肝豆状核变性患者流体智力的神经心理学研究

目的：通过神经心理学测验评估肝豆状核变性（HLD）患者的流体智力变化特点，探讨HLD患者流体智力变化的发生机制。方法：选取102例HLD患者为研究对象，根据颅脑影像学有无异常分为CT／MRI阳性组（70例）和CT／MRI阴性组（32例X另选正常对照组30例。记录瑞文标准推理测验（R＇SPM）总分和分项结果。结果：3组间比较在R’SPM总分、知觉辨别、系列关系及抽象推理上差异有显著统计学意义（P〈0．01）；在R’SPM比较推理上差异有统计学意义（P〈0．05）。与对照组比较，CT／MRI阳性组在R’SPM总分、知觉辨别、系列关系及抽象推理上差异有显著统计学意义（P〈0．01），在R’SPM类同比较、比较推理上差异有统计学意义（P〈0．05）；与CT／MRI阴性组比较，CT／MRI阳性组在R’SPM系列关系上差异有显著统计学意义（P〈0．叭），在总分、知觉辨别、抽象推理上差异有统计学意义（P〈0．05）。CT／MRI阳性组R’SPM与病程呈负相关（P〈0．05，P〈0．01）。结论：CT／MRI异常HLD患者存在流体智力缺陷；基底神经节器质性破坏及大脑广泛性萎缩可能是HLD患者流体智力缺陷的重要机制之一。     

脑型肝豆状核变性MRI诊断价值

目的：分析脑型肝豆状核变性（hepatolenticular degeneration ,HLD）的MRI特征,探讨MRI对脑型 HLD的诊断价值。方法回顾性分析经临床证实的52例脑型 HLD患者的颅脑MRI资料,所有患者均行MRI平扫及DWI成像。结果8例M RI平扫无明显异常,DWI呈等信号。38例表现为双侧基底节区及丘脑对称性分布的稍长 T1、稍长 T2信号,DWI呈稍高信号,其中11例累及中脑及脑桥,呈稍长T1稍长T2信号,4例双侧小脑齿状核呈长T2、长 T1信号影。6例表现为双侧基底节对称性短T1短T2信号,DWI呈稍低信号。36例见不同程度脑萎缩。结论 MRI对脑型HLD有很高的诊断价值,可作为影像检查首选方法,其信号变化可反映病程、判断疗效及预后。     

CT、核磁共振、诱发电位对多发性硬化的临床诊断价值

目的探讨CT、核磁共振（MRI）、诱发电位检查在多发性硬化（MS）临床诊断中的应用价值。方法对60例MS患者CT、核磁共振（MRI）、诱发电位检查结果与临床对比分析、结果MRI检查异常率最高．为85．1％，CT为41．7％．VEP为60％，SSEP为54．2％，BAEP为45．8％。如同时进行两项以上诱发电位检查．只要一项异常即判断为诱发电位异常，其异常率可达81．4％、结论CT、MRI、诱发电位检查结合应用可提高MS诊断的准确性。     

早期血糖水平对老年脑梗死132例近期预后影响

本文收集我院1998年1月至12月132例老年脑梗死病人进行回顾性分析，以探讨老年脑梗死病人早期血糖水平对近期预后的影响，以便更加合理治疗。1资料与方法1．1研究对象132例患者均符合1995年全国第四届脑血管病会议制定的脑梗死诊断标准，并经头颅Cf或MRI确诊。男78例，女54例。年龄65~90岁，平均72.6岁。其中16例（12．1％）为Ⅱ型糖尿病，均符合1985年WHO修定的糖尿病（DM）诊断标准。有高血压病史89例（67．4％），第二次脑梗死础冽（30．3％），冠心病史41例（31．l％），房颤14例（10．6％），脑萎缩49例（37．l％），脑白质脱髓鞘2…     

肝纤维化血清学指标对肝豆状核变性家族中无肝豆状核病变诊断依据的同胞兄妹的临床诊断意义

目的：探讨肝纤维化血清学指标对肝豆状核变性（HLD）家族中无肝豆状核病变诊断依据的同胞兄妹的诊断意义。方法：选取94例HLD家族中无肝豆状核病变诊断依据的同胞兄妹经检查确诊为HLD者为HLD组；70例HLD家族中无HLD病变诊断依据的同胞兄妹经检查排除HLD者为非HLD组；70名健康自愿者为健康对照组。对3组肝纤维化血清学指标透明质酸（HA）、层黏连蛋白（LN）及Ⅳ型胶原（Ⅳ-c）用放射免疫法进行检测和分析。结果：HLD组HA、LN和Ⅳ-C平均值分别为（139．26±74．15）ng·mL^-1、（126．85±24．44）ng·mL^-1。和（82．02±31．23）ng·mL^-1；明显高于非HLD组（51．32±19．54）ng·mL^-1、（65．72±20．35）ng·mL^-1、（34．82±11．56）ng·mL^-1及健康对照组（46．75±20．93）ng·mL^-1、（68．92±22．17）ng·mL^-1和（33．57±12．39）ng·mL^-1，P均〈0．0l；非HLD组与健康对照组间差异无统计学意义，P均〉0．05。结论：肝纤维化血清学指标检测可作为HLD家族中无HLD病变诊断依据的同胞兄妹筛查和临床诊断HLD的指标之一。     

