多发性硬化的MRI特征

目的 探讨多发性硬化(MS)患者脑及脊髓的MRI特征.方法 回顾性分析110例临床确诊的MS患者的MRI检查资料.结果 MS患者脑部病灶以侧脑室旁白质多见(55.8%),其次是额叶深部白质(54.7%)、顶叶深部白质(44.2%)、脑干(25.6%)、基底节(23.3%)、丘脑(11.6%)等,灰质也可受累;病灶大小不一,形态可为斑片状、斑点状、圆形、类圆形.脊髓病灶以颈、胸髓多见,分别占75.0%和68.8%,形态可为斑片状、条片状、类圆形,脊髓灰白质可同时受累,10.0%的患者出现脊髓形态改变,如增粗、萎缩.MS患者脑及脊髓内病灶在影像学上因病程不同可表现为长T1、长T2或等T1、长T2信号.结论 MS的MRI特点主要是以脑和脊髓白质出现多个大小、形状不同的病灶.     

Remyelination in multiple sclerosis

Remyelination in multiple sclerosis (MS) lesions has been described in several studies. It depends on the presence of myelinating oligodendrocytes and a functional interaction between these myelinating cells and axons. The imaging signal of remyelination in magnetic resonance imaging or spectroscopy is not yet defined. The present review will focus on the morphological appearance of remyelinating MS lesions, their correlation with oligodendrocyte pathology, and possible markers for remyelination in imaging.     

多发性硬化的MRI与临床

报告４６例多发性硬化（ＭＳ）患者的ＭＲＩ与临床资料。其中临床确诊者３９例，实验室支持确诊者４例，临床近似确诊者３例。ＭＲＩ阳性２９例（６３．０４％）。病灶分布依序为脑室周围、大脑半球、脑干、基底节、小脑、胼胝体及视神经；本组临床定位病灶９３个，ＭＲＩ显示病灶４９个，其中３７个病灶与临床相符，１２个属亚临床病灶。结果提示ＭＲＩ对发现ＭＳ病灶虽有很高的敏感性，但ＭＳ的诊断仍需结合临床。     

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 。     

The diagnosis of primary progressive multiple sclerosis

Primary progressive multiple sclerosis (PPMS) is a rather unique form of the more common relapsing inflammatory demyelinative disease. The absence of attacks that typify relapsing forms of MS imposes special challenges for diagnosis, but also provides an opportunity to study the pathogenesis of the more progressive aspects of the disease process in isolation of confounding transient clinical events. In this review, recent advances in diagnostic approaches are considered in relationship to baseline data from a large multinational study designed to better characterize and treat this clinical phenotype. PPMS subjects with cerebral spinal fluid (CSF) findings consistent with intrathecal immunoglobulin production may have a more tissue destructive disease process than those whose CSF lacks evidence of a B-cell immunopathogenic disease component.     

Role of MRI in diagnosis and treatment of multiple sclerosis

Magnetic resonance imaging (MRI) has played a unique role in the diagnosis and management of patients with multiple sclerosis (MS). In the recent years, there have been considerable changes in the diagnostic criteria for MS as MR-based studies demonstrated its power in earlier and more accurate diagnosis of the disease. Moreover, MRI metrics have become key supportive outcome measures to evaluate the efficacy of experimental treatments in randomized, controlled trials. MRI can also be used as a prognostic tool in patients with clinically isolated syndrome. Although advanced quantitative MRI measures such as magnetization transfer, spectroscopy, and diffusion imaging have added much more to our knowledge about pathogenesis and natural history of the disease but their cost, availability, complexity and lack of validation have limited their use in routine clinical practice. Conventional MR techniques including proton density, T1/T2-weighted images and fluid-attenuated inversion recovery sequences are now accepted in standard protocols for diagnosis and treatment outcome measures in clinical trials for MS.     

Pathological abnormalities in the normal-appearing white matter in multiple sclerosis

In established cases of multiple sclerosis (MS), the normal-appearing white matter (NAWM), as defined for magnetic resonance imaging (MRI), is abnormal in the majority of cases. The clinical significance of these NAWM abnormalities is the subject of debate, but there is strong correlation with degree and progression of disability. New lesions form in NAWM before blood-brain barrier breakdown, as evidenced by gadolinium enhancement. The pathological basis of these neuroimaging abnormalities is largely unknown. Definitive pathological studies on the NAWM are few and are often based on small numbers of samples and of cases. Despite a variety of MS NAWM pathological studies, major research questions, of importance to our understanding of basic pathogenetic mechanisms and consequent rational therapies, remain unanswered. These relate to the frequency and extent of oligodendrocyte/myelin and axonal abnormalities in MS NAWM, and to the cellular basis of very early MS lesions detected by neuroimaging. In a pilot study of MS NAWM, microglial activation was demonstrated in 9 of 10 MS cases. We are currently testing the hypothesis that microglial activation, as defined by altered phenotype and HLA-DR positivity, will act as a marker for oligodendrocyte/myelin and axonal pathology in MS NAWM.     

