干燥综合征伴周围神经病的临床研究

目的：探讨干燥综合征伴周围神经病的临床特征及治疗方法。方法回顾性分析我院2013‐04—2014‐07收治的4例干燥综合征伴周围神经病患者的临床资料。结果4例干燥综合征伴发的周围神经病各不相同,1例为周围神经病变,2例为感觉性周围神经病,1例为慢性吉兰‐巴雷综合征。4例患者均以周围神经病变为首发症状,预后不良。结论干燥综合征可伴严重周围神经病,早期可以周围神经病症状为首发症状,而口干、眼干等干燥综合征症状可不明显,故对周围神经病患者进行风湿免疫筛查,早期明确诊断原发病,对患者预后的改善有重要临床意义。     

副肿瘤性周围神经病15例临床分析

副肿瘤性周围神经病是指由癌肿引起的非转移性周围神经病变,在病变部位并无癌细胞可见[1]。约有50%患者神经系统症状早于肿瘤的发现[2],因而未发现癌肿之前患者常就诊于神经科。我科自2000年1月~2006年6月收治及会诊的该病患者15例,现分析如下。1临床资料1.1一般资料男9例,女6例;年龄50~74岁,平均62·5岁;发病至确诊时间4~16个月,平均10·5个月。6例曾误诊为Guillain-Barre综合征(GBS),9例为一般周围神经病。1.2临床表现1.2.1周围神经病表现均为隐匿或亚急性起病,均为首发周围神经病变症状,病情进行性发展。14例有四肢远端不适、刺痛、灼…     

以神经系统症状为首发的糖尿病40例分析

目的探讨糖尿病以神经系统症状为首发症状的临床类型。方法通过我院近10年来治疗的40例以神经系统症状为首发症状的糖尿病临床资料进行分析并结合文献对其发病机制、治疗进行讨论。结果40例中以周围神经病变为首发19例，以植物神经病变为首发3例，以脊髓病变为首发4例，以脑部病变为首发14例。结论凡是中老年人隐匿或急性起病的神经系统症状均应及时捡查血糖．以排除糖尿病。     

变应性肉芽肿性血管炎14例临床表现及周围神经损害

目的分析变应性肉芽肿性血管炎(Churg-Strauss sydrome,CSS)的临床特点以及周围神经受损的表现,以提高诊治水平。方法收集自2005年1月~2016年12月在北京大学第三医院收治的14例CSS患者的临床资料,回顾性分析其临床表现及周围神经受损的情况。结果 14例CSS患者中男性10例(71.4%),年龄20~78岁(53.6±16.2);外周血嗜酸性粒细胞平均升高20.7%(10.3%~55%);呼吸系统是最常受累的器官(100%),哮喘9例(64.3%),肺部浸润12例(85.7%);6例(42.9%)存在周围神经受损,以四肢周围神经尤其是下肢神经轴索损害为主,同时累及运动与感觉神经;CSS起病初期误诊率高达64.3%,起病至确诊间隔平均15.9 m(1~72 m)。结论 CSS是一种误诊率较高的全身多系统受累的血管炎,存在嗜酸性粒细胞升高,且临床和电生理结果提示非对称性以轴索损害为主的周围神经病患者,需注意CSS的可能。     

周围神经显微减压术治疗糖尿病性上肢周围神经病

目的 探讨周嗣神经显微减压术治疗糖尿病性上肢周围神经病的疗效.方法 应用腕管正中神经显微减压术及肘管尺神经显微减压、肌下转位术治疗15例糖尿病性上肢周围神经病患者(25侧手28根神经).结果 平均随访37个月.15例糖尿病性上肢周围神经病患者25侧手部麻木、疼痛症状术后100%缓解,手部力弱、运动功能不良症状术后缓解率60 %(15/25),随访期间症状复发1侧手正中神经(3%,1/28).并发症:手术切口愈合不良3处(11%,3/28).结论 周围神经显微减压术是治疗糖尿病性上肢周围神经病的有效方法,其改善手部感觉障碍的疗效好于改善运动功能不良的疗效.     

副肿瘤性周围神经病的诊断分析

副肿瘤性周围神经病(PPN)属于神经系统副肿瘤综合征(PNS)类型之一,是由于免疫反应引起的肿瘤远隔效应,其临床发病率较低,容易误诊或漏诊.诊断副肿瘤性周围神经病须首先排除肿瘤直接浸润、脑脊膜转移、放射治疗或药物化疗不良反应等因素引起的周围神经损害[1].笔者报告2006年3月-2009年8月首都医科大学宣武医院神经内科诊断与治疗的30例副肿瘤性周围神经病患者,并结合文献进行分析.     

