MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). Received: 21 February 1997 Accepted: 4 June 1997     

Value of MRI of the brain in patients with systemic lupus erythematosus and neurologic disturbance

Abstract Our objective was to review the frequency and pattern of signal abnormalities seen on conventional MRI in patients with suspected neuropsychiatric systemic lupus erythematosus (NP-SLE). We reviewed 116 MRI examinations of the brain performed on 85 patients with SLE, (81 women, four men, aged 21–78 years, mean 40.6 years) presenting with neurological disturbances. MRI was normal or nearly normal in 34%. In 60% high-signal lesions were observed on T2-weighted images, frequently in the frontal and parietal subcortical white matter. Infarct-like lesions involving gray and white matter were demonstrated in 21 of cases. Areas of restricted diffusion were seen in 12 of the 67 patients who underwent diffusion-weighted imaging. Other abnormalities included loss of brain volume, hemorrhage, meningeal enhancement, and bilateral high signal in occipital white-matter. The MRI findings alone did not allow us to distinguish between thromboembolic and inflammatory events in many patients. Some patients with normal MRI improved clinically while on immunosuppressive therapy. More sensitive and/or specific imaging methods, such as spectroscopy and perfusion-weighted imaging, should be investigated in these subgroups of patients with suspected NP-SLE.     

Brain white-matter volume loss and glucose hypometabolism precede the clinical symptoms of Huntington's disease.

We studied the anatomic and functional changes in various brain areas during the course of Huntington's disease (HD) in a large cohort of mutation-positive individuals (n = 71) encompassing the complete range of disability (presymptomatic through stage V), and in healthy controls, for the purpose of defining both degenerative and dysfunctional brain changes in the same subjects. METHODS: We used an MRI and unsupervised multiparametric segmentation procedure based on a relaxometric approach to measure in vivo brain volumes in 71 subjects with presymptomatic to advanced HD. The same population was evaluated by 18F-FDG PET to assess variations in brain glucose metabolism. To predict age at onset in unaffected mutation carriers, we considered the estimated number of years from each subject's age to manifested HD symptoms, for a given expanded triplet number. RESULTS: Age-adjusted analyses confirmed that the 71 subjects as a group, as well as the subgroup of 24 unaffected presymptomatic subjects at risk for HD, had significantly smaller gray-matter and white-matter volumes and larger cerebrospinal fluid volumes than did controls (P < 0.0001). In the 24 presymptomatic subjects, we observed a significant inverse linear correlation between white-matter volume reduction and the estimated time to symptom onset (r2= 0.39; P = 0.0011). Both clinically unaffected subjects at risk for HD and symptomatic patients had significantly decreased glucose uptake in the cortex (frontal and temporal lobes) and striatum (caudate and putamen). HD subjects who were followed up longitudinally showed progressive white-matter reduction in the preclinical subjects (n = 10) and decreased glucose uptake in the cortex and striatum in affected (n = 21) and preclinical (n = 10) subjects. CONCLUSION: White-matter volume loss may precede gray-matter atrophy and may be associated with neuronal dysfunction in early disease.     

MR imaging of acute intermittent porphyria mimicking reversible posterior leukoencephalopathy syndrome

Reversible posterior leukoencephalopathy syndrome (PLS) is characterized by headache, altered mental function, visual disturbances and seizures. Neuroimaging studies suggest a white-matter oedema, predominantly in the posterior parietal-temporal-occipital regions of the brain. We present the case of a 30-year-old woman who had suffered her first attack of acute intermittent porphyria (AIP). Following 1 week of abdominal pain she developed several generalized seizures, and hallucinations, and exhibited a progressive deterioration of the consciousness. T2-weighted images, especially fluid-attenuated inversion recovery (FLAIR) sequences showed bilateral lesions in the posterior frontal, parietal and occipital cortex and subcortical white matter. Following treatment with haematin and a high carbohydrate diet the patient's condition improved. Follow-up magnetic resonance imaging (MRI) revealed complete resolution of the lesions. To our knowledge, this is the first report concerning a completely reversible PLS in AIP.     

