脊髓型多发性硬化临床诊断分析

目的 总结分析脊髓型多发性硬化(MS)的临床特点和MRI表现.方法 回顾性分析21例脊髓型MS的临床特点和MRI表现,所有患者行脊髓和颅脑MRI检查.结果 脊髓型MS除有脊髓病变的临床表现外,临床症状和体征的多样性是其特点,如感觉障碍、肢体无力、视力障碍等;脊髓内病灶的MRI特点是不规则斑片状和条带状异常信号,位于脊髓两侧和后部,在T:WI像上为高或稍高信号,在T1WI上为等信号或稍低信号;80.9%(17/21)脊髓型MS合并脑内病灶.结论 脊髓型MS临床表现呈多样性,MRI可以准确显示脊髓内病灶,颅脑MRI检查有助于脊髓型MS的诊断.     

Magnetic resonance imaging in patients with multiple sclerosis and spinal cord involvement: 28 cases

In patients with clinically isolated spinal disease, magnetic resonance imaging (MRI) provides a non-invasive method of detecting surgically treatable causes and is also useful in detecting asymptomatic brain lesions where the cord syndrome is due to multiple sclerosis (MS). We report the findings of spinal and brain MRI in 28 patients with spinal cord disorder due to MS. It was possible to detect intrinsic plaques reliably in the majority of patients (60.7%) with clinical findings of spinal cord MS. The results of MRI are compared with the clinical status and with cerebrospinal fluid findings and evoked potentials.     

A spinal cord MRI study of benign and secondary progressive multiple sclerosis

This study was performed to achieve a better definition of the nature of the disability in multiple sclerosis (MS). Axial spinal cord magnetic resonance imaging (MRI) at C5 was obtained in 15 patients with benign MS, 17 patients with secondary progressive MS and 10 healthy controls. Patients with secondary progressive MS had smaller spinal cord cross-sectional area (P = 0.01) and transverse diameter (P = 0.006) than patients with benign MS. The degree of disability was inversely correlated with both the cross-sectional area (r = –0.6,P = 0.0018) and transverse diameter (r = –0.5,P = 0.0032) of the cord. Spinal cord atrophy was found in 7 (41%) patients with secondary progressive MS and in 2 (13%) with benign MS. These findings suggest that destructive pathology within MS lesions might play a relevant role in the development of disability in MS.     

多发性硬化脊髓受累临床与MRI对照研究

目的分析多发性硬化脊髓受累患者临床表现及MRI影像学特点,并探讨其临床诊断意义。方法回顾分析2006年1月-2009年12月住院治疗的46例多发性硬化脊髓受累患者的临床资料及影像学表现。结果起病形式以急性（58.70%,27/46）或亚急性（34.78%,16/46）为主,临床主要表现为肢体瘫痪（95.65%,44/46）、感觉障碍（84.78%,39/46）和尿潴留（67.39%,31/46）。MRI受累部位以颈髓最常见（45.65%21/46）,其次为胸髓（28.26%,13/46）,呈脊髓内单一或散在多发长T₁、长T₂斑片状异常信号影,病灶长度一般不超过2个椎体节段（84.78%,39/46）,个别患者（15.22%,7/46）病灶长度超过2个椎体节段;增强扫描可有不同程度强化（78.26%,36/46）。结论多发性硬化脊髓受累患者临床表现复杂多样,MRI脊髓受累可呈现单一或散在多发病灶,病灶长度较少超过2个椎体节段,但病灶节段延长不能排除多发性硬化。MRI是诊断多发性硬化脊髓受累最敏感和最特异的影像学检查方法。     

