儿童伴有胼胝体压部可逆性病变的轻微脑炎/脑病的MRI特点

【摘要】目的：探讨儿童伴有胼胝体压部可逆性病变的轻微脑炎/脑病的MRI特点。方法：回顾性分析24例经临床证实的伴有胼胝体压部可逆性病变的轻微脑炎/脑病的MRI表现,分析其影像学特点。结果：24例患儿中Ⅰ型伴有胼胝体压部可逆性病变的轻微脑炎/脑病20例,Ⅱ型4例。Ⅰ型患儿均表现为胼胝体压部椭圆形或圆形病灶,病变边界清楚,无占位效应。Ⅱ型患儿除胼胝体压部受累外,还出现其它部位受累,其中胼胝体膝部受累3例、侧脑室周围脑白质受累2例、半卵圆中心受累1例以及左侧顶叶脑白质受累1例,病灶均表现为T1WI等或低信号,T2WI、T2-FLAIR为稍高信号,DWI为明显高信号,ADC图呈低信号。所有患儿复查时（间隔8～30天）原有病灶完全消失。结论：儿童伴有胼胝体压部可逆性病变的轻微脑炎/脑病头颅MRI表现特异,短期内可完全恢复,以Ⅰ型为常见。MRI能为儿童伴有胼胝体压部可逆性病变的轻微脑炎/脑病的临床诊断及鉴别诊断提供重要依据,避免过度医疗。     

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.     

儿童白质消融性脑白质病的MRI表现

目的 探讨白质消融性脑白质病(VWM)的MRI特征表现.方法 回顾性分析2008年至2013年间经本院儿科基因确诊为VWM的10例患儿的临床及MR资料.由2名资深的神经放射医师进行阅片,对白质病变部位(大脑、小脑、脑干、胼胝体及内外囊)、范围、信号特点、有无囊变、脑萎缩及基底节受累等进行分析.结果 10例大脑中央白质均弥漫受累,9例部分皮层下白质受累,7例伴U形纤维受累;7例伴有囊变;8例内囊后肢受累,7例外囊受累,8例胼胝体内缘(透明隔缘)受累.2例丘脑和苍白球出现异常信号,6例脑干白质受累,7例小脑白质受累.结论 大脑中央白质出现弥漫对称的异常信号伴囊变是VWM特征性的MRI表现,对该病的诊断具有重要价值.     

儿童轻微脑炎/脑病伴可逆性胼胝体压部病变MRI诊断价值

目的 探讨儿童轻微脑炎/脑病伴可逆性胼胝体压部病变(MERS)的临床表现及MRI影像特征.方法 对本院8例MERS患儿的临床特点、影像学变化以及脑电图等进行回顾性分析,检查设备采用GE Signa Excite 1.5T HD Echospeed及DiscoveryMR750 3.0T扫描仪.结果 8例患儿影像学改变均累及胼胝体压部,病灶特征呈类圆形,T1WI呈等或稍低信号,T2 WI及FLAIR呈稍高信号,DWI上呈明显高信号,相应表观扩散系数(ADC)序列上呈低信号为典型特征,其中6例在DWI显示胼胝体压部孤立性受累而被诊断MERSⅠ型;另2例中1例扩展到整个胼胝体,1例扩展到半卵圆中心白质被诊断MERSⅡ型,随访7 d～1月患儿复查头颅MRI均恢复正常.结论 MERS临床上表现为急性轻微脑炎/脑病症状,为临床-影像学预后良好的一种综合征;MRI对其诊断、鉴别诊断及为临床治疗提供依据具有很大价值,为首选影像学检查方法.     

