dal trattamento con gammaglobuline e.v. ad alte dosi in quattro pazienti affetti da polineuropatia cronica infiammatoria demielinizzante (CIDP), selezionati in base alla inefficacia o alla comparsa di gravi effetti collaterali da corticosteroidi e/o immunosoprressivi. Il trattamento fu prontamente seguito in ogni caso da sensibile miglioramento clinico, particolarmente vistoso in due pazienti; nessun effetto collaterale o complicanza risultò evidenziabile. I possibili meccanismi d'azione delle immunoglobuline intere e.v. ad alte dosi vengono discussi.

     

Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: A long-term follow-up study

This long-term follow-up study examined patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and only sensory symptoms at first presentation, with emphasis on the development of motor symptoms and long-term disability. From all CIDP patients referred to our Department between 1987 and 1995, seven had only sensory symptoms at first clinical presentation. These were investigated according to a standard protocol, including a quantified clinical neurological examination and nerve conduction studies. The mean duration of the disease before weakness developed was 3.1 years, but varied considerably (0.8–6.3 years). At follow-up, weakness developed in five patients and persisted in three of them. Five patients were not seriously incapacitated by their disease (Rankin 1 or 2), four of them being in remission now and one showing a very slow progression of disease. Two patients were moderately disabled (Rankin 3); one had severe persistent sensory ataxia and only weakness during relapses and one had stepwise progression and moderate weakness. Motor nerve conduction studies revealed that the most notable worsening in the entire group of patients was a decrease in distal compound muscle action potential amplitudes, indicating the development of distal conduction block or axonal degeneration. These findings show that CIDP with only sensory symptoms is a transient clinical stage that precedes the appearance of weakness in about 70% of patients. The long-term prognosis does not differ from that of patients with CIDP who have weakness at the beginning of the disease. Received: 3 December 1998 Received in revised form: 17 May 1999 Accepted: 2 July 1999     

Clinical and electrophysiological parameters distinguishing acute‐onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy

Up to 16% of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely. We performed a retrospective chart review on 30 acute inflammatory demyelinating polyneuropathy (AIDP) and 15 acute‐onset CIDP (A‐CIDP) patients looking for any clinical or electrophysiological parameters that might differentiate AIDP from acutely presenting CIDP. A‐CIDP patients were significantly more likely to have prominent sensory signs. They were significantly less likely to have autonomic nervous system involvement, facial weakness, a preceding infectious illness, or need for mechanical ventilation. With regard to electrophysiological features, neither sural‐sparing pattern, sensory ratio >1, nor the presence of A‐waves was different between the two groups. This study suggests that patients presenting acutely with a demyelinating polyneuropathy and the aforementioned clinical features should be closely monitored as they may be more likely to have CIDP at follow‐up. Muscle Nerve, 2010     

Nerve root hypertrophy in chronic inflammatory demyelinating polyneuropathy

A patient with chronic inflammatory demyelinating polyneuropathy (ClDP) and centrel demyelinating disease is desoribed in whom striking nodular filling defects on multiple lumbar–sacral nerve roots, mimicking neurofibromata, were observed at myelography and magnetic resonance imaging. We suggest that these lesions are secondary to recurrent segmental demyelination and remyelination and that the differential diagnosis of this radiological feature should include CIDP. © 1994 John Wiley & Sons, Inc.     

Pattern analysis of nerve enlargement using ultrasonography in chronic inflammatory demyelinating polyneuropathy

ObjectiveFocal nerve enlargement is a characteristic finding in chronic inflammatory demyelinating polyneuropathy (CIDP). We performed this study to assess the distribution of nerve enlargement through ultrasonographic examination of peripheral nerves and to correlate the ultrasonographic findings with clinical features.MethodsTo compare the ultrasonographic features of 10 subjects with CIDP with those of 18 healthy controls, we bilaterally measured the cross-sectional areas (CSA) of the vagus, brachial plexus, musculocutaneous, median, ulnar, radial, sciatic, tibial, common peroneal, and sural nerves. We also analyzed correlations between CSAs and various clinical and electrophysiological features.ResultsMean CSAs were significantly larger in CIDP patients than controls, especially at proximal and non-entrapment sites. CSAs were significantly correlated with muscle strength at initial presentation, but not at the time of ultrasonography. The CSAs of the median and ulnar nerves at the mid-forearm, tibial nerve at 7 cm proximal to the medial malleolus, and sural nerve correlated with the nerve conduction velocity of the corresponding region.ConclusionUltrasonography revealed widely distributed nerve enlargement, especially in proximal regions and non-entrapment sites, in patients with CIDP compared with healthy controls. Nerve enlargement correlated well with the electrophysiologic function of the nerve, but not current clinical status.SignificancePattern analysis of nerve enlargement using ultrasonography is a supportive tool in the diagnosis of CIDP.     

慢性炎症性脱髓鞘性多发性神经病临床及神经电生理研究

目的 分析慢性炎症性脱髓鞘性多发性神经病(CIDP)的临床及神经电生理表现.方法 选取2010-07-2012-07我院7例CIDP患者,对其临床资料进行回顾性研究,分析临床表现、脑脊液及神经电生理检测结果.结果 7例CIDP患者均有四肢或双下肢肌力下降,腱反射减弱或消失,脑脊液蛋白升高,神经电生理异常.出院后3例恢复较良好,另外4例出现2～4次复发.结论 CIDP的诊断应结合临床表现、脑脊液检查和神经电生理检查,应依据具体情况采用免疫球蛋白和(或)皮质激素治疗.     

