慢性酒精性多发性神经痛的临床,病理及电生理特征

报告３５例慢性酒精性多发性神经病患者，全部为男性，年龄３３￣７０岁，平均５３．７岁，主要临床症状是四肢疼痛，麻木、袜套一手套式痛温觉减退，震动觉消失和肌肉萎缩，运动神经和感觉神经传导速度均有不同程度的减慢，腓浅神经活检见有髓神经纤维髓鞘变性，轴索变性或消失，Ｓｃｈｗａｎｎ细胞变性，也累及神经束膜，此种改变来源于长期饮酒所引起的维生素缺乏所致。     

慢性酒精性周围神经病的电生理改变

目的探讨慢性酒精性周围神经病的电生理特点和诊断价值.方法检测36例慢性酒精中毒患者与35例正常人肌电图对照,统计分析.结果观察组中确诊为临床或亚临床周围神经病的共30例(83.3 3%).观察组的正中神经、尺神经、胫后神经、腓总神经、腓肠、腓浅神经传导速度(NCV)及波幅明显低于正常对照组,感觉神经异常率高于运动神经,下肢异常率高于上肢.各神经传导速度与酒精摄入总量(TLDE)呈负相关.结论酒精对周围神经的毒性呈剂量依赖性.神经电生理检查能敏感地评价慢性酒精中毒患者的周围神经受损程度.     

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。     

神经传导速度检测诊断酒精性周围神经病的价值

目的探讨神经传导速度(NCV)对慢性酒精中毒性周围神经病(CAPN)的诊断价值。方法采用肌电图检测52例CAPN患者的正中神经、尺神经、腓神经和胫神经的NCV,并与26例健康者进行对照比较。结果CAPN患者的NCV异常率为73.12%,明显高于对照组;下肢NCV异常率(80.77%)高于上肢异常率(76.47%);感觉神经传导速度(SCV)异常率(82.73%)高于运动神经传导速度(MCV)异常率(75.26%)。结论NCV检测可作为酒精中毒性周围神经病的方法之一。     

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.     

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.     

定量感觉检查及神经传导速度测定对多发性神经病的诊断价值

目的探讨定量感觉检查(QST)及其联合神经传导速度(NCV)测定对多发性神经病的诊断价值。方法对60例多发性神经病患者进行QST以及感觉神经传导速度(SCV)、运动神经传导速度(MCV)检测,并比较各项检查的异常率。结果 QST的异常率(83.3%)显著高于SCV和MCV(50.0%,26.7%)(均P<0.05);SCV的异常率显著高于MCV(P<0.05)。QST联合SCV的异常率为95.0%,显著高于MCV的异常率(P<0.05)。结论 QST对多发性神经病的检出率较高,QST联合SCV对多发性神经病具有很高的诊断价值。     

糖尿病周围神经病97例临床特征与神经电生理分析

目的:探讨糖尿病周围神经病(DPN)患者的临床特征与神经电生理变化。方法:分析97例DPN患者的临床特征,比较DPN组和对照组的神经传导速度(NCV)、远端潜伏期、远端波幅3个参数。结果:①临床特征以肢体麻木(59%)最多见、其次为疼痛(42%)。②患者组NCV、远端波幅值低于对照组,远端潜伏期比对照组延长;两组3个参数比较,除腓总神经远端波幅、尺神经感觉传导速度和正中、尺、腓肠神经远端潜伏期外,差异均有统计学意义(P<0.05)。结论:①DPN患者临床特征以肢体麻木和疼痛最多见;②检测NCV、远端潜伏期、远端波幅,能较早发现临床患者。     

急性运动轴索型多发性神经病神经电生理异常分布的研究

吉兰-巴雷综合征(Guillain-Barre syndrome．GBS)是临床上急性迟缓性瘫痪的常见病因。根据其电生理和神经病理表现．可分为急性炎症性脱髓鞘性多发性神经病(acute inflammatory demyelinating polyradiculoneuropathy．AIDP)和轴索型多发性神经病。轴索型GBS进一步分为急性运动轴索型多发性神经病(acute motor axonal neuropathy．AMAN)和     

慢性酒精中毒患者周围神经损害的临床与神经电生理研究

目的：探讨总结慢性酒精中毒患者周围神经损害的临床与神经电生理改变特点。方法对55例慢性酒精中毒患者的神经电生理（肌电图、运动传导速度、感觉传导速度）检测结果进行分析,并同期选择55例健康受试者作为对照,对比观察2组检测指标的差异。结果55例酒精中毒患者主要表现为对称性肢带肌萎缩、肌力减退和肢体麻木等,与健康受试者相比,慢性酒精中毒患者肌电图在静息状态、轻收缩相、重收缩相各相异常发生率较高,2组肌电图异常发生率比较差异均有统计学意义（P＜0.05）;神经传导速度比较,慢性酒精中毒患者组无论是MCV还是SCV传导速度减慢率均高于对照组,差异有统计学意义（ P＜0.05）。结论慢性酒精重度患者常出现周围神经损害,以神经末端损害为主,表现为对称性肢带肌萎缩、肌力减退和感觉障碍等,神经电生理改变以肌电图异常、感觉神经、运动神经的传导速度降低最为典型,可作为早期诊断的重要手段。     

Ultrastructure of the sural nerve in chronic polyneuropathy associated with Adie's syndrome

Summary The sural nerve of a woman of 35 with chronic polyneuropathy and Adie's syndrome was examined by electron microscopy. Myelinated nerve fibres were absent and there was marked reduction in the number of unmyelinated fibres. Onion bulb formation was not observed. Collagen fibres occupied the intercellular spaces.     

