腓骨肌萎缩症神经电生理及组织病理学研究

目的:研究腓骨肌萎缩症(charcot-Marie-Tooth disease,CMT)临床特征分析神经电生理检测在其诊断和分型中的价值。方法:CMT 20例其临床特征,神经电生理及腓肠神经活检结果进行分析。结果:20例中均表现为慢性进行性双下肢或四肢无力及肌萎缩。双上肢以前臂1/2远端明显。双下肢以大腿下1/3远端明显。骨骼变形,双手呈爪形,双足高弓。5例合并感觉障碍。15例运动神经传导速度减慢(15-28m/s)。10例腓肠神经活检符合慢性脱髓鞘性周围神经病。其中6例有剥洋葱样改变,4例符合慢性轴索神经病。结论:慢性进行性双下肢或四肢无力及肌萎缩为主要临床特征的腓骨肌萎缩征分为Ⅰ型(脱髓鞘型)和Ⅱ型(轴索型)两个主要亚型。周围神经电生理检查是诊断和区分不同亚型的可靠方法。而腓肠神经活检是进一步提高对CMT临床认识和优化基因突变检测程序,明确诊断和分型的客观依据。     

格林-巴利综合征的临床、电生理、病理对照研究

目的 探讨格林-巴利综合征（GBS）的临床，电生理分型与病理改变相关性及治疗前后电生理动态改变特征及预后，方法 对45例GBS的临床，电生理改变特点和10例腓肠神经病理特征及5例电生理动态随访结果进行相关性，对照组研究。结果 本组病例电生理改变特点为：运动神经传导速度（MCV）和f波异常率大于感觉神经传导速度（SCV）异常率；绝大多数电生理改变符合脱髓鞘型损害（96%），少数为轴索损害型（4%），腓肠神经病理结果与电生理改变相符合，电生理动态随访显示；临床症状恢复早于电生理的恢复；MCV和EMG恢复较SCV快。结论 电生理检查除了作为GBS诊断重要手段之一。其电生理分型与其病理改变相吻合，可作为临床判断疗效和预后的客观指标之一及神经康复的动态随访指标。     

Guillain-Barre综合征的临床、电生理和病理学特征(附19例分析)

目的 探讨Guillain-Barre综合征(GBS)的临床、电生理及病理学特征。方法 对19例GBS患者的临床特点、电生理、病理结果进行回顾分析。结果 19例中脱髓鞘型18例，伴有轴索变性1例；临床表现及电生理检查均以髓鞘脱失为主要形式，与病理检查结果一致。结论 本组病例的发病形式主要是原发性脱髓鞘型。电生理检查在GBS的分型中有较大诊断价值。     

Guillain-Barré综合征患者电生理分型分析

目的探讨Guillain-Barré综合征（GBS）的电生理学分型及各型间的差异。方法根据电生理检测结果对54例GBS患者作电生理分型,并对各型的临床表现和脑脊液检验结果进行比较分析。结果54例患者中;行电生理检查的有42例,其中脱髓鞘型占19例（45．24％）,轴索型11例（26．19％）,不明确型5例（11．90％）,正常型7例（16．67％）,失神经电位型0例。大部分脱髓鞘型GBS患者多半伴有不同程度的轴索损害。脱髓鞘型与轴索型之间脑脊液蛋白含量及脑脊液异常率之间有明显差异（P均〈0．05）;在前驱感染及F波异常率之间没有差异（P均〉0．05）。结论电生理检查对GBS具有快捷、经济、安全和微创等优点,但目前尚无统一的分型,其对GBS的诊断具有重要的意义。     

Guillain-Barré综合征患者电生理分型分析

目的探讨Guillain-Barré综合征（GBS）的电生理学分型及各型间的差异。方法根据电生理检测结果对54例GBS患者作电生理分型,并对各型的临床表现和脑脊液检验结果进行比较分析。结果54例患者中;行电生理检查的有42例,其中脱髓鞘型占19例（45．24％）,轴索型11例（26．19％）,不明确型5例（11．90％）,正常型7例（16．67％）,失神经电位型0例。大部分脱髓鞘型GBS患者多半伴有不同程度的轴索损害。脱髓鞘型与轴索型之间脑脊液蛋白含量及脑脊液异常率之间有明显差异（P均〈0．05）;在前驱感染及F波异常率之间没有差异（P均〉0．05）。结论电生理检查对GBS具有快捷、经济、安全和微创等优点,但目前尚无统一的分型,其对GBS的诊断具有重要的意义。     

