多发性硬化20例临床分析

目的：探讨多发性硬化的临床症状及MRI对其诊断的价值和意义。方法：综合分析20例多发性硬化患者一般资料、MRI、脑脊液检查及治疗转归。结果：多发性硬化好发于10－40岁，女性多见，起病形式及临床症状多样，病程呈复发与缓解，MRI对多发性硬化病灶的发现率可达100％。糖皮质激素治疗有效，但随着病程的延长，复发次数的增多，激素治疗欠佳。结论：根据临床特点、影像学检查、脑脊液及神经电生理检查可提高多发性硬化的确诊率。     

多发性硬化35例临床分析

目的 探讨多发性硬化（MS）的临床特点。方法 综合分析35例MS患者的一般临床资料、病变部位、重要辅助检查及治疗转归。结果 MS多见于青壮年女性,病程多缓解与复发、上呼吸道感染、劳累紧张为其主要诱因,脑脊液显示免疫活性增高,视神经、脊髓受累多见,电生理、免疫学及影像学检查有助诊断,糖皮质激素治疗有效。结论 根据临床特点、综合神经电生理、脑脊液免疫学及影像学检查能明显提高临床确诊率。     

脑脊液寡克隆区带和核磁共振检查对多发性硬化的临床诊断价值

目的 :探讨脑脊液寡克隆区带CSFOB和头颅MRI的变化对诊断多发性硬化 (MS)的临床价值。方法 :对 2 5例怀疑多发性硬化 (MS)的患者进行脑脊液的寡克隆区带和头颅MRI的检查 ,研究两者和MS间的关系。结果 :确诊多发性硬化的患者均有脑脊液的寡克隆带和头颅核磁共振 (头MRI)异常。结论 :脑脊液寡克隆带和头MRI两者结合对诊断发硬化 (MS)有重要临床价值。     

多发性硬化42例临床分析

目的 探讨多发性硬化(MS)的临床特点、诊断和治疗.方法 分析42例多发性硬化患者的一般资料、病变部位、主要症状、重要辅助检查及其治疗方法和效果.结果 42例MS患者中青壮年女性多见,以急性、亚急性起病为主,首发症状以肢体无力最常见,病变部位以脊髓和视神经最常见,实验室检查以脑脊液蛋白水平和IgG指数增高常见.MRI异常率高达85.96%.结论 MS是一种临床表现复杂、累及中枢神经系统白质,多部位、多时相的自身免疫性疾病.根据临床特点、神经电生理、脑脊液免疫学及磁共振成像检查能提高临床确诊率,激素治疗对大多数病人有效.     

多发性硬化66例临床分析

目的 探讨多发性化（ＭＳ）的临床特点。方法 综合分析６６例多发性硬化患者的一般临床资料,病变部位,重要辅助检查及治疗转归。结果 ＭＳ多见于青壮年女性,起病较急,病程多缓解复发,上呼吸道感染及其主要诱因这一脊、视神经受累多见,电生理,免疫学及影像学检查有助诊断,糖皮质激素治疗有效。结论 根据临床特点,综合神经电生理、脑脊液免疫学及影像学检查能大大提高临床确诊率,其中磁共振成像意义较大。     

以周围神经病变症状起病的多发性硬化15例临床分析

目的探讨以周围神经病变首发的多发性硬化临床特点和发病机制。方法回顾性分析15例首发周围神经病变症状的多发性硬化的临床特点、脑脊液生化常规、电生理检查、头颅或脊髓MRI特点。结果 15例均为首发周围神经病变症状的多发性硬化患者,电生理提示广泛周围神经病损,既有脱髓鞘改变,又有轴突病变,MRI提示均有中枢神经多发性的脱髓鞘病变。结论周围神经病变症状可以为多发性硬化的首发症状。     

连续每周MRI对多发性硬化活动性测定的敏感性

每周连续脑MRI有助于多发性硬化自然病史的 了解,增加MRI检查次数有可能增加对多发性硬化活动性损害治疗效果监测的敏感性。 选择临床上明确的复发—缓解及加重型的多发性硬化患者11人中仅有3人首次钆加强MRI扫描有     

中枢神经系统脱髓鞘病变与复发-缓解型多发性硬化

参照Poser诊断标准．对1999年1月～2004年1月问经临床、头部和脊髓MRI、脑脊液IgG合成率和VEP检查后，诊断为中枢神经系统脱髓鞘病的53例患者．经5年临床和MRI随访．有36例符合缓解-复发型多发性硬化(MS)的临床确诊和实验室支持诊断标准，现将病情演变及诊断经过进行分析．以探讨复发-缓解型MS的早期诊断，为预防复发、控制病情进展提供治疗方面依据。     

