Cerebral cortical and white matter lesions in amyotrophic lateral sclerosis with dementia: correlation with MR and pathologic examinations

BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis with dementia (ALSD) is a progressive neurodegenerative disorder, characterized clinically by motor neuron symptoms and dementia, and pathologically by degeneration of the motor neurons of the brain and spinal cord as well as atrophy of the frontal and/or temporal lobes. So far, there has been no study on the correlation of MR images with histologic findings in ALSD. We studied the correlation of antemortem and postmortem T2-weighted MR images with histologic findings in autopsy-proved cases of ALSD. MATERIALS AND METHODS: Antemortem and postmortem T2-weighted images were compared with histologic findings in 3 autopsy-proved cases of ALSD. RESULTS: Antemortem MR images showed atrophy of the frontal and temporal lobes, which were asymmetric in the medial-ventral part of the temporal lobe. Faint linear T2-hyperintensity was seen in the medial-ventral part of the temporal subcortical white matter in 1 case. Postmortem T2-weighted images showed linear subcortical hyperintensity in the ventral-medial temporal lobe in each case. Histologically, cortical atrophy on MR images showed spongiform change with neuronal loss and gliosis especially in the superficial layers and linear subcortical hyperintensity on T2-weighted images showed degeneration and gliosis in each case. These findings are characteristic histologic changes of ALSD. CONCLUSION: MR imaging of atrophy of the frontal and temporal lobes with linear subcortical hyperintensities in the anteromedial temporal lobe is useful for diagnosis of ALSD.     

Alteration of white matter MR signal intensity in frontotemporal dementia.

PURPOSETo determine the diagnostic potential of MR imaging to show white matter involvement in frontotemporal dementia.METHODSWe evaluated MR signal intensity in cerebral white matter by visually inspecting and by quantitatively measuring signal intensity on MR images in 22 patients with frontotemporal dementia. The findings were compared with those in 22 age- and sex-matched patients who had had Alzheimer disease for the same length of time and with 16 age- and sex-matched healthy control subjects.RESULTSPatients with frontotemporal dementia had a significant increase in white matter signal intensity in the frontal and/or temporal lobes on T2- and proton density-weighted images. Visual inspection of regular proton density-weighted images and measurements made on the T2- and proton density-weighted images were sensitive to changes in white matter signal.CONCLUSIONIncreased MR signal intensity in the frontotemporal white matter on T2- and proton density-weighted MR images is a useful diagnostic sign of frontotemporal dementia and distinguishes this condition from Alzheimer disease.     

新生儿低血糖脑损伤的MRI诊断及鉴别

目的:探讨新生儿低血糖脑损伤的MRI特征及扩散加权成像(DWI)在低血糖脑损伤诊断中的应用价值。方法:回顾性分析13例经临床确诊的新生儿低血糖脑损伤病例的MRI资料,所有病例均在出生后38h～7d行MRI检查,扫描序列包括常规T1WI、T2WI及DWI序列。结果:常规T1WI表现为受累部位稍低信号4例,稍高信号2例;9例T2WI表现为受累部位稍高信号或灰白质分界不清。13例在DWI图上出现异常高信号,受累部位在ADC图上呈低信号(其中2例低信号范围内出现部分高信号),受累脑区包括双侧枕顶叶皮层及皮层下脑白质10例、双侧顶枕叶皮层下脑白质1例、双侧颞叶后部皮层下脑白质4例、双侧额叶后部皮层下脑白质4例、胼胝体压部13例(整个胼胝体受累1例)、双侧内囊后肢5例、双侧内囊前肢1例、双侧外囊1例、脑干1例,13例中10例病变呈对称性分布。结论:新生儿低血糖脑损伤以双侧枕顶叶后部脑组织及胼胝体压部最常受累,常对称性分布,具有一定特征性。DWI序列较常规T1WI及T2WI序列在低血糖脑损伤的诊断及评估上更具优越性。     

2个CADASIL患者的弥散张量成像与20例正常人的比较

目的　分析2例CADASIL患者的MR特点及弥散张量指标的变化。方法　收集2例通过病理和基因检查确诊为CA DASIL的先证者的临床资料,对其进行常规MR扫描和弥散张量成像,将弥散张量成像的指标与20例正常志愿者的指标进行比较。结果　2例CADASIL患者的MR主要表现为双侧额顶叶白质内多发腔梗、脱髓鞘改变和双侧颞叶前部白质脱髓鞘。1例患者双侧外囊、内囊后肢、胼胝体膝部和压部的部分各向异性(FA)值均小于正常组的平均值减去2倍标准差,另1例患者左侧外囊的FA值小于对照组的平均值减去2倍标准差。结论　常规MR表现和弥散张量成像指标的测量均反映了CADASIL患者中存在严重的白质病变。     

