MRI of autosomal dominant pure spastic paraplegia

We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a “corpus-callosum index” expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and “corpus-callosum index” than controls. This finding, not reported previously, might indicate that the disease process in pure HSP is not confined to the spinal cord. The anteroposterior diameters of the spinal cord at T 3 and T 9 were significantly smaller in patients than in controls. This might correspond to the degeneration of the pyramidal tracts and the dorsal columns described at neuropathological examination. Received: 8 August 1996 Accepted: 16 December 1996     

MRI and CT in an autosomal-dominant, adult-onset leukodystrophy

We report MRI findings in a family with an autosomal-dominant, adult-onset neurological disorder. The clinical picture, the white matter changes detected on MRI and the absence of any laboratory abnormality suggested the diagnosis of leukodystrophy with an unknown biochemical defect. Autosomal-dominant inheritance is extremely rare in this kind of disease, and most reported families have not undergone MRI. We performed MRI and clinical examination of 17 members of our family; 9 affected subjects, at different stages of the disease, were detected. The most characteristic MRI findings were initially symmetrical areas of signal change in the white matter of the trigonal region; demyelination extending thereafter to the frontal and parietal regions, partially involving subcortical white matter; the temporal lobe and optic radiations were less involved; the internal capsule and corpus callosum were involved later, in a dorsoventral direction; patchy demyelination was evident in the late stages in the brain stem; the cerebellum was spared even in the latest stages of the disease. While pathological examination is essential to characterise and classify these kinds of diseases, MRI can make substantial contributions to understanding their natural history, and to detect early signs of the disease. Received: 6 June 1996 Accepted: 9 August 1996     

脑膜型囊虫病的磁共振成像诊断

目的探讨磁共振成像(MRI)对脑膜型囊虫病的诊断价值。资料与方法回顾性分析经临床证实的21例脑膜型囊虫病的MRI表现特点。2l例均行MR平扫，7例同时行MR增强扫描。结果21例脑膜型囊虫病中，单纯性脑膜型囊虫8例(38．1％)，混合型13例(61．9％)。其中19例脑膜型囊虫多发，呈葡萄串状生长，2例单发，呈分叶状。囊虫发生于外侧裂9例，鞍上池6例，小脑桥脑角池4例，前纵裂池1例，同时发生于鞍上池与小脑桥脑角池1例。囊虫囊泡在T1WI上等于或稍高于脑脊液(CSF)信号，T2WI上等于CSF信号。囊泡壁在轴面T1WI上显示较为清楚，一般呈边缘光滑的细线样稍高信号影，增强后轻度强化；所有脑膜型囊虫内均未见头节显示。结论MRI是诊断脑膜型囊虫病的首选影像学检查方法。     

骨母细胞瘤的MRI诊断价值

目的:探讨骨母细胞瘤的MRI表现及其诊断价值。方法:对 10 例均行 MRI平扫和增强检查并经手术病理证实的骨母细胞瘤病例进行回顾性分析。结果:脊柱6例,颅骨3例,骨盆1例。骨母细胞瘤以脊柱好发。MRI检查多呈不均匀性长T1、T2 囊状膨胀性骨质破坏,增强扫描多呈不均匀性强化,部分见液 液平和/或软组织肿块。结论:MRI能较准确地显示肿瘤形态、部位、内部结构及与邻近结构的关系,平扫和增强 MRI检查对骨母细胞瘤定性诊断有较高的诊断价值。     

肝门区胆管癌的MRI应用价值

目的 探讨肝门区胆管癌(HC)的MRI检查技术及应用价值。资料与方法 HC34例，所有病例均行MR一体化扫描，包括MR平扫、MRCP、MR动态增强和MR动态增强血管成像(3D-DCE-MRA)，并作出能否手术切除的评价，与手术结果相对照。结果 34例HC均可见肝门肿块、肝内胆管扩张、肝门胆管中断，动态增强扫描31例表现为延迟强化，3D-DCE-MRA中6例在动脉期表现为螺旋样动脉，18例可见门静脉受侵，表现为门脉侧壁浸润、门脉缩窄或闭塞，MRI对能否手术切除评估的准确性为88．2％(30／34)。结论 MRI检查能充分显示HC病变及其侵犯范围，对HC进行准确的诊断和术前评估。     