Diagnosis of multiple sclerosis

There is no pathognomonic symptom, sign, or paraclinical result that provides an unfailingly accurate diagnosis of multiple sclerosis (MS), and hence, MS remains largely a clinical diagnosis. However, being a clinical diagnosis does not mean that the diagnosis of MS is one of exclusion. Increasingly sophisticated guidelines and objective paraclinical findings are generally sufficient to allow the clinician to confirm or rule out the diagnosis with confidence. This article presents the most recent guidelines for using clinical, radiological, and other paraclinical information and the red flags that should alert the clinician to investigate other diagnostic possibilities.     

Disclosing the diagnosis of multiple sclerosis

Context The question of how best to disclose to patients the diagnosis of serious and/or incurable neurological diseases has been much explored, but that of when has received little rigorous study. The present study investigates this question in relation to multiple sclerosis (MS), a disease marked by its incurability, unpredictability and predilection for young adults. Objectives We aimed to ascertain the preferences of Greek MS sufferers concerning when they should ideally be informed they have the disease, and their preferences and reactions regarding disclosure of the diagnosis. Design, setting and patients 1200 Greek MS patients,members of the MS Society, were asked to complete a questionnaire regarding their experience of and attitudes towards receiving the diagnosis. Design, setting and patients 1200 Greek MS patients,members of the MS Society, were asked to complete a questionnaire regarding their experience of and attitudes towards receiving the diagnosis. Results 657 patients (55 %) responded. 91% favoured learning the diagnosis immediately, but only 44% had had this experience: 29% had been informed within 1–3 years, and 27% later. Interestingly, however, a significant minority (9 %) suggested a possible preference for delayed delivery of diagnosis and 23.2% stated that concealing the diagnosis would not lead to loss of confidence in their doctor. Conclusion This study—the largest of its kind—provides objective data supporting prompt disclosure of diagnosis as the clearly–expressed preference amongst most patients. Interestingly, however, the results also reemphasise the importance of a difficult medical art: attempting to judge whether an individual patient is one (of the 91%) preferring immediate disclosure—or of the nearly 1–in–10 (9%) who may not.     

多发性硬化诊断与治疗进展

随着越来越多的疾病修正药物应用于临床,多发性硬化的治疗已向前迈出了可喜的一步,同时也为患者提供了更多的选择。本文重点介绍多发性硬化当前临床研究热点,包括临床表现、辅助检查、诊断标准和治疗进展等内容。。     

Magnetic resonance imaging and cognitive functioning in multiple sclerosis

Summary The relationship between cognitive impairment in multiple sclerosis and brain lesions seen on magnetic resonance imaging (MRI) was studied. Three groups of 11 patients with multiple sclerosis, matched for the variables of disability, duration of illness, age and sex, were included. On the basis of neuropsychological testing, the groups were seen to differ in their level of cognitive impairment. The first group showed no cognitive impairment, the second group a moderate, and the third group a serious cognitive impairment. These differences between the groups were reflected by MRI, which revealed more abnormalities in the groups with cognitive impairment compared with the group with normal cognitive function. However, by MRI it was not possible to distinguish between the groups with moderate and that with serious cognitive impairment.     

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。     

颅脑多发性硬化的临床及MRI诊断

目的 探讨多发性硬化(MS)的临床及MRJ特征,提高对多发性硬化的认识及诊断水平.方法 对20例颅脑MS患者临床资料、病灶部位、形态、MR信号及强化特点、胼胝体改变进行回顾性分析评价.结果 MS以青、中年女性稍多见,急性、亚急性起病,多以视觉障碍或肢体感觉、运动障碍为首发症状.视觉诱发电位大多数异常.MRJ检查18例发现病灶,敏感性90%(18/20).病灶以双侧侧脑室旁、额叶皮层及皮层下、半卵圆中心多发.病灶大、小不等,多数为圆形、卵圆形."直角脱髓鞘征"及"白质变脏征"是两个较为典型的征象.T1WI上表现为等、低信号,T2WI及Flair序列上表现为高信号,Flair序列显示病灶更清晰.增强扫描病灶可呈结节状强化、环状强化、弧形强化或无强化.结论 MS的临床及MRI表现具有一定特征.MRI有助于脑部MS的诊断及鉴别诊断,是诊断MS最敏感的成像方法.     

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.     