多发性硬化患者颈髓扩散张量成像研究

目的 通过扩散张量成像研究多发性硬化患者常规MRI表现正常脊髓(NASC)的改变,并探讨其临床应用价值.方法 采用平面回波成像技术对13 例多发性硬化患者和13 例健康志愿者施行颈髓扩散张量成像检查,分别测量第2 ~ 5 颈椎(C2 ~ 5)水平前索、后索、侧索及灰质兴趣区的部分各向异性(FA)值及平均扩散率(MD)值,比较两组之间所存在的差异性;相关分析检验多发性硬化组患者FA值和MD 值与扩展残疾状态量表(EDSS)评分之间的关系.结果 与正常对照组相比,多发性硬化组患者C2 ~ 5 前索、侧索、后索和灰质NASC 的FA 值降低、MD 值升高(均P < 0.05);相关分析显示,FA 值与EDSS 评分呈负相关(r = - 0.328 ~ - 0.207,P = 0.001 ~ 0.035),各兴趣区MD 值与EDSS 评分呈正相关(r =0.234 ~ 0.409,P = 0.000 ~ 0.018).结论 多发性硬化患者常规MRI 表现比正常脊髓的FA 值降低,提示存在隐匿性病变.脊髓扩散张量成像对多发性硬化患者临床评价和疗效判断有一定应用价值.     

多发性硬化脊髓受累临床与MRI对照研究

目的分析多发性硬化脊髓受累患者临床表现及MRI影像学特点,并探讨其临床诊断意义。方法回顾分析2006年1月-2009年12月住院治疗的46例多发性硬化脊髓受累患者的临床资料及影像学表现。结果起病形式以急性（58．70％,27／46）或亚急性（34．78％,16／46）为主,临床主要表现为肢体瘫痪（95．65％,44／46）、感觉障碍（84．78％,39／46）和尿潴留（67．39％,31／46）。MRI受累部位以颈髓最常见（45．65％,21／46）,其次为胸髓（28．26％,13／46）,呈脊髓内单一或散在多发长T。、长T：斑片状异常信号影,病灶长度一般不超过2个椎体节段（84．78％,39／46）,个别患者（15．22％,7／46）病灶长度超过2个椎体节段;增强扫描可有不同程度强化（78．26％,36／46）。结论多发性硬化脊髓受累患者临床表现复杂多样,MRI脊髓受累可呈现单一或散在多发病灶,病灶长度较少超过2个椎体节段,但病灶节段延长不能排除多发性硬化。MRI是诊断多发性硬化脊髓受累最敏感和最特异的影像学检查方法。     

Autonomic dysfunction in multiple sclerosis

Multiple sclerosis (MS) is the leading cause of neurological disability in young adults. Since the pathophysiology of MS is characterized by dissemination in space, as well as in time, the autonomic nervous system is inevitably damaged in the course of the disease in many patients and the proportion of affected patients increases with disease duration. Autonomic dysfunction (AD) in MS is explained by lesions in regions responsible for autonomic regulation such as nuclei in the periventricular region of fourth ventricle in the brainstem as well as medullar lesions. Reports about frequency of AD in MS patients vary notably between groups. Nevertheless its impact on quality of life is substantial but, unfortunately, often overlooked. The aim of this article is to present a concise review of various symptoms and signs of autonomic system dysfunction in MS.     

Imaging of the spinal cord and brain in multiple sclerosis: a comparative study between fast flair and fast spin echo

Recent reports have suggested that fluid attenuated inversion recovery (FLAIR) is a technique superior to conventional (CSE) or fast spin echo (FSE) T2-weighted sequences in detecting intrinsic lesions both in the brain and spinal cord. We report our experience of an inversion recovery prepared FSE, which we refer to as fast FLAIR, in a comparative study of ten patients with clinically definite multiple sclerosis (MS) who underwent cervical cord and brain imaging with both FSE and fast FLAIR. The results showed that in the cerebral hemispheres fast FLAIR detected more lesions than FSE (P < 0.001). However, FSE detected more lesions than fast FLAIR in the posterior fossa (P = 0.02) and in the cord fast FLAIR was much inferior detecting only 2 of 33 lesions seen on FSE. Estimating the T2 relaxation times of lesions in each of three areas (periventricular, posterior fossa, cervical cord) showed that the T2 value of posterior fossa and cervical cord lesions was significantly lower than that of periventricular lesions, suggesting that the lesion composition is different and consequently their imaging appearances are different. In conclusion, although fast FLAIR improves the detection of MS lesions in the cerebral hemispheres, its substantially lower sensitivity in the posterior fossa and spinal cord is a potentially important limitation to its use as a tool for the diagnosis of MS and for monitoring therapies. Further studies are needed to elucidate the mechanisms underlying the loss of sensitivity. Received: 23 March 1992 Received in revised form: 1 July 1996 Accepted: 5 August 1996     

