Brain magnetic resonance imaging findings and radiologic review of maple syrup urine disease: Report of three cases

BACKGROUNDMaple syrup urine disease (MSUD) is a rare autosomal-recessive disorder that affects branched-chain amino acid (BCAA) metabolism and is named after the distinctive sweet odor of affected infants’ urine. This disease is characterized by the accumulation of BCAAs and corresponding branched-chain ketoacids of leucine, isoleucine, and valine in the plasma, urine, and cerebrospinal fluid. However, the mechanisms of MSUD-induced brain damage remain poorly defined. The accumulation of BCAAs in the brain inhibits the activity of pyruvate dehydrogenase and α-ketoglutarate, disrupting the citric acid cycle and consequently impacting the synthesis of amino acids, causing cerebral edema and abnormal myelination.CASE SUMMARYWe report three neonates admitted to our hospital with the classic subtype of MSUD. All three patients, with a transient normal period, presented with poor feeding, vomiting, poor weight gain, and increasing lethargy after birth. Laboratory testing revealed metabolic acidosis. The serum tandem mass spectrometry amino acid profile showed elevated plasma levels of BCAAs (leucine, isoleucine, and valine). Brain magnetic resonance imaging (MRI) presented abnormal signals mainly involving the globus pallidus, thalamus, internal capsule, brainstem, and cerebellar white matter, which represent the typical myelinated areas in normal full-term neonates.CONCLUSIONIn our patients, MRI showed typical features, in concordance with the available literature. Early detection and timely treatment are very helpful for the prognosis of MSUD patients. Therefore, we discuss the neuroimaging features of MSUD to enhance the knowledge of pediatricians about this disease.     

Sonographic and magnetic resonance imaging findings in uterine incarceration

We show the sonographic and magnetic resonance imaging features of uterine incarceration. Clinical data and imaging findings were retrospectively reviewed for 8 confirmed cases identified by sonography from 2000 to 2010. Two patients had magnetic resonance imaging. Seven of 8 patients (87.5%) presented with abdominal pain; 4 of 8 (50.0%) also had urinary symptoms. All had a retroverted uterus with an elongated anterosuperiorly displaced or poorly visualized cervix on sonography. Magnetic resonance imaging showed similar features, but in both cases, the placental position was misinterpreted because of severe uterine retroversion. Radiologists should be aware of this condition and its imaging features to reduce associated morbidity and mortality.     

磁共振影像联合带厚度指标与T2WI高信号对子宫腺肌症的诊断价值

目的:探讨磁共振成像(MRI)中联合带厚度指标和病灶区T_2WI高信号灶联合应用对子宫腺肌症的诊断价值。方法:回顾性分析56例经手术和病理证实为子宫腺肌症患者及35例正常对照组的MRI资料,在观察子宫腺肌症MRI病灶特征的基础上,测量联合带厚度最大值(JZ_(max))、最大值与最小值差(JZ_(dif))、联合带与肌层厚度比(JZ_(rat))。子宫腺肌症组和对照组JZ_(max)、JZ_(dif)、JZ_(rat)比较采用Mann-Whitney U检验。联合带增厚和高信号灶单独应用与联合应用诊断子宫腺肌症的差异采用Pearsonχ2检验。结果:弥漫性子宫腺肌症35例,局限性21例。弥漫性子宫腺肌症中,3例联合带测量困难;另外32例中,JZ_(max)、JZ_(dif)和JZ_(rat)三者平均值分别为20.6mm、12.8mm和70.1%。局限性子宫腺肌症中,3例联合带显示欠清,其他18例JZ_(max)、JZ_(dif)和JZ_(rat)平均值分别为16.4mm、10.2mm和61.0%。子宫腺肌症组和正常对照组JZ差异有统计学意义(P0.05)。子宫腺肌症48例(85.7%)见T_2WI高信号灶。JZ_(max)、JZ_(dif)和T_2WI高信号3个参数联合应用诊断子宫腺肌症的灵敏度、特异度和准确度分别为92.9%、97.1%、94.5%,其中敏感度和准确度高于JZ_(max)、JZ_(dif)单独应用(P0.05)。结论:JZ_(max)、JZ_(dif)、JZ_(rat)联合T_2WI高信号有助于提高子宫腺肌症诊断价值。     

职业棒球运动员的优势肩关节:MRI检查

目的利用MRI明确职业棒球运动员优势肩关节的损伤情况.方法对国内某棒球队12名主力队员的优势肩关节进行了MRI普查,其中4例同时接受了MR关节造影检查.结果 10名队员(83%)的优势肩关节在MR检查中呈现不同程度的损伤,主要包括肩峰下间隙复合体的损伤、盂肱关节不稳定以及肩锁关节的损伤,而且同一肩关节经常合并出现多种损伤.结论职业棒球运动员中,其优势肩关节损伤的几率较高,应引起足够的重视.     

Manual ventilation during magnetic resonance imaging

Mechanical ventilation during magnetic resonance imaging (MRI) requires a specially constructed ventilator that contains no ferromagnetic components; however, the expense of acquiring such a ventilator may not be warranted if projected use is infrequent. We adapted a Laerdal manual resuscitator to allow its use during MRI, and report the simulation and monitoring procedure that we developed and the successful ventilation of three pediatric patients with the device.     