有机磷中毒后迟发性周围神经病的临床和神经病理四例

目的探讨有机磷中毒后迟发性周围神经病(OPIDP)的临床及神经病理改变特点。方法对4例口服有机磷后出现周围神经损害症状的患者进行电生理和腓肠神经活检检查。结果 4例患者于急性中毒后平均20.5(10～24)天出现以下肢受累为主的逆行性运动感觉周围神经病,其中2例患者存在锥体束征。电生理检查提示以运动神经受累为主的轴索性周围神经损害。腓肠神经活检主要表现为与病程相关的轴索损害及再生现象,可见急性期的炎性反应、小纤维损害和继发的髓鞘改变。结论有机磷中毒后迟发性周围神经病表现为以运动障碍为主的逆行性神经病,中枢神经系统亦可能受累及;周围神经病理表现为轴索损害为主,同时存在小纤维损害及继发的髓鞘改变。     

复发性吉兰-巴雷综合征的临床与病理研究(附23例报告)

目的:研究复发性吉兰-巴雷综合征(GBS)的临床和病理特征,以及急性复发性脱髓鞘性多发性神经病(复发型AIDP)与慢性复发性脱髓鞘性多发性神经病(复发型CIDP)的鉴别。方法:23例患者,复发型AIDP10例,复发型CIDP13例,分析比较二组病人的临床表现,病程特点,腰穿脑脊液(CSF)检验,肌电图电生理检查,以及腓肠神经活检病理诊断。结果:二组病例首次发病前有上呼吸道感染史者多见,复发均无诱因;临床表现为对称性肢体肌无力,末梢型感觉障碍;多数可见CSF蛋白细胞分离;肌电图呈周围性神经源性损害。与复发型CIDP比较,复发型AIDP起病较快,临床发病期病程短,恢复较为完全,复发间距长,颅神经损害更为多见。腓肠神经病理示:复发型AIDP可见炎症细胞浸润,神经纤维密度明显减少;复发型CIDP以脱髓鞘病变为主,施万细胞增生明显,可有洋葱头样改变。结论:复发型AIDP与复发型CIDP的临床与病理均有所不同,腓肠神经活检对复发性GBS具有重要的诊断价值。     

慢性格林-巴利综合征49例临床、电生理与病理研究

目的研究慢性格林-巴利综合征(CIDP)的临床、电生理及病理特征,探讨腓肠神经活检的诊断价值.方法总结49例CIDP患者的临床表现,病程特点,腰穿脑脊液(CSF)检查、肌电图检查以及21例患者的腓肠神经活检病理结果.结果本组49例CIDP患者大多无明显前驱因素,临床表现为对称性肢体运动和感觉障碍,少数(16例)可伴颅神经损害;脑脊液蛋白量波动较大,单次腰穿24例可见蛋白细胞分离;大多数肌电图(39例/466例)提示有脱髓鞘损害,少数有轴索损害(15例/46例).21例患者行腓肠神经病理检查,显示明显脱髓鞘病灶17例,轴索变性5例,洋葱头样改变5例,炎性细胞浸润11例.结论电生理和病理检查均提示CIDP是以神经脱髓鞘改变为主,对临床表现不典型者腓肠神经活检有较大的诊断价值.     

副肿瘤性周围神经病的临床、电生理及病理改变特点

目的探讨副肿瘤性周围神经病(PPN)的临床、电生理及病理改变特点。方法回顾性分析4例PPN患者的临床资料。结果 4例患者均以疼痛、麻木等感觉症状起病,其中3例首先累及双下肢,3例双下肢不对称受累,3例累及植物神经,2例累及颅神经,所有患者周围神经症状均由远端向近端进行性发展。4例患者均出现感觉神经传导异常,其中3例以轴索损害为主,1例以脱髓鞘损害为主;1例伴有运动神经传导异常,以脱髓鞘损害为主。4例行腓肠神经活检的患者中,3例为活动性轴索改变,1例为轴索和髓鞘混合性改变,4例均无小血管周围炎性改变。结论 PPN多为不对称分布的感觉神经病,进行性发展,颅神经和植物神经受累常见。PPN的电生理以感觉神经传导异常为主、轴索损害较重。活动性轴索变性是PPN的主要病理改变,极少伴有小血管周围炎。     