Cerebral white matter degeneration in frontotemporal dementia detected by diffusion-weighted magnetic resonance imaging

RATIONALE AND OBJECTIVE: Brain tissue damage in frontotemporal dementia (FTD) has never been systematically studied using diffusion-weighted imaging (DWI). We studied FTD patients using DWI to determine whether microstructural changes in white matter can be detected in vivo. MATERIALS AND METHODS: Thirteen FTD patients and 15 aged healthy subjects were studied. Mean diffusivity (MD) abnormalities in 28 white matter regions were visually evaluated. In addition, MD values in 10 white matter regions relative to that in the ipsilateral postcentral gyrus were measured. The results were compared between healthy subjects and FTD patients. RESULTS: The visual rating resulted in a significant MD elevation in FTD patients in the bilateral high superior frontal gyri, right orbitofrontal gyrus, bilateral anterior temporal lobes, and left middle temporal lobe (P < .01, respectively). Relative MD comparison revealed a significant MD elevation in FTD patients in the bilateral high superior frontal gyri, bilateral orbitofrontal gyri, and bilateral anterior temporal lobes (P < .05 after Bonferroni correction, respectively). CONCLUSION: Our results demonstrated white matter MD abnormalities in FTD patients. It was suggested that the observed white matter MD abnormalities are secondary to damage in the overlying cortex.     

Association of right-to-left shunt with frontal white matter lesions in T2-weighted MR imaging of stroke patients

Introduction  Cardiac right-to-left shunt (RLS), mainly due to patent foramen ovale (PFO), is a risk factor for paradoxical embolism and stroke. Results of studies about brain lesions in diffusion-weighted imaging (DWI) in PFO patients were controversial. DWI only detects acute ischemic lesions. We assessed the hypothesis that, in T2-weighted magnetic resonance imaging (T2WI) of stroke patients, RLS is associated with a typical distribution of small white matter lesions. Materials and methods  In this retrospective case–control study, T2WI images of 162 stroke patients were evaluated. From stroke patients admitted between 1999 and 2003, 81 stroke patients with RLS were identified with contrast-enhanced transcranial Doppler (bubble test). Controls were 81 age-matched stroke patients without RLS (negative bubble test). In T2WI images, small lesions (<2 cm) were categorized depending on their location in subcortical white matter, peritrigonal white matter, deep and paraventricular white matter, and basal ganglia. Additionally, larger territorial infarcts were rated. Results  In T2WI frontal or predominantly frontal-located subcortical small white matter, lesions are significantly associated with RLS (p < 0.0001, chi-square test). Forty-three patients with RLS (53%) and only 19 control patients (23%) showed this frontal dominance. Odds ratio is 3.7 (95% confidence interval = 1.9–7.1) for having a RLS when T2WI shows this lesion pattern in a stroke patient. No patient of the RLS group and 6% of the control group had parietal dominance. Distribution of small lesions in other locations like basal ganglia or deep white matter showed no significant difference for the groups. Conclusion  A distribution of mainly frontal subcortical small white matter lesions in T2WI is significantly associated with RLS in stroke patients.     

Radiologic and neuropathologic findings in patients in a family with dentatorubral-pallidoluysian atrophy

We describe the cases of 2 patients, a father and his son, with DRPLA who underwent MR examinations prior to death and in whom postmortem examinations of the brain were obtained. MR imaging findings had the following features: 1) atrophy of the cerebellum and brain stem were the common findings, 2) high-signal-intensity lesions in the cerebral white matter and brain stem were observed on T2-weighted images in the patient with adult-onset DRPLA, 3) signal-intensity changes in the cerebral white matter were restricted to the periventricular white matter in the patient with juvenile-onset DRPLA, but these changes appear in the advanced stage, and 4) progressive cerebral atrophy was more marked in the patient with juvenile-onset DRPLA. In the patients with DRPLA, the abnormal high signal intensity of the cerebral white matter or brain stem on MR images reflect the loss of myelinated fibers. Cerebral atrophy mainly reflects atrophy of the neuropile.     

