亚急性联合变性的临床与辅助检查的诊断意义

目的:探讨亚急性联合变性(ＳＣＤ)的临床特点及电生理和核磁共振成像的诊断价值。方法:对１４例ＳＣＤ患者的临床资料进行回顾性分析。结果;发现所有ＳＣＤ患者发病均由维生素Ｂ１２缺乏所引起的肢体感觉异常,深感觉减退,共济失调及痉挛性轻瘫是ＳＣＤ常见的症状和体征。该病早期易误诊,电生理检查有极高的敏感性,核磁共振成像可以确定脱髓鞘的部位。结论:血清维生素Ｂ１２浓度测定、电生理及核磁共振检查对诊断和治疗有重要作用。     

系统性红斑狼疮合并周围神经病的临床特点和肌电图改变

分析２２例临床有或疑有神经系统损害的系统性红斑狼疮（ＳＬＥ）患者，其中仅３例被临床诊断为ＳＬＥ合并周围神经病，然神经电生理检查证实１３例为周围神经损害。主要临床特点为对称性和非对称性四肢远端麻木、疼痛，深浅感觉障碍，肌力减退，肌萎缩等。神经电生理检查显示神经传导速度减慢，波幅降低，异常自发电位（纤颤电位和正锐波），运动单元多相电位增加。电生理检查与病理改变相符：ＳＬＥ合并周围神经病既有轴索损害，又有脱髓鞘改变。提示神经电生理检查可以为ＳＬＥ患者提供早期或亚临床周围神经损害的依据。     

亚急性联合变性四例磁共振成像特点

报道４例亚急性联合变性（ＳＣＤ）患者的临床特点及脊髓磁共振成像（ＭＲＩ）所见，观察病灶于治疗前后的变化并分析ＭＲＩ在诊断ＳＣＤ中的价值。例１男，６８岁，因双足麻木２周、双下肢无力１０天入院。查体：双下肢肌张力偏高，深感觉减退。血ＲＢＣ２．９９×１０１...     

系统性红斑狼疮合并周围神经病的临床特点和肌电…

分析２２例临床有或疑有神经系统损害的系统性红斑狼疮（ＳＬＥ）患者，其中仅３例被临床诊断为ＳＬＥ合并周围神经病，然神经电生理检查证实１３例为周围神经损害。主要临床特点为对称性和非对称性四肢远端麻木，疼痛，深浅感觉障碍，肌力减退，肌萎缩等，神经电生理检查显示神经传导速度减慢，波幅降低，异常自发电位（纤颤电位和正锐波）运动单元多相电位增加。电生理检查与理改变相符，ＳＬＥ合并周围神经病既有轴索损害，又有脱     

脊髓亚急性联合变性的磁共振特点

脊髓亚急性联合变性（ＳＣＤ）是由维生素Ｂ１２（ＶｉｔＢ１２）代谢障碍引起的神经系统疾病。随着磁共振成像（ＭＲＩ）在神经科的广泛应用，为ＳＣＤ提供了有效的影像学诊断依据。现将我院有ＭＲＩ异常的３例ＳＣＤ患者及国内报道的７例总结如下。 临床资料 １．一般资料：１０例均为我院住院患者，男９例，女１例，年龄３４～６８岁，平均年龄５５岁。起病至就诊时间２周至４年，平均６．８个月。病程在半年以内６例，半年以上４例。病因：胃大部切除术２例，恶性贫血４例，另４例病因不清。 ２．临床表现：在１０例ＳＣＤ中，均有肢体…     

亚急性联合变性的磁共振表现

目的评价磁共振成像（ＭＲＩ）对亚急性联合变性（ＳＣＤ）的诊断价值。方法对临床和实验室检查确诊的７例ＳＣＤ患者进行ＭＲＩ检查。结果有３例异常，均位于胸段脊髓，２例Ｔ２加权为片状或条状高信号区，１例胸髓变细。结论ＭＲＩ对ＳＣＤ有一定的诊断价值。     

鞍膈脑膜瘤的诊断和治疗

目的探讨鞍膈脑膜瘤（ＤＳＭ）的分类、诊断及手术方法。方法经磁共振成像（ＭＲＩ）检查发现１２例ＤＳＭ，其中６例为Ａ型（鞍膈上垂体柄前），３例为Ｂ型（鞍膈上垂体柄后），２例为Ｃ型（鞍膈下蝶鞍内），１例为Ｄ型（累及鞍膈上下和垂体柄前后）。结果１２例病人均行手术治疗，其中１１例全切除，１例大部切除，无手术死亡率和严重功能障碍。结论ＭＲＩ是诊断ＤＳＭ的最好方法。有助于鉴别垂体瘤和鞍结节脑膜瘤及其手术方法的选     