SURF-1基因604G→C突变所致Leigh综合征的MRI表现

目的 探讨SURF-1基因604G→C突变引起的Leigh综合征(Leigh syndrome,LS)的MRI表现.方法 对8例确诊为SURF-1基因604G→C突变的Ls患儿进行头颅MR检查,观察基底节、下丘脑、脑干及小脑灰质核团,大小脑白质异常及萎缩情况,并与典型LS患儿MRI改变进行比较.结果 3例Ls患儿MRI表现为脑干和下丘脑核受累,其中2例同时合并基底节异常;3例仅有大脑白质异常信号,无灰质核团受累;8例均存在脑萎缩,其中2例仅表现为单纯的脑萎缩,无灰质核团受累及白质异常.结论 SURF-1基因604G→C突变患儿的MRI表现具有多样化的特点.     

周围型肝内胆管细胞癌的MR诊断价值

目的:探讨周围型肝内胆管细胞癌(PCC)的MRI诊断价值。方法:回顾性分析经病理证实的PCC患者14例,对MR平扫及动态增强扫描的资料进行分析。结果:14例PCC块状型8例,结节型4例,弥漫型2例。于T1WI、T2WI主要表现为混杂的异常信号,MR动态增强扫描13例动脉期瘤灶边缘淡薄环形或不规则强化、静脉期及延迟期扫描瘤灶内实性部分的信号呈渐进性、填充式增强,强化程度更加明显,1例动脉期扫描瘤灶边缘明显强化,延迟扫描瘤体边缘仍保持厚环状明显强化。其他表现包括瘤内含有扩张胆管7例,腹膜后淋巴结转移者5例,均无门静脉癌栓。结论:PCCMR平扫及动态增强扫描具有一定的特征性,可为PCC的MR诊断及鉴别诊断提供重要的根据。     

Moyamoya disease: diagnostic accuracy of MRI

Our purpose was to evaluate the diagnostic accuracy of MRI in moyamoya disease. We studied 30 patients with this disease, comparing MRI and angiographic findings. The diagnostic value of MRI was evaluated for occlusive lesions, collateral vessels, and parenchymal lesions. In all patients bilateral occlusion or stenosis of the supraclinoid internal carotid artery and proximal anterior and middle cerebral arteries was clearly shown by MRI, and staging of the extent of occlusion agreed with angiographic staging in 44 (73%) of 60 arteries. MRI, particularly coronal images, clearly showed basal cerebral moyamoya vessels in 54 hemispheres, and 45 of a total of 71 large leptomeningeal and transdural collateral vessels were identified. MRI also showed parenchymal lesions in 48 (80%) hemispheres, and the extent of occlusion in the anterior and posterior circulations respectively correlated with white matter and cortical and/or subcortical infarcts.     

MRI of acute cerebral infarcts: increased contrast enhancement with continuous infusion of gadolinium

We compared contrast enhancement on T1-weighted MRI of acute cerebral infarcts after conventional bolus administration and continuous infusion of gadolinium. We examined 12 patients with a history of acute stroke with contrast-enhanced MRI once a week for a 1 month. Only ischaemic lesions were investigated after cerebral haemorrhage had been excluded by CT. Each MRI study included T2- and proton density-weighted sequences for determination of the size and site of the infarct, immediate postinjection T1-weighted imaging after bolus administration of 0.1 mmol/kg gadolinium-DPTA and delayed T1-weighted imaging after additional continuous infusion of 0.1 mmol/kg over 2 h. A total of 42 MRI studies was performed. In the first week after the onset of stroke, most infarcts (8 of 10) did not enhance after bolus administration, whereas all showed distinct contrast enhancement after the infusion. In the following weeks all but two infarcts showed contrast enhancement after bolus administration; after continuous infusion contrast enhancement could be seen in all cases. While contrast enhancement after bolus administration showed the typical gyriform pattern, enhanced areas were more extensive after the infusion and usually covered the entire infarcted area shown on T2- and proton density-weighted images. We presume that the disturbed blood-brain barrier in ischaemic areas favours delivery of contrast medium to the infarcted tissue if it is offered continuously so that a steady state can develop. Received: 8 April 1998 Accepted: 1 September 1998     

Hypocaeruloplasminaemia with heteroallelic caeruloplasmin gene mutation: MRI of the brain

