神经纤维瘤病MR影像表现

目的　分析并探讨神经纤维瘤病的分型及各型的MR影像学表现。方法　回顾性地分析 8例神经纤维瘤病患者的临床及影像学资料 ,所有病例均常规行MRI及钆剂强化扫描 ,归纳所有患者的临床表现及影像学特点。结果　根据NIH的诊断标准 ,8例中 ,4例属NF -1型 ,均在脑内胶质瘤、椎管内神经纤维瘤或脑内对称部位的异常信号基础上 ,合并有皮肤的咖啡牛乳色斑或多发神经纤维瘤 ,其中 1例有家族史 ;4例属NF -2型 ,均患双侧听神经瘤 ,2例合并髓内星型细胞瘤。结论　NF -1型在所有神经纤维瘤病中占大多数 ,且多合并有皮肤损害 ;NF -2型较少见 ,典型表现为双侧听神经瘤 ,皮损少见。MRI及强化扫描对检出神经纤维瘤病中枢神经系统的损害方面有优势     

神经纤维瘤病中枢神经系统的MRI表现

目的 探讨神经纤维瘤病(NF)的中枢神经系统(CNS)MR表现。方法 回顾性分析10例神经纤维瘤病患的临床资料并对其中枢神经系统MR表现进行分析，所有病例均行常规MR平扫及增强扫描。结果 10例病例中，NF—1型6例，表现为脑内胶质瘤、椎管内神经纤维瘤，4例合并皮肤损害，其中一例有家族史，NF—2型4例，表现为双侧听神经瘤、多发脑膜瘤或神经纤维瘤，皮肤损害少见。所有病例中增强扫描均发现平扫未发现之病灶。结论 MR能很好显示病变的大小、形态及信号特点，对检出神经纤维瘤病中枢神经系统的损害方面有优势，增强扫描可帮助发现平扫未见病灶。     

Ⅱ型神经纤维瘤病的头部MRI表现

目的：强调Ⅱ型神经纤维瘤病头部ＭＲＩ的诊断和增强扫描价值。材料与方法：搜集我院行ＭＲ检查并经手术证实符合神经纤维瘤病诊断标准者３例，分析其头部ＭＲＩ表现。结果：３例中２例为双侧听神经鞘瘤，其中１例伴有双侧三叉神经鞘瘤及脑膜瘤，另１例为单侧听神经鞘瘤伴有多发小的其他颅神经鞘瘤和脑膜瘤。结论：Ⅱ型神经纤维瘤病最常表现为三叉神经和听神经肿物，ＭＲ增强扫描对发现小的病变最为敏感。     

Ⅰ型神经纤维瘤病周围神经病变的超声应用价值

 目的 探讨Ⅰ型神经纤维瘤病 (neurofibromatosis type Ⅰ,NF1)周围神经病变的超声应用价值。方法 回顾性分析11例经临床或手术病理证实的NF1周围神经病变的超声表现,观察NF1周围神经病灶的数目、位置、范围、内部回声、血流信号分布,及其与周围神经、组织的关系。结果 NF1周围神经病灶的超声特性表现为大小不等、多灶性分布、表现多样化的神经纤维瘤,分为三型:多发结节型5例,呈类圆形、分叶状及纺锤形,边界清晰,结节内多为实性低回声;丛状型4例,表现为受累神经干变形、扭曲、不均匀增粗,呈蔓丛状低回声沿神经干长轴生长;弥漫型2例,表现为皮下脂肪层增厚,边界不清,呈片状高回声,其间可见线状、条索状低回声分布。本组3例恶变为恶性周围神经鞘膜瘤,肿物体积较大,回声不均,内部血流信号丰富,周围组织可见浸润征象。结论 超声能够清晰显示NF1受累周围神经的病变特征,为临床诊断提供客观、确切依据,同时超声随访对发现NF1的周围神经病灶复发、恶变也有重要意义。     

神经纤维瘤病Ⅰ型的头颈部CT和MRI表现

目的 探讨神经纤维瘤病Ⅰ型(NF-1)的CT和MRI表现.资料与方法 回顾性分析33例经病理证实的NF-1患者的临床资料及头颈部CT、MRI特征.结果 单侧听神经瘤2例;单侧视神经瘤1例及单发凸面脑膜瘤1例;视神经增粗4例;眼球体积增大伴外凸(青光眼)6例;脉络丛粗大钙化3例;面颊部肿块2例;颌面骨及周围改变6例;蝶翼缺损及颞叶疝21例,其他部位颅骨缺损8例;颈深部肿块2例.结论 CT与MRI相结合能全面显示NF-1的头颈部病变,对NF-1具有重要诊断价值.     

