A case of acute multiple sclerosis mimicking tumor on the neuro-imaging studies]

A 31-year-old woman with a history of suspected optic neuritis was admitted with weakness of right-sided extremities and confusion. On admission general physical examination revealed no abnormality. Neurological examination revealed central facial palsy, mild hemiparesis and hemisensory deficit on the right side. Cerebrospinal fluid analysis showed monocytosis of 29 cells, protein of 82 mg/dl, glucose of 62 mg/dl and myelin basic protein of 6.8 ng/ml. No oligoclonal bands were seen. X-ray computed tomographic (CT) scans showed multiple homogeneous contrast-enhancing lesions in the white matter of bilateral parietal lobes without distinct edema or mass effect. Follow-up CT scans showed ring-enhanced lesions. Magnetic resonance image (MRI) showed multiple Gadolinium-enhanced lesions. Additionally, 123I-IMP SPECT [Gamma view-SPCT 2000 H-20 (Hitachi Co.)] was performed at 30 minutes and 5 hours after intravenous administration of IMP (3 mCi). It showed high IMP uptake corresponding to the CT and MRI lesions. Cerebral angiography was considered to be normal. Other laboratory findings were within normal limits. A biopsy was performed. Histological examination showed spongiosis, gliosis and perivascular cuffing. The histological diagnosis was acute demyelinating disease. After therapy with methylprednisolone, she improved gradually. Enhanced lesions in CT and MRI may correspond to active demyelination at acute MS. High uptake of SPECT may also appear in acute stage, although it has not been reported. We should perform neuro-imaging studies including SPECT on acute MS.     

MRI findings of multiple sclerosis with acute transverse myelopathy

We classified clinically definite multiple sclerosis (MS) patients who underwent brain MRI with superconductive magnet into 2 subgroups: group A, consisting of 14 patients who had shown acute transverse myelopathy (ATM) during the course of illness; and group B, 35 patients without ATM, and the same neuroradiologist, blinded to clinical profiles of the patients, investigated the MR scans. We analyzed some parameters such as distribution, size, shape and number of the lesions. Although the total number of lesions were similar in each group, and the number of small size or patchy shape solitary lesions were not different, the moderate, large, ovoid or confluent lesions were lower in number in group A, compared with group B. The degree of cerebral white matter lesions and periventricular lesions were highier in group B. Brainstem lesions were significantly less common in group A. These results show characteristics differences in MR-detected, possibly pathological, changes between these 2 groups, and support our previous report that group A may constitute a distinct subgroup in patients with MS.     

Diagnostic brain MRI findings in primary progressive multiple sclerosis

The clinical course of multiple sclerosis can be classified as relapsing from onset (relapsing-remitting), or progressive from onset (primary progressive - PPMS). These clinical phenotypes have been based on historical and clinical observations. It has been reported that PPMS patients tend to have quantitatively less MRI activity and disease burden. We evaluated the sensitivity and diagnostic value of conventional brain MRI scan in 143 PPMS patients. Brain MRIs were blindly evaluated to determine if they satisfied Paty and/or Fazekas diagnostic criteria. Patients were divided into those with typical, atypical or normal scans. They satisfied brain MRI criteria in 92% cases. Findings included: 131 typical, four atypical, and eight normal scans. All 12 non-typical scans' subjects had spinal onset; spinal MRI scans were positive in four of seven cases. Sex, age of onset, site and number of symptoms involved at onset among those groups were not significantly different but accumulation of disability had a tendency to be slower in these few individuals with normal or atypical head MRI's. Although there may be quantitative differences in lesion activity/burden, MRI scanning in PPMS unexpectedly has diagnostic sensitivity very similar to that seen in RRMS. A normal brain MRI is unusual in PPMS patients.     