Neonatal citrullinemia: comparison of conventional MR, diffusion-weighted, and diffusion tensor findings

Conventional MR, diffusion-weighted, and diffusion tensor imaging were performed in an 8-day-old girl with citrullinemia. She had severe hyperammonemia for several days. On conventional T2-weighted MR images, symmetric, confluent high signal intensity was found in the bilateral thalami, basal ganglia, cortex, and subcortical white matter. Diffusion-weighted imaging demonstrated decreased apparent diffusion coefficient in these areas, reflecting cytotoxic edema. Follow-up MR imaging at the age of 4 months revealed subcortical cysts, ulegyric changes, and atrophy, which were most prominent in the occipital lobes. Diffusion tensor imaging revealed decreased anisotropy throughout the brain, consistent with diffuse injury to the oligodendro-axonal unit. Diffusion-weighted and diffusion tensor imaging are valuable techniques for the detection of irreversible brain damage and for the characterization of hyperintense lesions on T2-weighted MR images in patients with the neonatal form of citrullinemia.     

出血性脑梗死的MRI特征性表现及其分型

目的：探讨出血性脑梗死（HI）的MRI特征性表现和分型。方法：采用1．5T MR,对50例HI行常规序列扫描（T1WI、T2WI、FLAIR）。必要时行MRA（42例）、增强扫描（4例）及T2＊WI（27例）。结果：根据梗死、出血的形态、部位进行MRI分型。Ⅰ型：脑叶HI：22例（44．0％）脑回状、斑片状出血位于皮层和／或皮层下白质。Ⅱ型：脑深部（基底节及丘脑）HI：17例（34．0％）;Ⅲ型：小脑HI：14例（28．0％）;斑片状、线状出血位于梗塞区内、边缘。混合型（Ⅰ型＋Ⅱ型）：3例（6％）。T2＊WI呈低信号。结论：HI的MRI表现有一定特征性,与脑梗死部位、面积有密切关系。新的MRI分型有助于对HI发生机制的理解,从而指导临床治疗。T2＊WI有助于HI的检出。     

用MR扩散加权成像评价新生儿缺氧缺血性脑病的初步研究

目的应用扩散加权成像(DWI)方法评价新生儿缺氧缺血性脑病(HIE)，并同常规MRI对照，以了解扩散成像的价值及限度。方法对临床确诊的36例缺氧缺血性脑病的新生儿(年龄3h至22d，平均8．44d)，进行常规MRT，WI和DWI检查(b=700s／mm^2)。观察皮层及皮层下白质、深部白质、基底节及丘脑、脑室及脑外间隙等部位。结果缺血缺氧所致脑损害在DWI表现为弥漫性损害：区域性皮层、皮层下及深部白质较广泛的高信号19．4％(7／36)；局灶性损害：沿侧脑室壁和三角部白质的高信号27．8％(10／36)，额叶深部白质点状高信号5．6％(2／36)。相应部位的T1WI分别为16．7％(6／36)，36．1％(13／36)，30．6％(11／36)。出血性病变，在T1WI上为高信号，而在DWI上表现为无信号。结论MR DWI适于早期检查HIE，T1WI适于亚急性期和慢性期。     

急性甲醇中毒性脑病的CT及MR表现

目的:分析甲醇中毒患者的颅脑CT及MR表现,以提高影像诊断水平。方法:搜集40例甲醇中毒患者的颅脑及眼部的CT和MR资料,回顾性分析其CT及MR表现。结果:40例中额顶叶白质、外囊壳核CT呈低密度及MR呈长或短T1 长T2 改变的18例,占45%。其中双侧外囊壳核CT呈低密度、MR呈长或短T1 长T2 改变共16例,占40%,右侧壳核出血1例。额、顶叶皮层下白质密度减低共6例,迟发(入院后1周)表现1例。双侧颞叶海马CT呈低密度、MR呈长T1 长T2 改变,共8例。红核、黑质边缘模糊共4例。视神经呈长T1 长T2 改变共8 例。结论:双侧外囊壳核出血性坏死、白质水肿为急性甲醇中毒性脑病的CT及MR主要表现,少数有外囊壳核出血表现。视神经筛板板层内段早期表现为水肿、晚期表现为视神经萎缩。MR检查病灶更加明确,更有特征性。     

环孢霉素A致可逆性后部白质脑病综合征的MRI表现

目的探讨环孢霉素A诱发可逆性后部白质脑病综合征(PRLS)的MRI表现。资料与方法回顾性分析1例PRLS的临床资料和MRI表现并复习文献。结果MRI显示PRLS以顶枕叶为主的皮层及皮层下白质病变,病变多呈双侧对称性分布,偶可累及双侧颞叶后部及额叶,T2WI和液体衰减翻转恢复序列(FLAIR)对病变的显示有独特的作用,扩散加权成像(DWI)能够确定PRLS的临床分期。结论PRLS在MRI上具有特征性。MRI表现结合临床资料,对PRLS多能作出正确的诊断。     

Cortical thickness difference across the central sulcus visualized in the presence of vasogenic edema