特发性全身性癫痫分数低频振幅及局部一致性的静息态f MR I研究

目的：观察特发性全身性癫痫(IGE)患者静息状态下全脑功能改变情况。方法采用3.0T MR扫描仪对23例 IGE患者(IGE组)及23例健康志愿者(对照组)行全脑3D 结构相及静息态功能磁共振成像(RS-fMRI)扫描,进行全脑分数低频振幅(fALFF)及局部一致性(ReHo)功能分析,并对比 IGE组相比对照组 fALFF及 ReHo 改变的脑区,分析 IGE 组差异脑区与患者病程的相关性。结果与正常对照组相比,IGE组 fALFF升高的脑区位于双侧中央前回、左侧辅助运动区、左侧扣带回、左侧中央旁小叶、左侧距状裂周围皮层、左侧枕上回、左侧枕中回及右侧楔前叶;fALFF降低的脑区位于双侧颞下回、右侧海马旁回、右侧岛叶、右侧楔前叶及左侧顶下小叶(P<0.005)。ReHo 升高的脑区位于左侧距状裂周围皮层、左顶上小叶、左中央后回及右中央前回;ReHo降低的脑区位于右侧梭状回、左侧豆状核、右侧额下回、右内侧额上回、左侧枕中回、右侧岛叶及双侧顶下小叶(P<0.005)。IGE组 fALFF及 ReHo的差异脑区与患者病程均无相关性。结论 IGE患者脑内广泛的脑区功能异常改变可能是 IGE复杂临床表现的神经病理基础。联合应用 RS-fMRI的2种分析方法能较全面地评价静息状态下脑功能状态的改变情况,为 IGE 神经病理生理机制的研究提供可靠的功能神经解剖学依据。     

Regional brain atrophy evolves differently in patients with multiple sclerosis according to clinical phenotype

BACKGROUND AND PURPOSE: Progressive brain atrophy is a well-known feature of multiple sclerosis (MS). We characterized the spatial evolution of atrophy in different MS phenotypes. METHODS: Dual-echo and T1-weighted MR images were obtained in 70 patients with MS and 10 healthy control subjects at entry and after 15 months. Within-group changes in regional atrophy were assessed by applying Structural Image Evaluation Using Normalization of Atrophy software and statistical parametric mapping analysis. Reported differences are for P <.001. RESULTS: During follow-up, patients with relapsing-remitting MS (RRMS) differences significant atrophy around the ventricular system; pericerebellar spaces; cerebellar tentorium; putamen; corpus callosum; cingulate sulcus; hippocampus; parieto-occipital fissure; lateral fissure; and frontal, parietal, temporal, and occipital cortex. Patients with secondary progressive MS developed significant atrophy of the cingulate sulcus; pulvinar; caudate nucleus; anterior orbital gyrus; mammillary body; fourth ventricle; and regions of frontal, parietal, temporal, and occipital cortex. Patients with primary progressive MS developed significant atrophy of the bilateral central sulcus; caudate nucleus; prepontine and quadrigeminal cisterns; lateral ventricle; and regions of frontal, parietal, temporal, and occipital cortex. In all phenotypes, the development of atrophy in some regions was significantly correlated with the accumulation of T2- and T1-visible lesions and clinical disability (r = -0.57 to -0.86). CONCLUSION: In MS, brain atrophy develops involving different structures in the different phenotypes. While ventricular enlargement is predominant in RRMS, cortical atrophy seems to be more important in the progressive forms. Measures of regional brain atrophy were significantly correlated with disability, suggesting that this approach is promising for bridging the gap between clinical and MR imaging findings in MS.     

Accelerating regional atrophy rates in the progression from normal aging to Alzheimer’s disease

We investigated progression of atrophy in vivo, in Alzheimer’s disease (AD), and mild cognitive impairment (MCI). We included 64 patients with AD, 44 with MCI and 34 controls with serial MRI examinations (interval 1.8?±?0.7 years). A nonlinear registration algorithm (fluid) was used to calculate atrophy rates in six regions: frontal, medial temporal, temporal (extramedial), parietal, occipital lobes and insular cortex. In MCI, the highest atrophy rate was observed in the medial temporal lobe, comparable with AD. AD patients showed even higher atrophy rates in the extramedial temporal lobe. Additionally, atrophy rates in frontal, parietal and occipital lobes were increased. Cox proportional hazard models showed that all regional atrophy rates predicted conversion to AD. Hazard ratios varied between 2.6 (95% confidence interval (CI) = 1.1–6.2) for occipital atrophy and 15.8 (95% CI?=?3.5–71.8) for medial temporal lobe atrophy. In conclusion, atrophy spreads through the brain with development of AD. MCI is marked by temporal lobe atrophy. In AD, atrophy rate in the extramedial temporal lobe was even higher. Moreover, atrophy rates also accelerated in parietal, frontal, insular and occipital lobes. Finally, in nondemented elderly, medial temporal lobe atrophy was most predictive of progression to AD, demonstrating the involvement of this region in the development of AD.     