多发性硬化的临床特点分析

目的探讨多发性硬化(MS)的临床表现特点。方法回顾性分析2007-01—2010-06于我院确诊的68例多发性硬化病例,总结其一般资料、临床表现、病变部位、重要辅助检查及治疗转归等。结果 68例患者中,首发症状以肢体无力(28例,41.2%)最常见;肢体无力、感觉障碍、视觉损害是MS患者最常见的症状;MS好发于青壮年,以急性和亚急性起病为主;视、听、体感诱发电位(VEP、BAEP、SEP)有助于发现亚临床病变;脑脊液(CSF)检查可有异常;磁共振(MRI)检查阳性率高;临床定位以大脑半球、脊髓和视神经受累最多见。糖皮质激素及免疫球蛋白治疗有效。结论根据临床特点,结合神经电生理脑脊液及影像学等检查能大大提高MS的临床确诊率。     

多发性硬化临床特征分析及治疗

目的 探讨多发性硬化(multiple sclerosis,MS) 的临床特征及治疗方法.方法 回顾分析42例MS患者的临床资料.结果 MS 好发于青年女性(29例),常见症状为肢体无力(29例)、视力障碍(22例)和感觉障碍(21例).病变累及大脑半球30例,视神经28例,脊髓23例,脑干9例,小脑4例,所有病例MRI检查均有阳性改变,CSF检查IgG鞘内合成率阳性16例,寡克隆区带阳性19例,抗髓鞘碱性蛋白(MBP)抗体阳性15例.视觉诱发电位(VEP)异常28例,脑干听觉诱发电位(BAEP)阳性16例,体感诱发电位(SEP)异常20例,急性期甲强龙冲击治疗患者临床症状均有改善,缓慢激素减量及小剂量激素长期维持治疗,随访至2008-01仅有7例复发.结论 结合临床特点、MRI、脑脊液免疫学、诱发电位检查能明显提高临床确诊率.急性期甲强龙冲击治疗可改善临床症状,缩短急性期病程,缓慢激素减量及小剂量激素长期维持可能有助于降低复发率.     

Clinical, magnetic resonance imaging, cerebrospinal fluid and electrophysiological characteristics of the earliest multiple sclerosis

OBJECTIVES: The vast majority of clinically isolated syndrome (CIS) patients with at least two silent brain MRI lesions progress to multiple sclerosis (MS) as early as after 2 years meaning that they actually have MS, the earliest MS. Effective therapy with interferon beta preparations in patients with the earliest MS demands early and accurate diagnosis of the disease. PATIENTS AND METHODS: In order to find the differentiating clinical and paraclinical characteristics of patients with the earliest MS we compared clinical, MRI, CSF and evoked potential findings in patients with the earliest MS and patients with relapsing-remitting (RR) MS. Retrospective analysis included 149 patients (103 women), among them 40 patients with the earliest MS and 95 patients with RR MS. RESULTS: Patients with the earliest MS had more often predominant afferent symptoms (p=0.023) but less often predominant cerebellar (p=0.033) and efferent symptoms (p=0.012) than patients with RR MS. They were less likely to fulfill the Barkhof brain MRI criteria (p=0.050) and had less often prolonged latencies of visual evoked potentials (VEP) (p=0.006) than patients with RR MS. On the other hand they were more likely to have elevated CSF cells (p=0.010) than patients with RR MS and had as often present CSF oligoclonal bands (p=0.112). CONCLUSION: The differentiating characteristics of patients with the earliest MS are predominance of afferent symptoms, less brain MRI dissemination and more frequently normal VEP, but on the other hand abnormal CSF findings with elevated CSF cells and positive oligoclonal bands.     