A brain MRI study of different types of chronic-progressive multiple sclerosis

Introduction –This study was performed to define the pattern of brain magnetic resonance imaging (MRI) abnormalities in chronic-progressive MS (MS). Material and methods — Brain MRIs were obtained for 17 patients with secondary progressive MS (SPMS), 14 with primary progressive MS (PPMS) and 5 with "transitional" progressive MS (TPMS). Results — Total lesion loads were different for the three groups of patients (p<0.01). At post-hoc analysis, there was no difference between patients with TPMS and those with PPMS, while both these groups had lesion loads lower than those of patients with SPMS. Patients with PPMS with clinical signs indicating involvement of both brain and spinal cord had greater total lesion loads than those with clinical isolated spinal cord involvement (p<0.05). Conclusion — These data indicate that brain MRI patterns of abnormalities are related to the clinical manifestations in patients with chronic-progressive MS.     

A follow-up study of very low field MRI findings and clinical course in multiple sclerosis

Seventy-three consecutive patients with definite multiple sclerosis (MS) were examined with very low field (0.17 and 0.02 tesla) magnetic resonance imaging (MRI) of the brain. Fifty-seven patients were examined a second time after 6 months, 41 patients a third time after 1 year, and 25 patients a fourth time after 2 years. The initial finding was abnormal in 57/73 patients (78%). The number of lesions increased with age, duration of disease, and increasing disability. Plaques were more numerous in progressive than in remitting MS. Of the patients with abnormal MRI 65% had more than 3 lesions, and in 82% lesions were smaller than 2.5 cm in diameter. After follow-ups of 1 year and 2 years the size of MRI lesions remained unchanged in 55% and 64%, respectively. Unchanged plaque numbers were found in 72% and 60%. Unchanged MRI was most common among patients with remitting MS who were in a stable phase. Decrease in size and disappearance of plaques correlated well with clinical remissions. Increase in size was rare even during relapses. New plaques could appear during all phases of clinical course although they reflected better a relapse of remitting or progressive disease.     

Nocardia brain abscess mimicking high-grade necrotic tumor on perfusion MRI

Differentiating a pyogenic cerebral abscess from a cystic brain tumor can be a challenge when using morphological and functional imaging techniques. Several studies on MRI perfusion-weighted imaging (PWI) have demonstrated that enhancing abscess capsules have lower cerebral blood volume ratios (rCBV) than the enhancing rims of necrotic tumors. We report a 67-year-old male with a Nocardia cerebral abscess showing restricted diffusion in the necrotic center, but high values for rCBV in the enhancing capsule on PWI, therefore mimicking a high-grade necrotic tumor. Differential diagnosis between cerebral abscesses and necrotic tumors is greatly improved by the adjunct of diffusion-weighted imaging (DWI) and PWI to the morphological magnetic resonance findings; yet there is still overlap. That an abscess may show increased rCBV along the capsule, therefore mimicking a hypervascular brain tumor on PWI, should be considered when attempting a radiological diagnosis of a ring-enhancing brain lesion.     

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.     

多发性硬化患者的MRI及多种诱发电位研究

目的探讨磁共振成像(MRI)和诱发电位(EPs)在诊断多发性硬化中的价值。方法对68例多发性硬化患者的头颅MRI、脑干听觉诱发电位、视觉诱发电位以及体感诱发电位等指标进行回顾性分析和比较。结果多发性硬化患者的头颅MRI、脑干听觉诱发电位、视觉诱发电位以及体感诱发电位的异常率分别为91.2%(62/68)、80.9%(55/68)、82.4%(56/68)和77.9%(53/68),且均发现多发性硬化的亚临床病灶;两项或多项联合检查的异常率较单项检查的异常率增高,差异有统计学意义(P<0.01)。结论头颅MRI和诱发电位检查有助于临床早期确诊多发性硬化,联合应用可使其敏感性提高。     

Fatigue in multiple sclerosis: cross-sectional correlation with brain MRI findings in 71 patients.

Fatigue is an unexplained but common and disabling symptom in MS. We assessed fatigue in 71 patients with MS and identified MS-fatigue (MSF) and MS-nonfatigue (MSNF) groups. Fatigue severity did not correlate with regional or global MRI plaque load or atrophy assessed by conventional sequences. No significant differences were noted in any MRI measures between MSF and MSNF groups. We suggest that brain MRI disease topography or severity does not explain fatigue in MS and that fatigue is likely due to mechanisms poorly characterized by conventional MRI.     

