散发性Creutzfeldt-Jakob病的临床和影像学特点

目的探讨散发性Creutzfeldt-Jakob病(sCJD)的临床和影像学特点。方法回顾性分析4例sCJD患者的临床资料。结果4例sCJD患者均表现为亚急性起病,进行性痴呆,伴有肌阵挛;头颅MRI显示对称性或非对称性大脑皮质彩带样和(或)基底节弥散加权成像(DWI)高信号。结论sCJD的临床特点为进展性痴呆伴肌阵挛,头颅MRI特别是DWI出现高信号为其病变特点。     

Marchiafava-Bignami病的临床和影像学特点(附2例报告)

目的探讨Marchiafava-Bignami病(MBD)的临床及影像学特点。方法回顾性分析2例MBD患者的临床资料。结果本组2例患者均为男性,发病年龄分别为54、49岁,均有长期大量饮酒史。例1急性起病,以手抖、意识不清为主要症状。例2为慢性起病,以反应迟钝、记忆力减退以及头痛为主要症状。影像学检查示2例患者胼胝体区均存在异常病灶。给予B族维生素后患者情况均出现明显好转。结论 MBD临床表现各异,影像学检查常于胼胝体处发现异常,及时给予B族维生素治疗效果较好。     

Marchiafava-Bignami病9例临床分析

M arch iafava-B ignam i病(M BD)又称原发性胼胝体变性,临床以精神症状和多灶性神经系统体征为主要表现,我院自1997年3月~2005年8月共诊治该病9例,现分析报告如下。1临床资料1.1一般资料9例均为男性,年龄33~65岁,平均58岁;职业中工人6例,农民2例,市民1例;8例有长期持续饮酒史15~35年,每日饮酒量45~60度白酒250~600m l,空腹饮酒者6例,占66.7%。1例因胃部手术14年长期进食少,营养不良。1.2临床表现急性起病3例,亚急性2例,慢性起病4例,所有病例均出现精神症状,以兴奋躁动、谵妄、幻视、淡漠为主;智能减退7例,主要表现记忆力、计算力下降、定…     

Marchiafava-Bignami病合并皮质受累一例

原发性胼胝体变性（Marchiafava—Bignamidisease,MBD）是一种罕见的胼胝体病变,多为慢性乙醇中毒引起的胼胝体不同程度脱髓鞘或坏死,其临床表现多样,主要为智能减退、意识障碍、癫痫发作、精神行为异常等。     

散发型克-雅病影像学特点与临床症状分析

散发型克雅病患者共计5例,临床主要表现为快速进展性痴呆,发病时间1～4个月。其中2例脑电图呈现特征性周期性同步放电,4例脑脊液14-3-3蛋白检测阳性。MRI扩散加权成像均显示皮质或基底节对称性或非对称性"彩带"样高信号,提示扩散加权成像对克雅病的诊断敏感且具有特异性,可作为临床首选检查方法。     

桥脑梗死患者临床及影像学特点

目的分析经磁共振成像(MRI)证实的15例桥脑梗死患者的临床及影像学特点。方法通过对15例桥脑梗死患者进行常规MRI和弥散加权成像(DWI)扫描,比较同一层面的所有磁共振图像,分析其临床表现及影像学特点。结果除1例急性桥脑梗死患者的临床表现符合经典定位理论外,其余大部分桥脑梗死患者均以一侧中枢性面、舌瘫,肢体偏瘫为主要临床表现。15例患者经MRI和DWI扫描,均可发现梗死灶,其中1例为发病3 h内的超急性期患者,DWI可见病灶显影,T_2WI未见异常。随着时间的推移,DWI逐渐变为等信号和低信号。结论大部分桥脑梗死患者的临床表现与幕上大脑半球基底核区的梗死相似。DWI可以显示超急性期桥脑梗死病灶,DWI结合T_2WI有助于多次脑梗死患者责任病灶的确定。     

胼胝体变性六例影像学特点和临床表现

目的 探讨胼胝体变性(Marchiafava-Bignami disease,MBD)的影像学特点和临床表现.方法 对我院诊治的6例MBD患者进行回顾性分析,包括影像学检查(如头颅CT、MRI)、实验室检查、临床症状和随访情况.结果 6例患者MRI检查均表现为胼胝体肿胀及T1WI等或低信号,T2WI高信号,液体衰减反转恢复序列高信号,弥散加权成像示弥散受限.其中例1、例4和例6以急性意识障碍起病,CT和MRI检查除累及整个胼胝体外双侧半卵圆区也受累,预后较差.例2和例3以反应迟钝、记忆力下降起病,CT和MRI检查病变主要累及胼胝体膝部和压部,经积极治疗,患者基本恢复正常.例5以双下肢麻木无力、反应迟钝起病,MRI检查病变累及胼胝体压部及体部,经治疗后也恢复正常.结论 MBD临床表现复杂,但具有特征性影像学表现,其起病形式和影像学特征可作为反映其预后的重要因素之一.     