肝豆状核变性广泛大脑皮质病变的MRI特征

目的探讨肝豆状核变性(HLD)广泛大脑皮质病变的MRI特征。方法回顾性分析3例临床确诊HLD患者的临床资料。结果本组3例HLD患者中男1例,女2例;年龄14~18岁,平均(16.0±1.6)岁;病程3个月~4年,平均(2.4±1.6)年。本组患者均以言语不清为首发症状,主要临床表现为动作笨拙、行走不稳等。头颅MRI检查示双侧皮质及皮质下白质广泛对称性分布大片状长T1、长T2异常信号,Flair相呈高信号,部分同时存在混杂信号,无强化效应;其中2例还伴脑萎缩,脑室系统异常扩大。结论 HLD患者MRI表现除典型基底节区病变外,还可以出现脑多部位受累。对于青少年期起病,临床表现为锥体外系症状,头颅MRI示广泛大脑皮质病变者,应行铜代谢检查以鉴别HLD的诊断。     

磁共振成像及脑脊液细胞学对病毒性脑（膜）炎的诊断性研究

目的探讨MRI和脑脊液细胞学检测阳性率、异常程度和发病时间对病毒性脑（膜）炎诊断和制定治疗方案的指导价值。方法回顾189例病毒性脑（膜）炎患者的头部MRI及脑脊液检查资料,分析MRI正常或异常患者在疾病早期的脑脊液细胞学改变。结果189例患者中96例（50．79％）呈现MRI异常影像、129例（68．25％）脑脊液细胞学检测异常。MRI异常患者中脑脊液细胞学检测异常率为72．92％（70／96）,与MRI正常患者（63．44％,59／93）比较差异具有统计学意义（P=0．000）。结论病毒性脑（膜）炎患者在疾病早期MRI改变晚于脑脊液细胞学改变,但二者对提示诊断和制定治疗方案均具有重要临床意义。     

肝豆状核变性的磁共振影像学所见及其形成机理

肝豆状核变性(HLD)，是以遗传性铜代谢障碍所致的肝硬变和以基底节为主的脑部变性为特征的疾病。HLD的磁共振影像(MRI)研究较少，自1990年以来笔者观察了12例HLD患者的MRI改变，为研究HLD患者MRI表现与临床症状及治疗效果的关系提供依据。     

肝型和无症状型肝豆状核变性颅脑磁共振分析

目的观察肝型和无症状型的肝豆状核变性颅脑MRI表现。方法对我院2014年5月-2016年5月收治的97例肝型和28例无症状型WD患者,均行头部MRI检查。结果 125例患者中颅脑磁共振检查发现有108例患者(87.8%)有脑部异常信号样改变,其中肝型83例,无症状型25例;83例有脑萎缩改变,肝型63例,无症状型20例。结论肝型和无症状型肝豆状核变性患者颅脑MRI异常信号比例较高,主要集中在壳核;信号改变主要为对称性长T_1和长T_2,未发现有混杂样信号改变;病程与脑干和壳核相关,发病年龄与壳核、苍白球、尾状核密切相关;Child-Pugh分级与颅脑磁共振改变没有明显相关性。     

肝豆状核变性患者的执行功能障碍

目的 通过神经心理学测验评估肝豆状核变性(HLD)患者的执行功能变化特点,探讨其发生机制. 方法 选取102例HLD患者为研究对象.根据颅脑影像学有无异常分为CT/MRI阳性组(70例)和CT/MRI阴性组(32例),另选择30名健康志愿者做为正常对照组.分别记录测试者瑞文标准推理测验(R'SPM)、Stroop色-字干扰测验(CWT)、连线测验OMT)的结果. 结果 3组比较在R'SPM总分、知觉辨别、比较推理、系列关系、抽象推理,CWT的字义干扰时间、颜色十扰时间,TMT的B项耗时数及干扰量上差异有统计学意义(P<0.05).结论 HLD患者存在执行功能障碍,CT/MRI阳性组的损害严重程度和范围更为明显.额叶-纹状体环路上的破坏可能是HLD患者执行功能障碍的重要机制之一.     