Acute disseminated encephalomyelitis that meets modified McDonald criteria for dissemination in space is associated with a high probability of conversion to multiple sclerosis in Taiwanese patients

Background: Patients with acute disseminated encephalomyelitis (ADEM) may relapse and some may ultimately convert to multiple sclerosis (MS); however, no criteria that can predict MS conversion are available to date. Our aim was to describe the clinical and magnetic resonance imaging (MRI) features of patients with an initial ADEM attack and evaluate which MRI criteria can predict conversion to MS. Methods: We retrospectively reviewed the records of 36 patients diagnosed with ADEM. We determined clinical signs/symptoms, examined the cerebrospinal fluid (CSF), and performed brain MRI scans and compared the findings between patients who did and did not convert to MS. Results: Clinical signs/symptoms, and CSF analysis show no significant difference between the two groups. The rate of conversion to MS from ADEM in Taiwanese patients is low (11%) after a mean follow‐up period of 28.36 months. Modified McDonald criteria were fulfilled in 19/36 patients: 21% (4/19) of those patients developed MS according to Poser criteria subsequently. Of the other patients (17/36) who did not fulfill these criteria, none converted to MS. (log rank test; P = 0.027). Conclusions: It is difficult to predict from initial clinical presentations to address which patients with ADEM will convert to MS. Patients with ADEM whose brain MRI findings met the modified McDonald criteria may have clinically isolated syndrome because they have a significantly higher probability of conversion to MS. In contrast, patients whose brain MRI findings did not meeting these criteria may be considered as having classic ADEM because they have a lower probability of conversion to MS.     

Relapsing-remitting inflammatory disease of the central nervous system with normal MRI: multiple sclerosis or phenocopy in a series of 15 patients

Multiple sclerosis is a demyelinating disease of central nervous system. Although many sub-types and clinical forms are identified, diagnosis is clearly related to the detection of MS lesions on brain MRI. We report data of 15 patients admitted in Nice for suspicion of MS after clinical relapsing-remitting or progressive symptoms. Extensive screening tests (i.e blood sample, CSF, MRI, spectroscopy) were performed at onset and at each relapse. All patients had normal-appearing white matter on spinal cord and brain MRI. Nevertheless, 11 patients can be considered as MS according to McDonald criteria.     

Diagnostic problems in “clinically definite” multiple sclerosis patients with normal CSF and multiple MRI abnormalities

Among patients who underwent cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) examination during a 5-year period, we found 18 patients at the multiple sclerosis center of the University of Rome and clinically definite multiple sclerosis, MRI white matter abnormalities, normal CSF examination, a disease duration of at least 1 year and an upper age of < 45 years at onset These patients were examined again with a variety of tests screening for different diseases mimicking multiple sclerosis. Alternative diagnoses reached after laboratory tests were: Lyme disease, two cases of vasculitis, mitochondrial encephalomyopathy, multiple ischemic lesions caused by atrial septum aneurysm and olivopontocerebellar atrophy. Hence, six of these 18 patients had a final diagnosis other than multiple sclerosis, while 12 remained with a final diagnosis of “MS with normal CSF”. Our study suggests that in patients with a clinical picture of multiple sclerosis and disseminated white matter MRI lesions but no CSF abnormalities, the classical clinical criteria may not be sufficiently specific, unless confirmed by a very prolonged clinical history with repeated MRI.     

视神经脊髓炎——是否是多发性硬化的一个亚型？

目的 探讨视神经脊髓炎与多发性硬化的方法。方法 对１３例视神经脊髓炎患者的临床表现,脑脊液,电生理学及影像学检查结果进行分析。结果 ８５％的视神经脊髓炎患者有多次（平均３．１次）的缓解复发,复发时症状仅限于视神经和／或脊髓,其脑脊液中寡克隆区带阳性率３３％,脑干诱发电位异常率８％,头颅ＣＴ和ＭＲＩ未发现异常。结论 视神经脊髓炎和多发性硬化之间有所不同,支持视神经脊髓炎是一个单独的疾病单元学说。     

Intravenous immunoglobulin treatment of multiple sclerosis and its animal model, experimental autoimmune encephalomyelitis

Intravenous immunoglobulin (IVIG) is an established treatment of immune-mediated demyelinating neuropathy. Since IVIG possesses multiple immunomodulatory and anti-inflammatory properties, IVIG therapy may represent a way of interfering with the disease process in multiple sclerosis (MS). In the MS animal model experimental autoimmune encephalomyelitis (EAE), infusions of IVIG significantly reduced disease symptoms as well as the underlying CNS pathology. IVIG was only effective in EAE when administered in a prophylactic treatment protocol, since IVIG infusions during the established phase of EAE did not alter the disease course or the degree of inflammation found in the central nervous system. IVIG also has the potential to act through myelin repair mechanisms as evidenced by work done in the Theilers murine encephalomyelitis virus model of demyelination. Together these observations have led to certain expectations for IVIG as a treatment for MS, and have resulted in various clinical trials. Several controlled trials report beneficial effects of IVIG on relapse rate, new MRI lesions, and disease progression in relapsing-remitting MS, while a remyelinating effect of IVIG has not been documented. IVIG is, therefore, presently regarded as a second-line therapy of MS.     

