儿童异染性脑白质营养不良的MRI表现

目的 观察异染性脑白质营养不良（MLD）的特征性表现。方法 回顾分析我院经生化检查证实的MLD共11例,观察MRI上脑内白质病变的部位和特殊结构（白质纤维束、丘脑、胼胝体）受累的情况。结果 11例均表现为双侧脑室周围白质T2WI对称高信号,10例双侧半卵圆中心T2WI对称高信号;4例累及皮层下白质。8例累及胼胝体膝部,9例累及压部,两者同时受累为8例;7例累及内囊后肢,4例累及外囊,4例累及脑干皮质脊髓束（3例中脑,1例桥脑）。1例累及小脑白质,8例半卵圆中心T2WI有虎纹征,8例T2WI丘脑呈低信号;5例侧脑室扩大,1例全脑萎缩。结论 MLD的典型表现为双侧半卵圆中心和侧脑室周围白质T2WI对称高信号,早期皮层下白质不受累,胼胝体膝部和压部受累为重要征象;另外征象包括脑干皮质脊髓束和内、外囊受累及虎纹征。     

佩梅病的头颅MRI表现及其与临床、基因分型的关系

目的 探讨佩梅病( PMD)患儿的头颅MRI特点,以及与临床及基因分型的关系.方法 回顾性分析16例经临床诊断为佩梅病患儿的临床和影像资料.患儿均为男性,年龄5个月至9岁8个月.由儿科神经医师对患儿的症状和体征进行检查,将其按照临床症状进行临床分型.由影像科医师对头颅MRI图像的特点进行分析,病灶的位置包括苍白球、锥体束、胼胝体、小脑白质、半卵圆中心,判断大、小脑是否萎缩、是否有“豹纹征”.结果 临床诊断经典型佩梅病8例,中间型7例,先天型1例.16例佩梅病患儿MRI均以广泛的脑白质髓鞘化延迟为特点,病灶累及苍白球13例、锥体束7例、胼胝体11例、小脑白质7例、半卵圆中心12例、脑萎缩5例、小脑萎缩1例,5例有“豹纹征”.锥体束和小脑白质受累在点突变患儿中多见;临床症状较重的中间型及先天型患儿出现小脑白质病变频率较高;“豹纹征”多见于经典型患儿,提示患儿的髓鞘化程度相对较高.结论 佩梅病患儿的头颅MRI具有典型的影像特点,其影像学表现与临床分型和基因突变类型有一定的相关性.     

遗传性弥漫性脑白质病变合并轴索球样变的磁共振特征附2例报告

目的探讨遗传性弥漫性脑白质病变合并球状轴索(HDLS)的磁共振特点,以加深对该病的理解和认识。方法报道2例确诊为集落刺激因子1受体基因突变的HDLS患者的临床诊疗经过,结合文献对其磁共振不同序列的影像学特征进行讨论及分析。结果2例均为青年,慢性起病,亚急性进展,临床表现为体重减轻,口齿不清,反应迟钝,近记忆力减退及肢体运动功能障碍。头颅MRI表现为双侧侧脑室旁、半卵圆中心对称性白质病变、DWI显示弥散受限,MRS提示病变区域NAA峰减低,Cho峰增高,2例均有不同程度脑萎缩,以顶叶及胼胝体体部较明显。结论HDLS为罕见疾病,临床表现多样,容易误诊,双侧侧脑室体旁、半卵圆中心区多发持续性DWI高信号伴有Cho峰增高为本病较特异性的影像学表现,有助于HDLS的早期诊断。     

Delayed encephalopathy after acute carbon monoxide intoxication: MR imaging features and distribution of cerebral white matter lesions.

