原发性进行性失语的临床、影像及语言特征

目的 探讨原发性进行性失语（PPA）的临床、影像及语言特征.方法 PPA患者7例,其中语义性痴呆（SD）6例,进行性非流利性失语（PNFA）1例,收集患者的人口学资料、病史,进行头MRI检查,采用汉语失语成套测验进行语言评估.结果 患者平均发病年龄56岁,均缓慢起病,以语言表达、命名障碍为首发症状.MRI示左侧颞极萎缩为主,病程长的患者左侧额叶和顶叶、右侧颞叶也明显萎缩.语言评估发现所有患者的自发语言、复述、命名、听理解、阅读和书写均不同程度损害.SD患者言语流利,复述、朗读能力下降相对较轻,命名、复杂语句的理解能力损害突出.PNFA言语顿挫吃力,患者列名能力明显下降,但命名相对保存完好.结论 PPA多为老年前期发病,语言障碍为最早、最突出的症状.MRI特征性的改变为额叶和颞极萎缩,左侧为著.其中SD表现为命名性失语和经皮质感觉性失语,PNFA表现为经皮质运动性失语的特征.     

原发性进行性失语一例临床分析

目的 原发性进行性失语（PPA）是一种少见的中枢神经系统变性疾病，国内罕见报道。现报道1例，以提高临床医生对该病的认识。方法 采用韦氏一表、认知能力筛选检查、积木测验、数字广度测验和社会功能问卷等全套神经心理学量表方法，检查和描述了PPA的临床和神经心理学特征；并进行了MRI和PET影像学检查。结果 病人除有单纯性命名性失语外，不伴有其他类型的失语与智能损害及神经系统体征；MRI和PET检查均发现左颞叶明显萎缩。结论 PPA以缓慢进行性失语而不伴有认知功能障碍和神经系统体征为特点，优势半球局灶性额、颞叶病变有诊断意义。     

原发性进行性失语1例报告

原发性进行性失语(primary progressive aphasia,PPA)是指患者语言功能进行性下降2年或2年以上,早期日常生活能力和认知功能正常保留,病理上以额颞叶萎缩为特点,但不具有Pick小体的一种中枢神经系统变性疾病.PPA临床相对少见,国内只报道数例.我科近年诊断1例,报道如下.     

丘脑卒中与失语的神经心理学与BAEP分析

目的：研究丘脑卒中与失语的关系。方法：对２５例丘脑卒中的神经心得学检查结果进行分析，并与脑干听觉诱发电位（ＢＡＥＰ）检查结果对照。结果：发现左侧丘脑卒中可致失语。发生率为８２％，右侧丘脑卒中语言正常。有无丘脑失语ＢＡＥＰ异常率相近，均表现为Ⅲ－Ⅴ波波间期〉Ⅰ－Ⅲ波波间期，及Ｖ波，Ⅳ波潜伏期的延长。结论：丘脑内侧膝状体与脑干听觉通路其它结构与语言这一复杂心理活动无直接关系。     

原发性进行性失语伴精神障碍1例

1病例患者女,60岁,因＂渐起失语,记忆差,行为乱5年,加重1年＂而住院。于2005年起无明显诱因渐出现说话有时停顿,突然想不出要说物体的名称,明知道是什么却怎么也说不出来,后逐渐变得说话不流利,口吃,逐渐加重,2008年起出现反应迟钝,与之交谈只能用简单的语句作答,不能正确表达自己的想法,经常重复,迂回及赘述。     

进行性非流利性失语及其研究进展

进行性非流利性失语（PNFA）是一种以语言功能损害为主要特征的神经系统变性病,是原发性进行性失语（PPA）3种常见分型中的一种。PNFA起病隐匿且症状多样,影像学与相关语言量表结合临床表现对PNFA诊断及鉴别诊断具有重要作用。本文拟从PNFA的临床表现、影像学表现、语言学相关检测等方面进行综述。     

