慢性炎症性脱髓鞘性多发性神经病31例临床与病理分析

目的　探讨慢性炎症性脱髓鞘性多发性神经病 (CIDP)的临床及病理特征。方法　总结 31例CIDP临床及病理资料进行回顾性分析。结果　 31例CIDP患者腓肠神经活检均表现为轻度髓鞘脱失、轻度髓鞘增生及轻度胶原纤维增生“三联征” ,且主观感觉障碍愈重 ,病理改变愈明显。结论　CIDP与AIDP临床与病理特征不同 ,腓肠神经活检对CIDP具有重要的诊断价值     

非职业性慢性汞中毒周围神经损害的临床表现及电生理特征

目的 评价非职业性慢性汞中毒患者周围神经损害的临床表现及其神经电生理特征.方法 选取军事医学科学院附属医院神经内科2011年12月～2013年9月收治的慢性汞中毒患者31例为中毒组,选取年龄、性别匹配的31例健康体检者为对照组.收集两组的相关资料并进行比较分析.结果 慢性汞中毒患者周围神经损害的临床表现以肢体无力、疼痛、麻木为主.运动神经检测显示中毒组正中神经、尺神经、腓总神经远端潜伏期较对照组延长,正中神经、尺神经、腓总神经传导速度减慢,差异有统计学意义（P＜0.01或P＜0.05）.感觉神经检测显示中毒组腓肠神经、正中神经潜伏期较对照组延长,尺神经、桡神经波幅降低,腓肠神经、正中神经、尺神经、桡神经传导速度减慢,差异有统计学意义（P＜0.01或P＜0.05）.结论 非职业慢性汞中毒性周围神经损害起病隐匿,神经电生理检查能够无创、客观地反映患者的周围神经损害程度.     

燃煤型砷中毒所致神经肌肉损害的临床病理观察

目的探讨燃煤型砷中毒所致神经肌肉损害的临床病理特征。方法对4例中、重度燃煤型砷中毒患者的腓肠神经与腓肠肌进行活检,光镜与电镜观察周围神经与肌肉的病理变化。结果光镜下见神经纤维变性萎缩,部分区域呈现脱髓鞘改变,间质中未见炎症细胞浸润;骨骼肌呈现节段性凝固性坏死(蜡样坏死),肌肉中未见炎症细胞浸润。电镜下见有髓神经纤维,部分有髓鞘分层、溶解塌陷,髓鞘下可见大空泡,轴浆内结构基本正常,部分血旺氏细胞轻度肿胀;肌丝灶性溶解,肌膜呈城垛样改变,T小管增多,肌细胞部分发生萎缩,间质增多。结论燃煤型砷中毒可致周围神经、肌肉病理改变,从而导致一系列临床症状,并引起功能障碍。     

多灶性运动神经病的临床、电生理和病理分析

目的　对 15例诊断为多灶性运动神经病 (multifocalmotorneuropathy ,MMN)的病例的临床、电生理和病理进行研究 ,并就诊断易混淆的问题进行讨论。方法　行肌电图检查 ,以ELISA法检测血、脑脊液抗GM1抗体 ,5例病人行腓肠神经活检。结果　上肢起病 7例 ,下肢起病 8例 ,肌无力以肢体的远端为重 ,与肌萎缩并不平行。 4例病人血抗GM1(IgM )抗体升高。所有病人均有运动传导阻滞。病理为不同程度髓鞘脱失 ,有轻度或散在的轴索变性。结论　结果表明MMN为免疫介导的脱鞘性周围神经病 ,有特征性的运动传导阻滞 (CB)。CB的诊断标准和判断的准确性对MMN的诊断具有决定性作用     

肢体骨损害为首发症状的多发性骨髓瘤31例临床分析

分析31例以肢体骨损害为首发症状的多发性骨髓瘤(MM)患者的临床资料,股骨损害率最高(54.8%);股骨骨折率76.5%,其次为肱骨.骨髓穿刺阳性率80.6%,17例病变部位骨活检病理诊断为浆细胞瘤,骨活检与骨髓穿刺阳性符合率64.7%.13例骨折患者行手术加化疗,病情均缓解.用双磷酸盐治疗者较单纯化疗者的平均骨痛缓解时间缩短,骨病新发事件少.提示肢体骨损害为首发症状的MM患者股骨损害率最高,骨组织穿刺病理学检查可提高其诊断率;双磷酸盐加常规化疗可延缓骨病发展,手术有助于改善患者的生活质量.     