Magnetic resonance (MR) images obtained in 15 patients with delayed encephalopathy after acute carbon monoxide (CO) intoxication were reviewed. Images had been obtained 4-9 weeks after exposure to CO, during the relapse of neuropsychiatric symptoms after initial recovery. Bilateral symmetric confluent high signal intensity in the periventricular white matter and centrum semiovale was seen on long-repetition-time images (n = 15). The high intensity extended into the corpus callosum (n = 11), subcortical U fibers (n = 12), and external (n = 9) and internal (n = 7) capsules. Bilateral diffuse low-intensity signal in the thalamus and putamen on T2-weighted images, suggesting iron deposition, was demonstrated in 10 patients. Bilateral ischemia or necrosis of the globus pallidus was seen in nine patients. In three of four patients with follow-up MR imaging studies, a decrease in extent and signal intensity of white matter lesions accompanied lessening of clinical symptoms. These results suggest that the main pathologic feature of delayed encephalopathy associated with CO intoxication is a reversible demyelinating process of the cerebral white matter.     

常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病患者的MR定量指标及其与临床的关系

目的 对常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病(CADASIL)患者的白质高信号和脑体积进行定量分析,并探讨其与临床的关系.方法 15例通过病理检查确诊为CADASIL的患者进行常规MR扫描,统计脑半卵圆中心、内囊后肢、外囊、胼胝体和颞极白质受病变累及情况.利用计算机后处理软件计算标准化颅脑体积和白质高信号占颅脑体积的百分比,并与年龄、美国国立卫生研究院卒中量表(NIHSS)和简易精神状态检查(MMSE)量表评分进行Spearman相关性分析.结果 CADASIL患者的白质病变累及部位依次为:半卵圆中心(13/15)、颞极白质(10/15)、外囊(8/15)、内囊后肢(5/15)、胼胝体(4/15).白质高信号占颅脑体积的百分比为(5.7±1.4)%,标准化颅脑体积为(1602±58)×103mm3.年龄与标准化颅脑体积呈负相关(r=-0.555,P＜0.05);白质高信号百分比与NIHSS、MMSE量表评分分别呈正、负相关(r=0.522,P＜0.05;r=-0.679,P＜0.01);标准化颅脑体积与NIHSS评分呈负相关(r=-0.624,P＜0.05).结论 CADASIL患者的白质高信号和脑体积可以定量测量,这两种影像学指标可以在一定程度上反映患者的病情.白质高信号的发展可能预示患者认知功能的下降.     

Delayed encephalopathy of acute carbon monoxide intoxication: diffusivity of cerebral white matter lesions

BACKGROUND AND PURPOSE: Carbon monoxide intoxication has delayed effects on the cerebral white matter characterized by bilateral, confluent lesions that reflect diffuse demyelination. To increase our understanding of this process, we assessed the diffusion characteristics of these lesions. METHODS: Five consecutive patients with delayed encephalopathy of CO intoxication were examined with diffusion MR imaging. Diffusion-weighted images (DWIs) were obtained 25-95 days after their exposure to CO and during a relapse of neuropsychiatric symptoms, which occurred after an initial recovery. Imaging was performed at 1.5 T by using a spin-echo echo-planar sequence with diffusion gradients of 0, 500, and 1000 s/mm(2). DWIs and apparent diffusion coefficient (ADC) maps were visually evaluated, and mean ADCs were calculated from the periventricular white matter and the centrum semiovale, where confluent hyperintensity was seen on T2-weighted images. Findings were compared with those of normal-looking white matter. RESULTS: In all five patients, both T2-weighted images and DWIs showed the white matter lesions as bilateral, diffuse, confluent areas of hyperintensity in the periventricular white matter and centrum semiovale. On ADC maps, these lesions were isointense, with focal areas of hypointensity (n = 4) or diffuse hypointensity (n = 1). Mean ADC values of the white matter lesions were significantly lower than those of normal-looking white matter, regardless of their isointensity or hypointensity on ADC maps (P <.05). CONCLUSION: Bilateral, confluent, white matter lesions in patients with delayed encephalopathy of CO intoxication show decreased diffusivity.     

Brain MR in chronic fatigue syndrome.