神经梅毒的临床及影像特征分析

目的分析神经梅毒的临床及影像特征。方法回顾性分析24例神经梅毒的患者的临床特征、实验室检查及神经影像学检查结果。结果 24例神经梅毒患者中实质型4例,间质型20例。所有患者均经梅毒螺旋体明胶凝聚试验(TPPA)、血清快速血浆反应素试验(RPR)显示为阳性,经脑脊液TPPA、RPR检查显示为阳性20例。神经梅毒患者影像学检查显示多样,但无显著特征。结论神经梅毒临床症状表现多样,对其进行血清及脑脊液的RPR、TPPA检查,并结合影像学检查,有利于早期明确诊断。     

Gerstmann综合征临床与神经心理学研究

目的研究Gerstmann综合征(GS)的临床与神经心理学特点。方法采用汉语失语症成套测评(ABC)、书写及视空间检查,对259例经CT或MRI检查确诊为脑卒中或脑损伤的患者进行评价。结果 (1)259例患者中有19例患有Gerstmann综合征(GS),其中9例有GS四主征、10例有GS的核心症状、命名性失语(AA)5例、经皮质运动性失语(TCM)2例、经皮质感觉性失语(TCS)4例、Wernicke失语3例、构音障碍2例、无失语5例;(2)病变部位:左顶枕、左侧颞顶、左侧颞顶枕、左侧额顶枕和左侧基底节共17例,右侧基底节、顶叶损害2例。结论 (1)Gerstmann综合征不是一个独立的综合征;(2)其病变部位不仅仅限于优势半球顶叶。     

非流利失语型额颞叶痴呆患者神经心理学特征及皮质代谢功能变化

目的 正规的认知功能评价结合脱氧葡萄糖正电子发射断层摄影术(FDG-PET)检查和影像学改变,分析非流利失语型额颞叶痴呆(FTD)患者的神经心理学特征.方法 选择临床、影像、皮质葡萄糖代谢功能以及神经心理学评价均符合非流利失语型FTD患者,动态评价其神经语言学以及多种认知功能.按其特殊的神经心理学特点结合FDG-PET分析神经心理学改变的皮质基础.结果 5例患者的核心症状均为隐袭起病、慢性进行性的非流利型失语,伴随的突出改变为视空间、字词级阅读保留好,记忆相对好,以非语言记忆保留更突出.生活适应能力损害相对晚.患者均有相应的皮质糖代谢改变基础.结论 非流利失语型FTD患者的视空间能力、字词级阅读能力以及生活适应能力损害晚,与相应的皮质代谢功能相关,可以帮助照料者有效指导、调整患者的适应能力训练.     

语义性痴呆患者的临床、影像及神经心理学特点

目的 探讨语义性痴呆( semantic dementia,SD)患者的临床、影像和神经心理学特点.方法 SD患者18例,详细收集患者的临床资料,进行头MRI检查和神经心理评估,神经心理评估包括语义记忆(物体命名任务)、总体认知功能评估[简易精神状态检查(Mini-mental State Examination,MMSE)]、视空间能力[画钟测验(Clock Drawing Test,CDT)]、日常生活能力[日常生活能力量表( Activities of Daily Living,ADL)]、精神行为症状[神经精神问卷(Neuropsychiatric Inventory,NPI)]及总体严重程度[改良额颞叶变性临床痴呆评定量表( Frontotemporal Lobar Degeneration Modified Clinical Dementia Rating Scale,FTLD-CDR)]的评估.结果 患者发病年龄(60.6±8.5)岁,5例于65岁后发病,均以找词困难、命名障碍为首发症状,10例主诉有记忆障碍,14例出现人格行为改变.5例曾被诊断为阿尔茨海默病( Alzheimer's disease,AD),1例诊断为精神分裂症,无一例患者此前被明确诊断为SD.语义记忆评估显示所有患者对生命类和非生命类物体的名称及功能均有明显遗忘.MMSE(10.94±8.86)分,16例患者命名完全错误,定向力、记忆力、计算力、阅读和书写能力亦有损害,但与命名比较相对较好.CDT临摹(4.61±0.85)分,14例完全正常.ADL (29.72±8.75)分,病程5年的患者日常生活能力全面下降.NPI(8.00±7.22)分,14例患者出现异常.FTLD-CDR显示所有患者的语言障碍得分最高.头MRI检查显示多数患者以左侧颞叶萎缩为主,但有1例患者右侧颞叶萎缩重于左侧.早期萎缩局限于左侧颞极,随病情进展,累及右侧颞极、左侧额叶和顶叶皮质.结论 SD多为老年前期发病,但1/3的患者发生于老年期.语义记忆障碍最突出,行为和人格异常亦常见.临床上存在较高的误诊率和漏诊率,最常被误诊为AD.在病程早期,患者的视空间及其他能力相对保留,病程5年的患者功能全面衰退.头颅MRI可反映疾病的进展过程和临床特征,但个别患者以右侧颞叶萎缩为主.     