原发性中枢神经系统恶性淋巴瘤 3例报告

目的 报告3 例原发性中枢神经恶性系统淋巴瘤,以提高人们对本病的认识.方法 回顾性分析3例患者的临床资料.结果 临床表现均以颅内压增高和神经功能缺损(头痛、肢体无力)为主要症状,头颅MRI检查示占位性病变,3例均为多发病灶,分别经脑活检及腰穿脑脊液病理诊断为淋巴瘤.结论 本病经影像学检查很难确诊,脑活检及腰穿脑脊液细胞学检查提供了可靠的临床诊断方法.     

血管炎合并周围神经病变2例

例1:女,18岁.因发热伴四肢关节疼痛半年,双下肢疼痛,麻木10天入院.患者于半年前出现膝关节疼痛,伴双下肢红斑、浮肿、发热,当地医院按下肢静脉炎给予青霉素和复方丹参治疗,浮肿消退,症状缓解.3个月后再次出现发热,伴肩肘关节、掌指关节及踝关节疼痛,但经上述治疗无好转,以后出现间断发热,四肢远端皮肤反复红斑,压之褪色,以胫前和足底明显.10天前出现双下肢剧痛、麻木,无遇冷加重遇热减轻.查体:T37.5℃,P80次/min,Bp112/75mmDHg(1mmDHg=0.133kPa) .患者神志清、精神差,颅神经检查(-).四肢远端肌力差,双足伸屈受限,无肌萎缩和肌束颤动.肱二头肌及肱三头肌反射减退,膝反射及踝反射消失.四肢肘膝关节以下浅感觉减退,足底、足背、胫前皮肤有青紫色斑,形态不规则,压之退色.脑膜刺激征(-),病理反射未引出.腓肠神经活检示神经外膜内多个血管及周围有以淋巴细胞为主的单核细胞浸润,有两条动脉几乎完全闭塞,动脉壁增厚呈纤维化,约50%的神经纤维髓鞘脱失.诊断为血管炎继发性周围神经损伤.     

肿瘤化疗引起的神经系统并发症

抗肿瘤药物对神经系统的毒副作用与药物种类、用药的剂量、方式及途径有关。这些毒副作用可表现在周围神经、颅神经和中枢神经系统。1 周围神经及颅神经损害 长春碱类药物最常引起周围神经病变,以周围神经炎较为多见。患者表现为肢体远端麻木、疼痛等感觉功能障碍,运动功能障碍较少见。少数患者可出现颅神经病变,表现为声音嘶哑、眼睑下垂等。有的在第一次应用长春新碱后数小时即发生严重下颌疼痛,可能与三叉神经毒性有关。此种疼痛为自     

临床常用药物的神经毒性

药物的神经毒性作用是药物与机体相互作用的结果,根据毒性作用发生在神经组织的部位不同,可将药物对神经系统毒性作用类型分为神经元损害、轴索损害、髓鞘损害、影响神经递质功能四种类型.药物对神经系统的毒性作用,表现为对神经系统结构和功能的损害,神经症状多为眼睑下垂、眼肌麻痹、嗅觉障碍、味觉障碍、听觉障碍、平衡失调、手和足出现对称性麻木和针刺感、肌肉萎缩、膝腱和踝反射异常、肌肉无力等,也可表现为多样化的精神病样发作;神经系统的毒性作用严重者可致死亡.现将临床常用药物产生神经毒性作用的机制及其临床表现综述如下.     