Normal brain activation in hemiatrophy due to multiple sclerosis: a functional MRI case study

Evidence from recent functional magnetic resonance imaging studies suggests that adaptive cortical changes ('plasticity') could participate in the maintenance of function in multiple sclerosis (MS). Here, we addressed the impact of brain atrophy on the pattern of cerebral activation in an MS patient with a relapsing-remitting course. This patient showed mildly disabling hemiparesis of the left side (EDSS 2.0), and corresponding brain hemiatrophy (15% volume reduction) of the right hemisphere. The clinical syndrome was considered to result from a lesion in the corona radiata involving corticospinal fibers. Motor-evoked potential recordings confirmed substantial axonal damage to the pyramidal tract arising from that hemisphere. Irrespective of these asymmetries, normal brain activation was found for hand and foot movements for both brain sides. This demonstrates that brain atrophy itself does not necessarily induce cortical adaptive changes, even if mild disability is present. On the other hand, significantly disabling distinct clinical syndromes e.g. arising from spinal cord lesions may evoke cortical changes irrespective of brain atrophy. This issue has to be studied in longitudinal investigations.     

MR imaging of multiple sclerosis simulating brain tumor

Multiple sclerosis (MS) may sometimes present as a mass lesion that is clinically and radiologically indistinguishable from brain tumor. The initial recognition of such cases is essential to avoid a surgical intervention and supplementary treatment. Sometimes areas adjacent to a tumor may present similar histopathological features with non-neoplastic demyelinating lesions. Thus, the proper preparation of the specimen is the key for correct pathological diagnosis. We report a case of MS with large cranial involvement showing a mass effect. The operative procedure associated with medical treatment was performed, and a good result was obtained. Our case presentation and others in the literature suggest strategies for detecting MS presenting as a mass lesion.     

Age-related gadolinium-enhancement of MRI brain lesions in multiple sclerosis

There is evidence that inflammatory processes in multiple sclerosis (MS) are age-dependent. In this study we evaluated the impact of aging on gadolinium (Gd) enhancement of brain magnetic resonance imaging (MRI) lesions in MS patients. Pre- and post-contrast MRI scans, acquired using a standardized procedure by the same MRI scanner, at least 1 month far from clinical relapse or steroid treatment, were examined in 200 disease-modifying treatment free MS patients. Seventy-three patients (36.5%) showed at least one enhancing lesion. Age at MRI examination (p=0.0001), disease duration (p=0.002) and EDSS score were significantly (p=0.02) lower, whereas relapse rate in the preceding 2 years was higher (p=0.003) in patients with enhancing lesions than in patients with unenhancing scans. Multivariate logistic analysis showed that current age was the variable better predicting Gd enhancement (p=0.004). The odds ratios were 0.95 (CI: 0.92-0.98) for each year of patient's age and 0.64 (CI: 0.48-0.87) for each age decade. The main changes in enhancement risk occurred after 35 years of age. Multivariate Poisson regression model showed that relapse rate in the preceding 2 years (p<0.0001) and current age (p=0.0003) were the best predictors of the number of enhancing lesions. This information can be used to increase the statistical power of clinical trials using Gd-enhancing lesions as an outcome measure.     