We present two patients with hypocaeruloplasminaemia and a heteroallelic caeruloplasmin gene mutation (HypoCPGM). These patients had diabetes mellitus and tremor of the hands, respectively. T2-weighted fast spin-echo MRI showed mildly reduced intensity of the putamen, much more marked on echo-planar imaging. Received: 26 May 1998 Accepted: 10 August 1998     

MRI联合磁共振静脉成像诊断脑静脉窦血栓形成的价值

目的:探讨MRI及磁共振静脉成像(MRV)对脑静脉窦血栓形成的诊断价值.方法:回顾性分析37例DSA确诊脑静脉窦血栓形成患者的临床资料及影像表现.37例中有30例行常规MRI检查,29例行MRV(2D TOF)检查.结果:30例常规MRI检查中28例静脉窦有异常信号,其中26例MRI表现为静脉窦T1WI、T2WI高信号,2例T1WI等信号、T2WI低信号,并部分伴有脑组织梗死、出血.29例MRV检查均发现静脉窦充盈缺损或中断.结论:MRI与MRV结合对诊断脑静脉窦血栓形成具有高度敏感性,是诊断静脉窦血栓形成的首选检查方法.     

卒中患者脑微出血的MRI特点及其临床意义

目的 探讨卒中患者脑微出血（CMBs）的MRI特点及其临床意义，并比较MRI不同扫描序列检出CMBs的能力。方法 卒中患者60例，分为脑缺血组（34例）和脑出血组（26例），以同期查体的60岁以上健康老年人为对照组（30例）。采用常规MRI（包括SE T1WI和FSE T2WI）、扩散加权成像（DWI）、梯度回波T2加权成像（GRE-T2WI）和平面回波成像（EPI）检查，分别统计各组CMBs、腔隙性梗死、脑白质稀疏情况，同时记录卒中患者的高血压、糖尿病、卒中病史，比较不同扫描序列检出CMBs的差异。结果 CMBs在缺血组、出血组和对照组的发生率分别为29．4％，61．5％和6．7％。CMBs最常见于基底节／丘脑区。CMBs与高血压、卒中病史相关（P〈0．01），而与糖尿病无关（P〉0．05）。CMBs的数目与腔隙性脑梗死的数目和脑白质的改变程度成正相关（P〈0．01）。常规MRI和DWI均不能显示CMBs，EPI与GRE-T2WI检出CMBs的差异无统计学意义（P〉0．05）。结论 脑卒中患者多发性CMBs的存在提示微血管病变的严重程度和出血倾向，对于临床治疗决策具有重要的指导意义。GRE-T2WI是检测CMBs的首选方法，EPI可作为GRE-T2WI的补充手段。     

脑曼氏裂头蚴的特征性MRI表现

目的：研究脑曼氏裂头蚴的常规MRI特征性表现。材料和方法：收集经手术病理确诊的6例脑内曼氏裂头蚴，回顾性分析其常规MRI表现，包括病灶分布、数目、形态、大小、信号及强化情况。结果：脑曼氏裂头蚴的常规MRI表现为：6例均单发病灶，病灶位置表浅，3例位于顶叶，1例位于额叶，1例位于颞叶，1例位于脑干后部；5例病灶形态不规则，3cm左右大小，T1WI呈不均匀低或略低信号，T2WI呈不均匀高信号，中央略高，周围高信号；5例强化后表现为多个小环状强化，并不规则相套，呈“绳结状”；1例表现为环形边缘强化，壁中等厚度，内外壁较光整，强化病灶周围均可见轻微水肿未见以往文献中描述的白质退变或出血。MRI3例诊断为裂头蚴，3例诊断为寄生虫感染。5例MRI表现“绳节状”强化病例手术中可见活虫，1例MRI表现环状强化病灶者，环内囊腔中仅见少量虫体碎片。结论：“绳结状”强化是脑内曼氏裂头蚴的典型MRI特征，与胶质瘤及其他感染性病灶有一定的鉴别诊断价值。但病灶呈单发囊腔表现时，易与脑脓肿及其他寄生虫感染混淆。     

寰枢椎失稳的MRI诊断价值

目的评价MRI在诊断寰枢椎失稳中的价值。资料与方法回顾性分析26例寰枢椎失稳的MRI表现和临床资料。结果骨性结构的改变包括骨性畸形11例,枢椎骨折12例,肿瘤所致寰枢椎椎体破坏3例。软组织改变包括横韧带断裂7例,咽后壁软组织挫伤出血3例,齿状突与前弓或两侧块间组织嵌入3例。颈髓的改变包括硬脊膜和颈段脊髓受压12例,脊髓信号改变10例。结论MRI对寰枢椎失稳的诊断具有重要价值。它能清晰显示骨性结构异常、韧带断裂情况及颈段脊髓损伤,因而对指导临床治疗有重意义。     