免疫抑制状态下并发脑脓肿的MRI表现

目的:探讨免疫抑制状态下并发脑脓肿的MRI表现.方法:回顾性分析7例免疫抑制并发脑脓肿的MRI表现,常规行T1WI、T2WI和增强扫描.结果:7例患者共发现50个小脓肿灶,其中位于额叶10个,项叶17个、枕叶9个、颞叶8个、丘脑1个,小脑5个.主要MRI表现为脑内多发类圆形病灶,位置较深,T1WI呈等、低信号,T2WI呈高信号,较大病灶周边有轻度水肿,Gd-DTPA增强扫描示病灶呈小环状强化,壁薄、光滑.结论:免疫抑制状态下并发脑脓肿的MRI表现具有一定特征性.     

MRI of cortical dysplasia – correlation with pathological findings

Cortical dysplasia (CD) is the most epileptogenic structural lesion associated with epilepsy and patients with intractable seizures caused by this condition are good surgical candidates. MRI plays an important role in detecting the abnormalities of CD. We clarified the MRI characteristics of CD by comparing imaging and histological findings in 20 patients with intractable seizures who underwent surgical resection. There were 12 males and eight females, mean age at operation was 15 years. MRI was performed at 1.5 tesla; T1-weighted, T2- and proton density-weighted spin-echo and fluid-attenuated inversion-recovery (FLAIR) images were obtained. The lesions were in the frontal lobe in nine cases, temporal in two, occipital in another two, insular in one and multilobar in six. Blurring of the grey/white matter junction was seen in all patients, and T2 prolongation in white matter and/or at the grey/white matter junction in 19. Abnormal signal intensity was more frequent in the white matter or at the grey/white matter junction than in the grey matter. FLAIR images made this abnormal high signal easier to appreciate, and we thought them very useful in this context. In areas of T2 prolongation, we saw dysplastic neurones and/or balloon cells, dysmyelination, and ectopic neuronal clustering histologically; glial proliferation played an important role in prolonging T2. Received: 24 November 2000/Accepted: 6 February 2001     

基于局部一致性方法探究电针本神穴的即刻脑效应

目的:观察电针本神穴前后不同阶段脑效应的变化,基于局部一致性(ReHo)方法探究电针本神穴的即刻脑效应及相对特异性.方法:采用3.0 T MRI扫描仪与新型头颅柔性线圈(AHC12),对20例健康受试者行电针前及电针即刻2个阶段BOLD-fMRI扫描.利用RESTplus软件对图像进行预处理,获取2期扫描的ReHo值....     

NMR relaxation times in the human brain at 3.0 tesla

Relaxation time measurements at 3.0 T are reported for both gray and white matter in normal human brain. Measurements were made using a 3.0 T Bruker Biospec magnetic resonance imaging (MRI) scanner in normal adults with no clinical evidence of neurological disease. Nineteen subjects, 8 female and 11 male, were studied for T1 and T2 measurements, and 7 males were studied for T2. Measurements were made using a saturation recovery method for T1, a multiple spin-echo experiment for T2, and a fast low-angle shot (FLASH) sequence with 14 different echo times for T2. Results of the measurements are summarized as follows. Average T1 values measured for gray matter and white matter were 1331 and 832 msec, respectively. Average T2 values measured for gray matter and white matter were 80 and 110 msec, respectively. The average T2 values for occipital and frontal gray matter were 41.6 and 51.8 msec, respectively. Average T2 values for occipital and frontal white matter were 48.4 and 44.7 msec, respectively. ANOVA tests of the measurements revealed that for both gray and white matter there were no significant differences in T1 from one location in the brain to another. T2 in occipital gray matter was significantly higher (0.0001 < P < .0375) than the rest of the gray matter, while T2 in frontal white matter was significantly lower (P < 0.0001). Statistical analysis of cerebral hemispheric differences in relaxation time measurements showed no significant differences in T1 values from the left hemisphere compared with the right, except in insular gray matter, where this difference was significant at P = 0.0320. No significant difference in T2 values existed between the left and right cerebral hemispheres. Significant differences were apparent between male and female relaxation time measurements in brain.     

