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。     

周围神经病

周围神经病是由多种病因引起的常见的周围神经系统病变之一，可影响患者的运动、感觉和植物神经功能。正确认识周围神经病的病因、发病机制及临床特征有助于周围神经病的诊断和治疗。本文就周围神经解剖，周围神经病的发病机制，病理改变和临床表现、分类、诊断和治疗方法作一介绍。     

慢性特发性轴索性周围神经病

慢性特发性轴索性周围神经病(chron ic id iopath ic axonalpolyneuropathy,C IAP)是于50岁以后发病的一种原因不明的慢性轴索性周围神经病[1],多为隐袭起病,出现缓慢进展的感觉或感觉运动性周围神经病变症状,神经电生理特点为周围神经轴索性损害。病理检查证实周围神经以轴索损害为主,伴随或不伴随髓鞘损害。自从1993年由Noterm ans等[1]提出该诊断后,在发病机制以及诊断和鉴别诊断等方面存在诸多争议。一些遗传性、慢性炎症性和中毒性轴索性周围神经病常归于C IAP的范畴[2,3]。我们对C IAP在病因、临床、电生理、诊断和治疗等方面的…     

癌性周围神经病的研究进展

癌性周围神经病系癌症对周围神经系统的远隔损害而非其继发性作用引起的,如感染、凝血异常、营养代谢障碍、放化疗的副作用所致,更非肿瘤转移所致;此病发病率低;免疫系统针对癌神经元共同抗原作出的免疫应答导致神经系统损害是本病的主要发病机制,血管炎机制及M-蛋白与该病的发病也密切相关;临床表现多样,有亚急性感觉周围神经病、运动神经元病、感觉运动周围神经病、周围性自主性神经病等。     

急性B淋巴细胞白血病性周围神经病的临床特征分析(附2例报告及文献复习)

目的提高对急性B淋巴细胞白血病(B-ALL)性周围神经病临床特征的认识。方法分析2例B-ALL性周围神经病患者的临床特征、实验室检查、电生理、治疗和预后,并结合相关文献复习进行讨论。结果 (1)2例患者均在化疗后一段时间出现神经系统症状及体征。(2)1例有脑脊液蛋白增高;另1例脑脊液蛋白正常,神经电图示周围神经脱髓鞘改变、轴索损害。均支持多发性周围神经病的诊断。(3)经免疫球蛋白和(或)激素冲击治疗后,患者神经系统症状及体征快速好转。结论 B-ALL患者化疗后一段时间突然出现周围神经系统缺损症状及体征,应完善腰穿、神经电图检查,明确有无周围神经病存在; B-ALL性周围神经病的严重程度与是否伴发肺部感染等有关;拟诊B-ALL性周围神经病,应尽早予以免疫球蛋白冲击治疗,疗效良好;白血病性周围神经病可能与免疫重建有关。     

运动对糖尿病周围神经病影响的研究进展

正糖尿病周围神经病(Diabetic peripheral neuropathy,DPN)是糖尿病的常见并发症之一,它是指在排除其他病因的情况下糖尿病患者出现的与周围神经功能障碍相关的症状或体征,可累及感觉,运动以及自主神经,其中最常见为多发、对称性神经病变和植物神经病变。目前,有关糖尿病周围神经病的发病机制尚未完全明确,治疗措施除营养神经,改善循环外也无特殊治疗。运动疗法作为糖尿病管理的五个要点之一,很多国家已经将相关体育运动列为T2DM重要的非药物治疗方案~([1])。有研究表明运动有助于改善糖尿病周围神经病变,为研究运动对DPN的影响,作者查阅了近年的相关研究,对DPN的发病机制及运动强度、运动方式对与DPN     