50例肝豆状核变性临床分析

目的探索肝豆状核变性的临床特点,为早期诊断提供依据。方法我院1988～2008年收治的50例肝豆状核变性患者,对其临床表现,实验室检查及CT和MRI检查进行分析。结果铜代谢异常损害以肝脑为主。结论青少年患者以肝硬化、贫血、骨骼畸形、锥体外系损伤临床表现为首发症状,无常见病因可寻,应考虑到本病的可能,尽早进行检查,以便能早期确诊及时治疗。     

肝豆状核变性56例临床分析

目的回顾分析肝豆状核变性患者临床资料,总结其临床特点。方法回顾分析1994-01～2011-01本院收治的56例肝豆状核变性患者临床资料。结果该病从发病到确诊时间中位数2.6 a;临床表现以肝损害为主26例,以神经系统损害为主16例,二者兼有14例;角膜K-F环阳性42例,血铜蓝蛋白降低52例,头颅CT或MRI阳性31例。结论该病从发病到确诊时间长。临床表现主要以肝损害为主或以神经系统损害为主或二者兼有,血铜蓝蛋白测定、K-F环检查、头颅CT和MRI检查对诊断具有重要意义。     

Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders? An analysis of 100 cases

Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only “face of the giant panda” sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5–40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty‐six patients had WD (M:F = 28:30, age at onset: 14 ± 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 ± 9.8 years) that included Huntington's disease‐4, young‐onset Parkinson's disease‐7, mitochondrial disorders‐2, Hallervorden‐Spatz disease‐8, non‐Wilsonian hepatolenticular degeneration‐2, toxic/metabolic disorder‐1, and others‐20. The duration of illness at the time of MRI was comparable (WD: 3.1 ± 4.9 years; Other EOEPD: 2.8 ± 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: “Face of giant panda” sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)‐like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides “Face of giant panda” sign, hyperintensities in tectal‐plate and central pons (CPM‐like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD. © 2010 Movement Disorder Society     

Proton magnetic resonance spectroscopic imaging for metabolic characterization of demyelinating plaques.

We used proton magnetic resonance (MR) spectroscopic imaging to determine the serial changes in MR signals from choline, creatine, lactate, and N-acetylaspartate in and around a large demyelinating lesion followed over a period of 8 months. Elevated lactate and choline signals were observed at the first examination 3 days after the onset of symptoms. Reduced N-acetylaspartate signals were observed a few days afterward. The abnormal metabolite signals varied in different regions of the lesion and extended beyond the borders of abnormal signal intensity seen on conventional MR imaging (MRI). On the last examination at 8 months when the lesion appeared much smaller on MRI, choline signals from the center of the lesion were still high, but were falling. Choline signals outside the lesion on MRI had returned to normal as had lactate signals everywhere. Importantly, there was no recovery of N-acetylaspartate signals in or adjacent to the lesion on MRI. This serial study demonstrates the potential of MR spectroscopic imaging for characterizing the chemical pathological evolution of demyelinating lesions in ways that conventional MRI cannot. We propose that abnormal signals from choline can indicate recent regional demyelination, while persistent abnormal signals from N-acetylaspartate can provide an index of irreversible damage in the nervous system.     

脑型肝豆状核变性临床特征与MRI分析(附12例报告)

目的:探讨肝豆状核变性的临床特点与头颅MRI特征,为早期诊断及治疗提供参考。方法:回顾性归纳分析12例肝豆状核变性患者的临床表现及神经影像学特点。结果:平均发病年龄23.5岁;以锥体外系症状为首发10例;性格改变1例;学习成绩下降1例,K-F环均为阳性;头颅MRI特征为对称性基底节区、丘脑、中脑及桥脑异常信号。结论:肝豆状核变性临床表现多样,血清铜蓝蛋白检测、角膜K-F环及头颅MRI检查对诊断本病有重要意义。     