反应性嗜酸性粒细胞增多症诱发多发性周围神经病的临床特点(附1例报告)

目的探讨反应性嗜酸性粒细胞增多症(HE)引起周围神经病变的临床特点。方法回顾性分析1例HE继发周围神经病患者的临床资料。结果本例老年男性以多发性周围神经炎为主要表现,粪便病原学蛔虫感染。经过系统的药物驱虫、激素治疗后,患者肢体无力及肌痛的症状明显好转,外周血中嗜酸性粒细胞的数量明显下降,并且与肌酸激酶下降趋势及临床症状的改善一致。结论 HE以多发性周围神经病为主要临床特点。     

阿米三嗪-萝巴新对糖尿病性周围神经病的疗效

糖尿病性周围神经病是糖尿病最常见的并发症之一,临床较为常见。其发病率与病程和糖尿病分型有关,病程越长其发病率越高,2型糖尿病较1型糖尿病的发病率为高。可呈对称性复发性神经病、单神经病或复发性单神经病．可累及感觉、运动和自主神经,多以感觉症状为主。由于其发病机制尚未完全阐明,故至今仍无确切有效的治疗药物。在临床实践中我们发现在传统治疗的基础上加用阿米三嗪-萝巴新(都可喜)能够明显改善糖尿病性周围神经病患者的临床症状。为观察其治疗糖尿病性周围神经病的临床疗效并探讨其作用机制,笔者于1998年1月-2004年3月对42例伴有周围神经病的2型糖尿病患者进行了为期4周的随机对照的临床治疗观察。     

神经活检在周围神经疾病诊断中的意义及与电生理检查相关性分析

目的探讨神经活检在周围神经病诊断中的意义,并分析病理检查与神经电生理检查结果的一致性。方法收集2009-2011年作者医院行腓肠神经活检的16例周围神经病患者的临床诊断、电生理诊断和病理诊断资料;分析电生理诊断、光镜诊断和电镜诊断在判断轴索损害或/合并髓鞘损害的一致性,并分析其结果不一致的可能原因。结果电生理检查结果示异常12例(12/16),其中表现为轴索损害为主5例(5/16),髓鞘损害为主7例(7/16);病理检查结果示15例(15/16)患者有不同程度的髓鞘或轴索损害;4例(4/16)患者经神经活检后原有的诊断得到了补充或修改;进一步分析神经病理对于周围神经损害的诊断与电生理诊断无统计学差异。结论 (1)神经活检能够发现一些间质改变和亚临床、亚电生理的神经损害,从而对疾病的认识和治疗提供帮助。(2)神经病理对于周围神经损害的诊断与电生理诊断相关性较好,但当电生理表现为轴索损害时其一致的趋势欠佳,可能与轴索损害的多样性有关。     

正中神经显微减压术治疗糖尿病性上肢周围神经病

目的探讨正中神经显微减压术治疗糖尿病性上肢周围神经病的疗效。方法应用正中神经显微减压术治疗12例糖尿病性上肢周围神经病患者（19侧手）。结果平均随访35个月。12例糖尿病性上肢周围神经病患者19侧手部麻木、疼痛症状术后100%缓解,手部力弱、运动功能不良症状术后缓解率58.8%,随访期间症状复发1侧手（6%）。并发症：手术切口愈合不良2侧（12%）。结论周围神经显微减压术是治疗糖尿病性上肢周围神经病的有效方法,其改善手部感觉障碍的疗效好于改善运动功能不良的疗效。     

Content validity of symptom‐based measures for diabetic,chemotherapy, and HIV peripheral neuropathy

Introduction: No treatments for axonal peripheral neuropathy are approved by the United States Food and Drug Administration (FDA). Although patient‐ and clinician‐reported outcomes are central to evaluating neuropathy symptoms, they can be difficult to assess accurately. The inability to identify efficacious treatments for peripheral neuropathies could be due to invalid or inadequate outcome measures. Methods: This systematic review examined the content validity of symptom‐based measures of diabetic peripheral neuropathy, HIV neuropathy, and chemotherapy‐induced peripheral neuropathy. Results: Use of all FDA‐recommended methods to establish content validity was only reported for 2 of 18 measures. Multiple sensory and motor symptoms were included in measures for all 3 conditions; these included numbness, tingling, pain, allodynia, difficulty walking, and cramping. Autonomic symptoms were less frequently included. Conclusions: Given significant overlap in symptoms between neuropathy etiologies, a measure with content validity for multiple neuropathies with supplemental disease‐specific modules could be of great value in the development of disease‐modifying treatments for peripheral neuropathies. Muscle Nerve 55 : 366–372, 2017     

