结节性多动脉炎合并神经系统损害3例报告

本文报告３例结节性多动脉炎合并神经系统损害均具有体重减轻、肾、消化系统损害及血白细胞与血小板增多。其中２例还有发热及心脏损害，３例均合并有神经系统损害（２例合并有周围神经病，１例合并有多发性肌炎）。１例为组织活检所证实，另２例虽缺组织活检及血管造影资料（家属拒绝），但根据国内外学考提出的诊断标准仍可诊断为结节性多动脉炎。     

结节性多支脉炎合并神经系统损害3例报告

本文报告３例结节性多动脉炎合并神经系统损害均具在体重减轻，肾，消化系统损害及血白细胞与血小板增多，其中２例还有发热及心脏损害，３例均合并有神经系统损害（２例合并有周围神经病，１例合并有多发性肌炎），１例为组织活检所证实，另２例虽缺组织活检及血管造影资料（家属拒绝）但根据国内外学考提出的诊断标准可诊断为结节性多动脉炎。     

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。     

甲状腺功能亢进并发周围神经病(附一例报告并文献复习)

目的　探讨甲状腺功能亢进并发周围神经病的临床表现、发病机制、电生理 (EMG)和病理特点、治疗以及预后。方法　详细收集病史及体格检查资料并进行随访观察 ,动态观察甲状腺功能 ,对比肌电图检查结果 ,并电镜观察腓肠神经活检标本。结果　此例甲状腺功能亢进并发周围神经病表现为运动重于感觉、远端重于近端 ;肌电图提示运动感觉传导速度均减慢 ;神经活检证实同时有脱髓鞘和轴索变性。甲状腺功能亢进控制后肌无力明显改善 ,神经传导速度亦明显恢复。结论　甲状腺功能亢进可并发周围神经病 ,但较罕见 ,其机制可能为自身免疫和代谢异常 ,预后与甲状腺功能亢进控制情况密切相关     

亚急性炎性脱髓鞘性多发性神经根神经病的临床和病理研究(附3例病理报告)

目的 探讨亚急性炎性脱髓鞘性多发性神经根神经病（SIDP）的临床和病理特点。方法 对7例SIDP患者临床资料和腓肠神经活检所见进行分析。结果 患者呈亚急性起病，达到高峰时间均在4周－2个月；运动障碍较感觉障碍明显，上升性呼吸肌麻痹很少发生，对激素治疗有效且无复发。3例腓肠神经活检，病理显示：以脱髓鞘为主要表现，伴有单核细胞浸润，胶原纤维增生，但无洋葱样肥大神经改变。结论 SIDP不论临床表现还是病理特点都不同于急、慢性炎性脱髓鞘性多发性神经根神经病（AIDP、CIDP），而是介于两者之间。     

儿童慢性炎症性脱鞘性多神经病的临床和病理改变特点

目的探讨儿童慢性炎症性脱鞘性多神经病(chronic inflammatory demyelinating polyneuropathy,CI-DP)的临床及病理改变特点。方法根据欧洲神经肌肉病中心修订的儿童CIDP诊断标准诊断的10例17岁以下患者,收集其临床资料,进行周围神经电生理以及腓肠神经的病理检查。结果所有患者主要表现为肢体无力,分别有4例和3例出现四肢感觉减退和颅神经损害。9例有脑脊液蛋白细胞分离现象。10例均出现运动或感觉神经传导速度减慢及远端潜伏期延长,9例患者的动作电位波幅降低。所有患者的有髓神经纤维出现轻-重度减少,其中3例患者的纤维脱失程度在不同束间存在差异,6例患者以脱髓鞘为主;3例以轴索损害为主。1例患者仅出现轻微改变。9例患者存在炎细胞浸润。结论儿童CIDP以肢体无力为主。部分患者以轴索损害为主,神经纤维脱失程度可以存在束间差异。     

以周围神经病变为首发表现的原发性干燥综合征的临床病理分析

本文通过分析1例以双下肢运动感觉功能受损、口眼干燥为主要表现的52岁女性原发性干燥综合征患者的临床、实验室检查和病理学资料,表明原发性干燥综合征患者可以以神经系统损害为首发表现.     