Peripheral nerve electrophysiology studies in relation to fatigue in patients with chronic inflammatory demyelinating polyneuropathy

ObjectiveTo explore the relationship between fatigue, standard electrophysiological parameters and number and size of functioning motor units in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).MethodsExperienced fatigue was assessed using the linearly-weighted, modified Rasch-built fatigue severity scale (R-FSS) and the multidimensional Checklist of Individual Strength (CIS). Averaged electrophysiology values were calculated from multiple nerves. Motor Unit Number Index (MUNIX) technique was utilised to assess motor unit function. Assessments were repeated in 15 patients receiving regular intravenous immunoglobulin therapy, with changes in parameters calculated.ResultsR-FSS and CIS scores did not correlate MUNIX or MUSIX sum scores from 3 different muscles. Inverse correlation was observed only between distal CMAP area and R-FSS but not CIS scores. However, changes in distal CMAP area and R-FSS scores on repeat assessment were not correlated.ConclusionsExperienced fatigue does not appear to correlate with loss of functioning motor units in patients with CIDP. Changes in experienced fatigue on repeat assessment did not correlate with changes in any of the electrophysiological parameters, suggesting fatigue experienced in CIDP is not strongly correlated with peripheral nerve dysfunction.SignificanceNerve conduction studies and MUNIX values do not appear to be useful surrogate markers for fatigue in CIDP.     

慢性酒精中毒性肌病的临床与神经电生理学研究

目的　探讨慢性酒精中毒性肌病的临床和电生理改变特点。方法　对 2 6例慢性酒精中毒性肌病、13例慢性酒精中毒性周围神经病、2 1例慢性酒精中毒性神经和肌肉混合损害患者 ,以及 2 0例正常受试者进行详细询问病史、查体 ,并记录酒精摄入量和测定相关神经电生理指标 ,包括肌电图、单纤维肌电图、肌纤维传导速度、周围神经传导速度和诱发电位。结果　 (1)酒精中毒性肌病的主要临床表现为肢带肌的无力、肌肉疼痛和萎缩 ,肌病的症状和体征往往先于周围神经的损害。 (2 )与正常对照受试者比较 ,各组患者神经肌肉颤抖值均增大 (t检验 ,P<0 .0 5 ) ,纤维密度增加 (t检验 ,P<0 .0 5 ) ,周围神经病组患者的神经肌肉颤抖值和纤维密度改变尤为显著 (t检验 ,P<0 .0 1)。 (3)肌病组患者肌纤维传导速度明显减慢 (t检验 ,P<0 .0 5 ) ,其余两组患者无显著变化 (t检验 ,P>0 .0 5 )。结论　 (1)酒精中毒性肌病临床主要表现为对称性肢带肌萎缩、肌力减退和肌肉疼痛。 (2 )肌电图提示肌源性改变 ,特别是肌纤维传导速度减慢 ,是诊断酒精中毒性肌肉病变的客观指征。     

Demyelinating polyneuropathy associated with monoclonal IgM-paraproteinaemia. Histological, ultrastructural and immunocytochemical studies

Histological, ultrastructural and immunocytochemical findings of sural nerve biopsies from 2 patients with monoclonal IgM-paraproteinaemia are presented. In both cases the pathological IgM antibodies reacted with a myelin antigen which was identified as myelin-associated glycoprotein (MAG) by immunoelectroblot . Histology and electron microscopy showed typical features of a chronic demyelinating neuropathy with accompanying axonal degeneration. Immunohistochemical studies demonstrated IgM in the vicinity of endoneurial vessels and on some of the myelinated fibres. The localisation of IgM on the myelin sheath showed a typical pattern, which was similar to that found in binding studies with the patients sera on control nerves. It resembled the characteristic immunocytochemical staining pattern of MAG. Binding studies with the patients' sera on human and canine CNS material exhibited a clear labelling of white matter and certain, as yet unidentified structures within the cerebral and cerebellar cortex. In mixed glial cell cultures, the sera of both patients bound specifically to oligodendrocytes. Our observations are interpreted as immunohistochemical evidence that the anti-MAG antibodies of the patients' sera had bound to their antigenic target in the peripheral nerves. Because the antibodies clearly react to central myelin, oligodendrocytes and other not yet identified cortical structures, CNS involvement in such disorders should be considered.     