急性和慢性多发性周围神经病的电生理及形态学观察

目的寻找神经、肌肉电生理和腓肠神经病理在急性和慢性炎性脱髓鞘性多发性周围神经病(GBS和CIDP)的诊断价值。方法总结GBS和CIDP(15例和17例)的临床、电生理及病理资料进行回顾性分析。结果EMG异常而神经电生理正常共8例;临床和电生理均未提示感觉异常的患者病理发现髓鞘和轴突的丧失、髓鞘再生及许旺细胞内结构改变。结论腓肠神经活检及神经、肌电生理的测定是本组疾病相辅相成的辅助检查手段。     

Physiologic-pathologic correlation in Guillain-Barré syndrome in children

OBJECTIVE: To correlate electrophysiologic patterns with sural nerve pathology in children with Guillain-Barré syndrome (GBS). BACKGROUND: Based on electrophysiologic and pathologic observations, GBS has been divided into demyelinating and axonal subtypes. The acute motor axonal neuropathy (AMAN) involves predominantly motor nerve fibers with a physiologic pattern suggesting axonal damage, whereas the acute inflammatory demyelinating polyneuropathy (AIDP) involves both motor and sensory nerve fibers with a physiologic pattern suggesting demyelination. In this study, we sought to confirm these observations by correlating sural nerve pathology with electrophysiologic findings in GBS patients. METHODS: Biopsies of sural nerve from 29 of 50 prospectively studied GBS patients were obtained. Nerves were examined by light and electron microscopy, and with immunocytochemistry for macrophages, lymphocytes, and complement activation products. RESULTS: Sural nerves from AMAN patients were normal or had only a few (0.1% to 0.7%) degenerating fibers without lymphocytic infiltration or complement activation. One patient with reduced sural sensory nerve action potential classified as acute motor sensory axonal neuropathy (AMSAN) had many degenerating fibers (2.3%) in the sural nerve. All three AIDP patients displayed active demyelination, and in two patients, lymphocytic infiltration and complement activation products were observed on the abaxonal Schwann cell surface. CONCLUSION: Classification of Guillain-Barré syndrome subtypes based on motor conduction studies correlates closely with pathologic changes seen in sural nerve. In acute motor axonal neuropathy cases, the sural nerve is almost completely spared pathologically. In acute inflammatory demyelinating polyneuropathy cases, macrophage-mediated demyelination and lymphocytic infiltration are common in the biopsies of sural nerves.     

Optimizing the electrodiagnostic accuracy in Guillain-Barré syndrome subtypes: Criteria sets and sparse linear discriminant analysis

ObjectiveTo optimize the electrodiagnosis of Guillain-Barré syndrome (GBS) subtypes at first study.MethodsThe reference electrodiagnosis was obtained in 53 demyelinating and 45 axonal GBS patients on the basis of two serial studies and results of anti-ganglioside antibodies assay. We retrospectively employed sparse linear discriminant analysis (LDA), two existing electrodiagnostic criteria sets (Hadden et al., 1998; Rajabally et al., 2015) and one we propose that additionally evaluates duration of motor responses, sural sparing pattern and defines reversible conduction failure (RCF) in motor and sensory nerves at second study.ResultsAt first study the misclassification error rates, compared to reference diagnoses, were: 15.3% for sparse LDA, 30% for our criteria, 45% for Rajabally’s and 48% for Hadden’s. Sparse LDA identified seven most powerful electrophysiological variables differentiating demyelinating and axonal subtypes and assigned to each patient the diagnostic probability of belonging to either subtype. At second study 46.6% of axonal GBS patients showed RCF in two motor and 8.8% in two sensory nerves.ConclusionsBased on a single study, sparse LDA showed the highest diagnostic accuracy. RCF is present in a considerable percentage of axonal patients.SignificanceSparse LDA, a supervised statistical method of classification, should be introduced in the electrodiagnostic practice.     

Serum antibody against a peripheral nerve myelin ganglioside, LM1, in Guillain-Barré syndrome.