Neuropathies and almitrine. 14 cases

Previously reported cases of peripheral neuropathies occurring during almitrine therapy had only a few weeks follow-up after having stopped the drug. We have studied clinical and electrophysiological data 6 to 12 months after almitrine had been given up in 9 patients from a group of 14 whose epidemiologic, clinical, electrophysiological and pathological data had been registered. In 7 of them, without any chronic respiratory deficiency, almitrine was administered as almitrine bismésilate and raubasine, and in 7 others (6 with chronic respiratory deficiency) as almitrine bismesilate alone. In patients who had another possible cause of neuropathy, clinical disorders appeared after a lesser total quantity of almitrine (p less than 0.05). Clinical data were suggestive of sensory peripheral neuropathies of feet and lower third of legs. Electrophysiological data suggested distal axonopathy in spite of the absence of denervation: amplitudes of sensory potentials were reduced and nerve conduction velocities were moderately decreased. Biopsies revealed mild neurogenic atrophy of muscles and distal axonopathy. Clinical improvement was very slow and 6 to 12 months later, most of the patients still presented decreased vibration sense and ankle reflexes loss, but all of them were still improving. Amplitudes of sensory potentials and sensory nerve conduction velocities were significantly improved (p less than 0.05) but motor nerve conduction velocities were not (p greater than 0.05). Our study shows: 1) clinical, electrophysiological and pathological data similar to those previously reported; 2) subclinical disturbances of motor functions in lower limbs and sensory functions in upper limbs; 3) some patients presented with unusual signs: posture tremor (3 cases), painful legs and moving toes (1 case); 4) peripheral neuropathies may occur during almitrine therapy even in patients without any chronic respiratory insufficiency; 5) peripheral neuropathies occurred with lower doses in patients with other factors predisposing to neuropathies; 6) patients' improvement was very slow; 7) in 9 cases the imputability of these peripheral neuropathies to almitrine is plausible. We suggest not to prescribe almitrine without caution, especially in patients with other factors of neuropathy. Treatment should be regularly interrupted.     

Phe 84 deletion of the PMP22 gene associated with hereditary motor and sensory neuropathy HMSN III with multiple cranial neuropathy: clinical, neurophysiological and magnetic resonance imaging findings

Hereditary motor and sensory neuropathy (HMSN) is a heterogeneous group of peripheral neuropathies which are diagnosed on the basis of clinical, electrophysiological and neuropathological findings. Among the hypertrophic demyelinating neuropathies, HMSN III is the most severe. It is often associated with de novo mutations in the genes encoding for peripheral myelin proteins. While peripheral nerve hypertrophy is an expected finding in HMSN III, cranial nerve hypertrophy is exceptional. Here we describe a mutation in the PMP22 gene in a 19-year-old man with infantile onset of sensory motor polyneuropathy without family history and multiple cranial nerve hypertrophy shown by cranial magnetic resonance imaging. Received: 3 May 2000, Received in revised form: 29 August 2000, Accepted: 7 September 2000     

Peripheral neuropathy in Tangier disease: A literature review and assessment

Tangier disease (TD) (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high‐density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with TD with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue, and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in TD. Our search identified 54 patients with TD with peripheral neuropathy. Syringomyelia‐like neuropathy subtype (52.4%) was more frequent than multifocal sensorial and motor neuropathy subtype (26.2%), focal neuropathy subtype (19.1%), and distal symmetric polyneuropathy subtype (2.4%). Splenomegaly was the most common (40.7%) clinical manifestation in these patients. The pattern of electrodiagnostic abnormalities are: (1) demyelinating abnormalities were more predominant in the upper extremities than in the lower extremities and (2) slowing of motor nerve conduction was more prominent in the intermediate segment than in distal nerve segments. The sural‐sparing pattern was present in 34.6% and conduction block was present in 11.5% of the patients. Our literature review and our case showed the clinical spectrum of TD neuropathy is quite wide and that it should be considered in the differential diagnosis of non‐uniform demyelinating neuropathies.     