PURPOSE: To confirm the cortical thickness difference across the central sulcus (CS) visualized in the presence of vasogenic edema on MRI. MATERIALS AND METHODS: T2-weighted images of 70 cerebral hemispheres showing vasogenic edema infiltrating into subcortical white matter around the CS were studied retrospectively. Two neuroradiologists measured the cortical thickness of the anterior and posterior banks of the CS, precentral sulci (PrCS), and postcentral sulci (PoCS). Additionally, we compared the cortical thickness of the anterior and posterior banks of each sulcus visually using a grading scale. RESULTS: On T2-weighted images, the cerebral cortex was highlighted by a high signal-intensity vasogenic edema in the adjacent white matter, and its thickness was readily measurable. The unique cortical thickness difference between the anterior and posterior banks of the CS were confirmed with measurements of 2.67 and 1.48 mm (p<0.0001). The cortical measurements across other cerebral sulci were 2.04 and 1.95 mm (NS) for the PrCS, and 1.67 and 1.77 mm (NS) for the PoCS. The cortical thickness ratios were 1.86 for the CS, 1.05 for the PrCS, and 0.96 for the PoCS. On visual evaluation, the anterior bank of the CS was thicker than the posterior bank in 93% (65/70). For the PrCS and PoCS, the thickness of the anterior and posterior banks appeared to be equal in over 70% of the patients. CONCLUSION: A prominent cortical thickness difference across the CS in the presence of vasogenic edema was confirmed. This finding is considered to facilitate the identification of the CS in patients with brain tumors.     

MRI对成人脑体积定量分析与临床对照研究

目的：探讨共振成像对成人脑体积定量分析的方法及其价值。方法：对20例正常对照组和20例实验组行垂直于海马长轴的倾斜冠状位磁共振T1及T2加权像扫描,测量海马、颞叶、额叶体积及颅腔容积,对其比值进行定量分析。结果：海马萎缩与临床痴呆密切相关。结论：采取垂直于海马长轴的倾斜冠状位磁共振T1及T2加权像扫描,测量海马体积与颅腔体积并计算其比值的方法,对判断与临床痴呆相关的脑萎缩敏感、可靠、切实可行。     

Terminal zones of myelination: MR evaluation of children aged 20-40 months

BACKGROUND AND PURPOSE: MR imaging is the method of choice for assessment in vivo of the development of myelination of the human central nervous system. During the first months of life, the myelination process follows well-defined steps, whereas little information exists about the later phases of myelination. To improve our understanding of this aspect and to identify the specific sites involved in the process of myelination in its terminal phase, we evaluated normal MR brain studies in children aged 20-40 months. METHODS: We retrospectively evaluated 85 MR brain studies of 81 children aged 20-40 months who were without diseases potentially affecting white matter. The MR studies were performed with a 1.5-T system, with T2-weighted spin-echo and turbo spin-echo sequences. Subjective analysis of the signal intensity of the white matter was made in four areas: subcortical frontal, temporal, and parietal lobes and peritrigonal region. Extension of myelination was graded on an ordinal scale; 0 indicated the absence of myelin, and the maximum value indicated complete myelination. RESULTS: A persistent T2 hyperintensity of the subcortical areas was noted after 20 months of age. With advancing age, a progressive increase in the grade of myelination was noted in these regions, and at about 40 months of age myelination was complete. However, in most of our patients aged 20 months, myelination in the peritrigonal areas appeared complete. CONCLUSION: The only area that can still exhibit a persistent T2 hyperintensity on MR images at about 2 years of age is considered to be the peritrigonal region: the so-called terminal zone. At this age in our patients, however, a persistent T2 hyperintensity was noted in the frontotemporal subcortical regions. In these areas, the myelination appeared complete at 36-40 months of age. The so-called terminal zones were the subcortical areas rather than the peritrigonal area, and complete myelination took place by about age 3 years.     

MRI of the brain in chronic carbon monoxide poisoning

We examined 13 patients with chronic carbon monoxide (CO) poisoning by magnetic resonance imaging (MRI); all of them had been in an explosion in a coal mine 25 years previously. Symmetrical globus pallidus lesions were observed in 12, as was degeneration of the white matter, with focal cortical atrophy. The temporal parietal and occipital lobes were usually affected, the parietooccipital region being the most frequently and extensively damaged. Of the 12 patients with white matter degeneration 7 had definitely asymmetrical cortical and subcortical lesions. There were 6 patients with dilated temporal horns, probably due to atrophy of the hippocampal gyri. A history of CO inhalation and an awareness of the typical distributions of lesions are important for recognition of the effects of CO poisoning, especially when patients are in the chronic stage.     