以胸闷为首发症状的肌原纤维肌病一例并文献复习

肌原纤维肌病是一种罕见的遗传性骨骼肌疾病,以包涵体或蛋白质沉积为病理学特点,临床表现复杂多样,主要有慢性进行性肢体无力,以远端肌无力为主,部分患者可兼有周围神经损害表现,部分患者可出现心肌病甚至以心肌病变为主要症状。现报道1例以胸闷为首发症状的成人肌原纤维肌病患者,通过临床特点、病理活检及文献复习,了解其病理特点及诊治方法。     

Common and less common peripheral nerve disorders associated with diabetes

Diabetes can be associated with a number of peripheral nerve disorders. The commonest is slowly-progressive axonal distal symmetrical sensori-motor neuropathy. Sensory loss and positive sensory symptoms are its main manifestations. Lumbosacral radiculoplexus neuropathy (LSRPN) is a distinct entity, accompanied by severe lumbar, hip, leg pain and weight loss, with subsequent weakness. Although typically unilateral, bilaterality is described, with spontaneous recovery usual over several months. The upper limb counterpart, cervical radiculoplexus neuropathy is rare. Acute painful neuropathies, including "diabetic neuropathic cachexia", are infrequent. Accompanying weight loss is usual and burning pains in the extremities are severe. Insulin-triggered acute painful neuropathy is well-described although infrequent and still poorly-understood. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represents an immune-mediated treatable disorder, usually causing prominent diffuse motor weakness, which was described as more common in diabetics. More recent epidemiological data have however been conflicting and it is possible that CIDP is no more frequent in diabetics than in the general population. Diagnosis is made by electrophysiology and cerebrospinal fluid analysis. A painless diabetic motor neuropathy, thought to be caused by ischaemic injury and microvasculitis, has recently been postulated as separate from LSRPN and CIDP. Other focal and multifocal neuropathies that can occur in diabetics are cranial or truncal. Entrapment neuropathies are more often of median and ulnar nerves, and may in some cases benefit from decompression. Finally, autonomic neuropathies are well-described in diabetes and can be diverse in presentation with cardiovascular, gastrointestinal, urogenital and sudomotor manifestations. Their management can be difficult with debilitating symptoms despite treatment.     

Acute inflammatory demyelinating polyneuropathy associated with pegylated interferon α 2a therapy for chronic hepatitis C virus infection

The combination of pegylated interferon （Peg-IFN） and ribavirin is the standard of care for chronic hepatitis C virus （HCV） infection treatment. In general, common side effects related to this combination therapy are mild and are very well tolerated. However, peripheral neuropathy including demyelinating polyneuropathy related to Peg- IFN is extremely rare. We present the first case of an acute inflammatory demyelinating polyneuropathy （AIDP） associated with Peg-IFN-α 2a （Pegasys） after 16 wk of a combination therapy with Pegasys and ribavirin in a 65-year-old woman with chronic HCV infection. She developed tingling, numbness, and weakness of her upper and lower extremities and was hospitalized for acute neurological deficits. Her clinical course, neurological findings, an electromyogram （EMG）, nerve conductions studies （NCS）, muscle biopsy, and a sural nerve biopsy were all consistent with AIDP likely related to Pegasys use. The patient recovered completely with the use of intravenous immunoglobulin （IVIG） including physical therapy and neurological rehabilitation. It is very important that gastroenterologists and/or hepatologists recognize this rare neurological complication related to Peg-IFN treatment very early, since it requires a prompt discontinuation of therapy including an immediate referral to a neurologist for the confirmation of diagnosis, management, and the prevention of long-term neurological deficits.     