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。     

干燥综合征伴周围神经病变临床研究

目的：探讨干燥综合征伴周围神经病的机制、临床表现及治疗措施。方法对1例以周围神经病变为首发症状的干燥综合征患者的诊疗经过进行分析。结果本例患者以右侧眼睑闭合不全发病,临床免疫学检测抗核抗体胞浆型1∶320、抗SSA／Ro60抗体阳性、抗SSB抗体阳性,唇腺活检提示（下唇）间质慢性炎,可见淋巴细胞灶。经糖皮质激素、营养周围神经等药物治疗后症状改善。结论干燥综合征伴周围神经系统损害的患者,早期临床症状常不明显且缺乏典型性,应结合血清学及唇腺病理学等检查,有助于早期诊断,避免漏诊误诊。     

伴有周围神经病变的多发性硬化

目的分析5例伴周围神经病变的多发性硬化的临床电生理、实验室检查和MRI改变,探讨多发性硬化合并周围神经损害的临床特点和发病机制。方法回顾性总结5例伴发周围神经病损的多发性硬化的脑脊液生化,免疫学检查、寡克隆带,电生理检查,MRI及其它有助于鉴别诊断的检查。结果5例均为伴有周围神经病变的多发性硬化,电生理提示广泛周围神经病损,既有脱髓鞘改变,又有轴突变性。结论由于中枢神经系统与周围神经系统髓鞘存在着共同的抗原性成分,故MS患者的中枢神经系统与周围神经系统可以同时发生脱髓鞘改变。     

吉兰-巴雷综合征合并低钠血症的相关研究进展

吉兰-巴雷综合征是一种自身免疫介导的急性炎性周围神经病,常急性起病,症状多于2周左右达到高峰,表现为多发神经根及周围神经损害。现有研究显示部分吉兰-巴雷综合征患者合并低钠血症,但对于其发病机制尚不明确。本文针对吉兰-巴雷综合征合并低钠血症的发病机制、危险因素、诊断及治疗等方面的进展进行综述。     

Infectious neuropathy

PURPOSE OF REVIEW: Infectious neuropathy affects a large number of people worldwide. There is evidence of direct involvement of nerves by the infective agent, from the immune reaction of the patient or secondary to the toxicity of the drugs used during treatment. This group of neuropathies is often treatable or preventable. RECENT FINDINGS: There is a complex clinical picture of the neuropathy of leprosy, different pathological features and immunological mechanisms. If the skin is unaffected in leprosy it is not always easy to demonstrate that the neuropathy is due to leprosy. Peripheral neuropathy in patients with chronic infection with hepatitis C virus may be due to the virus, the development of vasculitis or direct neurotoxic effects of the treatment. Peripheral neuropathy has become the chief neurological syndrome in individuals infected with HIV-1. The antiretroviral therapies themselves can cause peripheral neuropathies clinically indistinguishable from those caused by the virus. The occurrence of chronic polyneuropathy as a late manifestation in Lyme disease is extremely rare and is not well understood. SUMMARY: Although infectious neuropathies are very frequent, mainly in developing countries, further studies are needed to elucidate their mechanisms of action, focusing on preventive interventions.     

Chronic neuropathy associated with immune complexes of hepatitis B virus

In 7 patients, including one autopsied case, with neuropathy associated with hepatitis B virus infection, histologic examination of sural nerve biopsies revealed small vessel vasculitis in the vasa nervorum. In all cases, immunofluorescent deposits of hepatitis B surface antigen, immunoglobulin and C3 complement were detected in the vasa nervorum. That these deposits could represent immune complexes composed of hepatitis B virus was supported by the serologic demonstration of high serum-level of immune complexes and by the ultrastructural demonstration of electron-dense deposits around the endoneural capillary and in the endoneurium. The densities of large myelinated fibers were significantly lower than controls (P less than 0.01) in 6 of 7 cases. These results suggest that immune complexes composed of hepatitis B virus might play a significant role in the pathogenesis of endoneural and epineural vascular lesions, through which neuropathy may be induced in patients with hepatitis B virus infection.     