II型神经纤维瘤病的头部MRI表现

目的：强调Ⅱ型神经纤维瘤病头部ＭＲＩ的诊断和增强扫描价值。材料与方法：搜集我院行ＭＲ检查并经手术证实符合神经纤维瘤病诊断标准者３例，分析其头部ＭＲＩ表现。结果：３例中２例为双侧听神经鞘瘤，其中１例伴有双侧三叉神经鞘瘤及脑膜瘤，另１例为单侧听神经鞘瘤伴有多发小的其他颅神经鞘瘤和脑膜瘤。结论：Ⅱ型神经纤维瘤病最常表现为三叉神经和听神经肿瘤，ＭＲ增强扫描对发现小的病变最为敏感。     

神经纤维瘤病Ⅱ型的MRI表现三例

神纤维瘤病Ⅱ型是神经纤维瘤病中的罕见类型 ,笔者遇到 3例 ,现报告如下并结合文献进行复习。例 1　女 ,18岁。左侧肢体乏力伴行走不稳 2年余 ,无恶心、呕吐及四肢抽搐 ,数月后发现左耳听力下降 ,并缓慢进行性加重。查体无特殊。曾行左前臂神经纤维瘤手术治疗。ＭＲＩ表现 :双侧桥小脑角见稍长Ｔ1、稍长Ｔ2 信号肿块 ,双侧听神经明显增粗 ;Ｃ3 ～Ｃ5平面左侧椎管内外见“哑铃”状稍长Ｔ1、稍长Ｔ2 异常信号 ,增强后上述病灶明显强化 ,右侧颈部下斜肌处亦见梭形强化灶 (图 1)。手术所见 :双侧桥小脑角肿瘤 ,质中 ,灰白色 ,与听、面神经粘连 ;…     

神经纤维瘤病I型的MRI研究

目的：回顾神经纤维瘤病I型（NF1）患者MRI表现，分析MR扫描序列及其诊断价值，以建立合适的MR成像方案，为NF1影像诊断提供有价值的依据。方法：对30例临床确诊为NF1患者采用本组MR成像方案进行扫描，主要包括：轴面SE序列T2WI；平扫矢状面SE脉冲序列T1WI；增强轴面或矢状面SE脉冲序列T1WI；轴面或冠状面液体衰减反转恢复（FLAIR）序列，同时分析病变的发病部位、数目、形态、信号的变化和病变的强化情况等。结果：MRI可见下列3种表现：（1）多发性脑内错构瘤：30例中25例在SE脉冲序列T2WI和FlAIR脉冲序列见高信号病灶，病灶主要位于苍白球、小脑和脑干。另外，25例中20例可见海马回、海马旁回等区晕状高信号改变。（2）视通道或下丘脑胶质瘤：视神经、视交叉增粗、扭曲；视交叉或下丘脑肿块，SE脉冲序列T2WI和FlAIR序列表现为不规则分叶状混杂信号肿块，在增强SE脉冲序列T1WI有明显不规则强化。（3）脊柱多发性神经纤维瘤：SE脉冲序列T2WI和脂肪抑制短时反转恢复（STIR）序列显示高信号沿脊神经分布的多发性肿瘤。结论：MRI能够作为1种 常规的影像检查方法对NF1患者进行诊断和追踪。本组MR成像方案能较好地显示NF1的多发性或多灶性病变。     

鞍旁肿瘤的MR诊断及鉴别诊断

目的:探讨鞍旁肿瘤的MR影像学表现,提高诊断准确率.方法:回顾性分析64例经手术病理证实的鞍旁肿瘤MR影像学表现,所有病例均行MR平扫及增强扫描.结果:64例中脑膜瘤17例,侵袭性垂体瘤14例,神经鞘瘤9例,海绵状血管瘤6例,转移瘤6例,表皮样囊肿6例,脊索瘤4例,淋巴瘤2例.鞍旁脑膜瘤信号和强化表现与颅内其他部位脑膜瘤类似;侵袭性垂体瘤累及鞍旁,颈内动脉海绵窦段被包绕;神经鞘瘤以三叉神经鞘瘤多见,呈哑铃形跨颅中、后窝生长;海绵状血管瘤呈鞍内向鞍旁生长的哑铃形肿块,增强后强化显著,均匀强化或不均匀周边强化逐渐向内充填;鞍旁转移瘤多为双侧性,常见于鼻咽癌颅内侵犯;表皮样囊肿T1WI信号高于脑脊液,DWI上呈高信号改变;脊索瘤为不均匀信号,伴斜坡骨质破坏;鞍旁淋巴瘤可为双侧性,伴颅中窝底骨质破坏.结论:鞍旁肿瘤M R表现各有特点,把握其特征可作出正确诊断.     

神经纤维瘤病的脑部CT评价

目的：探讨神经纤维瘤病的脑部CT表现及其相关问题。方法：对15例经临床手术病理证实的神经纤维瘤病患者的临床资料与脑部CT资料进行了回顾性分析。结果：15例CT检查诊断为颅内肿瘤,其中双侧听神经瘤13例,脑膜瘤6例,单侧听神经瘤、三叉神经瘤、双侧脑室内肿瘤、胶质瘤各1例,侧脑室内脉络丛广泛钙化斑块6例。结论：CT扫描能直接全面地显示神经纤维瘤病在脑部的表现,对本病的诊断具有很高的价值。     

Spontaneous involution of a non-optic astrocytoma in neurofibromatosis type I: serial magnetic resonance imaging evaluation