脊髓型多发性硬化的MRI表现

目的总结分析脊髓型多发性硬化的MRI表现。方法搜集经临床证实的脊髓型多发性硬化11例,均行MRI检查,对其临床及MRI资料进行回顾性分析。结果脊髓型多发性硬化的特征性MRI表现为,11例患者的病灶以颈髓多见,病变脊髓在T1WI像为低或等信号,T2WI像为高信号,病灶位于脊髓两侧和后部,病灶活动期呈斑片状或边缘强化,应用糖皮质激素试验性治疗对脊髓出现的可疑脱髓鞘病灶者有一定的帮助。结论脊髓型多发性硬化有其特征性MRI表现,MRI有助于脊髓型多发性硬化的诊断,是目前诊断脊髓型多发性硬化最敏感的影像学方法 。     

Long spinal cord lesions in a patient with pathologically proven multiple sclerosis

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system. We report the case of a 50-year-old man who presented with progressive gait ataxia. Brain magnetic resonance imaging (MRI) on fluid-attenuated inversion recovery revealed a hyperintense lesion in the right temporal white matter. The spinal cord showed a long hyperintense lesion between the vertebral levels C6 and L1 on T2-weighted MRI. Biopsied tissues from the brain lesion demonstrated features of inflammatory demyelination with preservation of astrocytes, consistent with typical MS. This is the first reported case of pathologically proven MS with longitudinally extensive spinal cord lesions.     

Multiple system atrophy masking multiple sclerosis

Multiple system atrophy (MSA) and multiple sclerosis (MS) are progressive neurological disorders with overlapping clinical signs and symptoms. However, due to the course of the disease and the age of onset both disorders are rarely differential diagnosis for each other. We here report the remarkable association of the two diseases in one patient. As MSA dominated the clinical presentation, diagnosis and therapy of MS were delayed. We discuss the clinical symptoms in our patient and highlight the features that allow to differentiate both diseases.     

Prognostic value of spinal cord MRI in multiple sclerosis patients

Multiple sclerosis [MS] is a common inflammatory, demyelinating and neurodegenerative disease of the central nervous system that affects both the brain and the spinal cord. In clinical practice, spinal cord MRI is performed far less frequently than brain MRI, mainly owing to technical limitations and time constraints. However, improvements of acquisition techniques, combined with a strong diagnosis and prognostic value, suggest an increasing use of spinal cord MRI in the near future. This review summarizes the current data from the literature on the prognostic value of spinal cord MRI in MS patients in the early and later stages of their disease. Both conventional and quantitative MRI techniques are discussed. The prognostic value of spinal cord lesions is clearly established at the onset of disease, underlining the interest of spinal cord conventional MRI at this stage. However, studies are currently lacking to affirm the prognostic role of spinal cord lesions later in the disease, and therefore the added value of regular follow-up with spinal cord MRI in addition to brain MRI. Besides, spinal cord atrophy, as measured by the loss of cervical spinal cord area, is also associated with disability progression, independently of other clinical and MRI factors including spinal cord lesions. Although potentially interesting, this measurement is not currently performed as a routine clinical procedure. Finally, other measures extracted from quantitative MRI have been established as valuable for a better understanding of the physiopathology of MS, but still remain a field of research.     