PURPOSETo determine the prevalence of MR white matter abnormalities in patients with chronic fatigue syndrome (CFS).METHODSBrain MR studies of 43 patients (29 women and 14 men, 22 to 78 years old) with a clinical diagnosis of CFS (n = 15), CFS with associated depression (n = 14), and CFS with associated other psychiatric disorders, namely, anxiety and somatization disorder (n = 14), were compared with brain MR studies in 43 age- and sex-matched control subjects.RESULTSMR findings were abnormal in 13 (32%) of the patients in the study group (ages 34 to 78 years) and in 12 (28%) of the control subjects (ages 26 to 73 years). One patient with CFS had multiple areas of demyelination in the supratentorial periventricular white matter. Another patient with CFS and associated depression had a single focus of probable demyelination in the supratentorial periventricular white matter. In four patients with CFS (ages 34 to 48 years) MR abnormalities consisted of one or several punctate hyperintense foci in the corona radiata, centrum ovale, and frontal white matter. The remaining seven patients (ages 50 to 78 years) had frontoparietal subcortical white matter foci of high T2 signal. The prevalence of white matter hyperintensities was not different between the patients and the control subjects.CONCLUSIONSOur findings suggest that no MR pattern of white matter abnormalities is specific to CFS.     

Cerebral White Matter in the Centrum Semiovale Exhibits a Larger N-acetyl Signal than Does Gray Matter in Long Echo Time 1H-Magnetic Resonance Spectroscopic Imaging

Long echo time (272 ms) ¹H magnetic resonance spectro-scopic imaging was used to measure the relative magnitudes of the N-acetylaspartate (NAA) signal in a variety of anatomically defined brain structures (centrum semiovale, thalamus, medial frontal cortex, and genu of the corpus callosum) composed primarily of gray matter or white matter. Six normal young adult humans aged 30–40 were studied. With a 95% level of statistical confidence, the white matter in the centrum semiovale (CSO) produced a more intense NAA signal than did the gray matter in the thalamus and the frontal cortex. Differences between the white matter regions were also noted. The CSO white matter's NAA signal yielded a larger NAA signal than did the white matter of the genu of the corpus callosum. Possible reasons for the anatomical variation in the cerebral NAA signal intensity are discussed.     

MR imaging of periventricular leukomalacia in childhood

Eight children with clinical and radiologic abnormalities consistent with periventricular leukomalacia were investigated with MR imaging of the brain that employed both inversion-recovery and T2-weighted spin-echo imaging sequences. The more precise delineation of white and gray matter on inversion-recovery images as compared with CT allows a detailed demonstration of the anatomic features of periventricular leukomalacia; specifically, a reduced quantity of white matter in the periventricular region and centrum semiovale and, in more severe cases, cavitated infarcts that replace the immediate periventricular white matter. The T2-weighted spin-echo and short inversion time inversion-recovery images demonstrated abnormally increased signal in white matter that appeared normal on CT scans and only minimally abnormal on conventional inversion-recovery images. These abnormalities most probably represent white matter gliosis that extends beyond the immediate periventricular regions. MR recognition of cerebral white matter abnormalities associated with periventricular leukomalacia may confirm the clinical suspicion of this diagnosis in children with spastic diplegia or quadriplegia.     

Acute and chronic brain infarcts on MR imaging in a 20-year-old woman with acute posterior multifocal placoid pigment epitheliopathy

A 20-year-old woman recently diagnosed with acute posterior multifocal placoid pigment epitheliopathy developed headaches, weakness, and paresthesias. MR imaging of the brain revealed an acute infarct (demonstrated by diffusion-weighted images) in the head of the right caudate nucleus, a chronic infarct with encephalomalacia in the body of the corpus callosum, and multiple foci of abnormal signal intensity in the white matter of the centrum semiovale.     

3.0T MR弥散梯度编码方向对脑组织FA值测量的影响

目的:探讨超高场强MR下弥散张量成像中弥散梯度编码方向对脑组织弥散各向异性分数(FA)的影响。方法:使用3种不同的弥散梯度编码方向(6、13和21个)在3.0T MRI上对14名健康志愿者进行头颅弥散张量成像(DTI)。在FA图上分别测量两侧半卵圆中心、胼胝体膝部、胼胝体压部、两侧内囊、丘脑及桥脑FA值,并进行统计学分析。结果:胼胝体压部FA值最高,其次为胼胝体膝部、内囊和桥脑,丘脑FA值最低。随着弥散梯度编码方向的增加,FA图质量提高,对白质纤维束细节的显示也更清楚,尤其是对脑干结构的分辨,但成像时间延长;3种不同弥散梯度编码方向的DTI扫描方案所观察到的半卵圆中心、胼胝体膝部、胼胝体压部、内囊、丘脑及桥脑的FA值不存在统计学显著性差异。结论:超高场MRI弥散梯度编码方向数目对脑组织FA值的测量无显著性影响,在临床运用中可根据患者状况选择弥散梯度编码方向,以提高DTI检查的成功率。     