Creutzfeldt-Jakob病的磁共振诊断

目的　探讨头部磁共振成像对Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病（ＣＪＤ）的诊断价值。方法　２例均经脑活检、免疫组化及实验鼠传递证实,分别于发病后２、１２个月进行头部磁共振检查。结果　２ 例双侧尾核、壳核于Ｔ２加权像均呈对称性均质高信号。结论　头部磁共振成像的异常信号对ＣＪＤ诊断虽非完全特异,但结合临床具有一定价值。     

遗传性运动感觉性神经病合并肥厚性心肌病的3D—TCD,rCBF,BAEP…

目的了解遗传性运动感觉性神经病（ＨＭＳＮ）合并肥厚心肌病（ＨＣＭ）的经颅多普勒（ＴＣＤ）局部脑血流量（ｃＣＢＦ），脑干听觉诱发电位（ＢＡＥＰ）和心电图（ＥＣＧ）的变化。方法，对一家三代ＨＭＳＮ合并ＨＣＭ１２例患者和１例无症状者常规进行了这四项检查，结果ＴＣＤ，ｒＣＢＦ，ＢＡＥＰ和ＥＣＧ的异常率分别为８５％，７６％，９２％和９２％。结论绝大多烽ＨＭＳＮ合并ＨＣＭ患者的ＴＣＤ，ｒＣＢＦ，ＢＡＥＰ和ＥＣ     

多系统萎缩合并周围神经病的临床和病理研究

多系统萎缩（ＭＳＡ）合并周围神经病７例，其中临床诊断ＭＳＡ３例，橄榄体－桥脑－小脑萎缩３例，原发性姿位性低血压１例。全组锥体束征４例，锥体外束征２例。７例均有小脑性共济失调。姿位性低血压４例，感觉障碍４例，健反射减低６例；阳萎６例，膀胱障碍４例，电生理检查均示不同程度异常，６例颅脑ＣＴ和２例磁共振成像均示小脑脑干萎缩。腓肠神经活检病理特征为慢性脱髓性病变。２例作酶标免疫组化法检测ＩｇＧ、ＩｇＭ、ＩｇＡ和Ｃ３均呈阳性的反应。文章结合文献对ＭＳＡ临床分类、病理特点、病因和发病机制进行了讨论。     

笑气滥用的神经系统损害(附1例报道并文献复习)

目的 探讨笑气(氧化亚氮)滥用中毒所致的神经系统损害的临床特点。方法 分析1例滥用笑气中毒所致的神经系统损害病例的临床特点及转归。结果 ①笑气中毒所致神经系统病变在临床易被漏诊,以青年多见; ②笑气中毒所致神经系统病变的临床表现为亚急性联合变性样的脊髓损害、以运动轴索损害为主的长度依赖性的周围神经病变以及共济失调等,并常有维生素B12水平下降、高同型半胱氨酸血症; ③磁共振可见颈髓和或胸髓后索呈不规则的白质脱髓鞘,脊髓横断面T₂WI显示颈或胸髓后索倒“V”形高信号; ④肌电图主要表现为以运动轴索损害为主的周围神经病变,多以下肢为重。结论 亚急性或慢性起病的青年患者,表现亚急性联合变样、周围神经损害等,需详细询问有无笑气接触史,行血维生素B12、同型半胱氨酸、颈髓磁共振、肌电图等检查,以明确诊断,排除笑气中毒可能。     

笑气滥用导致神经功能缺损的临床特点

目的探讨笑气滥用导致神经功能缺损的临床特点。方法回顾性分析2例笑气滥用导致神经功能缺损患者的临床资料,并对相关文献资料进行复习。结果 1例以模拟格林-巴利综合征的症状发病,表现为四肢对称性的麻木无力,脊椎横断面T2WI显示颈髓后索倒"V"形高信号,EMG呈多发性周围神经脱髓鞘和轴索损伤。另1例以模拟急性脊髓炎症状起病,表现为双下肢无力伴小便潴留,EMG呈脊髓损害表现,但脊椎MRI未发现明显病灶。2例患者血同型半胱氨酸水平均明显升高,伴或不伴维生素B12缺乏,停止吸食笑气并补充维生素B12后症状逐渐好转。结论当健康青年人出现维生素B12缺乏的神经系统症状,如表现为亚急性联合变性、周围神经病等,应考虑笑气中毒的可能。     