EFNS guidelines on the use of neuroimaging in the management of multiple sclerosis

Magnetic resonance (MR)-based techniques are widely used for the assessment of patients with suspected and definite multiple sclerosis (MS). However, despite the publication of several position papers, which attempted to define the utility of MR techniques in the management of MS, their application in everyday clinical practice is still suboptimal. This is probably related, not only, to the fact that the majority of published guidelines focused on the optimization of MR technology in clinical trials, but also to the continuing development of modern, quantitative MR-based techniques, that have not as yet entered the clinical arena. The present report summarizes the conclusions of the 'EFNS Expert Panel of Neuroimaging of MS' on the application of conventional and non-conventional MR techniques to the clinical management of patients with MS. These guidelines are intended to assist in the use of conventional MRI for the diagnosis and longitudinal monitoring of patients with MS. In addition, they should provide a foundation for the development of more widespread but rational clinical applications of non-conventional MR-based techniques in studies of MS patients.     

Regional specificity of magnetization transfer imaging in multiple sclerosis.

BACKGROUND:The goal of this study was to develop and validate a method for generation of regional magnetization transfer ratio (MTR). We also studied the topography of MTR changes in multiple sclerosis (MS) and in normal controls (NC), and preliminarily examined the clinical usefulness of this method.METHODS:We examined 45 patients with MS (relapsing remitting [RR] = 28 and secondary progressive[SP] = 17] and 19 NC. Mean disease duration was 14.3 years and median Expanded Disability Status Scale was 3.0. Regions of the brain were determined using semiautomated brain region extraction (SABRE). Twenty-six regional masks were automatically applied to MTR maps that were further split into gray matter (GM) and white matter (WM)compartments.RESULTS:Mean MTR from 12 SABRE regions differed significantly between MS patients and NC. For WM, all regional mean MTRs differed significantly between RR, SP, and NC participants(P < .001). In regression analysis, only 3 regions remained significantly different when corrected for total T2-LV. The regression model predicting disability selected GM mean MTR of the right medial inferior frontal region (P = .031).CONCLUSIONS:The study results showed that this regional MTR approach is reproducible, reliable and clinically relevant. MTI changes occur selectively in specific sub-regions.     

多发性硬化的临床诊断

多发性硬化是中枢神经系统炎性脱髓鞘疾病.目前病因未阐明,发病机制与自身免疫性反应有关.临床表现的显著特点为时间多发性(多次发作)和空间多发性(多个病变部位).常用辅助检查手段包括头部或脊髓MRI、脑脊液免疫学、诱发电位等.最新的诊断标准为2010年McDonald标准.目前认为,多发性硬化与视神经脊髓炎在临床、病理、影像等方面不同,由于该病临床表现的多样性及缺乏特异性生物学诊断指标,其诊断仍然是临床医师面临的最大难题之一.     

Choreoathetosis in multiple sclerosis

Extrapyramidal symptoms are extremely rare in multiple sclerosis. We examined a patient with a ten year history of multiple sclerosis, who developed choreoathetoid movements of both upper extremities. To our knowledge this is the second case report of this association.     

A case of multiple sclerosis triggered by organic solvents

The neurotoxicity of organic solvents has long been recognized. Some are used as anesthetic agents, others in various industries. Their acute effect has been well documented since the nineteenth century, but more recently they have become notorious as the cause of addiction to glue sniffing. They may alter the immune system by causing lymphopenia, impairing phagocytosis and decreasing the level of serum complement, as well as altering the impermeability of the blood-brain barrier and leading to the appearance of white matter lesions in the brain. The following case study explores the possible role of organic solvents in the pathogenesis of multiple sclerosis.     

Communicating the diagnosis of multiple sclerosis

In the frame of current treatment options for multiple sclerosis (MS) and recommendations for early intervention, we investigated the practice and attitudes of neurologists towards MS-diagnosis communication in Greece. We constructed and sent out a 22-item questionnaire to neurologists practising in different employment settings and geographic regions in Greece. Overall, 217 (37.41 %) of 580 neurologists replied. The vast majority (94.9 %) informs the patient of a definite MS diagnosis, and 73.6 % do so immediately, but only 41.7 % use the term multiple sclerosis. Furthermore, neurologists strongly agreed that timing of diagnosis communication depends to a large extent on the individual patient’s personality (62.5 %) and mental state (52.3 %). Most neurologists (78.7 %) inform relatives about the diagnosis, but only in the presence of the patient. In cases where disclosure was delayed, 59.5 % noted that they did not observe any changes as regards the trust or confidence of their patients towards them. Most neurologists also noted that education level (72 %) and mental state (51.9 %), at the time of disclosure influenced patients who did not fully understand the meaning of their diagnosis. This survey provided some useful new findings with respect to MS diagnosis communication; however, the questions of how and possibly how much to communicate warrant further cross-cultural investigation.