瘤样炎性脱髓鞘病的影像学特点观察

目的分析瘤样炎性脱髓鞘病(TIDD)影像特点,探讨磁共振(MRI)特别是强化和弥散加权成像(DWI)检查技术对于TIDD的诊断价值。方法对16例TIDD患者行头颅CT和MRI扫描。结果病灶在头颅CT均为低信号,在MRI平扫上呈T1WI低、T2WI高信号,强化像上可见强化,57%可见开环征,发病早期DWI可见高信号,FLAIR较T1WI及T2WI能显示更清晰的病灶及范围。结论使用多种影像技术可以辅助诊断TIDD,并与脑缺血或肿瘤相鉴别,能区分病灶所处时期,因此,影像检查是诊断TIDD的有效辅诊方法之一。     

儿童烟雾病临床和影像学特征

目的探讨儿童烟雾病的临床和影像学特征。方法详细询问患儿病史,进行体检和影像学检查。对儿童烟雾病患者的病因、临床特征、影像学等进行分析总结。结果发病年龄2岁2个月～12岁。6例临床表现为肢体无力或偏瘫,其中3例以此为首发症状;惊厥4例,头痛2例,头晕2例,感觉障碍1例。2例行头颅CT检查者均显示有缺血性改变。6例均行头颅磁共振成像(MRI)检查,4例显示急性脑梗死或小腔隙灶或软化灶,2例无脑实质无异常。6例头颅磁共振血管成像(MRA)检查,均显示颈内动脉虹吸部末端和大脑前或中动脉狭窄或闭塞,脑基底部有异常血管网形成。结论儿童烟雾病多表现为短暂性脑缺血发作(TIA),临床以肢体无力或偏瘫、惊厥为常见症状。MRI联合MRA检查是诊断儿童烟雾病的主要方法之一。     

散发性Creutzfeldt-Jakob病的临床、病理及影像学研究

目的探讨散发性Creutzfeldt—Jakob病(sCJD)的临床、病理及影像学特点方法同顾性分析12例sCJD患者的临床表现、脑电图(EEG)、影像学特点及病理资料。结果(1)本组男7例,女5例,平均发病年龄49岁;3例以视觉缺失急性起病,9例以智能下降,精神、行为异常或共济失调亚急性起病;12例均有痴呆、肌阵挛和锥体外系体征。(2)9例脑电图(EEG)表现典型的、1例表现不典型的三相波,(3)12例头颅MRI检查,5例出现双侧基底节区T2加权像WI对称性高信号;8例同时行弥散加权(DWI)扫描,均表现为额叶或／和枕叶DWI高信号,并有5例伴双侧基底节区对称性DwI高信号．(4)1例尸检及6例脑活检均具备CJD病理特点。结论sCJD在具备典型临床表现基础上,动态EEG及头颅MRI DWI扫描可为CJD的早期临床诊断提供依据?     

Diffusion Tensor Imaging and Fiber-Tracking in Marchiafava-Bignami Disease

Marchiafava-Bignami disease, a rare disorder most commonly seen in patients with a history of alcohol consumption, involves demyelination and subsequent necrosis of the corpus callosum. Diffusion tensor imaging demonstrates regional abnormalities in the corpus callosum that are not evident by conventional magnetic resonance imaging (MRI). Furthermore, fiber-tracking demonstrates significant disruption of axonal fiber bundles within the corpus callosum, most marked within the body, corresponding to the clinical finding of interhemispheric disconnection, which is characteristic of the disease.     

The value of diffusion-weighted imaging in the diagnosis of Marchiafava-Bignami disease: apropos of a case.

Marchiafava-Bignami disease (MBD) is characterized by demyelination and necrosis of corpus callosum encountered in chronic alcoholic patients. Etiology is the deficiency of vitamin B complex. Magnetic resonance imaging (MRI) in MBD typically reveals focal lesions of high T2 and FLAIR signal intensity in the corpus callosum. We here present a 42-year-old male alcoholic diagnosed as MBD on the basis of MRI and diffusion-weighted imaging (DWI) features. The patient totally recovered following appropriate vitamin B complex replacement therapy, despite reduced diffusion in the initial setting. This case report emphasizes on the important role played by MRI and DWI in the early diagnosis and follow-up of this potentially fatal disease.     