婴幼儿先天性TORCH感染颅脑CT、MRI表现

目的探讨婴幼儿先天性TORCH感染颅脑受累时的CT、MRI表现特点。方法回顾性分析17例先天性TORCH感染婴幼儿脑部受累时的颅脑CT、MRI影像资料,总结其CT及MRI特点。结果 17例患儿均出现不同程度室管膜下和脑白质内多发小钙化灶,15例出现继发性脑室扩张或梗阻性脑积水,13例出现脑室周围环形脑白质髓鞘化延迟区,神经元移行障碍畸形7例,胼胝体发育不良9例,小脑发育不良2例,脑穿通畸形2例。结论脑发育不良、室管膜下和脑白质内多发弥漫性小钙化灶、继发性脑室扩张或梗阻性脑积水是婴幼儿先天性TORCH感染颅脑的特征性影像学表现,CT、MRI表现结合母子血清学检测可对先天性TORCH感染脑部受累做出正确诊断。     

黏多糖病患者神经系统CT及MRI表现

目的 分析黏多糖病(mucopolysaccharidosis,MPS)患者神经系统CT和MRI表现,探讨其对MPS诊断和不同类型MPS鉴别的意义. 资料与方法 回顾性分析24例MPS患儿中枢神经系统的CT和MRI表现.11例行头颅加脊柱MRI,5例行头颅MRI,4例行CT检查,2例行头颅MRI及MRA,1例行头颅MRI及CT检查,1例行颈部MRI. 结果 Ⅰ-H型6例,Ⅰ-S型1例,Ⅱ型1例,Ⅲ型1例,Ⅳ型5例,Ⅵ型9例,Ⅶ型1例.舟状头3例,脑室扩张5例,脑萎缩4例,脑室旁脑白质、放射冠及胼胝体筛网状改变13例,室旁斑片状长T2信号11例,枕骨大孔狭窄,脊髓受压10例,其中1例髓内示异常长T2信号,双侧颞极蛛网膜囊肿3例,寰枢椎畸形7例. 结论 MPS患者中枢神经系统的CT及MRI表现有一定的特征,对临床诊断MPS和鉴别不同类型MPS有帮助.     

Hallervorden—Spatz病的临床和MRI特点（附2例分析）

目的: 探讨苍白球、黑质、红核色素变性的临床和MRI特点.材料和方法: 回顾性分析2例苍白球、黑质、红核色素变性的临床和MRI表现.结果: 主要临床特点: 少年期起病,病情缓慢进展,主要表现为锥体外系症状,均有言语障碍,其中1例有精神障碍、视神经萎缩、步态姿势异常和双侧巴宾斯基征( ); T2 WI均表现为苍白球、黑质等部位对称性低信号,其中1例为"虎眼征".结论: 根据临床和MRI特点可以准确诊断HSD.     

儿童甲基丙二酸血症颅脑常规MRI影像分析

目的探讨甲基丙二酸血症（MMA）患儿颅脑常规MRI特点及其临床应用价值。资料与方法对13例MMA患儿的常规MRI图像进行回顾性分析。全部病例均经尿有机酸分析确诊。结果MRI主要表现为脑发育延迟和脑组织受损。其中，脑沟、脑池增宽者12例，合并侧脑室增宽者9例；脑自质容积减少者8例，脑自质髓鞘化延迟7例；胼胝体形态幼稚6例，另有1例胼胝体压部信号异常；苍白球显示长T1、长T2信号者1例。结论颅脑常规MRI可反映MMA对脑发育的影响和脑损伤，有助于了解病情和评估预后。     

儿童急性坏死性脑病的影像学分析

目的分析儿童急性坏死性脑病(ANEC)的颅脑影像学特征,探讨其对临床的指导价值。方法回顾性分析2013年1月至2018年10月武汉儿童医院22例确诊为ANEC患儿的临床及影像资料。患儿病初均有高热,前驱感染后迅速出现神经功能恶化。首次影像检查中,所有患儿均行头颅MRI检查,其中6例MRI检查前行头颅CT检查。MRI随访中,4例失访,6例仅行1次短期随访(<14 d),12例接受1~2次短期及1~4次长期随访(>14 d)。影像学检查重点观察丘脑、脑干、脑白质和基底节区等部位,以及整个随访中是否有出血及软化灶形成。结果所有ANEC患儿的影像学表现中均累及双侧丘脑,其他对称病变部位包括大脑白质(14例)、基底节区(15例)、脑干(16例)、小脑(9例)、胼胝体(2例)和海马(1例),不对称病灶患儿3例,分别见于大脑白质(2例)及小脑(1例)。最典型的头颅MRI表现为急性期在表观扩散系数(ADC)图上,丘脑呈"三色模式"(中央高信号和周围低信号环,以及丘脑周围高信号)或"双色模式"(丘脑中央低信号及边缘高信号)。影像学随访中,MRI上出现出血、脑软化可能提示临床预后差。结论ANEC为快速进展型脑病,具有典型影像学特点,出血、脑软化可能提示不良预后。     

儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病首次发作的MRI分析

目的探讨儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)首次发作的MRI特点.资料与方法回顾性分析2018年5月—2021年2月湖南省儿童医院临床诊断为MOGAD的40例患儿首次发作时头颅、脊髓及视神经MRI表现,患儿首次发作年龄1.2～13.8岁.结果40例患儿中,37例(92.5％)头颅MRI可见异常病灶,30...     

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。     

MR-revealed myelination in the cerebral corticospinal tract as a marker for Pelizaeus-Merzbacher's disease with proteolipid protein gene duplication

BACKGROUND AND PURPOSE: Pelizaeus-Merzbacher's disease (PMD) is caused by mutations in the proteolipid protein (PLP) gene. Recent studies have shown that an increased PLP dosage, resulting from total duplication of the PLP gene, invariably causes the classic form of PMD. The purpose of this study was to compare the MR findings of PMD attributable to PLP duplication with those of PMD arising from a missense mutation. METHODS: Seven patients with PMD, three with a PLP missense mutation in either exon 2 or 5 (patients 1-3), and four with PLP duplication (patient 4 having larger PLP duplication than patients 5-7) were clinically classified as having either the classic or connatal form of PMD. Cerebral MR images were obtained to analyze the presence of myelination and T1 and T2 shortening in the deep gray matter. Multiple MR studies were performed in six of the seven patients to analyze longitudinal changes. RESULTS: Four patients (patients 1-4) were classified as having connatal PMD, whereas the other three (patients 5-7) were classified as having classic PMD. Myelination in the cerebral corticospinal tract, optic radiation, and corpus callosum was observed in three cases of classic PMD with PLP duplication. In patient 4, myelination extended to the internal capsule, corona radiata, and centrum semiovale over a 3-year period. No myelination was observed in three PMD cases with a PLP point mutation. T2 shortening in the deep gray matter was recognized in all patients with PMD. CONCLUSION: The presence of myelination in the cerebral corticospinal tract with diffuse white matter hypomyelination on MR images could be a marker for PMD with PLP duplication. It is suggested that progression of myelination may be present in connatal PMD with large PLP duplication.     

MR of childhood metachromatic leukodystrophy.

PURPOSETo investigate the MR findings of childhood metachromatic leukodystrophy (MLD).METHODSNine MR imaging studies in seven children (five girls and two boys, 10 to 32 months old) with MLD were evaluated retrospectively for the extent and progression of white matter abnormalities and the presence of contrast enhancement.RESULTSAll seven cases showed symmetric, confluent high signal intensity on T2-weighted images in the periventricular white matter and centrum semiovale. A posterior predominance of white matter abnormalities was noted in all cases. Although initially spared from demyelination in all cases, in one case, the subcortical U fibers were later involved in demyelination of follow-up MR studies. Other sites of involvement were the genu (n = 5) and splenium (n = 6) of the corpus callosum, the posterior limbs of the internal capsule (n = 5), the descending pyramidal tracts (n = 4), the claustrum (n = 4), and the cerebral white matter (n = 2); diffuse brain atrophy was seen in two cases. No enhancement of the lesion was seen on any of the five postcontrast examinations. A "tigroid" pattern, previously described in cases of Pelizaeus-Merzbacher disease, was noted in the centrum semiovale in six cases.CONCLUSIONIn late-infantile MLD, demyelination is more prominent in the occipital region. In addition to demyelination of the periventricular white matter, common manifestations include a "tigroid" pattern and involvement of the corpus callosum, the internal capsule, and the corticospinal tract.     