脊髓亚急性联合变性的临床特征分析

目的总结脊髓亚急性联合变性患者的临床表现及磁共振成像、肌电图的特点,并探索血中有关标志物水平与神经损害程度的相关性。方法回顾性分析2016-12—2018-05于河南省人民医院临床确诊的50例脊髓亚急性联合变性患者的临床特点,用Spearman秩相关分析血标志物水平与神经损害程度的相关性。结果首次发病年龄16~86(58.92±16.52)岁,其中60岁以上首次发病27例(54%),男34例(68%);50例患者中累及周围神经者31例(62%),后索者46例(92%),侧索者27例(54%),大脑白质与视神经损害10例(20%);36例(72%)患者行神经传导速度检查,25例(69%)有异常改变;50例患者全部行常规MRI检查,19例(38%)行常规加增强MRI检查,其中25例(50%)患者发现病灶;血清平均血红蛋白浓度、血清VitB12水平与神经功能损伤程度无显著相关性(P>0.05),血清平均红细胞体积、叶酸、同型半胱氨酸水平与神经功能损伤程度呈正相关(r>0),红细胞计数、血红蛋白、平均血红蛋白量、总蛋白、白蛋白与神经功能损伤程度呈负相关性(r<0)。结论SCD患者临床上以脊髓后索损害较为常见,辅助检查中神经电生理检查检出率高于MRI;且对于院前补充过VitB12或者功能性VitB12缺乏的SCD患者,血清中的其他标志物可以作为间接参考指标。     

大剂量甲钴铵治疗亚急性联合变性的临床、影像及电生理分析

目的：观察亚急性联合变性（SCD）患者的临床特点、影像学（MRI）、电生理表现及甲钻铵治疗前后的变化,评价其诊断价值和疗效。方法：选取15例SCD患者,检测血常规和血清维生素B12、叶酸浓度,进行周围神经传导速度、体感诱发电位、视觉诱发电位、听觉诱发电位及脊髓MRI检查,观察治疗前后血红蛋白、血清维生素B12浓度、MRI及电生理的变化,并进行神经功能评定。结果：经甲钴铵治疗后患者临床症状明显改善,脊髓MRI显示的颈胸髓内后索、侧素长条状等T1长T2异常信号,治疗后多数病灶缩小或消失,电生理改变亦明显好转。结论：MRI诊断SCD准确度高;电生理检查敏感度高,且可发现亚临床病灶。两者结合可有助于SCD的早期明确诊断。大剂量甲钻铵静脉滴注可迅速提高血清维生素B12浓度,吸收利用度好,能有效改善SCD患者的神经功能。     

Central vs peripheral nerve conduction. Before and after treatment of subacute combined degeneration

Central and peripheral nerve conduction was studied in two patients with subacute combined degeneration by using the short-latency somatosensory evoked potentials and the peripheral nerve conduction study during treatment with cyanocobalamin. Before the treatment, somatosensory evoked potentials with median nerve stimulation were normal, but those with peroneal nerve stimulation revealed prolonged central conduction indicating dysfunction within the posterior column. Peripheral sensory and motor nerve action potentials were reduced with normal or slightly reduced conduction velocity. After treatment, marked shortening of the central conduction time (by 24% and 31%, respectively) was observed with mild or no recovery of peripheral nerve action potentials. These physiologic findings suggest that the main pathologic changes in the central nervous system may be demyelination in the posterior column in addition to axonal degeneration in the peripheral nerve. The former was responsive to treatment but the latter was poorly responsive to treatment. Sensory symptom in subacute combined degeneration appears to be, at least partially, attributed to the spinal cord lesion.     

Central nervous system involvement in hereditary neuropathy with liability to pressure palsies: description of a large family with this association

OBJECTIVE: To describe a large family with hereditary neuropathy with liability to pressure palsies associated with central nervous system demyelination. DESIGN: We examined the 18 members of a pedigree. Genetic analysis was performed on 15 subjects, standard nerve conduction studies on 10 subjects, and brain magnetic resonance imaging studies on 8 subjects. RESULTS: Hereditary neuropathy with liability to pressure palsies was confirmed in 9 patients of the pedigree. Brain magnetic resonance imaging findings showed multiple areas of demyelination in 6 of 6 affected members and were normal in 2 of 2 healthy relatives. Magnetic resonance imaging abnormalities were predominantly located in the subcortical frontal white matter. All patients had acute and recurrent nerve palsies, while clinical features of central nervous system involvement were not a characteristic of this pedigree. CONCLUSIONS: We demonstrate that this association, previously reported in sporadic cases, is not coincidental. Therefore, patients with hereditary neuropathy with liability to pressure palsies can present central nervous system white matter lesions, and the role of the PMP22 (peripheral myelin protein 22) gene deletion in the central nervous system should be further studied.     