原发性胼胝体变性临床特点

目的探讨原发性胼胝体变性(MBD)的临床及影像学特点。方法回顾性分析7例MBD患者的临床资料。结果 7例均有大量饮酒史,临床上以意识障碍、精神症状、智能下降、反应迟钝、肢体震颤为主要表现。急性患者主要以意识障碍、精神异常起病;亚急性以快速进展的痴呆起病;慢性以智能下降、反应迟钝起病。患者MRI检查均可见胼胝体异常信号,为长T_1、T_2信号,Flair高信号。急性及亚急性起病者以胼胝体膨胀性改变为特征,慢性起病以"三文治征"为特征;2例伴胼胝体邻近白质病灶。结论 MBD多由于慢性酒精中毒所致,临床表现无特异性。MRI检查发现胼胝体对称性病灶有助于诊断。     

Clinicoradiologic subtypes of Marchiafava-Bignami disease

Abstract. The clinical diagnosis of Marchiafava-Bignami disease (MBD) has considerably changed during recent decades with brain MRI providing the opportunity of a reliable in-vivo diagnosis. However, semiologic and neuroimaging characteristics of the currently known spectrum of MBD have not been investigated systematically, and knowledge of clinicoradiologic associations is sketchy. We report an illustrative case with limited callosal involvement on MRI and a favorable outcome and have reviewed literature on clinical and radiologic features in 50 cases of MBD diagnosed in vivo since 1985. Our reviewed data suggest the differentiation of two clinicoradiologic subtypes: Type A is characterized by major impairment of consciousness, T2-hyperintense swelling of the entire corpus callosum on early MRI and poor outcome. Type B shows at most slight impairment of consciousness, partial callosal lesions on MRI and a favorable outcome. Differentiation of these clinicoradiologic subtypes may help resolve inconsistencies of the established clinical classification resulting from new insights into the clinical course and prognosis of MBD by structural neuroimaging.     

强迫症患者脑结构异常的多模态研究

目的探讨强迫症患者脑灰质和白质结构改变是否在同一样本中反映了相同环路的异常。方法对54例强迫症患者(强迫症组)和54名健康对照(对照组)进行3D结构磁共振成像扫描和弥散张量成像扫描。基于SPM分析软件,采用基于体素的形态学分析方法分析强迫症组全脑灰质体积与对照组的差异;基于FSL软件,采用基于纤维束示踪的空间统计学探讨强迫症组各向异性分数(fractional anisotropy,FA)与对照组的差异。结果与对照组相比,强迫症组左侧额中回、左侧前扣带和旁扣带脑回、左侧中央前回及右侧颞下回灰质体积减小(P<0.05,Alphasim校正),胼胝体体部和胼胝体膝部FA值减小(P<0.05,FWE校正)。结论强迫症患者的灰质体积和白质完整性均存在异常,且异常区域多位于皮质-纹状体-丘脑-皮质环路相关脑区,强迫症的灰、白质结构异常可能同时出现。     

12例儿童烟雾病的临床及影像研究

目的研究儿童型烟雾病（MMD）的临床及影像学特征。方法回顾性分析12例儿童型MMD的临床资料，观察其临床及影像学特点。结果本组病例女性居多，病程为反复多发．起病主要有短暂脑缺血发作、脑梗死、出血等形式，临床表现为肢体麻木、无力、头痛、智力减退等症状：数字减影血管造影（DSA）在脑底动脉环发现血管闭塞、狭窄及炯雾状毛细血管网形成，MRA对本病有一定阳性发现。结论对于儿童反复发作的脑卒中患者要考虑MMD的可能，目前仍依靠DSA确诊，MRA对本病有一定阳性发现。可考虑血管重建手术治疗。     

阿尔茨海默病脑磁共振研究

目的 了解Ａｌｚｈｅｉｍｅｒ病（ＡＤ）脑结构性变化的特点及与健康老人增龄性改变的差异。方法 对２２例ＡＤ和２９例健康老人分别按年龄为６０～６５岁、６６～７０岁和７０岁以上三组,ＡＤ为ａｄ１、ａｄ２和ａｄ３组,健康老人为ｎｏｒ１、ｎｏｒ２和ｎｏｒ３组。全部对象作ＭＲＩ检查。结果 ｎｏｒ３的灰质体积、总脑脊液体积及右侧海马体积与ｎｏｒ１和ｎｏｒ２相比有显著差异;与健康老人相比,ＡＤ的灰质体积,双侧海马体积显著降低,而总脑脊液、脑室外脑脊液、侧脑室、三脑室和四脑室体积显著升高。结论 与健康老人增龄性变化相比,ＡＤ脑结构性改变的涉及范围广,变化幅度大。     

Marchiafava-Bignami disease with recovery diagnosed by CT and MRI: demyelination affects several CNS structures

Summary Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism. Most reported cases have been diagnosed at autopsy. With CT and, especially, MRI it is possible to diagnose MBD in its early stages. Lesions of CNS structures other than the typical demyelination of the corpus callosum are described ante mortem in a patient with MBD. The more frequent use of CT and MRI in sudden onset encephalopathies of alcoholics could reveal the real incidence of MBD, and the consequent detection of other involved CNS systems might improve our knowledge about the aetiology, pathogenesis, prognosis and therapy of MBD.