Peripheral neuropathy in hepatitis C virus-related mixed cryoglobulinaemia: existing treatments and a positive symptomatic response to oxcarbazepine

Peripheral neuropathy is the most common symptom in patients with hepatitis C virus (HCV) associated mixed cryoglobulinaemia, in whom it may be the first clinical manifestation. Very frequently, the medical therapy proposed to treat HCV and cryoglobulinaemia causes an exacerbation of the disabling neuropathy. Therefore, other neuropathy treatments have been proposed, such as alternative immunosuppressive agents (steroids or cyclosporine) and plasma exchange, which, according to case reports, have yielded inconsistent results and presumably exert only temporary effects as they do not promote clearance of HCV. We present five cases of cryoglobulinaemia-related neuropathy resistant to steroids and gabapentin. Oxcarbazepine was introduced and produced moderate and persistent relief of symptoms without side effects.     

The presentation of viral hepatitis C infection without cryoglobulinemia with a peripheral neuropathy. Five case reports

INTRODUCTION: Peripheral neuropathies are the most common neurological complication of viral hepatitis C infection with mixed cryoglobulinemia. CASES REPORT: We report five cases (three men, two women) of peripheral neuropathies revealing viral hepatitis C infection without cryoglobulinemia; the patients' mean age was 56 years. Paresthesias were the most frequent symptom. Electroneuromyographic examination found one case of polyneuropathy and four cases of multiplex mononeuropathies; the complement level was normal in all patients and the rheumatoid factor positive in two cases. Etiological investigations for peripheral neuropathy remained negative. Treatment and outcome were variable. DISCUSSION: Negative cryoglobulinemia in cases of VHC infection with neurological features has been described in the last few years, suggesting the possibility of other mechanisms such as direct action of the virus on the nervous system. There is no consensus on the treatment and outcome is variable. CONCLUSION: Peripheral neuropathy may reveal VHC infection, underscoring the need for VHC serology testing in etiological investigations for peripheral neuropathies.     

替比夫定与聚乙二醇干扰素α-2a联用导致感觉神经病二例

目的 报道2例使用替比夫定和聚乙二醇干扰素α-2a联合治疗乙型肝炎导致的感觉神经病患者,对其临床、电生理和病理改变规律进行讨论.方法 2例男性患者分别为48岁和20岁,均为慢性乙型肝炎病毒感染者,在应用替比夫定和聚乙二醇干扰素α-2a治疗4个月后出现双下肢麻木和疼痛,症状进行性加重.体检发现均出现四肢远端痛觉减退,例2出现腱反射减低.2例均出现四肢远端无汗,例1出现手指和足趾的甲根部灰白样改变.例2伴随出现下肢轻度肌力下降和血清肌酸激酶轻度升高.对2例患者进行电生理检查及腓肠神经病理检查.结果 2例患者的电生理检查发现感觉神经动作电位波幅显著减低,传导速度轻度下降,其中1例出现运动神经动作电位波幅减低.腓肠神经活体组织检查显示有髓神经纤维中度减少、有髓神经纤维轴索变性和轻度再生簇形成.电镜检查进一步发现无髓神经纤维也出现减少.停止药物治疗并给予B族维生素、辅酶Q10和左旋肉碱治疗后症状好转.结论 替比夫定联合聚乙二醇干扰素α-2a治疗可以导致感觉神经病,出现轴索性神经病的电生理和病理改变特点.此病具有可逆性.     

New psychiatric and psychological aspects of diagnosis and treatment of hepatitis C and relevance for opiate dependence