The dropped head syndrome with chronic inflammatory demyelinating polyneuropathy

The dropped head syndrome occurs in a variety of neuromuscular disorders. We present a woman with chronic inflammatory demyelinating polyneuropathy who developed this syndrome, likely reflecting severe demyelination of nerves to cervical paraspinal muscles. © 1994 John Wiley & Sons, Inc.     

Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy

ObjectiveAim of this study was to develop and evaluate the applicability of an ultrasound score (Bochum ultrasound score – BUS) in distinguishing chronic (CIDP) from acute inflammatory demyelinating polyneuropathy (AIDP).Methods

• •Step 1: For the development of BUS 75 healthy-controls, 20 CIDP, 20 AIDP patients underwent US 4.55 ± 3.5 and 3.4 ± 2.91 years, respectively after onset. After comparing the distribution pattern and frequency of pathological US changes between the two study groups, we developed BUS, summarizing the cross sectional area (CSA) of: (1) the ulnar nerve in Guyons’ canal, (2) the ulnar nerve in upper-arm, (3) the radial nerve in spiral groove, (4) the sural nerve between the gastrocnemius muscle.
• •Step 2: The BUS underwent blinded evaluation in further 10 CIDP, 21 AIDP patients 3.8 ± 2.7 and 2.3 ± 1.5 years, respectively after onset.
• •Step 3: The BUS underwent blinded, prospective evaluation in 8 patients with acute/subacute polyradiculoneuropathy (5 CIDP, 3 AIDP) 2.6 ± 1.8 weeks after onset.

ResultsThe BUS showed a sensitivity of 90% and specificity of 90.4% (positive predictive value, PPV = 81.8%; negative predictive value, NPV = 95%) in distinguishing CIDP from AIDP, when they showed no differences in disease duration (p = 0.0551).In addition, the BUS distinguished subacute-CIDP from AIDP with a sensitivity of 80%, specificity of 100% (PPV = 100%, NPV = 75%).ConclusionThe BUS seems to allow a reliable distinction of CIDP from AIDP.SignificanceThe BUS may be helpful in distinguishing subacute-CIDP from AIDP.     

Concomitant chronic inflammatory demyelinating polyneuropathy and myasthenia gravis following cytomegalovirus infection

We describe a patient who concomitantly developed chronic inflammatory demyelinating polyneuropathy (CIDP) and myasthenia gravis (MG) following cytomegalovirus (CMV) infection. Whereas CIDP and MG presumably have different immunopathogeneses, a number of reports presented cases with CIDP and MG, some of which were concomitant cases. Several reports described association between CIDP or MG, and CMV infection, although the association is still a matter of controversy. This is the first report of patients with concomitant CIDP and MG following CMV infection. The association may be coincidental, but the possibility that CMV infection triggered development of both CIDP and MG simultaneously cannot be excluded.     

Unmyelinated fibers in sural nerve biopsies of chronic inflammatory demyelinating polyneuropathy

Summary Seven patients aged 29 to 76 years with various clinical subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated. Sural nerve biopsies were performed between 7 months and 19 years after onset of disease. Quantitative electron microscopy revealed involvement of primary unmyelinated fibers (UF) in all cases. When compared with age-matched controls from the literature and two controls of our own, there was an increase of degenerating primary UF in all cases, a definite decrease of density per mm² or number per nerve after subtraction of regenerates of myelinated and unmyelinated fibers in five cases, an increase of denervated Schwann cell complexes of the unmyelinated type in three cases, and an increased incidence of a high ratio (3) of primary UF per Schwann cell complex in five cases. Presumably due to the small number and heterogeneity of cases, the results did not correlate with type and duration of CIDP, but were obviously influenced by the degree of demyelination. The possible causes of UF damage in CIDP are discussed.     

儿童慢性炎症性脱髓鞘性多发性神经病的临床特征及肌肉回声强度与疾病严重程度的相关性分析

目的 探讨儿童慢性炎症性脱髓鞘性多发性神经病(Chronic inflammatory demyelinating polyneuropathy,CIDP)的临床特征及肌肉回声强度与疾病严重程度的相关性。方法 收集2015～2022年本院诊断为肯定型和可能CIDP患儿23例,其中男13例,女10例; 根据诊断年龄分为早期起病组8例(诊断年龄<4岁)和晚期起病组15例(诊断年龄≥4岁); 根据起病到病程高峰期时间分为慢性起病CIDP组(起病到病程高峰期时间≥2个月)和急性和亚急性起病CIDP组(起病到病程高峰期时间<2个月); 10例CIDP进行肌肉超声检查,肌肉超声回声强度分级按照赫克马特分级,并与患儿疾病严重程度进行相关性分析。结果 入组CIDP中首次诊断分别为CIDP7例,吉兰-巴雷综合征13例,运动发育迟缓1例,遗传代谢病1例、分离转换障碍1例; 早期起病CIDP和晚期起病CIDP组间临床特征无明显差异(P>0.05); 与慢性起病CIDP组比较,急性和亚急性起病CIDP组缓解一复发例数更多(χ²=10.879,P=0.001),起病到确诊时间更短(χ²=-11.340,P=0.000),误诊率更高(χ²=6.188,P=0.013); 肌肉超声中胫前肌和股内肌肌肉回声强度分级与疾病严重程度呈正相关(r=0.832,P=0.003)。结论 儿童CIDP最易误诊为GBS; CIDP肌肉超声回声强度与疾病严重程度呈正相关。