3.0-T磁共振成像在儿童臂丛神经分娩损伤的作用

Brachial plexus birth injuries in children are usually diagnosed using 1.5-T magnetic resonance imaging,while the application of high-field magnetic resonance imaging is rarely reported.Therefore,a retrospective comparison of 18 cases of children with brachial plexus injury was performed to investigate the characteristics of 3.0-T magnetic resonance imaging and intraoperative observations.Magnetic resonance examinations in 18 cases of children showed that pseudo-meningocele sensitivity,specificity,accuracy,and positivity rates were 83.3%,79.6%,81.1%,and 40.0%,respectively.As for the neuroma and fibrous scar encapsulation,the sensitivity,specificity,accuracy,and positivity rates were 92.9%,50.0%,83.3%,and 77.8%,respectively.These results confirm that 3.0-T high-field magnetic resonance imaging can clearly reveal abnormal changes in brachial plexus injury,in which pseudo-meningocele,fibrous scar encapsulation,and neuroma are the characteristic changes of obstetric brachial plexus preganglionic and postganglionic nerve injury.     

Vitamin B12 extensive thoracic myelopathy: clinical, radiological and prognostic aspects. Two cases report and literature review

The myelopathy caused by vitamin B12 deficiency is known as subacute combined degeneration. It is rare, but a well known cause of demyelination of the dorsal columns of the spinal cord. The magnetic resonance imaging is characterized by an increased signal on T2-weighted images involving the posterior columns of cervical and thoracic cord. There have been few cases in literature with extensive lesions (more than seven levels) of the thoracic spinal cord. The clinical and radiological improvements are possible if the replacement of vitamin B12 is initiated precocious. We present two rare cases of extensive thoracic myelopathy due to vitamin B12 deficiency. The first is a young woman with complete clinical recovery and important radiologic improvement after early treatment. In addition, the second case is an older man with partial response to the treatment. Those cases illustrate the importance of considering vitamin B12 deficiency in any patient, who presents with myelopathy.     

The spectrum of changes on 20 nerve biopsies in patients with HIV infection

Nerve and muscle biopsies were performed on 20 patients with HIV infection and peripheral neuropathy. Nine patients had distal symmetrical peripheral neuropathy (DSPN) (six ARC and three AIDS), six had inflammatory demyelinating polyneuropathy (IDP) (three ARC, one AIDS, and two otherwise asymptomatic patients), one had mononeuropathy multiplex (MM) (AIDS), 1 had mononeuropathy (ARC), one had meningoradiculitis (AIDS), and two had areflexia-associated lymphocytic meningitides (ARC), DSPN exhibited axonal degeneration in four of nine cases and was associated with segmental demyelination in five of nine cases. IDP exhibited segmental demyelination associated with axonal degeneration in four of six cases. Demyelination was more frequent in asymptomatic patients (2 of 2 cases) and in ARC (7 of 12 cases), whereas axonal degeneration was predominant in AIDS (6 of 6 cases). Mononuclear cell infiltration was seen in 1 of 2 asymptomatic patients and in 11 of 12 ARC patients but was exceptionally found in AIDS (1 of 6 cases). Involvement of the walls of small vessels, mostly venules ("subacute microvasculitis"), was found in 1 of 2 asymptomatic patients, in 8 of 12 ARC patients, and never in AIDS. The polyclonal mononuclear cell population was composed mainly of Leu 2 (T8) positive cells in seven cases of ARC. No virions were seen in electron microscopy. HIV was isolated in two cases from the CSF or the nerve biopsy.     

MR Findings of Intrathecal Chemotherapy-Related Myelopathy in Two Cases: Mimicker of Subacute Combined Degeneration

We report the findings of spinal magnetic resonance imaging (MRI) in 2 patients who had undergone intrathecal chemotherapy and presented with the subacute onset of ascending numbness and weakness. MRI revealed a symmetric hyperintensity at the posterior columns of the spinal cord from the lower cervical region down to the conus medullaris level on T2-weighted images, and no abnormal enhancement. The imaging findings are similar to those seen in subacute combined degeneration (SCD), but the serum vitamin B(12) levels were normal in these 2 cases.