慢性炎性脱鞘性多发性神经根神经病并脑内脱髓鞘病变一例

<正>慢性炎性脱鞘性多发性神经根神经病(chronic inflammatory demyelinating polyradiculoneuropathy,CIDP)并脑内脱髓鞘病变临床鲜有报道。本文对宁夏医科大学总医院神经内科收治的1例CIDP合并脑内脱髓鞘病结合相关文献进行报道。1病例报告患者女性,66岁,主因"四肢麻木、无力半     

糖尿病合并慢性炎症性脱髓鞘性多发性神经病-4例临床分析并文献回顾

目的探讨糖尿病(diabetic mellitus,DM)合并慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)的临床、电生理特点,并与糖尿病周围神经病(diabetic peripheral neuropathy,DPN)进行早期鉴别诊断。方法回顾性分析4例DM合并CIDP患者的临床表现、电生理检查及诊疗特征。结果 4例DM合并CIDP患者中,1例仅表现为对称性肢体乏力,其余3例均伴有对称性的麻木或疼痛,仅1例患者伴有颅神经损害;4例患者均存在腱反射均减弱或消失,病程均超过2个月,且均有脑脊液蛋白-细胞分离现象;4例患者肌电图检查均提示脱髓鞘病变为主,使用激素冲击治疗后症状均好转,其中2例复发患者分别采用丙种球蛋白和血浆置换术治疗后症状好转,4例患者目前均恢复良好。结论当糖尿病患者出现周围神经病变时,早期根据其临床特征及辅助检查,诊断其是否合并CIDP,并对DM合并CIDP患者合理使用免疫抑制治疗效果良好。     

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。     

急、慢性炎性脱鞘性多神经根神经病患者CD+4T细胞、CD+8T细胞及NK细胞活性的动态研究

目的ＣＤ＋４Ｔ细胞、ＣＤ－８细胞以及ＮＫ细胞杀伤活性在炎性脱鞘性神经病中的变化规律和意义。方法分离外周单核淋巴细胞,采用ＡＰＡＡＰ法研究Ｔ细胞亚群、ＬＤＨ释放试验检测ＮＫ细胞活性。结果。ＡＩＤＰ发病初期ＣＤ４＋Ｔ细胞为主．在疾病的恢复期以ＣＤ８－Ｔ细胞为主,ＮＫ细胞的活性早于Ｔ细胞亚群的改变。ＣＩＤＰ病人ＣＤ＋８Ｔ细胞远远超过ＣＤ－４Ｔ细胞,在整个临床疾病过程中ＮＫ细胞活性持续较低。结论,ＡＩＤＰ和ＣＩＤＰ存在着不同的免疫损伤机制在疾病的不同时期ＣＤ４＋Ｔ和ＣＤ８－Ｔ细胞发挥了不同的作用,ＮＫ细胞可能具有重要的调节作用。     

慢性炎症性脱髓鞘性多发性神经病的治疗分析

目的 比较肾上腺皮质激素（ACH）、血浆置换（PE）、免疫球蛋白（Ig）的治疗慢性炎症性脱髓鞘性多发性神经病（CIDP）的疗效。方法 对46例CIDP患者分别采用ACH、PE、Ig治疗,观察其治疗后1个月的疗效、停药后的反跳复发现象和再治疗的疗效。结果 治疗1个月时各组的有效率分别为ACH组50％、PE组75％、Ig组67％,3组间比较无显著性差异（P＞0．05）。起效时间ACH组5－45天,中位数15天;PE组20－50天,中位数4天;Ig组4－15天,中位数5天。PE、Ig组起效时间较ACH组明显缩短（P＜0．001）。各组治疗后反跳率,ACH组为50％、PE组83％、Ig组50％,3组间比较无显著性差异（P＞0．05）。3组再治疗后均获改善。结论 ACH、PE、Ig都是治疗CIDP的有效方法。PE、Ig组志效较ACH组快。3组有反跳复发,而ACH组过快减量,过早停药时尤著。     

