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.     

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.     

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     

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.     

Mandibular nerve involvement in diabetic polyneuropathy and chronic inflammatory demyelinating polyneuropathy

Sensory complaints in the area of the mandible and mouth often escape notice or remain undiagnosed. Using electromyographic recording of the trigeminal reflexes and motor responses, we sought trigeminal dysfunction in 50 patients with peripheral neuropathy, and tried to gain pathophysiological information on the mechanisms provoking trigeminal damage. Trigeminal reflex recordings (early and late blink reflex after supraorbital stimulation, early and late masseter inhibitory reflex after mental stimulation, and jaw jerk) disclosed abnormalities caused by sensory trigeminal neuropathy in 8 out of 15 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 13 out of 23 patients with severe diabetic polyneuropathy, and in none of 12 patients with mild diabetic polyneuropathy. Six patients had abnormal motor responses in facial or masseter muscles. The response affected most frequently was the masseter early inhibitory reflex (also called first silent period, SP1) after mental nerve stimulation, its latency being strongly delayed. We found these long delays not only in patients with CIDP, but also in diabetic patients with severe polyneuropathy. We conclude that peripheral polyneuropathies often cause subclinical damage to the trigeminal nerve, especially to its mandibular branch. We believe that the nerve fibers running along the alveolar–mandibular pathway are more exposed to damage because of their cramped anatomical route in the mandibular canal and below the internal pterygoid muscle and fascia. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1673–1679, 1998     

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.

     

Differences between acute‐onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A‐CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed. Demographic characteristics, clinical manifestations, cerebrospinal‐fluid (CSF) findings, treatment and clinical evolution were analyzed. Nerve conduction studies were performed in all patients with at least 12 months follow‐up. A total of 91 patients were included (AIDP, n = 77; A‐CIDP, n = 14). The median age was 55.5 years in patients with A‐CIDP vs 43 years in AIDP (P = .07). The history of diabetes mellitus was more frequent in A‐CIDP (29% vs 8%, P = .04). No significant differences between groups were observed with respect to: human immunodeficiency virus (HIV) status, presence of auto‐immune disorder or oncologic disease. Cranial, motor and autonomic nerve involvement rates were similar in both groups. Patients in the A‐CIDP group showed higher frequency of proprioceptive disturbances (83% vs 28%; P < .001), sensory ataxia (46% vs 16%; P = .01), and the use of combined immunotherapy with corticoids (29% vs 3%; P = .005). There were no significant differences in CSF findings, intensive care unit (ICU) admission, or mortality rates. During the first 8 weeks both entities are practically indistinguishable. Alterations in proprioception could suggest A‐CIDP. Searching for markers that allow early differentiation could favor the onset of corticotherapy without delay.     

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.     

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。     

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.     

Treatment of chronic inflammatory demyelinating polyneuropathy

The management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the main topic of this review. A few comments will also be made about treatment of the demyelinating form of paraproteinaemic demyelinating polyneuropathy (PDN) and of multifocal motor neuropathy (MMN). The review briefly describes the main characteristics of these neuropathies, and examines case series and trials which evaluated the principal therapeutic strategies for CIDP, PDN and MMN, such as intravenous immunoglobulin (IVIg) therapy, steroid treatment, plasma exchange and immunosuppressor administration. Controlled trials demonstrated that IVIg, steroid treatment and plasma exchange are effective in CIDP. For PDN the therapeutic strategies are the same as for idiopathic CIDP, but usually the clinical response is poorer. For MMN, IVIg therapy is definitely the first choice treatment.

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Sommario Lo scopo principale di questa review è di esaminare le strategie terapeutiche nella neuropatia infiammatoria cronica demielineizzante (CIDP). Alcuni commenti vengono fatti anche riguardo il trattamento della forma demielinizzante della neuropatia demielinizzante paraproteinemica (PDN) e della neuropatia motoria multifocale (MMN). La review delinea le principali caratteristiche cliniche delle diverse forme di neuropatia demielinizzante ed esamina i principali studi clinici, controllati e non controllati, che hanno valutato il trattamento deila CIDP e della PDN e MMN con immunoglobuline endovena (IVIg), con la terapia steroidea, la plasmaferesi e farmaci immunosoppressori. Studi controllati hanno dimostrato che le IVIg, to steroide e la plasmaferesi sono efficaci nella CIDP. Per quanto riguarda la PDN le strategic terapeutiche sono analoghe a quelle adottate nella CIDP anche se la risposta clinica è solitamente meno evidente. Infine, nella MMN le IVIg sono sicuramente il trattamento di prima scelta.

     

Clinical heterogeneity in mild chronic inflammatory demyelinating polyneuropathy

We describe the clinical presentation, progression and electrodiagnostic features of three patients with a mild form of chronic inflammatory demyelinating polyneuropathy (CIDP). The unusually mild but also variable clinical picture was a cause of diagnostic uncertainty in all, but CIDP was eventually confirmed by extensive electrophysiological studies in each case, as well as by histology in one. Cerebrospinal fluid protein was raised in only one patient. Two patients were treated by intravenous immunoglobulins and both improved. Awareness of the existence of this relatively benign form of CIDP in its various presentations is essential as it can be functionally disabling, progress to more severe symptomatology, and as patients may benefit from immunomodulatory therapy.     

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.