多发性硬化脑内病灶MRI典型及不典型表现:附82例MRI分析

目的分析我国多发性硬化病例典型及不典型影像学改变。方法回顾经McDonald诊断标准明确诊断的82例多发性硬化患者的头部MRI检查结果,分析病灶部位、大小、数目、形状、信号强度及强化方式等。结果我国多发性硬化的典型影像学表现:（1）病灶部位以脑室旁（62例,75.61%）及幕上深部白质（55例,67.07%）多见。（2）病灶数目多在10个以上（62例,75.61%）。（3）病灶直径以≤1 cm为主（62例,75.61%）,>5 cm次之（23例,28.05%）。（4）信号强度及强化特征为T₁WI平扫呈等或略低信号,T₁WI高信号,PDwI高于脑脊液中水信号;T₁WI增强图像黑洞从微小至大片状均可见,形状多呈类圆形或条片状;<1 cm病灶显示结节状强化,1～5 cm病灶一般呈环状强化,>5 cm病灶以边缘强化或内部呈不规则环状强化为主,皮质病灶多见弓状强化。我国多发性硬化的不典型影像学表现为:病灶较大,最大直径>5 cm（23例,28.05%）;强化显著且强化持续时间较长;脑干病灶多见（61例,74.39%）。结论侧脑室旁或深部白质内多发小病灶是多发性硬化的典型MRI表现;侧脑室旁病灶致侧脑室变形、大脑凸面的微小黑洞及侧脑室边缘的条片状黑洞,以及近皮质或围绕侧脑室颞角的弓状强化具有重要诊断价值。病灶大、强化显著及脑干病灶多见,是我国多发性硬化不同于欧美地区的主要表现。     

A case of tuberous sclerosis with congenital brain tumor; an immunohistochemical study]

A case of tuberous sclerosis with congenital brain tumor was reported. The diagnosis was made on the basis of the pathological findings of subependymal giant cell astrocytoma, after surgery at the newborn period. After the neonatal period, the patient presented the classical triad of seizures, white spot of skin and mental retardation. The exact nature of tumor-forming giant cells remains controversial. We studied the nature of these cells using immunohistochemical method. GFAP, S-100 protein and NSE stains were all positive. This result suggested that the tumor cells had the features of both neurons and astrocytes.     

CADASIL mimicking multiple sclerosis: The importance of clinical and MRI red flags

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small vessel disease, manifesting as recurrent ischaemic events, migraine with aura, behavioural disturbance and cognitive decline. We report two patients with CADASIL masquerading as multiple sclerosis (MS). A 23 year old female presented with a visual scotoma and was discovered to have a corresponding retinal cotton wool spot. MRI brain revealed diffuse T2 hyperintensities suggestive of demyelination. A 56 year old male presented with transient sequential paraesthesia, initially of the perineum followed by the right leg. He also reported memory and mood impairment with a history of migraine with aura. MRI of the brain showed diffuse bilateral white matter lesions with sparing of the anterior temporal poles. Both patients satisfied the modified McDonald diagnostic criteria and were initially thought to have MS. However, they did not satisfy the caveat of “no better explanation” and on subsequent testing NOTCH 3 mutations were identified in both patients [1]. These cases highlight the importance of careful clinical assessment and neuroimaging findings in identifying clinical and paraclinical ‘red-flags’ for a diagnosis other than MS.     

Correlations of brain MRI parameters to disability in multiple sclerosis

OBJECTIVES: The objective was to correlate magnetic resonance imaging (MRI) T2-weighted lesion load and measures of white matter atrophy in the brain to disability in a population-based sample of patients with multiple sclerosis (MS). MATERIAL AND METHODS: A well defined cohort of patients was drawn at random from the general MS population by using the Danish Multiple Sclerosis Registry. A semi-automated local thresholding technique was used to quantify T2-weighted lesions on MRI; whereas manual tracing was applied to measure the corpus callosum brain ratio (CCR) and the ventricle brain ratio (VBR). RESULTS: A sample of 86 patients with a mean age of 43.3 years (SD 4.3), mean disease duration of 13.6 years (SD 4.4) and a median Expanded Disability Status Score (EDSS) of 6.0 was identified. The correlation between total lesion area of the brain (TLA) and disability (EDSS) for the whole sample was moderate (Spearman rank correlation coefficient r=0.48, P<0.001). Also correlations of CCR and VBR to disability (r=0.32-0.46) were significant. CONCLUSIONS: Correlations of TLA and disability in this study were rather strong. Hence, T2-weighted MRI lesion load in the brain still plays an important role as a surrogate marker of disease and as a secondary outcome measure in phase III treatment trials.