Carbon disulfide induced polyneuropathy: sural nerve pathology, electrophysiology, and clinical correlation

We report the clinical features, electrophysiological studies, sural nerve pathology and recovery course of carbon disulfide-(CS2) induced polyneuropathy in a 48-year-old man who worked in a viscose rayon plant. Sural nerve biopsy 2 years later still showed degeneration of both axon and myelin with a predominant loss of large myelinated fibers and remyelination. Electrophysiologic studies revealed mixed axonal and demyelinating polyneuropathy. To our knowledge, this is the first human report of sural nerve pathology in the recovery stage due to CS2 intoxication. After diagnosis, the patient was removed from the toxic environment. In the following three years, he showed part recovery predominantly in motor function compatible with the serial nerve conduction studies. We conclude that CS2 polyneuropathy may partly recover years after cessation of exposure.     

Marchiafava-bignami disease,striatal degeneration,and other neurological complications of chronic alcoholism in a Japanese

Summary A Japanese man with a variety of neurological complications, had drunk Japanese rice wine (sake) daily for about 25 years. There was a progressive development of parkinsonism, cerebellar ataxia, and mental deterioration by the time he was 32. He died of pneumonia at age 50 and the autopsy revealed Marchiafava-Bignami disease (MBD), striatal degeneration, pseudolaminar sclerosis of Morel, atrophy of the corpus mamillare and pons, cortical cerebellar atrophy, pseudopellagra, and polyneuropathy. This is the first case of MBD in a Japanese related to the ingestion of Japanese sake, and it is also a rare case in that almost all of the neurological complications seen with chronic alcoholism were apparent. Striatal degeneration seems to be a rare complication of chronic alcoholism.     

腓骨肌萎缩症电生理、病理和基因定位研究

目的研究腓骨肌萎缩症(CMT)临床特征、基因测定、病理及神经电生理检查在其诊断和分型中的价值。方法收集50例CMT患者临床资料,对其进行肌电图检查及腓肠神经活检,并采用PCR技术直接测序进行基因突变分析。结果 40例CMT患者双下肢运动及感觉传导速度减慢(双胫、腓总神经为15~28 m/s,腓肠神经为12~30 m/s),10例双下肢未引出反应电位;50例正中神经运动及感觉传导速度亦减慢分别为19~48 m/s和20~52 m/s。CMT患者神经传导速度减慢的程度和临床表现的严重程度并不平行。腓肠神经活检符合慢性脱髓鞘部分伴轴索改变性周围神经病。PMP22、Cx32、MPZ、MFN2、GDAP1致病基因的突变分析发现14例患者存在PMP22基因的大片段重复突变(28%),13例患者存在Cx32基因的点突变(26%),4例患者存在MPZ基因的点突变(8%),3例患者存在MFN2基因的点突变(6%),未发现GDAP1基因的突变,16例患者未检测出上述基因突变。结论电生理、病理、基因测定在CMT的诊断及分型中有重要价值。     

Central and peripheral motor conduction time in chronic alcoholics with polyneuropathy and/or spasticity.

We measured central and peripheral motor conduction time to demonstrate lesions in the upper and lower motor neurons of 11 chronic alcoholics with polyneuropathy without spasticity, 7 chronic alcoholics with spasticity with and without alcoholic polyneuropathy, and 16 healthy volunteers as controls. Peripheral motor conduction time was significantly prolonged in all extremities in all of the chronic alcoholics, and was accompanied by a considerable reduction in motor conduction velocity. Central motor conduction time was considerably prolonged in the lower extremities of the alcoholics with spasticity compared with both the controls and the alcoholics without spasticity. Central motor conduction time in the patients with alcoholic polyneuropathy without spasticity was slightly prolonged in comparison with the controls, but not significantly. Based on the electrophysiological findings, we conclude that peripheral neuropathy is a lesion common to chronic alcoholics whether or not they have clinically evident polyneuropathy. Chronic alcoholics with spasticity have significantly longer central motor conduction time in the lower extremities. Spasticity in chronic alcoholics develops not independently but concomitantly with peripheral neuropathy, suggesting that peripheral neuropathy develops earlier than spasticity.     

Ulnar neuropathy with abnormal non-localizing electrophysiology: Clinical,electrophysiological and ultrasound findings

Objective

To systematically study demographic, clinical, electrophysiological and nerve ultrasound characteristics of ulnar neuropathy with abnormal non-localizing electrophysiology (NL-UN) and further define the utility of ultrasound over and above the conventional electro-diagnostic approach.

Acute‐onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement,dysautonomia, respiratory failure,and autoantibodies

We examined a 27‐year‐old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain–Barré syndrome (GBS) or acute‐onset chronic inflammatory demyelinating polyradiculoneuropathy (A‐CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium‐sensing receptor (CaSR) autoantibodies. The patient was initially diagnosed with GBS and treated with intravenous immunoglobulin (IVIg). After almost complete recovery, relapse with quadriplegia and respiratory failure was observed 12 weeks after motor symptom onset. She then received IVIg and steroid pulse therapy followed by maintenance oral methylprednisolone and plasma exchange. She recovered completely 4 months after the relapse. The further clinical and serological course was consistent with systemic lupus erythematosus (SLE)‐associated CIDP. Herein we evaluate the association between A‐CIDP and some biological markers of autoimmunity. Muscle Nerve, 2010