Clinical characteristics of patients with late-onset multiple sclerosis

We evaluated clinical presentation, cerebrospinal fluid (CSF), and magnetic resonance imaging (MRI) in patients with late-onset multiple sclerosis (LOMS). Fifty-two patients with definitive multiple sclerosis (MS) diagnosed after the age of 50 years were identified between 1991 and 2002. Data pertaining to clinical characteristics, CSF analysis, and cerebral and spinal MRI were compared with those of 52 young-onset MS (YOMS) patients matched for sex and disease duration. Mean age at the time of diagnosis was 57 years in the LOMS group - the oldest patient was 82 - and 29 years in the YOMS group. Motor symptoms were significantly more often present in the LOMS than in patients with YOMS (90 % vs. 67 %, p = 0.014). Visual symptoms, residual signs of optic neuritis, and dysarthria were less frequent for LOMS. Sensory symptoms, ataxia, oculomotor symptoms, cognitive disorder, or fatigue did not differ between both groups. The majority of LOMS patients (83 %) had a primary progressive disease course, whereas 94 % of the YOMS group had a relapsing-remitting course. MRI showed typical multifocal supratentorial (LOMS vs. YOMS: 96 % vs. 98 %) and infratentorial (44 % vs. 62 %) lesions without significant group differences. Of particular interest, spinal lesions were more common (81 %) in LOMS compared to YOMS (48 %, p = 0.024), and cerebellar lesions were less frequent in the LOMS group (11 % vs. 44 %, p = 0.001). Gadolinium-enhanced lesions were initially present in less LOMS patients (15 %) than in YOMS (63 %, p < 0.001). CSF analysis revealed pleocytosis less frequently in LOMS (34 %) compared to YOMS (67 %, p = 0.006) but oligoclonal banding occurred without in both groups without differences. YOMS patients responded to corticosteroids (93 %) to a significantly greater degree than LOMS patients (73 %; p = 0.004). For individuals who develop LOMS, a primary progressive course is frequent, with motor symptoms as the prominent feature. Vigilance is necessary to recognise MS in this population because of its unusual presentation.     

Clinically isolated syndromes suggestive of multiple sclerosis, part I: natural history, pathogenesis, diagnosis, and prognosis

In 85% of young adults with multiple sclerosis (MS), onset is a subacute clinically isolated syndrome (CIS) of the optic nerves, brainstem, or spinal cord. Methods of assessing the prognosis for patients who present with a CIS have been sought, because only 30-70% of patients with a CIS develop MS. When clinically silent brain lesions are seen on MRI, the likelihood of developing MS is high. MS can be diagnosed within 3 months of CIS presentation with certain MRI and CSF criteria. Disability from MS is less likely in patients with a CIS of optic neuritis or sensory symptoms only, few or no MRI lesions, a long period to the first relapse, and no disability after the first 5 years. Development of more reliable prognostic markers will enable new treatments to be targeted for those who are most likely to benefit. We encourage continued clinical and laboratory assessment of patients with a CIS.     

Diagnostic value of magnetic resonance imaging in multiple sclerosis]

Brain MRI findings in 16 clinical cases of MS were compatible with multiple lesions in the brains in 14 cases. Cerebral lesions were detected in 12 cases without symptoms or signs referable to the cerebral hemispheres. When compared with t*he findings CT scannings, evoked potentials, and oligoclonal bands in the CSF, MRI findings appeared to be the most effective means for confirming the diagnosis of MS, but they would not indicated the course, the times of relapse and the activity of the disease process.     

Concentric sclerosis: imaging diagnosis and clinical analysis of 3 cases

Baló's Concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis (MS). The typical magnetic resonance imaging (MRI) changes associated with BCS consist of concentric rings or onions' cross-section on T1-weighted (T1W) images. Because MRI reveals pathological changes consistent with autopsy in the focus of BCS, it plays an important role in the before-death diagnosis of BCS. We report three cases of BCS diagnosed antemortem on the basis of the typical concentric rings pattern on MRI and on the basis of clinical findings and cerebrospinal fluid (CSF) examination. BCS often occurs in the prime of life, acutely or subacutely. Then come cerebral multifocal symptoms and signs. We find that BCS is not always an acute and irreversible pathological process as described in the past.