A patient with neurofibromatosis type I (NF1) was followed-up with serial magnetic resonance imaging (MRI) studies over a period of 6 years. A contrast-enhancing lesion of the internal capsule, histologically proven to be pilocytic astrocytoma through stereotactic brain biopsy with mass effect and associated edema, was detected to reveal spontaneous involution on follow-up MRI studies. Although spontaneous regression of gliomas of the optic pathway-hypothalamus in patients with NF1 is relatively common in the literature, spontaneous involution of non-optic (i.e. areas other than optic pathways and hypothalamus) gliomas is rarely reported. Conservative management with follow-up MRI studies should be considered for non-optic glial tumors and tumor-like masses in patients with NF1, and surgical treatment should not be considered unless the lesions exhibit a rapid or unrelenting growth on serial MRI studies or produce significant clinical deterioration.     

Stone formation in substitution cystoplasty

We describe an unusual case of urinary bladder neurofibromatosis replaced by ileocecum with large stone formation in the neoformed bladder.     

FDG-PET/CT findings of sarcomatous transformation in neurofibromatosis: a case report

We herein report FDG-PET/CT findings of sarcomatous transformation in a patient with neurofibromatosis type 1 (NF-1). About 5% of patients with NF-1 develop sarcomatous transformation of a malignant peripheral nerve sheath tumor which arises from plexiform neurofibromas and is often associated with a poor prognosis. Morphologic imaging techniques such as Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) are the standard methods to define the anatomic extent of the tumor, although tumor heterogeneity prevents reliable differentiation between benign and malignant lesions. The degree of fluoro-deoxyglucose (FDG) uptake correlates with histologic grade in neurogenic tumors in NF-1 patients. Our patient had a huge mass in the left gluteus area with a large nearly circular focus of increased FDG uptake in the tumor. The mass had a photopenic center. The maximum Standard Uptake Value (SUVmax) of this mass was 6.6. There was CT evidence of invasion of the left iliac wing, left acetabulum, and left superior pubic ramus; however there was no increased FDG uptake in these areas on the PET study. We surmised that the high FDG uptake indicated a high grade sarcoma, which was confirmed histologically. There was also a focal region of increased uptake in the L5 vertebral body, correlating with the CT hypodense lesion, with 2.9 SUVmax. FDG-PET/CT can identify sarcomatous change from benign neurogenic tumor with minimal misregistration, and can also detect metastatic disease. This case illustrates the importance of evaluating both metabolic and morphologic abnormalities to be able to formulate a proper treatment plan. This information can be obtained in a single session, using PET/CT.     

整体功能态在航天医学研究中的意义

航天员整体功能改变是航天医学研究方面的重要问题，但尚未得到解决根据在不同因素作用下神经、内分泌和免疫系统生理功能改变和相互关系，提出航天员在航天中可能出现的三种整体功能态类型，即抑制型、应激型和血瘀型。并提出了关于航天员功能态研究的几个方面。     

A case of a patient with neurofibromatosis type I who developed pneumothorax and eosinophilic pleural effusion after suffering from COVID-19 pneumonia

Coronavirus disease 2019 (COVID-19) has become a global pandemic since its discovery in December 2019, and as the disease continues to evolve, varying complications associated with it continue to arise. In this regard, computed tomography has played an extremely important role in the diagnosis and evaluation of COVID-19 pneumonia and its complications. We encountered a case of a male patient with neurofibromatosis (type I) who developed concurrent pneumothorax and pleural effusion during his recovery period from severe COVID-19 pneumonia. Pulmonary fibrosis and emphysema were also confirmed. Furthermore, an eosinophil pleural effusion appeared and was prolonged during the healing process of COVID-19. This clinical presentation suggests that fibrosis and emphysema formation due to neurofibromatosis may have caused pneumothorax and pleural effusion.     

Deep-seated segmental neurofibromatosis without café au lait spots

Segmental neurofibromatosis is a rare disease characterized by neurofibromas with or without café au lait spots localized to one segment of the body. The majority of reported cases have had cutaneous neurofibromas, and patients with deep involvement have rarely been described. We report on two patients with deep-seated segmental plexiform neurofibromatosis and review the literature. All reviewed cases including the present two had no café au lait spots, axillary freckling, Lisch nodules, family history or malignant progression of disease. Differential diagnoses from neuro-fibromatosis 1 (von Recklinghausen disease) and malignant peripheral nerve sheath tumor are important for genetic counseling and avoiding overtreatment. Received: 18 January 2000 Revision requested: 7 March 2000 Revision received: 12 April 2000 Accepted: 15 April 2000     

鲁中南地区先天性心脏病患者的ABO血型相关性研究

目的研究先天性心脏病发生与ABO血型抗原分布关系。方法选择鲁中南地区先天性心脏病患者444例,采用血型群体遗传学及血型血清学方法,进行ABO血型表现型及基因频率分布调查统计。结果发生先天性心脏病的ABO血型抗原分布与对照组无显著性差异。结论提示发生先天性心脏病与患者ABO血型间无显著相关性关系,血型因素不是重要的先天性心脏病易感因素。