PURPOSE OF REVIEW: This review highlights the aspects of the hepatitis C virus that are important to the psychiatrist. RECENT FINDINGS: Hepatitis C virus infection is frequently associated with mental clouding, depression, neurocognitive impairment, and deterioration in the quality of life. In recent studies psychiatric symptoms have been linked to psychiatric comorbidity rather than to direct hepatitis C virus neurotoxicity. Infection of the central nervous system, however, is thought to play a role at least in hepatitis C virus associated neurocognitive deficits. Application of the anti-hepatitis C virus agent interferon-alpha is regularly accompanied by psychiatric symptoms, most often depression. Antidepressant treatment may support interferon therapy, but its general indication and timing remain debatable. The problem of hepatitis C virus treatment in manic patients is still unsolved. Hepatitis C infection rates in injection drug users are often 90% and higher, while these patients in particular face barriers when trying to access treatment. Recent studies demonstrated feasibility of hepatitis C virus treatment in injection drug users in specialized treatment settings. SUMMARY: Hepatitis C virus infection is associated with psychiatric comorbidity and injection drug use, while treatment of the virus is frequently accompanied by neuropsychiatric symptoms. Psychiatrists are particularly qualified to support diagnosis of hepatitis C associated comorbidity and to render treatment feasible. Evaluation of treatment options and settings in infected patients with psychiatric comorbidity or injection drug users is required, as well as investigation of association of hepatitis C virus infection, and psychiatric and neurocognitive symptoms in properly defined samples.     

Hepatitis C virus: a rare manifestation--remitting relapsing central and peripheral demyelination

The most frequent neurologic manifestations of hepatitis C virus infection include peripheral neuropathy axonal type and central nervous system (CNS) vasculitis. Affected patients usually have cryoglobulinemia and other signs of vasculitis. Demyelinating lesions, both central and peripheral are rarely described. We present a case of simultaneous peripheral nervous system and CNS demyelination that comes in relapsing episodes, with negative cryoglobulins.     

Lewis-Sumner syndrome in hepatitis C virus infection: a possible pathogenetic association with therapeutic problems

A patient with chronic hepatitis from hepatitis C virus (HCV) infection developed Lewis-Sumner syndrome (LSS). The neuropathy worsened after intravenous immunoglobulins, remitted after intravenous methylprednisolone, relapsed during interferon-alpha, but responded again to steroids continued for 68 weeks with clinical remission and without worsening of hepatitis. We are not aware of other reports of HCV infection and LSS. This association may be coincidental or related to a virus-triggered immune-mediated process. Although the coexistence of a dysimmune neuropathy with hepatitis makes problematic the choice of treatment, we emphasize that the patient's condition during treatment with steroids and the 46 following weeks without therapy has been excellent.     

Neuromuscular disorders associated with hepatitis B virus infection

Approximately 400 million worldwide are chronically infected with the hepatitis B virus (HBV). During the course of illness, approximately 20% of patients develop disease manifestations outside the liver. Neuropathy develops in approximately 5% of patients with chronic HBV infection and rarely during acute HBV infection. The pathogenesis of the various HBV-associated neuropathy syndromes possibly involves deposition of immune complexes in nerves or blood vessel walls. Direct viral infection of nerves has not been demonstrated. Management entailed supportive care with antiviral and immunomodulatory treatment as clinically indicated. Rare cases of muscle disease, mostly inflammatory myopathy, have been associated with HBV infection. Presumably, HBV-associated antigens trigger immune mechanisms directed against components of muscle tissue. There is no evidence of replicative virus infection of muscle fibers. Management entailed immunomodulatory treatment, occasionally with anti-HBV therapy. Physicians should be aware that HBV infection has the potential to trigger presumed immune-mediated neuromuscular syndromes.     

AAEM minimonograph #41: Neuromuscular diseases associated with HIV-1 infection

Neuromuscular diseases occur in as many as 50% of patients infected with human immunodeficiency virus type 1 (HIV-1). All forms of neuromuscular disease have been reported, including axonal neuropathy, demyelinating neuropathy, mononeuropathy multiplex, polyradiculitis, ALS-like syndromes, disorders of neuromuscular transmission, myopathy, and toxic neuropathies due to medication side effects. Neuromuscular disease is often the presenting manifestation of HIV-1 infection. Infection with cytomegalovirus (CMV) is associated with different types of neuropathy including mononeuritis multiplex and polyradiculopathy. There is effective treatment for many of the associated disorders including chronic inflammatory demyelinating neuropathy, CMV-mediated neuropathies, and myopathy. Treatment of CMV-mediated mononeuropathy multiplex may be life saving. The different neuromuscular syndromes associated with different stages of HIV-1 infection may be due, in part, to different levels of immunocompetence.