Diffusion-tensor MR imaging in children with developmental delay: preliminary findings

PURPOSE: To determine whether diffusion-tensor magnetic resonance (MR) imaging can depict abnormalities in patients with a diagnosis of developmental delay but structurally normal brain MR imaging results. MATERIALS AND METHODS: Twenty pediatric patients who received a diagnosis of developmental delay underwent brain MR examinations, including diffusion-tensor MR imaging. The MR findings in these patients were compared with those in 10 age-matched neurodevelopmentally healthy children. Diffusion constant (Dav) and anisotropy were measured bilaterally in regions of interest in the centrum semiovale, corona radiata, internal capsule, corpus callosum, and subcortical white matter of the frontal and parieto-occipital lobes. By using a one-tailed Student t test in the positive direction for Dav and in the negative direction for anisotropy and P <.05 to indicate a significant difference, the Dav and anisotropy values for children with developmental delay were compared with those for children who were neurodevelopmentally healthy. RESULTS: The children with developmental delay had significant increases in Dav in all measured structures (P, <.001 to <.03). Significant decreases in anisotropy were detected in all white matter fiber tracts studied (P, <.001 to <.03) except the posterior limb of the internal capsule. CONCLUSION: In the children with developmental delay, diffusion-tensor MR imaging depicted decreases in anisotropy and increases in Dav in the white matter fiber tracts, which appeared to be normal at conventional MR imaging.     

MR of the brain in Sjögren-Larsson syndrome.

Cerebral MR was performed in three patients with Sjögren-Larsson syndrome. In each case, a 1.5-T system was used, and the patient was under general anesthesia. The MR findings included confluent hyperintense white matter lesions in the periventricular and deep white matter of the centrum semiovale, with sparing of the subcortical U fibers. The topography of the white matter abnormalities correlated well with the clinical signs and symptoms.     

Acute Marchiafava-Bignami disease with callosal,cortical, and white matter involvement

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism whose main pathology is symmetrical demyelination and necrosis of the corpus callous. The lesion may be found in hemispheric white matter, but the cortical involvement is extremely rare. We reported herein two cases of MBD with magnetic resonance (MR) findings, additional MR spectroscopy, and MR perfusion which revealed acute demyelination at the corpus callosum and symmetrical signal abnormality at cortical gray matter and hemispheric white matter.     

液体衰减反转恢复脉冲对DWI图像信噪比及ADC值的影响

目的:探讨液体衰减反转恢复(FLAIR)脉冲序列对扩散加权图像(DWI)信噪比及ADC值的影响.方法:17名健康志愿者在3.0T MRI上行常规轴位DWI和FIAIR-DWI扫描,分别测量两侧半卵圆区、扣带回、丘脑及胼胝体膝部和压部的ADC值及DWI信号强度,同时测量基底节层面的DWI信号强度作为噪声,计算以上5个部位DWI图像的信噪比.结果:扣带回和胼胝体压部的ADC值在施加FLAIR脉冲后显著下降,而半卵圆区、丘脑及胼胝体膝部在施加FLAIR脉冲前后无明显变化;5个部位的DWI图像信噪比在施加FLAIR脉冲后均显著下降(P＜0.01).结论:FLAIR显著提高了脑组织和脑脊液ADC值测量的准确性,但是也明显降低了DWI图像的信嗓比.     