MR and CT evaluation of intracranial sarcoidosis

Fourteen patients with CNS manifestations of neurosarcoidosis were evaluated by MR imaging and CT. Evaluations were done on a 0.5-T superconductive magnet with T1- and T2-weighted sequences. CT with contrast was obtained in all patients. The granulomatous lesions were classified by location into basilar, convexity, intrahemispheric, and periventricular white-matter involvement. Hydrocephalus with or without an associated lesion was also noted. MR determined the presence of disease in all patients (100%), but was less accurate than CT in depicting disease in two patients (14%). CT determined the presence of disease in 12 patients (85%) and was less accurate than MR in delineating hypothalamic involvement in two patients and periventricular white-matter disease in three patients. There was great variability in the appearance of intracranial sarcoidosis on MR. Three patients had lesions that were isointense or hypointense (relative to cerebral cortex) on both T1- and T2-weighted images while nine patients had lesions that were hyperintense on T2-weighted images. Convexity involvement and hydrocephalus were well documented by both CT and MR. These results indicate that both MR and CT are helpful in fully evaluating a patient with suspected intracranial sarcoidosis.     

新生儿低血糖脑损伤的MRI表现

目的 初步探讨新生儿低血糖脑损伤的MRI表现特征及扩散加权成像(DWI)存早期发现低血糖脑损伤中的应用价值.方法 回顾性分析12例低血糖新生儿(其中10例诊断为新生儿低血糖脑病)MRI资料,12例患儿于出生后3至10 d内进行了头部MR扫描,包括常规T1 WI、T2、WI 和DWI扫描.其中4例在第一次检杏后7至10 d后再次行MR扫描.结果 12例首次DWI检查中11例出现异常高信号,受累脑区包括双侧枕叶皮层2例、右侧枕叶皮层1例、左侧枕叶皮层及皮层下1例,双侧枕叶皮层及皮层下2例、双侧顶枕叶皮层2例、舣侧顶枕叶皮层及皮层下2例、胼胝体压部4例、双侧放射冠2例、左侧尾状核及苍白球1例、舣侧背侧丘脑1例、双侧内囊后肢1例.12例首次常规T1 WI、T2 WI中,4例T1 WI呈现异常信号,表现为受累部位皮层T1信号减低3例、受累的双枕叶皮层稍短T1信号1例;5例T2 WI见异常信号,均表现为受累部位皮层及皮层下T2信号稍增高且灰白质分界不清.4例复查中,受累枕叶局部白质软化4例,残存枕叶皮层见条状稍高T1信号2例,双侧大脑半球白质呈弥漫性脱髓鞘改变1例,胼胝体压部T2高信号消失1例,胼胝体压部仍见稍高T2信号1例.结论 在新生儿期,可能和低血糖相关的腑拟伤多发生在双侧顶、枕叶后部脑组织.早期的DWI扫描有助于低血糖脑损伤的早期发现和评估.     

Brain MRI and SPECT in the diagnosis of early neurological involvement in Wilson’s disease

Purpose To evaluate the impact of brain MRI and single-photon emission computed tomography (SPECT) in early detection of central nervous system abnormalities in patients affected by Wilson’s disease (WD) with or without neurological involvement. Methods Out of 25 consecutive WD patients, 13 showed hepatic involvement, ten hepatic and neurological manifestations, and twp hepatic, neurological, and psychiatric symptoms, including mainly movement disorders, major depression, and psychosis. Twenty-four healthy, age–gender matched subjects served as controls. All patients underwent brain MRI and ^99mTc-ethyl-cysteinate dimer (ECD) SPECT before starting specific therapy. Voxel-by-voxel analyses were performed using statistical parametric mapping to compare differences in ^99mTc-ECD brain uptake between the two groups. Results Brain MRI showed T2-weighted hyperintensities in seven patients (28%), six of whom were affected by hepatic and neurological forms. Brain perfusion SPECT showed pathological data in 19 patients (76%), revealing diffuse or focal hypoperfusion in superior frontal (Brodmann area (BA) 6), prefrontal (BA 9), parietal (BA 40), and occipital (BA 18, BA 39) cortices in temporal gyri (BA 37, BA 21) and in caudatus and putamen. Moreover, hepatic involvement was detected in nine subjects; eight presented both hepatic and neurological signs, while two exhibited WD-correlated hepatic, neurological, and psychiatric alterations. All but one patient with abnormal MRI matched with abnormal ECD SPECT. Pathologic MRI findings were obtained in six out of ten patients with hepatic and neurological involvement while abnormal ECD SPECT was revealed in eight patients. Both patients with hepatic, neurological, and psychiatric involvement displayed abnormal ECD SPECT and one displayed an altered MRI. Discussion These findings suggest that ECD SPECT might be useful in detecting early brain damage in WD, not only in the perspective of assessing and treating motor impairment but also in evaluating better the less investigated disorders in the cognitive domain.     