炎症性脱髓鞘性多发性神经病急性期临床评价

目的比较急性炎症性脱髓鞘性多发性神经病(AIDP)与慢性炎症性脱髓鞘性多发性神经病急性起病(A-CIDP)成年患者的临床和电生理特点。方法收集AIDP或A-CIDP患者44例(AIDP,n=30;A-CIDP,n=14)。分析人口学特征、临床表现、脑脊液特点、电生理检测特点。结果 AIDP与A-CIDP患者多见于男性(53%vs 72%,P=0.33),AIDP具有糖尿病病史患者比例相对较低(3%vs 28%,P=0.03),脑神经功能障碍较多见(56%vs 14%,P=0.01),从发病进展到病情最高峰的时间较短(8.2d vs 18.2d,P=0.04);MRC总分均值较低(40 vs 50,P=0.01),mNCV减慢的百分比较低(33%vs 71%,P=0.04)。结论发病进展到病情最严重时间为8 d左右,表现有脑神经功能障碍,MRC总分较低,mNCV减慢比例较低且有轴索损伤,应考虑诊断AIDP,反之则考虑A-CIDP。     

急、慢性炎性脱鞘性多神经根神经病患者CD+4T细胞、CD+8T细胞及NK细胞活性的动态研究

目的　CD     

重症肌无力合并慢性炎症性脱髓鞘性多神经病一例

患者女性,65岁,因“右眼睑下垂9个月,四肢无力3个月”于1998年2月就诊于我院,症状晨轻暮重,有贫血史.查体：睑裂左6 mm,右1 mm,左眼外展露白1 mm,双眼上下视不能,埋睫征消失,鼻音、语音嘶哑,饮水呛咳,无憋气,平卧位抬头不能,伸舌受限,四肢近端肌力Ⅴ-级,远端Ⅴ级,侧平举和直腿抬高时间为5 s.注射新斯的明1 mg后 30 min 睑裂增加为10 mm,侧平举时间45 s.重复神经刺激低频递减,诊断为“重症肌无力”.采用激素渐增法治疗1个月后可抬头18 s,侧平举40 s,外展露白、眼球运动受限、饮水呛咳以及伸舌受限均明显改善,肌力正常.CT和磁共振成像（MRI）发现胸腺瘤,     

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。     

急、慢性炎症性脱髓鞘性多发性神经病神经电生理对比研究

目的 比较分析急性炎症性脱髓鞘性多发性神经病(AIDP)与慢性炎症性脱髓鞘性多发性神经病(CIDP)的电生理表现.方法 收集2011年1月～2013年1月在吉林大学白求恩第一医院神经内科就诊的19例AIDP患者及15例CIDP患者,分析上下肢周围神经传导检查各项指标.结果 AIDP与CIDP均表现为运动传导速度(MCV)减慢、远端潜伏期延长、波幅降低、传导阻滞、F波及H反射异常,但CIDP组MCV减慢明显,与AIDP组存在显著差异,且CIDP组感觉传导检测异常明显,AIDP组感觉神经传导异常少见.结论 AIDP患者主要以周围神经运动纤维受损为主,存在明显的脱髓鞘及轴索的损伤,但周围神经感觉纤维受损不明显.CIDP患者周围神经运动纤维及感觉纤维受损均非常明显,且脱髓鞘程度明显重于AIDP患者.     

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.     

Treatment of pediatric chronic inflammatory demyelinating polyneuropathy: Challenges,controversies and questions

Pediatric chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon acquired disorder of unknown cause, presumed to have an immunological basis. We report 20 patients seen at Children''s Hospital Los Angeles over a period of 10 years. The outcome of our patients was favorable in a vast majority with good response to various treatments instituted. However, residual neurologic deficit was common. The choice of treatment modality was empirical and selected by the treating neurologist. Intravenous immunoglobulin (IVIG) and corticosteroids were most commonly utilized for treatment. Plasmapheresis, mycophenolate mofetil, rituximab, cyclophosphamide, azathioprine, and abatacept were added if the patients were refractory to IVIG or became corticosteroid dependent. The spectrum of disease severity ranged from a single monophasic episode, to multiphasic with infrequent relapses with good response to IVIG, to progressive disease refractory to multiple therapies.     

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.     

Chronic inflammatory demyelinating polyneuropathy in childhood: clinical and electrophysiological features

Five children with chronic progressive polyneuropathy but no familial history of it showed electrophysiological evidence of demyelination with partial conduction block, temporal dispersion, and focal slowing of nerve conduction velocities in multiple nerves. These findings are indicative of an acquired demyelinating polyneuropathy that is chronic and inflammatory and differentiate this condition from most of the inherited neuropathies. It is very important to recognize this entity because of the avaibility of various treatments.     

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.     

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.