MRI features of neurodegenerative Langerhans cell histiocytosis

CNS complications of LCH include “space occupying” lesions corresponding to histiocytic granulomas and “neurodegenerative” presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH.     

Marchiafava—Bignami病的影像学诊断（附4例报告及文献复习）

目的探讨Marchiafava—Bignami病（MBD）的CT和MRI表现特征。方法回顾性分析4例MBD的CT和MRI表现,总结其影像学特征。结果急性型2例,CT表现为苍白球密度降低,MR表现为胼胝体呈长T1、较长T2信号改变,DWI弥散受限;亚急性2例,胼胝体中层呈夹层状表现,MR呈长T1、长T2信号改变,1例增强后有边缘性轻度强化。4例均有双侧对称性侧脑室周围白质和半卯圆中心变性表现,局部有坏死。结论根据胼胝体及其伴随的双侧大脑半球白质和苍白球等部位的变性、坏死等影像表现,MBD可以得到明确诊断。     

枫糖尿病的临床和脑磁共振成像特点

目的：探讨枫糖尿病(MSUD)脑 MRI 表现及其诊断价值。方法经气相色谱、串联质谱、基因确诊9例 MSUD 患儿,通过 MRI 了解脑损伤特点,结合临床表现、生化特点进行分析,重点分析影像学表现。结果9例患儿于生后3 d~6岁发病,临床表现缺乏特异性,主要是喂养困难、昏睡、反应差、抽搐发作、智力下降。患者血亮氨酸、异亮氨酸、缬氨酸显著增高。9例均行 MRI 检查,2例同时行氢质子磁共振波谱(1 H-MRS)检查。脑 MRI 显示小脑半球、小脑脚、大脑脚、脑干、苍白球各有5例受累,4例累及丘脑、内囊后肢,3例累及半卵圆中心,2例同时合并胼胝体、枕部、颞叶深部、额叶、顶叶信号异常。受累的区域均表现为 T1稍低、T2稍高信号,而在扩散加权成像(DWI)上表现为明显高信号。1例影像正常。2例1 H-MRS 于0.9 ppm 处均未见甲基峰。结论MSUD 临床表现缺乏特异性,MRI 特点主要是生后已经开始髓鞘化的脑白质区域如小脑半球、内囊后肢、脑干内、小脑脚、大脑脚等在 DWI 上表现为明显高信号。     

Cree leukoencephalopathy: neuroimaging findings.

PURPOSE: To describe the computed tomographic (CT) and magnetic resonance (MR) imaging findings in Cree leukoencephalopathy. MATERIALS AND METHODS: The authors retrospectively reviewed the medical records and neuroimaging studies in 12 infants with Cree leukoencephalopathy (CT in 12 infants, MR in six). The diagnosis was established clinically in six patients and at autopsy in the other six. RESULTS: At CT, extensive, diffuse, and symmetric hypoattenuation was seen in the cerebral and cerebellar white matter in all 12 patients. Hypoattenuation was also seen in the corpus callosum in 11 (92%), internal capsule in 10 (83%), globus pallidus in nine (75%), brainstem in nine (75%), and thalamus in four (33%). The caudate nucleus and putamen were spared. On T2-weighted MR images in six patients, the cerebral and cerebellar white matter, including the subcortical arcuate fibers, was hyperintense as were the internal capsule, corpus callosum, corticospinal tracts, and globus pallidus. The thalamus was affected in four (67%) patients, pons in five (83%), and medulla in four (33%). The caudate nucleus and putamen were not affected. CONCLUSION: Cree leukoencephalopathy causes striking symmetric and diffuse involvement of the cerebral and cerebellar white matter and brainstem with sparing of the caudate nucleus and putamen.     

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.     

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.