Serum IgG antibody against LM1, the predominant ganglioside in the human peripheral nerve myelin, was found in 7 out of 140 patients with Guillain-Barré syndrome (GBS) in the acute phase, 1 out of 33 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), and 2 out of 47 patients with Miller Fisher syndrome (MFS). Anti-LM1 IgM antibody was detected only in 2 patients, each with GBS and MFS. The clinical and electrophysiological features of the seven GBS patients with anti-LM1 IgG antibody in the serum were investigated. Six patients recovered to grade 1 within one month of the onset of neuropathy. Electrophysiological studies revealed demyelination in five patients, of which one had axonal damage in addition, whereas sufficient evidence of demyelination or axonal degeneration was not observed in the remaining two. Five had a respiratory tract infection before the onset of neuropathy, and also had serum anti-GQ1b IgG antibody. IgG antibody against LM1 might be involved in the pathogenetic mechanisms of GBS, as a possible demyelinating factor. Presence of both anti-GQ1b and anti-LM1 antibodies may be associated with some infectious agent(s) affecting the respiratory tract.     

Guillain‐BarrÉ syndrome subtype diagnosis: A prospective multicentric European study

Introduction: There is uncertainty as to whether the Guillain‐Barré syndrome (GBS) subtypes, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), can be diagnosed electrophysiologically. Methods: We prospectively included 58 GBS patients. Electrodiagnostic testing (EDX) was performed at means of 5 and 33 days after disease onset. Two traditional and one recent criteria sets were used to classify studies as demyelinating or axonal. Results were correlated with anti‐ganglioside antibodies and reversible conduction failure (RCF). Results: No classification shifts were observed, but more patients were classified as axonal with recent criteria. RCF and anti‐ganglioside antibodies were present in both subtypes, more frequently in the axonal subtype. Discussion: Serial EDX has no effect on GBS subtype proportions. The absence of exclusive correlation with RCF and anti‐ganglioside antibodies may challenge the concept of demyelinating and axonal GBS subtypes based upon electrophysiological criteria. Frequent RCF indicates that nodal/paranodal alterations may represent the main pathophysiology. Muscle Nerve 58 : 23–28, 2018.     

Sural sparing pattern discriminates Guillain–Barré syndrome from its mimics

Introduction: Electrodiagnostic features of demyelination are essential for establishing the diagnosis in demyelinating subtypes of Guillain‐Barré syndrome (GBS), but they may also occur in disorders that mimic GBS clinically. Information about their frequency in GBS mimics is sparse. Methods: Evaluation of electrodiagnostic features from 38 patients with suspected GBS in whom the diagnosis was later refuted (GBS mimics). Their diagnostic accuracy was analyzed by comparison with nerve conduction studies (NCS) from 73 confirmed GBS patients. Results: Disorders that mimicked GBS clinically at the time of hospital admission included other inflammatory, metabolic, toxic, or infectious neuropathies and spinal cord disorders. The sural sparing pattern was the most specific electrodiagnostic feature for demyelinating GBS. Conclusions: Common electrodiagnostic abnormalities in early demyelinating GBS do not usually exclude other rare differential diagnoses. An exception to this is the sural sparing pattern described here, which strongly supports the diagnosis of demyelinating GBS. Muscle Nerve 50 : 780–784, 2014     

Clinical and nerve conduction features in Guillain−Barré syndrome associated with Zika virus infection in Cúcuta,Colombia

Background and purpose

Zika virus (ZIKV) infection has been associated with an increased incidence of Guillain?Barré syndrome (GBS) but the relative frequency of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and axonal GBS subtypes is controversial.

Methods

Twenty GBS patients diagnosed according to the Brighton criteria during the ZIKV outbreak in Cúcuta, Colombia, were evaluated clinically and electrophysiologically. The electrodiagnosis of GBS subtypes was made according to a recently described criteria set that demonstrated a high diagnostic accuracy on the basis of a single test. The electrophysiological features of 34 Italian AIDP patients were used as control.

Results

All patients had symptoms compatible with ZIKV infection before the onset of GBS and ZIKV infection was laboratory confirmed through a plaque reduction neutralization test (PRNT₉₀) in 100% of patients. The median time from onset of ZIKV infection symptoms to GBS was 5 days (interquartile range 1–6 days). Cranial nerve palsy was present in 85% of patients (facial palsy in 75%, bulbar nerve involvement in 60%), autonomic dysfunction in 85%, and 50% of patients required invasive mechanical ventilation. AIDP was diagnosed in 70% of patients. 40% of nerves of AIDP patients showed a prevalent distal demyelinating involvement but this pattern was not different from the Italian AIDP patients without ZIKV infection.