Autonomic Dysfunction with Orthostatic Hypotension1,2

Summary: Three cases of orthostatic hypotension with autonomic failure are presented and the physiological tests which were performed to assess the extent of their autonomic loss discussed.
In one patient, the diagnosis of widespread amyloid disease was made at autopsy, while in another patient, who is still living, amyloid material was found in a biopsy from the sural nerve. In both patients, rectal biopsy had been negative for amyloid. Rectal biopsy was also negative for amyloid in the third patient, though a sural nerve biopsy was not performed in her case.
The treatment of orthostatic hypotension arising from autonomic failure is briefly discussed.
Autonomic failure with orthostatic hypotension and loss of sweating was first described by Bradbury and Eggleston in 1925¹. Since then, several case reports have been published^2,3 of patients who also had loss of sphincter control and impotence. The patients may be divided into two groups, i.e. a group where the symptoms are secondary to a recognised disease, such as diabetes mellitus, amyloidosis or tabes dorsalis, and a second group for which no cause can be found. This paper describes three patients with autonomic dysfunction and the physiological tests which were performed to assess the extent of their autonomic loss. One of the patients died and, at autopsy, was found to have widespread amyloid disease. Studies on the two other patients, still living, are reported, and in one, a brother of the deceased patient, nerve biopsy shows the presence of amyloid.     

Mononeuritis multiplex in incomplete Behcet's disease: A case report and the review of the literature

Summary A case of Behcet's disease with peripheral nervous system involvement is described. A 58-year-old female with a 16-year history of Behcet's disease was admitted to our hospital because of numbness in multiple areas in both the upper and lower extremities. A biopsy of the sural nerve revealed degeneration of the axons and a capillary lesion. This finding was compatible with mononeuritis multiplex. This case of Behcet's disease with biopsy-proved mononeuritis multiplex is a very rare one.     

Nodular cutaneous amyloidosis and carpal tunnel syndrome due to the amyloidogenic transthyretin His 114 variant.

This is the second report of transthyretin (TTR) amyloidosis in a patient who had ATTR Tyr114His diagnosed by mass spectrometry and gene analysis. This case had some clinical features that differed from those of the first reported cases. The patient, 73-year-old man, complained of generalized cutaneous tubercula that had started at age 68. These tubercula gradually increased in size and became generalized. He felt a slight numbness in his extremities. Clinical and electrophysiological examinations revealed that he had bilateral carpal tunnel syndrome (CTS), whereas there was no clear evidence of sensory and/or motor polyneuropathy. Autonomic symptoms were not present. Biopsy studies revealed that both his tuberculum and his sural nerve contained TTR-related amyloid. In his sural nerve, amyloid deposits were observed mainly in the perineurium, not in the endoneurium, and there was no significant depletion of myelinated fibers. The features of this patient were clinically characterized by generalized cutaneous amyloid deposits and late-onset CTS with a lack of overt polyneuropathy and autonomic dysfunction. The unique clinical features in this case seemed to be consistent with the distribution of amyloid deposits.     

Chronic inflammatory demyelinating polyneuropathy in patients with systemic lupus erythematosus: prognosis and outcome

OBJECTIVES: To identify clinical characteristics, laboratory features, approaches to management, and predictors of outcome of chronic inflammatory demyelinating polyneuropathy (CIDP) in patients with systemic lupus erythematosus (SLE). METHODS: An analysis of 6 adults with the concurrent diagnosis of CIDP and SLE seen at a SLE Clinic from 1994 to 2004 with a review of 13 patients with SLE and CIDP reported in the medical literature from 1950 through 2004. RESULTS: Among our 6 patients with SLE and CIDP, 3 (50%) achieved a substantial clinical response to intravenous immunoglobulin (IVIg) and the remainder had a minimal response. The improved patients were more likely to have received treatment earlier (within 1 year of CIDP onset) and to respond faster (<1 to 3 months) than minimally improved patients. They tended to have CIDP features of weakness of all extremities, hyporeflexia of the upper extremities, and slowed nerve conduction velocity of the motor median nerve. Compared with minimal responders, responders had more serious internal organ manifestations and multiple autoantibodies associated with SLE. Review of the literature identified 13 previously reported CIDP patients with SLE. Many had neurological involvement of all extremities, nerve biopsies showing demyelination, and serious SLE internal organ manifestations. Most were treated with steroids, but the 1 treated with IVIg had similar characteristics to our subset of patients who improved with IVIg. CONCLUSIONS: CIDP is an uncommon, but not rare, manifestation of SLE. Certain characteristics including early CIDP diagnosis, involvement of all 4 extremities, hyporeflexia of the upper extremities, and slowed motor nerve conduction velocity of the median nerve in addition to SLE involvement of critical internal organs and the presence of multiple antibodies associated with SLE all appear to predict a good response to IVIg.     