正常成人大脑白质纤维各向异性特征的弥散张量磁共振成像研究

目的：利用弥散张量磁共振成像研究正常成人大脑白质纤维的各向异性特征。材料和方法：应用弥散张量磁共振成像方法，观察10例正常志愿者的大脑白质纤维的FA图像，分别测量两侧大脑半球相对称的5个感兴趣区即外囊、内囊前肢、内囊后肢、胼胝体前部、胼胝体后部的FA值，并进行比较。结果：在FA图像上可清楚地观察到大脑白质纤维结构，呈高信号，各个部位信号是不同的，胼胝体信号较其他部位高，尤以压部信号最高。5个感兴趣区的FA值是外囊0．41、内囊前肢0．48、内囊后肢0．71、胼胝体前部0．72、胼胝体后部0．86。各个兴趣区之间进行秩和检验，结果为胼胝体后部与外囊比较有统计显著差异，胼胝体后部与内囊前肢比较有统计显著差异，胼胝体前部与外囊相比较亦有统计显著差异，其余各兴趣区之间相比较无统计显著差异。结论：弥散张量磁共振成像方法有效地显示大脑白质纤维的各向异性特，缸，为正确认识大脑白质纤维的正常解剖提供更多有价值的信息。     

Leigh syndrome in a 3-year-old boy with unusual brain MR imaging and pathologic findings

We report unusual MR serial imaging and electron microscopy findings in a 3-year-old boy who had Leigh syndrome with cytochrome-c oxidase (cox) deficiency. The MR imaging findings included periventricular white matter involvement, posteroanterior progression, and extension through the corpus callosum and internal capsule; however, no basal ganglia or brain stem abnormality was found, which was suggestive of leukodystrophy. The most noteworthy findings were the cystic foci with contrast enhancement in the affected white matter.     

CT, MR, and pathology in HIV encephalitis and meningitis

The value and limitations of CT and MR in human immunodeficiency virus (HIV) infection of the brain was determined by a retrospective analysis of the CT scans (22) and MR images (7) in 22 patients with pathologically proved HIV encephalitis (21) or meningitis (1). Our clinical-radiologic-pathologic correlation suggested that, especially in the early stages of the disease, CT and MR were relatively insensitive in detecting the primary changes of HIV encephalitis. The multiple bilateral diffuse microscopic glial nodules with multinucleated giant cells of HIV found at autopsy in both gray and white matter were usually not directly visualized by either CT or MR. Secondary, nonspecific changes, however, were seen. These included cortical atrophy, found in virtually all patients with HIV encephalitis, and HIV-induced foci of demyelination found in the minority of cases. On CT the latter were seen in the white matter as nonenhancing, nonmass-producing areas of low density; on MR they were seen as frequently progressive high-intensity signal abnormalities on T2-weighted images, usually in the periventricular white matter and centrum semiovale. MR was more sensitive in detecting these demyelinative lesions than was CT. The clinical diagnosis of HIV encephalitis usually antedated the radiographic diagnosis. In HIV meningitis, contrast CT was more definitive than MR, showing striking enhancement of the subarachnoid spaces, although MR was more sensitive in detecting the secondary parenchymal changes.     

海洛因白质脑病的CT与MRI诊断

目的 研究CT与MRI对烫吸海洛因蒸汽引起海绵状白质脑病的特征性影像学诊断价值。方法 对2例烫吸海洛因引起的海绵状白质脑病的患者进行了临床和CT与MRI观察，查阅中外文献，对该病影像学特征结合临床表现、病理组织改变进行总结与讨论。结果 2例患者以小脑性共济失调、智力减退为主要临床表现，CT显示对称性分布的小脑白质、大脑白质为主的低密度改变；MRI显示对称性分布的小脑齿状核，大脑半球半卵圆中心、双基底节内囊、中脑、桥脑白质纤维为主的高信号改变。结论 烫吸海洛因引起海绵状白质脑病的CT与MRI影像学改变具有特征性，能为临床诊断提供可靠依据，从而使本病能够得到早期确诊。     

DTI和T1WI测值法在早产儿脑发育评估中的应用价值

目的:探讨磁共振扩散张量成像(DTI)和T1WI测值法在早产儿脑发育评估中的应用价值.方法:将32例早产儿根据分娩孕周分为早期早产儿组(出生胎龄≤32周,19例)和中晚期早产儿组(出生胎龄>32周,13例),均进行DTI检查,测量感兴趣区(ROI)的各向异性分数(FA)值和表观扩散系数(ADC)值.ROI包括胼胝体压部...