线粒体肌病合并周围神经病的临床病理研究

目的:分析线粒体肌病合并周围神经病的临床特点、电生理和病理特征。方法:对10例肌肉活检确诊为线粒体肌病同时又合并周围神经病的患者从临床症状与体征、电生理检查和腓肠神经活检等几方面予以分析,并探讨周围神经病的病理基础。结果:本组10例患者中1例仅有临床下受累,另外9例表现为轻中度感觉运动性周围神经病,以感觉症状为重。结论:对线粒体肌病患者应将详细的神经系统查体与神经电生理检查相结合,提高周围神经病的检出率。电生理检查和腓肠神经活检有助于诊断。周围神经病的发病机制可能与线粒体功能异常有关。     

3例干燥综合征合并周围神经病的临床及病理改变

目的探讨干燥综合征合并周围神经病者的临床及病理特点.方法分析3例干燥综合征合并周围神经病者的临床表现,并行胫神经活检、光学显微镜观察.结果3例患者神经纤维髓鞘脱失较为明显,其中1例损害较重,并且有轴索变性、神经营养血管纤维素样坏死、淋巴细胞浸润.结论干燥综合征者出现周围神经病可能与血管缺血、血管炎有关,早期活检可以提高诊断率及避免漏诊、误诊.     

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。     

Polyarteritis nodosa and peripheral neuropathy

Summary Peripheral nerves from 13 patients suffering from polyarteritis nodosa with multiple mononeuropathy were studied by light and electron microscopy. In the majority of cases, the vascular lesions were associated with Wallerian-like degeneration. Myelinated fibers presenting a normal axon with a disproportionately thin myelin sheath were less numerous. Unusual abnormalities consisted of swollen axons with an accumulation of organelles. Unmyelinated fibers were also damaged. A quantitative estimation of myelinated fibers loss did not show any selective vulnerability of either the large or the small diameter group.     

Ultrastructural changes of skeletal muscles in polyarteritis nodosa and in arteritis associated with rheumatoid arthritis

Summary Muscle biopsies from two cases of polyarteritis nodosa (PN) and one of arteritis in association with rheumatoid arthritis (RA) were examined by electron microscopy.The histological changes were similar in all three cases. The endothelial cells of the small blood vessels were often hypertrophied. Inflammatory reaction was present mainly in the vicinity of the blood vessels. Individual muscle fibres showed mostly nonspecific degenerative changes. In a case of PN, however, annulate lamellae were present in a small number of the muscle fibres. The annulate lamellae have been reported, to our knowledge, in the human skeletal muscles only in a few cases of polymyositis. In addition, two cases, one of PN and one of arteritis with RA, showed fine filamentous inclusions in the muscle fibres. Changes were also noted in the motor end-plate.A sural nerve biopsy in a case of arteritis with RA showed changes both in axons and myelin sheaths, in addition to the changes in the blood vessels similar to those in the muscle.     

Subperineurial space of the sural nerve in various peripheral nerve diseases

Summary The size of the subperineurial space of the sural nerve has been evaluated quantitatively in 69 cases of various peripheral nerve diseases and in controls. A significant increase was found in beriberi neuropathy (6 cases) and idiopathic polyradiculoneuropathy (9 cases) as compared with the control (8 cases). On electron microscopy a few macrophages, fibroblast processes, collagen fibrils with a diameter of 50 nm, microfibrils with a diameter of 8 nm, and amorphous material were observed in both the enlarged subperineurial space and the endoneurial intercellular space. They were less frequently observed in controls. No significant correlation was found between the size of subperineurial space and the density of myelinated fibers.     