脊髓多发性硬化的MRI表现与临床对照分析

目的 探讨脊髓多发性硬化(multiple sclerosis,MS)的MRI表现及其与临床的相关性。方法 分析13例脊髓MS患者,对病变的部位、范围及病变处脊髓的形态、MR信号及病变的强化程度进行分析评价并与临床症状进行对照。结果 13例脊髓MS主要发生在颈髓,急性期局部脊髓肿胀,T1加权像病变呈等信号或边缘模糊的稍低信号。T2加权像呈高信号。活动期病灶呈斑片状、环状或弓形强化。反复发作病例、多发病灶其强化多样性,临床症状多变性。结论 脊髓MS有其特征性MRI表现,其与临床有较强的相关性,能为临床诊断和治疗提供可靠的依据。     

Increased S-nitrosothiols are associated with spinal cord injury in multiple sclerosis

Multiple sclerosis (MS) is an immune-mediated disorder associated with inflammation, demyelination and axonal damage. In search of potential biomarkers of spinal cord lesions in MS related to nitric oxide metabolites, we measured total nitrite and nitrate levels, and protein-bound nitrotyrosine and S-nitrosothiol concentrations in the serum of MS patients at different stages of the disease. Sixty-eight patients and 36 healthy volunteers were included in the study. Total nitrite and nitrate levels were augmented in relapsing-remitting MS, while increased S-nitrosothiol concentrations were found both in relapsing-remitting and secondary-progressive MS. Further analysis demonstrated that S-nitrosothiol levels were selectively increased in patients with spinal cord injury. The data suggest that high S-nitrosothiol concentration may be a potential serum biomarker for spinal cord injury in MS.     

视神经脊髓炎和多发性硬化患者颈髓扩散张量成像研究

目的通过扩散张量成像(DTI)比较视神经脊髓炎和多发性硬化患者与正常对照者常规MRI表现正常脊髓的扩散性差异,并探讨其临床应用价值。方法采用平面回波成像技术对10例视神经脊髓炎、14例多发性硬化患者和13例正常对照者进行颈髓DTI检查,分别测量颈椎C2~5水平前索、侧索、后索和灰质兴趣区的部分各向异性(FA)和平均扩散率(MD)。结果与正常对照组相比,视神经脊髓炎组患者前索、侧索、后索FA值降低(均P0.05),左侧侧索、后索、灰质MD值升高(均P≤0.05);多发性硬化组患者右侧侧索、后索FA值降低(均P0.05)。与多发性硬化患者相比,视神经脊髓炎患者侧索FA值更低,左侧侧索和右侧后索MD值更高(均P0.05)。结论 DTI可以检出视神经脊髓炎和多发性硬化患者常规MRI表现正常脊髓的水分子扩散异常,进而发现二者脊髓扩散指标的差异性,为早期诊断与鉴别诊断提供重要信息。     

多系统萎缩患者143例临床和影像学特征分析

目的 探讨多系统萎缩(MSA)不同亚型的临床和影像学特征及其相关性.方法 对143例符合1999年Gilman诊断标准的MSA患者进行临床分型和诊断分级,根据Horimoto分期对108例影像学出现异常的患者脑桥十字征和壳核裂隙征进行分析,并探讨不同临床亚型及病程与影像学异常的相关性.结果 143例MSA患者男女比例为1.3:1,其中MSA小脑萎缩型(MSA-C)93例,MSA帕金森型(MSA-P)39例,两者同时出现的即为MSA-P+C型11例;很可能的MSA 90例,可能的MSA 53例.108例MSA患者影像学出现异常,其中MSA-C型患者36例(36/76,47%)出现脑桥十字征,10例(10/76,13%)出现壳核裂隙征;MSA-P型患者6例(6/24,25%)出现脑桥十字征,6例(6/24,25%)出现壳核裂隙征.MSA-C型中病程较短的患者脑桥十字征分期较早.结论 本组病例中MSA-C型患者明显多于MSA-P型,可能与种族遗传背景有关.脑桥十字征和壳核裂隙征为MSA患者的显著影像学特征,MSA临床分型与影像学特征具有一定的相关性,其中脑桥十字征在MSA-C型较为显著,壳核裂隙征在MSA-P型较为显著.     