脑海绵状血管瘤:γ-刀治疗后的MRI表现

目的 分析脑海绵状血管瘤γ-刀治疗后的MRI表现,评价γ-刀治疗脑海绵状血管瘤的疗效。资料与方法 搜集脑海绵状血管瘤γ-刀治疗患者36例,MRI随访24个月,分析γ-刀治疗后病灶MRI征象及临床症状的演变。结 果36例患者γ-刀治疗后6个月,MR／示28例患者瘤周水肿增大,瘤体信号无明显变化,16例患者症状加重。γ-刀治疗后12个月MR／示7例瘤体缩小及其信号减低,25例瘤周水肿增大,16例症状减轻;γ-刀治疗后24个月MR／示19例瘤体缩小,26例瘤体信号减低,26例瘤周水肿缩小,其临床症状减轻。结论 MRI可以准确地显示脑海绵状血管瘤γ-刀治疗后脑部的病理演变及转归,与临床症状的演变基本一致。     

弥散加权MRI对急性和非急性脑梗死的鉴别诊断价值

目的：评价弥散加权(DWI)MRI对急性和非急性脑梗死的鉴别诊断价值。方法：对41例脑梗死(急性12例，亚急性14例，慢性15例)患者行头颅DWI和常规MRI扫描。结果：急性和亚急性脑梗死在DWI上均表现为高信号，其中8例急性脑梗死在DWI上比常规T2WI显示的更清楚或范围更大；而慢性脑梗死在DWI上表现为等信号、混杂信号或低信号。结论：DWI可为急性和非急性脑梗死的鉴别诊断提供可靠的依据。     

脑灌注MRI与脑血管造影在评价脑缺血病变中的价值

目的 通过对脑缺血性疾病患者的脑动脉血管造影和脑灌注ＭＲＩ对照，评价脑缺血性疾病中侧支循环建立情况，并试图鉴别脑缺血性病变的性质。     

早期脑缺血MR灌注成像的实验研究

目的 应用脑灌注ＭＲＩ技术研究早期脑缺血缺血血流动力学改变并探讨其临床应用价值。     

少见和不典型椎管肿瘤的MRI诊断和误诊分析

目的：探讨少见和不典型椎管肿瘤MRI误诊原因，提高对该类疾病的认识。资料与方法：对16例少见和不典型椎管肿瘤进行回顾性手术病理对照分析。结果：8例实性神经鞘瘤，7例MRI误诊为脊膜瘤，1例误诊为室管膜瘤；2例神经母细胞瘤误诊为硬膜外淋巴瘤和脊膜瘤；2例海绵状血管瘤误诊为神经鞘瘤；3例血管畸形误诊为室管膜瘤、神经鞘瘤和硬膜外脓肿：1例血管球瘤误诊为硬膜外淋巴瘤。结论：在评价椎管肿瘤方面，MRI具有绝对优势，根据肿瘤的位置、信号强度和均匀度，再辅以对比剂的恰当应用，不仅对典型的肿瘤能做出正确诊断，对不典型的和少见的椎管肿瘤也可提供更多可靠的信息。     

侧脑室内室管膜下瘤的MRI诊断

目的 研究侧脑室内室管膜下瘤的MRI表现，以提高对该病的神经放射学认识。资料与方法 搜集8例经手术病理证实的侧脑室内室管膜下瘤，男5例，女3例，发病年龄16-47岁，平均32．9岁。着重分析肿瘤的好发年龄、部位，MRI信号特点，肿瘤增强情况。结果 左侧脑室内4例，右侧脑室内3例，双侧脑室内1例，除1例位于双侧脑室体部及三角区外，其余病例皆发生于侧脑室室问孔附近，肿瘤漂浮在侧脑室中。肿瘤最大直径4cm，最小1cm。T2WI肿瘤呈均匀高信号；T1WI肿瘤呈等、低信号，肿瘤内部可见小囊状低信号区。增强扫描，肿瘤无强化或有轻微强化。结论 室管膜下瘤的MRI表现具有特征性，术前MRI检查可以对其作出明确的定位定性诊断。