Role of HIV in the pathogenesis of distal symmetrical peripheral neuropathy

We report the results of a clinical, electrophysiological and pathological study conducted in 18 AIDS patients presenting a distal symmetrical predominantly sensory polyneuropathy (DSPN) characterized by painful dysesthesias as main complaint. Onset of the neuropathy was at CDC (Center for Disease Control) stage II in 2 patients, at CDC stage III in 5 patients and at CDC stage IV in the remainder. Electrophysiological investigation confirmed the presence of an axonal alteration in the sensory nerves, but also revealed motor involvement in all cases. The neuropathological features of sensory nerves were fiber loss and axonal degeneration with macrophagic activation. The expression of monocyte-macrophage markers and of major histocompatibily complex class II antigens appeared up-regulated in endoneurial ramified cells, while expression of CR3, a complement receptor involved in the process of phagocytosis, was down-regulated. In six nerve biopsy samples and in two out of five DSPN dorsal root ganglia we found HIV-related mRNA and protein located in scattered cells of the endoneurium which we presume to be macrophages. These data suggest that: (a) DSPN may occur early in the course of the disease and is not limited to later stages; (b) DSPN is not a ganglionitis but is actually a sensory-motor neuropathy; (c) the virus enters the peripheral nervous system and induces changes in the immunocompetent cell population with activation of macrophages. Storage of the virus inside macrophages may act both as a reservoir for the virus and as a putative cause of nerve damage, probably through release of cytotoxins and/or interaction with trophic factors.     

Is the sensory neuropathy in ataxia-telangiectasia distinguishable from that in Friedreich's ataxia?

Summary The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.     

多发性硬化周围神经损害的肌电图及病理研究

目的：探讨多发性硬化（MS）产生周围神经损害的肌电图，病理特点和影响MS累及周围神经的相关因素。方法：33例MS患者，均满足Poser的确定诊断标准，排除其他神经系统疾病，30名正常自愿受试者作为对照，排除周围神经损害的相关因素，两组分别进行运动，感觉神经传导检测，F波潜伏期及出现率，H反射潜伏期检测，腓肠神经活检，光镜及电镜观察周围神经病理变化。结果：（1）33例MS患者中，9例有根性疼痛，3例有手袜套样感觉障碍，6例不对称性肌萎缩，2例有明显的自主神经症状；（2）肌电图显示复合肌肉动作电位波幅降低，正中神经，尺神经感觉运作电位波幅增高，F波及H反射的潜伏期延长，F波出现率降低。MS周围神经损害的程度与神经功能缺损、病程及病变部位有关，神经功能缺损越重，病程越长，胫神经和腓总神经运动传导波幅降低越明显，正中神经、尺神经感觉动作电位波幅增高越明显；脊髓型MS周围神经受损明显高于脑型；（3）6例患者腓肠神经活检，光镜下可见有髓纤维呈不同程度的髓鞘脱失。电镜下以轴索变性为主，髓鞘板层解离及髓球形成。结论：MS是一种以CNS受损为主的脱髓鞘疾病，在部分患者可对同时累及周围神经系统，脱髓鞘改变主要发生在脊神经根，远端轴突可继发轴索损害，肌电图是比较理想的可全面评价MS周围神经损害的临床检测手段，对判断预后有一定的实用价值。     

Vasculitic neuropathy in panarteritis nodosa: Clinical and ultrastructural findings

Clinical involvement of the peripheral nervous system in panarteritis nodosa is common, but the histological aspects are little known. We describe the sural nerve, muscle and skin biopsy findings in a patient with panarteritis nodosa, affected by mononeuritis multiplex. The data are compared to those reported in other types of vasculitis neuropathy.

---

Sommario È noto l'interessamento del sistema nervoso periferico nella panarterite nodosa; gli aspetti istologici però sono ancora poco conosciuti. Si descrivono gli aspetti bioptici a carico del nervo, muscolo e cute in un paziente affetto da mononeurite multipla da panarterite nodosa. I dati sono stati paragonati in altri tipi di neuropatie vasculitiche.

     

Uncompacted myelin lamellae in two cases of peripheral neuropathy

Summary Peripheral nerve biopsies from two patients with chronic sensorimotor neuropathy were studied. The first case was a non-Hodgkin malignant lymphoma and did not show any dysglobulinemia. The second case had a benign monoclonal gammopathy IgG, Lambda type. Direct immunofluorescence showed no deposits in the first case and slight deposits of anti IgG sera on a few myelinated fibers in the second case. There were numerous fibers showing uncompacted myelin lamellae, 7% in the first case and 4% in the second case. Some of these fibers had axons containing more tubules than filaments. The very few cases reported on neuropathies showing that uncompacted myelin lamellae were frequently associated with dysglobulinemic neuropathy. However, this ultrastructural abnormality of the myelin sheath can be observed without any dysglobulinemia.