Conclusions

Guillain?Barré syndrome associated with ZIKV infection in Cúcuta is characterized by a high frequency of cranial nerve involvement, autonomic dysfunction and requirement of mechanical ventilation indicating an aggressive and severe course. AIDP is the most frequent electrophysiological subtype. Demyelination is prevalent distally but this pattern is not specific for ZIKV infection.

     

Inflammatory infiltrates in sural nerve biopsies in Guillain-Barré syndrome and chronic inflammatory demyelinating neuropathy

Prompted by observations in experimental autoimmune neuritis we reanalyzed immunohistochemically the inflammatory infiltrates in sural nerve biopsies of 22 cases with Guillain-Barré syndrome (GBS) and 13 cases with chronic inflammatory demyelinating polyneuropathy (CIDP). Endoneurial infiltration of CD3+ T cells was found in 20 cases of GBS (median 5.5 cells/mm²) and in 10 cases of CIDP (5 cells). Epineurial T cells were present in all GBS cases (19.5 cells) and in 11 CIDP cases (21 cells). CD68+ macrophages were abundant in these neuropathies and often occurred in endoneurial perivascular clusters. In GBS subgroups the number of endoneurial T cells was significantly higher in patients with hypoesthesia and abnormal electrophysiological findings in the sural nerve. In CIDP hypoesthesia was associated with significantly higher numbers of macrophages. Our study also indicates that other factors including the time point of biopsy or previous corticosteroid treatment may influence the inflammatory cell profile. Quantifying cell infiltration may aid in establishing the diagnosis of an immunoneuropathy in patients with mild and noncharacteristic pathology. © 1996 John Wiley & Sons, Inc.     

Proximal nerve lesions in early Guillain–Barré syndrome: implications for pathogenesis and disease classification

Guillain–Barré syndrome (GBS) is an acute-onset, immune-mediated disorder of the peripheral nervous system. In early GBS, arbitrarily established up to 10 days of disease onset, patients could exhibit selective manifestations due to involvement of the proximal nerves, including nerve roots, spinal nerves and plexuses. Such manifestations are proximal weakness, inaugural nerve trunk pain, and atypical electrophysiological patterns, which may lead to delayed diagnosis. The aim of this paper was to analyze the nosology of early GBS reviewing electrophysiological, autopsy and imaging studies, both in acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor/motor-sensory axonal neuropathy (AMAN/AMSAN). Early electrophysiology showed either well-defined demyelinating or axonal patterns, or a non-diagnostic pattern with abnormal late responses; there may be attenuated M responses upon lumbar root stimulation as the only finding. Pathological changes predominated in proximal nerves, in some studies, most prominent at the sides where the spinal roots unite to form the spinal nerves; on very early GBS endoneurial inflammatory edema was the outstanding feature. In the far majority of cases, spinal magnetic resonance imaging showed contrast enhancement of cauda equina, selectively involving anterior roots in AMAN. Both in AIDP and AMAN/AMSAN, ultrasonography has demonstrated frequent enlargement of ventral rami of C5–C7 nerves with blurred boundaries, whereas sonograms of upper and lower extremity peripheral nerves exhibited variable and less frequent abnormalities. We provide new insights into the pathogenesis and classification of early GBS.     

Guillain–Barré syndrome in Pakistan: similarity of demyelinating and axonal variants

Guillain-Barré syndrome (GBS) comprises multiple subtypes whose nosological and pathophysiologic interrelationships are unclear. In an attempt to better understand the relationship between the disease's major subtypes, we reviewed the characteristics of GBS cases consecutively admitted to a tertiary care hospital in Karachi, Pakistan, over a 13-year period. Of 175 cases, 80 (46%) were demyelinating and 55 (31%) axonal, whilst 40 (23%) had ambiguous electrophysiological findings precluding classification. The three groups differed in severity of weakness at presentation (axonal approximately ambiguous > demyelinating; P = 0.002 for arm strength and P = 0.025 for leg strength); mean age (demyelinating > axonal > ambiguous; P = 0.05); and mean cerebrospinal fluid protein concentration (demyelinating > ambiguous > axonal; P = 0.05). However, they were similar in several other respects, including gender ratio, proportion of pediatric cases, history of antecedent infection, length of hospital stay, need and duration of mechanical ventilation, and functional outcome at discharge. Stool culture data was available for 146 (83%) cases in the study; none was positive for Campylobacter jejuni. GBS in Pakistan comprises a high proportion of axonal cases. Similarity of outcomes in axonal and demyelinating variants and lack of C. jejuni stool culture positivity are atypical features.