Cysts of the canal of Nuck: ultrasound and magnetic resonance imaging findings

Cyst of the canal of Nuck is a rare cause of inguinal swelling in woman. We report a case of a cyst of the canal of Nuck in which sonography showed a tubular cystic structure with internal septae localized within the inguinal canal. Magnetic resonance examination demonstrated that the mass was hypointense on T1-weighted and hyperintense on T2-weighted series and that there were fine hypointense septae inside the mass on the T2-weighted sequence. Diagnosis of cyst of the canal of Nuck was confirmed by surgery and subsequent histopathologic evaluation.     

Cranial magnetic resonance imaging findings in patients with migraine

OBJECTIVE: To investigate the frequency of cranial magnetic resonance imaging abnormalities in patients with migraine and their relationship to type, duration, and frequency of migraine attacks. METHODS: Forty-five patients (43 women, 2 men) with migraine whose ages ranged between 19 and 53 years (mean, 40.91 [SD, 7.69]) were evaluated. Of the 45 patients, 20 had migraine with aura and 25 had migraine without aura, according to the diagnostic criteria of the International Headache Society. RESULTS: In 13 (28.8%) of 45 patients, white matter foci were present on magnetic resonance imaging. Eight of these patients (61.5%) had migraine with aura, and 5 patients (38.4%) had migraine without aura. The presence of white matter foci was significantly higher in the patients with aura (8 [40%] of 20) than in those without aura (5 [20%] of 25). It was found that as the frequency of attacks per month increased, the number of patients with white matter foci also increased. Although the mean duration of migraine was longer in patients with white matter foci (149.5 months [SD, 87.9]) than in those without white matter foci (134.1 months [SD, 88.3]), there was no significant difference (P >.05). CONCLUSION: Although there are no specific magnetic resonance imaging findings peculiar to migraine, detection of white matter foci should be taken into consideration in patients with migraine (especially migraine with aura). Frequency of attacks is an important indicator of existence of white matter foci.     

肾血管瘤CT和MRI特征分析

目的 探讨肾血管瘤的CT和MRI特征,为其诊断进一步提供参考。方法 回顾性分析经手术病理证实的10例肾血管瘤患者的临床特征及影像表现,其中3例行CT、5例行MRI、2例同时行CT和MRI检查,总结其影像特征。结果 10例肾血管瘤中,7例为吻合状血管瘤,6例位于肾髓质、3例位于肾窦、1例位于肾实质,肿瘤最大径1.4～6.4 cm,8个病灶表现为类圆形。所有病灶均以实性为主,3例有包膜。4例T₂WI呈明显高信号,6例DWI呈高信号;在ADC图上3例呈等信号,2例呈高信号,2例呈低信号;8例表现为皮髓质期瘤周明显结节、斑片状强化,后期逐渐向中心填充,1例明显不均匀强化,1例不强化。CT多期增强扫描中,肾血管瘤实质期强化率高于腹主动脉(P<0.05),皮髓质期强化率与腹主动脉差异无统计学意义;肾血管瘤在MRI增强扫描皮髓质期及肾实质期的强化率与肾皮质及腹主动脉差异无统计学意义。结论 肾血管瘤好发于肾髓质或肾窦部,多为吻合状血管瘤;影像表现具有一定的特征性：T₂WI多呈明显高信号,动态增强扫描中皮髓质期病灶周边明显结节样强化,逐渐向中心填充,强化...     

Magnetic resonance imaging of abdominal pain during pregnancy

The objective of this review is to demonstrate magnetic resonance imaging as an important adjunct to ultrasound and computed tomography in the evaluation of the pregnant patient with abdominal pain. With the advent of ultrafast T2-weighted pulse sequences, fetal and bowel motion cause minimal artifact on the images. An accurate diagnosis can often be made in a few minutes based on these high-contrast images performed in 2 or 3 planes. T1-weighted gradient echo images with and without fat saturation are useful for identifying blood and fat, especially in the case of adnexal masses. Gadolinium-diethylenetriamine pentaacetic acid is rarely used to diagnose inflammatory or obstructive disease and is reserved for those patients with suspected malignancies.     

磁共振对剖宫产瘢痕妊娠的诊断及临床价值

目的探讨研究3.0 T磁共振成像(magnetic resonance imaging,MRI)对于剖宫产瘢痕妊娠(cesarean scar pregnancy,CSP)的影像学特征及其对临床的应用价值。材料与方法回顾性收集2013年7月至2019年4月就诊于扬州大学附属医院并诊断为CSP共38例患者的基本临床资料,利用扫描仪器GE 750 W获取所有病例盆腔MRI图像,分析病灶特征、生长方式、有无血管流空、孕囊与瘢痕相交长度及孕囊距子宫浆膜面的距离及收集临床相关治疗方案。结果在MR上孕囊可表现为单纯囊性及混杂包块型,混杂包块型较单纯囊性妊娠囊有更大的最大径、孕囊体积以及与子宫瘢痕相交长度,且差异具有统计学差异(P<0.05);从孕囊的生长方式上可分为外生型及内生型,外生型较内生型孕囊血供更为丰富,距子宫浆膜面距离更薄,且差异具有统计学意义(P<0.05);38例患者进行相关治疗,在1~3个月内血人绒毛膜促性腺激素(human chorionic gonadotropin,HCG)恢复至正常值,预后良好。结论磁共振对CSP患者可进行全面评估,充分了解病灶的基本特征及其与周围组织关系,为定制个性化临床治疗方案提供依据。     