Significance of MRI-confirmed atrophy of the cranial spinal cord in Friedreich's ataxia

Summary The severity of Friedreich's ataxia was graded in ten patients by clinical examination and in five by use of posturography. These data were compared with neuroradiology findings. CT-confirmed infratentorial atrophy occured only in advanced cases of Friedreich's ataxia; the correlation with the clinical score was poor. On mid-sagittal MRI planes the diameters of fourth ventricle, brain stem at the level of the inferior olive and spinal cord at the levels of the foramen magnum and C3 were measured. Patients with Friedreich's ataxia had significant MRI-confirmed atrophy of the cranial spinal cord as compared with a normal, age-matched control group. This was also observed in patients with Friedreich's ataxia in the early stages. A reliable correlation between atrophy of the cranial spinal cord and the clinical score, however, could again not be found. MRI exploration of the cranial spinal cord may be recommended as an additional diagnostic marker in Friedreich's ataxia.The study was done at the Department of Neurology, University of Tübingen.Department of Neuroradiology, University of Mainz; guest at the Department of Neuroradiology, University of Tübingen, during the study.     

多系统萎缩患者52例的自主神经功能障碍与肛门括约肌肌电图的关系

目的探讨肛门括约肌肌电图(EAS-EMG)在评价多系统萎缩(MSA)患者自主神经功能障碍方面的作用。方法对52例临床诊断MSA(可能MSA 9例,拟诊MSA 43例)的患者进行自主神经功能障碍的临床特点分析(膀胱直肠功能、卧立位血压、性功能)和EAS-EMG检查,并分析两者的关系。结果44例(84.6%)患者早期出现自主神经功能障碍,但仅少数患者(38.5%)因此就诊。12例无卧立位血压异常的患者均有不同程度的尿便障碍和性功能障碍。EAS-EMG结果全部异常。尿频、尿失禁、排尿困难、夜尿增多患者的EAS-EMG指标异常均不同程度地高于无相应症状的患者,部分指标异常的差异有统计学意义。卧立位血压异常的有无并未造成EAS-EMG各项指标的显著性差异。EAS-EMG的平均时限在阳痿早期出现的患者[(13.30±0.14)m s]明显长于后期出现者[(15.31±2.50)m s,P=0.000]。结论MSA患者的自主神经功能障碍多数早于其他症状出现。卧立位血压的异常和尿失禁均非最早出现的自主神经功能障碍,对早期出现的各种难以解释的自主神经功能障碍均应加以重视。EAS-EMG对于临床怀疑MSA而无卧立位血压异常的患者更具诊断价值,有尿失禁等排尿障碍的患者更易出现EAS-EMG的异常。     

A modified protocol to improve the detection of enhancing brain and spinal cord leasions in multiple sclerosis

By detecting focal blood-brain barier (BBB) breakdown, gadolinium (GD-DTPA) contrast-enhanced T1-weighted magnetic resonance imaging (MRI) allows assessment of inflammatory activity in multiple sclerosis (MS) and provides a sensitive means of monitoring immunomodulatory therapies in exploratory trials. Serial monthly studies were performed in eight relapsing-remitting and eight secondary progressive patients to assess new and more sensitive techniques for enhanced MRI. Brain and spine imaging was carried out at 1.5-T on two occasions 24–72 h apart using a conventional imaging protocol with T1-weighted MRI at single-dose (0.1 mmol/kg) Gd-DTPA and a potentially more sensitive “modified” protocol with T1-weighted MRI at triple-dose (0.3 mmol/kg) Gd-DTPA (with addition of delay and magnetisation transfer presaturation for brain imaging). For each MRI protocol the total numbers of enhancing lesions (97 paired studies) and new enhancing lesions (81 paired studies) were assessed. The total number of enhancing lesions seen was 347/75 on conventional brain/cord MRI respectively, and 754/123 on modified brain/cord MRI. The respective numbers of new enhancing lesions were 168/40 on conventional and 276/71 on modified scans. Smaller increases were seen in the proportion of active scans using the modified protocol. Sample size calculations showed no reduction in sample sizes required for a parallel group study but a reduced sample size for crossover studies using the modified protocol: the addition of cord to brain imaging did not improve power for either trial design. A combined modified brain and cord imaging protocol markedly improves the detection of areas of focal BBB leakage in MS and many be useful in selected natural history studies. The modified brain protocol reduces sample size requirements for crossover studies but not necessarily for parallel design trials.. Received: 23 May 2000, Received in revised form: 25 September 2000, Accepted: 19 October 2000