Brain magnetic resonance imaging findings in relapsing neuromyelitis optica

BACKGROUND: Some studies showed abnormalities in brain magnetic resonance imaging (MRI) of relapsing neuromyelitis optica (R-NMO) from 12 to 46%. These abnormalities are described as compatible/non-compatible with multiple sclerosis (MS). OBJECTIVE: To describe the abnormal brain MRI lesions in R-NMO with imaging studies conducted with more sensitive white matter change techniques. METHODS: Thirty patients with R-NMO were selected. All MRI brain studies were performed with a 1.5-T Siemens MRI system according to the Standardized MR Imaging Protocol for Multiple Sclerosis from the Consortium of MS Centers Consensus Guidelines. RESULTS: Brain MRI images were evaluated in 29 R-NMO cases because in one case the MRI images were not appropriate for the study. Of these 29 brain MRI studies, 19 cases (65.5%) had at least one or more lesions (1-57) and 10 were negative (34.4%). Brain MRI findings in 19 cases were characterized in T2/fluid-attenuated inversion-recovery (FLAIR) by the presence of subcortical/deep white matter lesions in 16 (84.2%) cases (1-50), most of them <3 mm and without juxtacortical localization. Periventricular lesions were observed in 13 (68.4%) cases, but morphologically they were not oval, ovoid or perpendicularly orientated. Infratentorial lesions, all >3 mm, were observed in 4 (21.05%) cases without cerebellar involvement. T1 studies demonstrated absence of hypointense regions. Optic nerve enhancement was observed in 6/19 patients (31.5%). None of the brain MRI abnormalities observed were compatible with Barkhof et al. criteria of MS. CONCLUSIONS: This study, based on a Cuban patient population, with long duration of disease, good sample size and detailed characterization by MRI, demonstrated the brain MRI pattern of R-NMO patients, which is different from MS.     

Serial MRI findings of intracranial tuberculomas: a case report and review of the literature]

Intracranial tuberculoma is an infectious disorder, occurring with or without tuberculous meningitis. Although intracranial tuberculoma is rare in developed countries, its frequency has increased in recent years in association with aging and immunocompromised hosts. Because of the low sensitivity of Mycobacterium tuberculosis cultures or of DNA detection from cerebrospinal fluid, diagnosis of intracranial tuberculoma is often difficult. Conventional magnetic resonance (MR) imaging of the tuberculoma yields variable results and is indistinct from other inflammatory lesions or brain tumors. We report the case of a 74-year-old woman with progressive neurologic deterioration. MR imaging of the brain showed multiple ring-like enhancing lesions in the supra- and infra-tentorial regions, mimicking multiple metastatic brain tumors. Diffusion-weighted imaging (DWI) of the brain showed homogeneous high signals in each lesion. A cavity in the lung suggested systemic involvement of tuberculosis. Despite extensive examination, tuberculosis could not be detected. Nevertheless, anti-tuberculosis treatment was administered. The patient's neurologic condition initially deteriorated for 4 weeks, then gradually improved. MRI showed marked improvement of the lesions after anti-tuberculosis treatment. Whereas conventional MRI is not specific in such cases, DWI might be useful for early assessment of intracranial tuberculosis.     

Structural and metabolic damage in brains of patients with SPG11-related spastic paraplegia as detected by quantitative MRI

The goal of this work was to assess brain structural and metabolic abnormalities of subjects with SPG11 and their relevance to clinical disability by using quantitative magnetic resonance (MR) metrics. Autosomal recessive hereditary spastic paraplegia (AR-HSP) with thin corpus callosum and cognitive decline is a complex neurological disorder caused by mutations in the SPG11 gene in most cases. Little is known about the process leading to corticospinal and white matter degeneration. We performed conventional MRI/MR spectroscopic imaging ((1)H-MRSI) examinations in 10 HSP patients carrying an SPG11 mutation and in 10 demographically matched healthy controls (HC). We measured in each subject cerebral white matter hyperintensities (WMHs), normalized global and cortical brain volumes, and (1)H-MRSI-derived central brain levels of N-acetylaspartate (NAA) and choline (Cho) normalized to creatine (Cr). Clinical disability was assessed according to patients' autonomy in walking. Conventional MRI showed WMHs in all patients. Global brain volumes were lower in patients than in HC (p < 0.001). Decreased values were diffusely found also in cortical regions (p < 0.01). On (1)H-MRSI, NAA/Cr values were lower in SPG11 patients than in HC (p = 0.002). Cho/Cr values did not differ between patients and HC. Cerebral volume decreases and NAA/Cr in the corona radiata correlated closely with increasing disability scores (p < 0.05). Quantitative MR measures propose that widespread structural and metabolic brain damage occur in SPG11 patients. The correlation of these MR metrics with measures of patients' disease severity suggests that they might represent adequate surrogate markers of disease outcome.     

垂体MRI成像在儿童矮小症的应用

目的探讨儿童矮小症垂体MRI成像的表现和应用价值。方法 128例诊断为儿童矮小症的患儿,男83例,女45例,年龄4岁至15岁,平均9.92±2.82岁,接受垂体MRI扫描,对MRI影像进行分析。结果 128例患儿中垂体腺瘤9例(其中垂体微腺瘤6例),占7.03%;垂体发育不良18例,占14.06%;空泡蝶鞍10例,占7.81%;垂体柄异常变2例(变细、扭曲),占1.56%;垂体后叶缺失4例,占3.12%。垂体形态器质表现异常43例,占33.59%,垂体形态大小表现正常85例,占66.41%。结论垂体磁共振成像在儿童矮小症的检查中有重要作用,有助于了解病因和协助诊断。