骶椎神经鞘瘤MRI征象与临床病理特征分析

目的探讨骶椎神经鞘瘤的侵犯特点与MRI表现特征。材料与方法观察26例骶椎神经鞘瘤的MRI表现,根据生长部位、侵犯范围及有无囊变、"足突边缘"征,进行分型、分组测量瘤体最大径。比较型间及组间瘤体最大径的差异。对照分析瘤实质T2WI信号、强化效应及HE染色组织学表现。结果 1型4例,2型5例,3型10例,4型7例。骶前软组织肿块出现率高于骶板后(χ2=13.066,P=0.011)。骶椎中线与中线旁骨质破坏各5例、14例。第3型瘤体最大径大于其他型(t=2.655,P=0.014)。8例巨大侵袭性骶椎神经鞘瘤(giant invasive sacral Schwannoma,GTSS)瘤体最大径超过18例非GISS(t=3.027,P=0.006)。"足突边缘"征阳性12例与阴性14例间瘤体最大径差异无显著性(t=1.896,P=0.07)。囊变组12例的瘤体最大径、病程均长于非囊变组14例(t=2.928,P=0.007;t=-2.187,P=0.039)。Ⅰ区呈T2WI稍高信号、明显强化,对应Atoni A区;Ⅱ区呈T2WI高信号、无强化或轻微强化,对应Atoni B区。结论骶椎神经鞘瘤具有偏心性破坏和骶前侵犯特性。T2WI上稍高信号与高信号及其差异性强化是该瘤的MRI表现特征。     

Icteric-type hepatoma: magnetic resonance imaging and magnetic resonance cholangiographic features

Background: We evaluated the imaging features of magnetic resonance imaging (MRI) and magnetic resonance cholangiography (MRC) of icteric-type hepatoma and correlated these with the findings of endoscopic retrograde cholangiography (ERC), percutaneous cholangiography, and surgery. Methods: Thirteen patients with viral hepatitis complicated by cirrhosis of the liver and obstructive jaundice underwent MRC and dynamic MRI. Five patients received percutaneous transhepatic cholangiography and drainage; one of these patients also underwent resection of the left hepatic lobe. Another patient received MRC followed by thrombectomy and T-tube insertion. ERC and endoscopic nasobiliary drainage were performed in another patient for bile diversion. Results: Primary liver tumors and dilatation of biliary system were demonstrated in all patients. No capsule formation could be found in any primary liver tumors. MRI showed the simultaneous presence of an intraluminal tumor in the portal trunk and common hepatic duct in eight patients. Three different MRC features were found: (a) an oval defect in the hilar bile duct(s) with dilated intrahepatic ducts (n= 9), (b) dilated intrahepatic ducts with missing major bile ducts (n= 2), and (c) localized stricture of the hilar bile duct(s) (n= 2). Conclusion: The presence of one or more of the following features in multiplanar MRI and MRC help to identify this rare, specific type of hepatocellular carcinoma: (a) the presence of an intraluminal tumor in both the portal trunk and the common hepatic duct, (b) enhancement of the intraluminal tumor in the common hepatic duct on the arterial phase, (c) type I MRC feature, and (d) hemobilia, blood clot within the gallbladder, and/or type II MRC feature. Received: 12 January 2000/Revision accepted: 12 July 2000     

骶尾椎脊索瘤MRI征象与临床病理特征分析

目的探讨骶尾椎脊索瘤常规MRI及动态增强MRI(dynamic contrastenhanced magnetic resonance imaging,DCE-MRI)表现特征及其病理学基础。材料与方法观察21例骶尾椎常规MRI、DCE-MRI和HE染色病理表现,测取瘤体最大径与DCE-MRI的时间信号曲线(time-signal intensity curve,TSIC)和最大强化斜率(wash in rate,Wi R)、最大消退斜率(wash out rate,Wo R)及强化峰值时间(time to peak,TTP)。结果瘤体T2WI以高信号为显著特点。"足突边缘"征阳性18例的瘤体最大径大于阴性者(t=2.800,P=0.011)。脊索瘤信号在30 min内逐渐增强,TSIC缓慢持续上升,Wi R为0.75±0.05,Wo R为0,TTP为30 min。病理学主要表现是富含空泡细胞和液滴状细胞的瘤细胞巢间以大量黏液聚集。结论骶尾椎脊索瘤具有以椎体为中心的三维侵袭并椎前侵犯倾向的生物学行为特性,"足突边缘"征是其重要边缘形态特征。特征性的T2WI高信号、缓慢持续强化,与瘤细胞及黏液密切相关。