Late-onset mitochondrial neuromyopathy: an age-related phenomenon?

Peripheral neuropathy has been described in a number of cases of mitochondrial diseases. In these patients the onset of neuropathy varies from childhood to adulthood, whereas late onset is quite rare. We report here three males, ranging from 71 to 75 years with onset of peripheral neuropathy between 64 and 74 years of age. They complain of ataxic gait, muscle aches, weakness and mild muscle atrophy, sensory impairment with predominant glove and stocking distribution, reduced or absent deep tendon reflexes. Neurophysiological examinations and sural nerve biopsy studies showed a sensorimotor neuropathy with axonal degeneration in two cases and demyelination in one. Peroneus brevis muscle biopsy revealed, apart from frank neurogenic changes, presence of ragged-red fibers and cytochrome c oxidase negative fibers. Electron microscopy confirmed an abnormally increased presence of subsarcolemmal and intermyofibrillar mitochondria in muscle samples. These morphological features suggested a mitochondrial disease that was confirmed by biochemical investigations on muscle homogenate showing that the mitochondrial respiratory chain (MRC) enzyme activities were all reduced when compared to citrate synthase activity. In addition the presence of a partially inactive cytochrome c oxidase protein by ELISA was demonstrated in two cases. According to a recent "mitochondrial theory of aging", we think that a progressive decline of MRC function has affected either skeletal muscle or peripheral nerves in our patients. Being energy-requiring processes, muscle metabolism as well as active axonal transport may become progressively defective with age resulting in a late-onset neuropathy.     

Angio-immunoblastic lymphadenopathy and neurological manifestations (author's transl)

Cases of associated angio-immunoblastic lymphadenopathy (AIL) and peripheral neuropathies have been rarely reported in the literature. Three such cases are described, in two men and one woman aged 79, 59, and 45 years respectively. Diagnosis of AIL was confirmed by lymph node biopsy in the two latter patients during their lifetime, and in the first case from examination of cervical nodes at autopsy. All three patients presented neurological disorders, of the polyradiculoneuritis type in the first case, multiple neuritis of the lower limbs followed by radiculalgia in the second, and myalgia and neuralgia with neurogenic signs in the EMG in the third case. No evidence of a toxic, metabolic, or infections aetiology was found, histological examination of nerve and muscle specimens demonstrated localised AIL lesions in one case, and discrete lymphoplasmocytic infiltration of the peripheral nervous system in the other two patients.     

糖尿病周围神经病700例临床与神经电生理分析

目的探讨糖尿病周围神经病的临床和电生理特点,明确电生理检查的诊断价值。方法对700患者进行感觉和运动神经传导测定,240例患者进行针极肌电图测定。结果507例(724%)患者电生理检查异常,其中307例(606%)为多发性周围神经病,74例(146%)为腕管综合征;感觉神经传导异常程度重于运动神经,波幅的下降程度较传导速度减慢明显,下肢重于上肢(P<005)。仅有46%的患者针极肌电图异常而神经传导正常。结论糖尿病周围神经病的临床和电生理表现均以感觉神经受损为主;电生理检查有助于发现临床病变,但并非所有患者均能发现电生理异常;建议不将针极肌电图进行糖尿病周围神经病的筛查作为常规使用。