Molecular neuroimaging in primary progressive aphasia with predominant agraphia

A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy. Fluorodeoxyglucose-F18 positron emission tomography showed hypometabolism in the lateral left frontal lobe, including Exner’s area. Beta-amyloid and tau-positron emission tomography scans were negative, indicating the etiology was not Alzheimer’s disease. The underlying neurodegenerative process is most likely related to TDP-43, although a 4-repeat tauopathy cannot be excluded. Following his clinical evolution, and ultimately identifying the underlying pathology from autopsy, will elucidate the etiology of this interesting clinical presentation.     

Reading disorders in primary progressive aphasia: A behavioral and neuroimaging study

Previous neuropsychological studies on acquired dyslexia revealed a double dissociation in reading impairments. Patients with phonological dyslexia have selective difficulty in reading pseudo-words, while those with surface dyslexia misread exception words. This double dissociation in reading abilities has often been reported in brain-damaged patients, but it has not been consistently shown in patients with neurodegenerative diseases.In this study, we investigated reading impairments and their anatomical correlates in various neurodegenerative diseases. First, we performed a behavioral analysis to characterize the reading of different word types in primary progressive aphasia (PPA). Then, we conducted a voxel-based morphometry neuroimaging study to map the brain areas in which gray matter volume correlated with the accurate reading of exception and pseudo-words.The results showed a differential pattern of exception and pseudo-word reading abilities in different clinical variants of PPA. Patients with semantic dementia, a disorder characterized by selective loss of semantic memory, revealed a pattern of surface dyslexia, while patients with logopenic/phonological progressive aphasia, defined by phonological loop deficits, showed phonological dyslexia. Neuroimaging results showed that exception word reading accuracy correlated with gray matter volume in the left anterior temporal structures, including the temporal pole, the anterior superior and middle temporal and fusiform gyri, while pseudo-word reading accuracy correlated with left temporoparietal regions, including the posterior superior and middle temporal and fusiform gyri, and the inferior parietal lobule.These results suggest that exception and pseudo-word reading not only rely upon different language mechanisms selectively damaged in PPA, but also that these processes are sustained by separate brain structures.     

Clinical and pathological characterization of progressive aphasia

OBJECTIVE: The clinical and neuropathological categorization of patients presenting with progressive aphasia is an area of controversy. This study aimed to characterize a large group of progressive aphasic patients from a single center (n = 38), first clinically by case note review, and then pathologically. METHODS: Hierarchical cluster analysis of the cases according to their clinical language deficits was used to establish an unbiased, data-driven classification. RESULTS: This analysis revealed two groups of cases corresponding to the syndromes of progressive nonfluent aphasia (n = 23) and semantic dementia (n = 15). Postmortem analysis showed a majority in both groups of pathologies from the spectrum of frontotemporal lobar degeneration: the most frequent were non-Alzheimer's disease (AD) tauopathy in the nonfluent cases (10 of 23) and frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions in the fluent cases (8 of 15). Despite rigorous exclusion of cases with clinically significant memory deficits or other cognitive impairments, the pathology of AD was present in approximately one third of each group (overall 12 of 38), although often with an atypical neuroanatomical distribution. INTERPRETATION: Progressive aphasia is best seen as a composite of two conditions, on both clinical and pathological levels: progressive nonfluent aphasia and semantic dementia.     

Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features

Four patients with the clinical syndrome of primary progressive aphasia and a nonfluent aphasia profile were followed up over a period of 3 to 5 years. Extensive neuropsychological data for three patients revealed a progressive, quantitative decline of language with relative stability of memory, visuospatial skills, and reasoning. Comportment and most activities of daily living were preserved even when speech was unintelligible. Although several aphasia types may be associated with primary progressive aphasia, a nonfluent aphasia profile and phonemic paraphasic errors are most useful in differentiating it from the much more common clinical syndrome, "probable Alzheimer's disease." The clinicopathological correlates of probable Alzheimer's disease differ from those associated with primary progressive aphasia. Therefore, the clinical distinction between the two syndromes may be important for predicting the underlying pathophysiologic changes during the life of the patient.     

Pediatric epilepsy surgery: neuroimaging, neuropsychology, and anticonvulsants

Neuroimaging and the neuropsychological evaluation are important components of the presurgical evaluation for epilepsy surgery. Advances in neuroimaging over the last decade, to a large part, underlie improvements in pediatric epilepsy surgery outcomes. The neuropsychological evaluation plays an important role in the evaluation of the older child and adolescent, particularly in the evaluation of mesial temporal sclerosis. However, its role in the young child being considered for surgery remains to be defined. This section reviews the definition of medical intractability, issues related to medication withdrawal during video-EEG monitoring, recent neuroimaging advances, and the neuropsychological evaluation.     

低血糖脑病的临床和神经影像学特点

目的 探讨低血糖脑病(HE)的临床和神经影像学特点.方法 回顾性分析49例HE患者的临床和神经影像学资料.结果 本组HE患者临床表现为意识障碍26例,精神行为异常19例,癫癎发作6例,言语不清、反应迟钝10例,偏瘫和单肢瘫5例,去脑强直发作3例.23例行头颅CT检查,除10例见有脑梗死和脑萎缩外余无异常改变.13例行MRI检查,7例异常,其中显示弥漫性皮质和海马受累者3例(2例同时波及基底节区,1例仅累及右侧大脑皮质,MRS示病变区乳酸峰无明显异常),胼胝体压部受损2例(1例患者10 d后病变恢复,1例伴内囊后肢受累),壳核和放射冠区白质受累各1例,表现为等或稍长T1、长T2异常信号,DWI呈高信号伴表观扩散系数(ADC)降低.结论 HE的临床表现多不典型;MRI有高度特异性,DWI序列对病变更敏感.     

脑桥梗死的临床与神经影像学特征

目的阐述脑桥梗死的临床-影像-解剖学关系。方法回顾性分析69例急性孤立性脑桥梗死患者的临床特征及其与神经影像学改变的对应关系。结果主要临床特征有病灶对侧的中枢性面瘫和肢瘫、眩晕、构音障碍、偏身麻木、饮水呛咳、眼球水平运动异常等。9例（13．04％）患者在发病早期呈现进展性过程；51例（73．91％）梗死灶位于脑桥旁正中区域；59例（85．51％）患者病灶位于脑桥中上部水平；45例血管造影患者中．9人有椎-基底动脉狭窄。结论脑桥梗死多发生在脑桥中上部的旁正中区域，主要由椎-基底动脉狭窄、高血压性基底动脉穿通支闭塞所致。偏瘫、眩晕、构音障碍、感觉异常及眼球水平运动障碍等为主要临床表现。经典的脑桥综合征少见。少数患者在病程初期呈现进展过程，大多数患者预后良好。     

Activities of daily living in progressive non-fluent aphasia, logopenic progressive aphasia and Alzheimer's disease

Background/Aims: This study examined functional changes in progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA) and Alzheimer's disease (AD) and the association between function, cognition and behaviour. Methods: 59 patients were assessed with the Disability Assessment of Dementia (DAD), Addenbrooke's Cognitive Examination Revised (ACE-R) and the Cambridge Behavioural Inventory Revised (CBI-R). Results: No differences between groups in basic and instrumental activities of daily living (ADLs), and total ACE-R scores were found; there were correlations between total DAD and ACE-R scores for PNFA and LPA. Over 12 months, PNFA showed the marked decline of basic ADLs, whereas all three groups showed marked decline of instrumental ADLs. Conclusion: PNFA, LPA and AD appear functionally similar when matched for disease duration. The rate of decline of ADLs depends, however, on disease diagnosis.