多发性硬化周围神经损害的临床和肌电图研究

目的研究多发性硬化(MS)周围神经损害的临床及肌电图(EMG)特点?方法回顾性分析29例MS患者的临床及EMG检查资料。结果本组18例(62．1％)有周围神经损害的临床表现和/或EMG异常,其中肢体麻木16例(88．9％)、肢体乏力11例(61．1％)、神经根性疼痛5例(27．8％)、自主冲经损害症状3例(16．7％)、饮水呛咳和吞咽困难2例(11．1％);腱反射减低11例(61．1％)、末梢或根型感觉障碍9例(50％)、肌力减低7例(38．9％)、肌萎缩4例(22．2％)、咽反射减低1例(5．6％)．其发病年龄、病程、冲经功能缺损程度及预后与11例不伴周围神经损害的MS患者基本相似、EMG检查发现自发电位4例(13．8％),运动单位电位波幅增高、时限延长8例(27．6％)、运动神经传导速度减慢15例(51．7％)、感觉神经传导速度减慢13例(44．8％)、波幅减低9例(31．1％)、远端潜伏期延长5例(17．2％)、经皮质类固醇等治疗周围神经损害症状除1例双下肢麻木外,其余均随病情好转而恢复。结论部分MS患者伴周围神经损害表现,可随病情好转而恢复;EMG可帮助判断周围神经损害的部位和程度。     

New trends in neuropathy practice: clinical approach to CIDP]

Our recent study showed that the overall prevalence of CIDP was estimated as 2.2 per 100,000 population in Aomori Prefecture, in Northan Honshu of Japan. In our series of more than 80 cases with CIDP, a chronic acquired inflammatory demyelinating polyneuropathy, nearly 30% showed clear laterality of weakness, and electrophysiologic laterality or multifocality was apparent in almost all cases. Nearly 90% of patients were able to walk without walking aids or other assistance. Sixty% showed distal dominant muscular weakness. In 12 patients with age of onset under 15, pes cavus deformity was seen in 5. Two thirds complained numbness in the extremities during progressive phase. Four cases initially showed severe sensory ataxia associated with motor conduction block. It should be, thus, reminded that clinical spectrum of CIDP is enormously wide: chronic acquired demyelinating multiple mononeuropathy showing asymmetric involvement (Lewis-Summer syndrome) should be put on one side of the clinical presentation of CIDP. Multifocal motor neuropathy (MMN) is, on the other hand, an unique syndrome mimicking amyotrophic lateral sclerosis (ALS). There may be, however, true association syndrome of CIDP and ALS presenting both peripheral nerve demyelination and pyramidal sign with progressive bulbar involvement. Recently, several atypical varieties of CIDP showing only one-limb involvement, upper limb weakness rather than lower limb power loss, or proximal weakness, etc ... have been reported in the literature. To realize such clinical variations of chronic acquired demyelinating neuropathy is important for early diagnosis and commencement of treatment of CIDP. Clinical guideline for suspicion of CIDP could be useful for general physicians and neurologists unfamiliar to peripheral neuropathies.     

Clinical and electrophysiological characteristics of autosomal recessive axonal Charcot-Marie-Tooth disease (ARCMT2B) that maps to chromosome 19q13.3

Charcot-Marie-Tooth disease (CMT) comprises a heterogeneous group of hereditary motor and sensory peripheral neuropathies. The autosomal recessive axonal form of CMT (ARCMT2) is rare. Eight patients of a large consanguineous family of Spanish ancestry in Costa Rica were diagnosed with ARCMT2B; previous genetic studies of this family revealed linkage to chromosome 19q13.3. The clinical and electrophysiological features of these patients are reported. All patients presented with a symmetric motor and sensory neuropathy, which was more pronounced in the lower limbs. Further, distal muscle wasting and impaired deep tendon reflexes were found. Age at onset was between 26 and 42 years, and the disease duration ranged from 2 to 19 years. Electrophysiological studies revealed a primary axonal degenerative process. The clinical characteristics of this family differed in several aspects from previously reported families with ARCMT2.     