Cranial MRI findings in myotonic dystrophy

MRI was performed in 13 patients with the adult form of myotonic dystrophy (MD) and compared with that of sex- and age-matched normal controls. There was some cerebral atrophy in the patients and marked thickening of the skull in three of them, associated with ossification of the falx cerebri in two. We found high-signal areas on T 2-weighted images in the white matter in 9 (70 %) of the patients; five showed high-signal areas in the subcortical white matter of the temporal lobes. These findings were associated with intellectual impairment in only one patient, who had a history of a difficult birth and temporal lobe epilepsy. Received: 31 October 1995 Accepted: 5 March 1996     

Gray, White Matter Concentration Changes and Their Correlation with Heterotopic Neurons in Temporal Lobe Epilepsy

Objective

To identify changes in gray and white matter concentrations (GMC, WMC), and their relation to heterotopic neuron numbers in mesial temporal lobe epilepsy (mTLE).

Materials and Methods

The gray matter or white matter concentrations of 16 left and 15 right mTLE patients who achieved an excellent surgical outcome were compared with those of 24 healthy volunteers for the left group and with 23 healthy volunteers for the right group, by optimized voxel-based morphometry using unmodulated and modulated images. A histologic count of heterotopic neurons was obtained in the white matter of the anterior temporal lobe originating from the patients'' surgical specimens. In addition, the number of heterotopic neurons were tested to determine if there was a correlation with the GMC or WMC.

Results

The GMCs of the left and right mTLE groups were reduced in the ipsilateral hippocampi, bilateral thalami, precentral gyri, and in the cerebellum. The WMCs were reduced in the ipsilateral white matter of the anterior temporal lobe, bilateral parahippocampal gyri, and internal capsules, but increased in the pons and bilateral precentral gyri. The heterotopic neuron counts in the left mTLE group showed a positive correlation (r = 0.819, p < 0.0001) with GMCs and a negative correlation (r = -0.839, p < 0.0001) with WMCs in the white matter of the anterior temporal lobe.

Conclusion

The present study shows the abnormalities of the cortico-thalamo-hippocampal network including a gray matter volume reduction in the anterior frontal lobes and an abnormality of brain tissue concentration in the pontine area. Furthermore, heterotopic neuron numbers were significantly correlated with GMC or WMC in the left white matter of anterior temporal lobe.     

MRI及MR扩散张量成像对肌萎缩侧索硬化症的初步评价

目的　描述肌萎缩侧索硬化症 (ALS)的脑部MR影像学表现特点 ,探讨常规MR成像及MR扩散张量成像 (DTI)部分各向异性 (FA)值在本病诊断中的价值。方法　对 14例临床确诊的ALS患者和 12例正常人行MR扫描 ,其中 2例患者行增强扫描。对 10例患者及 12例正常人行DTI横轴面和冠状面扫描 ,使用自旋回波 回波平面成像 (SE EPI)序列 ,b值为 10 0 0s/mm2 ,扩散敏感梯度方向为 2 5。为定量评价锥体束 ,在内囊后肢和中脑大脑脚水平测量双侧锥体束的FA值并做统计学处理。结果　 6例患者双侧内囊后肢呈局限性T1WI略低信号及T2 WI高信号 (高于皮层信号 ) ,冠状面T2 WI可明确显示内囊后肢至大脑脚纵向连续的带状高信号影 ,符合锥体束走行 ;8例患者双侧内囊后肢呈局限性T1WI略低信号及T2 WI略高信号 (与皮层信号相等 )。 2例行增强扫描者均未见异常对比增强。 12例正常人双侧内囊后肢均呈局限性T1WI略低信号及T2 WI略高信号 (与皮层信号相等 )。病变组FA值在内囊后肢水平和中脑大脑脚水平均明显低于对照组FA值 (F =7.38,P <0 .0 1;F =7.31,P =0 .0 1)。结论　常规MR成像诊断ALS时须结合临床资料。ALS患者锥体束区域FA值的下降提示存在神经元变性及继发性脱髓鞘 ,DTI可以在活体无创性地对锥体束及其潜在病变进行检测和评价     

基于体素的形态学分析技术在评价脑灰质体积改变对皮质下缺血性血管性痴呆患者执行功能影响中的应用

目的:采用基于体素的形态学分析(VBM)技术分析皮质下缺血性血管性痴呆(SIVD)患者执行功能障碍与相关脑区灰质体积改变之间的关系.方法:分别纳入SIVD患者和健康志愿者各14例,应用VBM技术比较2组的脑灰质体积差异,分析脑灰质体积变化与执行功能的相关性.结果:与健康对照组相比,SIVD患者灰质体积显著减少,包括全脑...