MRI techniques and cognitive impairment in the early phase of relapsing-remitting multiple sclerosis

Correlation studies between various conventional and non-conventional MRI parameters and cognitive impairment in the early stages of multiple sclerosis (MS) are lacking, although it is known that a number of patients with early MS have mild cognitive impairment. Our aim was to explore whether this cognitive impairment is dependent on the extent and severity of the burden of disease, diffuse microscopic brain damage or both. We studied 63 patients with clinically definite relapsing-remitting (RR) MS, duration of disease 1–10 years and Expanded disability status scale scores ≤ 5.0. Mean age was 35.4 years, mean duration of disease 5.8 years and median EDSS score 1.5. Neuropsychological performance, psychological function, neurological impairment and disability were assessed. The patients also underwent MRI, including magnetisation-transfer (MT) studies. We quantified the lesion load on T2- and T1-weighted images, the magnetisation transfer ratio (MTR) of normal-appearing brain tissue (NABT) and the brain parenchymal fraction (BPF). No significant difference was found between lesion loads in patients with and without cognitive impairment. In 15 patients (23.8 %) with overall cognitive impairment, median BPF and average NABT MTR were significantly lower than those in patients without cognitive impairment (0.868 vs 0.892, P = 0.02 and 28.3 vs 29.7 P = 0.046, respectively). Multiple regression analysis models demonstrated that the only variables independently correlated with cognitive impairment were: BPF (R = 0.89, P = 0.001) and average NABT MTR (R = 0.76, P = 0.012). Our findings support the hypothesis that, cognitive decline in patients with MS, a low disability score and short duration of disease is directly associated with the extent and severity of diffuse brain damage. The loss of brain parenchyma did not correlate with the severity of microscopic damage in the NABT, indicating that the two processes could be distinct in the early stages of the disease. Received: 7 August 2000 Accepted: 18 October 2000     

Comparison of regional brain volume and glucose metabolism between patients with mild dementia with lewy bodies and those with mild Alzheimer's disease.

The aim of this study was to investigate regional differences between morphologic and functional changes in patients with mild dementia with Lewy bodies (DLB) compared with those with Alzheimer's disease (AD). METHODS: Twenty patients with very mild DLB (mean age, 74.5 y; mean Mini-Mental State Examination [MMSE] score, 24.0), 20 patients with very mild AD (mean age, 74.1 y; mean MMSE score, 24.0), and 20 age- and sex-matched healthy volunteers (normal controls [NC]) underwent both (18)F-FDG PET and 3-dimensional spoiled gradient echo MRI. Fully automatic volumetry of the MRI data was used to obtain whole brain, hippocampal, occipital, and striatal volumes, which were compared with the results of a similar analysis of glucose metabolic data. RESULTS: In DLB patients, volumetric data indicated a significant volume decrease in the striatum, whereas (18)F-FDG PET showed significant glucose metabolic reductions in the temporal, parietal, and frontal areas--including in the occipital lobe--compared with those in the NC group. In contrast, in AD patients, both the hippocampal volume and glucose metabolism were significantly decreased, whereas the occipital volume and metabolism were preserved. CONCLUSION: Comparison of very mild DLB and AD revealed different morphologic and metabolic changes occurring in the medial temporal lobes and the occipital lobe, demonstrating characteristic pathophysiologic differences between these 2 diseases.     