Chronic dysimmune neuropathy

The Chronic Dysimmune neuropathies (CDN) are a clinically heterogeneous group of polyneuropathies united by their presumed immune mediated aetiology. At present such neuropathies are classified as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Multifocal Motor Neuropathy (MMN) and the Neuropathies in association with serum Paraproteins (Paraproteinaemic Neuropathies). This classification fails to recognise other distinctive syndromes and is limited by heterogeneity within, and overlap between, subgroups. We have refined this clinical subclassification by a review of a consecutive series of 102 unselected patients with CDN referred to a single neurologist. We recognise 6 clinical subtypes of CDN: one sensory ataxic group; three motor-sensory subgroups (chronic motor sensory demyelinating neuropathy, subacute motor sensory demyelinating neuropathy and a multifocal motor sensory neuropathy); and two pure motor subgroups (symmetric pure motor demyelinating neuropathy and multifocal motor neuropathy). This subclassification allows distinct syndromes to be recognised and helps resolve problems of heterogeneity and overlap. Distinction between these subgroups is of immediate practical relevance to patient management. Although steroids are beneficial for most of the subgroups, this is not so for both of the pure motor syndromes which should be treated with intravenous immunoglobulin. Patients with chronic development of Motor Sensory Demyelinating Neuropathy respond less well to steroids than those with a subacute onset. An association was found between elderly patients with Subacute Motor Sensory Demyelinating Neuropathy and carcinomas. Within any clinical subgroup patients behave similarly regardless of the presence of associated paraproteins or nerve specific antibodies. Received: 19 March 2002, Received in revised form: 2 January 2003, Accepted: 13 January 2003 Correspondence to: Dr. M. Donaghy     

PERIPHERAL NEUROPATHIES AND CHRONIC HEPATITIS C

Objectives: Recent interest has been expressed in peripheral neuropathies in hepatitis C virus (HCV) patients. The aim of this prospective study was to evaluate the prevalence of peripheral neuropathies associated with chronic hepatitis and their clinical manifestations. Patients and method: Ninety anti‐HCV‐positive patients were consecutively interviewed and examined by the same operator. Forty‐five patients with end‐stage liver disease and awaiting liver transplantation were evaluated at the Liver Transplantation Center (Group 1). Further 45 patients were referred for neurological consultation during hospitalisation in the Department of Medicine (Group 2), where they had been admitted for different clinical reasons. One patient from group 1 and 5 patients from group 2 were excluded from the study because of previous neurological diseases. All patients underwent a standardized neurological evaluation, including history, neurological examination, mini mental state examination, neuropathy symptom score, and neurological disability score. In presence of symptoms and signs of peripheral neuropathy, an electrophysiological evaluation was performed. Results: Signs or history of encephalopathy were found in 23/44 patients of group 1 (52.2%) and in 8/40 patients of group 2 (20%). Clinical manifestations of neuropathy, confirmed by electrophysiological examination, were found in 11 subjects of group 1 (25%) and in 17 patients of group 2 (42.5%). Most patients had minor symptoms; sensory disturbances occurred more frequently than motor and autonomic dysfunctions. In group 1, peripheral neuropathy was associated with systemic illness (diabetes, renal failure, liver neoplasm, previous alcohol abuse) in 4/11 patients (36.3%); conversely, in all patients from group 2, other possible etiological factors were present: alcohol abuse in 3; diabetes in 4; renal failure in 1; mixed cryoglobulinemia in 7; and neoplasm in 2. Conclusions: Symptomatic but not disabling neuropathies were found in 33% of HCV in patients. Sensory involvement was prevalent. Systemic illness and mixed cryoglobulinemia were more frequently present in Group 2 than in Group 1. This finding suggests that the metabolic dysfunctions caused by liver disease may be the primary determinant of peripheral system damage only in patients with end‐stage liver disease.     

High prevalence of neuropathies in patients with end‐stage liver disease

Cocito D, Maule S, Paolasso I, Castelli L, Ciaramitaro P, Poglio F, Ottobrelli A, Grimaldi S. High prevalence of neuropathies in patients with end‐stage liver disease.
Acta Neurol Scand: 2010: 122: 36–40.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objectives – Peripheral neuropathy has been reported in association with end‐stage liver disease, but there is only a limited number of reports on the incidence and features of these neuropathies. Materials and methods – In this study, 83 patients awaiting liver transplantation were evaluated for the presence of peripheral and autonomic neuropathy. Results – Sixty‐five percent of the patients had evidence of neuropathy, in agreement with peripheral NCS or cardiovascular autonomic function test. The neuropathy was more frequent in patients with advanced hepatic failure, evaluated with the MELD score. The most frequent abnormalities in nerve conduction studies were sensory‐motor neuropathies and sensory neuropathies, with a length‐dependent pattern. Conclusion – Peripheral neuropathy and autonomic neuropathy are common in patients with end‐stage liver disease with different etiology and correlate with the severity of the liver disease.