婴幼儿神经系统遗传代谢病的脑MRI研究

目的探讨婴幼儿神经系统遗传代谢病的脑MRI表现。方法收集2014年1月—12月我院诊治的16例遗传代谢病病人临床及影像资料,并结合临床特点及实验室检查分析该组病人的脑MRI表现。结果 16例遗传代谢病病人中包括有机酸血症12例(甲基丙二酸血症8例,枫糖尿病4例),尿素循环障碍3例,异染性脑白质营养不良1例。12例有机酸血症中,7例基底节区信号异常,主要表现在T_1FLAIR上呈低信号,T_2FLAIR上呈稍高信号,DWI上呈明显高信号;4例脑干及2例小脑半球在DWI上呈明显高信号,2例脑萎缩,1例胼胝体在DWI上呈高信号,1例双侧半卵圆中心在T_1FLAIR上呈低信号、T_2FLAIR高信号、DWI高信号,1例脑室扩大。3例尿素循环障碍病人中2例脑室扩大,1例大脑皮质在DWI上呈弥漫性高信号。1例异染性脑白质营养不良表现为两侧侧脑室周围白质、半卵圆中心T_2FLAIR高信号。结论婴幼儿神经系统遗传代谢病脑MRI表现缺乏特异性,根据疾病类型不同MRI表现各异,其中有机酸血症患儿以基底节区受累为主。     

Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet's disease from other central nervous system

Two neuroradiologists reviewed MRI studies of 34 patients with neuro-Behçet's disease (NBD), 22 with multiple sclerosis (MS) and 7 with systemic lupus erythematosus (SLE) with central nervous system involvement, masked to the clinical diagnosis, age and sex of the patients. Of the patients with NBD 12 were in an acute attack; the others had chronic disease. MRI was assessed using a set of criteria, looking at atrophy, the site of discrete parenchymal lesions, regions of predominant involvement and the extent of the lesion(s). The observers also made a guess at the clinical diagnosis. The brain stem and/or basal ganglia were the most predominantly involved sites in all patients with acute NBD; 75 % of these lesions were large and confluent, mainly extending from the brain stem to the diencephalon and basal ganglia. However, in chronic cases, the predominant involvement was in the brain stem and/or basal ganglia in only 36 %, and in cerebral hemisphere white matter in another 36 %; 27 % of these patients showed no parenchymal lesion. Hemisphere white-matter lesions were equally distributed between periventricular and other areas in NBD, while in MS more were periventricular, and in SLE more were nonperiventricular. Brain-stem atrophy was seen in 21 % of patients with NBD, with a specificity of 96.5 %. In the absence of cortical atrophy, its specificity was 100 %. The attempt at making a radiological diagnosis was successful in all cases of acute NBD and 95.5 % of patients with MS, but in only 40 % of patients with chronic NBD. Most of this latter groups MRI studies were interpreted as MS. An extensive lesion involving the brain stem and basal ganglia seemed to be diagnostic of acute NBD. However, hemisphere white-matter lesions could not be differentiated from those in MS.     

一氧化碳中毒迟发性脑病的MRI表现

目的:研究一氧化碳(CO)中毒迟发性脑病的MRI特征.方法:回顾性分析32例CO中毒迟发性脑病患者的MRI和临床资料.结果:CO中毒迟发性脑病MRI表现可分为三型:①神经核团受累型;②脑白质受累型;③皮层受累型.MRI特征:苍白球为对称性的卵圆形长T1、长T2信号,皮层下白质为对称性的弥漫、模糊云雾状长T1、长T2信号,侧脑室周围、半卵圆中心白质亦为对称云絮状长T1、长T2信号,胼胝体常受累.MRI显示苍白球合并脑白质受累者及皮层受累者,临床表现较重.结论:CO中毒迟发性脑病MRI表现有一定特征性,且能反应其病理过程,并对CO中毒迟发性脑病的诊断和评价临床表现、预后均有意义.     

MRI of the brain in the Kearns- Sayre syndrome: report of four cases and a review

We report brain MRI findings in four patients with typical Kearns-Sayre syndrome (KSS) and correlate them with clinical manifestations. MRI was interpreted as normal in two patients; cerebral and cerebellar atrophy was seen in the other two. On T2-weighted spin-echo images, two patients had high-signal lesions bilaterally in subcortical white matter, thalamus and brain stem. In one patient, the white matter lesion extended into the deep cerebral white matter and the cerebellum was also affected. The other also had bilateral high-signal lesions in the globus pallidus. There was little correlation between neurological deficits and MRI findings. A review of the literature revealed that 10 of the 13 patients with typical KSS previously studied had bilateral subcortical white-matter lesions on T2-weighted images; at least 7 also had high-signal lesions in the brain stem, globus pallidus, thalamus or cerebellum. Although MRI may be normal or show atrophy, the characteristic finding in KSS is a combination of the high-signal foci in subcortical cerebral white matter and in the brain stem, globus pallidus or thalamus. Received: 23 October 1998 Accepted: 8 February 1999