原发性进行性失语综合征新认识

原发性进行性失语综合征是一种以进行性语言障碍为惟一或突出临床特征的痴呆综合征,言语障碍最显 著,进展也最迅速。无有效治疗。     

原发性进行性非流利性失语的研究进展

1概况原发性进行性非流利性／语法变异型失语（Thenon-flu—ent／agrammatic variant of primaryprogressiveaphasia,PN—FA）是额颞叶痴呆（Frontotemporal dementia,FTD）三种临床分型的一种,同时根据其临床语言能力缺陷的特点来说它也属于原发性失语（Primaryprogressiveaphasia,PPA）一种。     

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。     

不同量表对原发性进行性失语亚型的语言学分析

目的通过简易精神状态量表(MMSE)和蒙特利尔认识评估量表(Mo CA)中语言方面的损害进而应用不同语言学量表对原发性进行性失语(PPA)的亚型进行分析。方法对认知障碍门诊收集的2例以语言障碍和记忆力差伴命名困难为主要表现的患者,结合MMSE和Mo CA中语言某方面的异常,应用语言学量表进行流利性、言语产生(语法和运动言语)、命名、单词理解、复述及阅读的分析,最后结合患者病史及头颅MRI或SPECT检查作出初步诊断。结果例1和例2患者通过MMSE、MOCA和相关语言学量表检测,结合影像学头颅MRI或SPECT检查,得出例1拟诊为Logopenic型失语,例2拟诊为语义型痴呆。结论结合MMSE和Mo CA中语言某方面的异常和相关的语言学量表检测,最后根据病史及影像学检查有助于PPA亚型的诊断。     

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。     

Logopenic型进行性失语五例的临床、神经心理学及影像特征分析

目的研究Logopenic型进行性失语(LPA)的临床表现、神经心理学和影像特点。方法对5例患者进行病史、临床查体、神经心理学、语言评估和血液、脑脊液检查,以及头颅磁共振(MRI)、氟18-氟脱氧葡萄糖(FDG)、PET/CT或SPECT,碳11-匹兹堡复合物B(PIB)PET/CT检查。结果 5例LPA患者为自发语言和命名过程中单个词语的取词困难,语言复述和复杂长句理解障碍,伴语音错语和记忆力减退。头颅MRI示左侧颞顶叶明显萎缩。FDG-PET显示左侧额、颞、顶和枕叶葡萄糖代谢减低。2例患者显示皮质淀粉样蛋白沉积。结论取词困难是LPA语言损害的核心特点,详细的包含语言测评的神经心理学检查和头颅影像学有利于LPA诊断。     

原发性进行性失语语言学的量表诊断

原发性进行性失语是一种早期以语言功能损害为突出特点的神经系统退行性综合征,最新共识对其语言学检测任务及评估内容做了阐述,但没有规定统一的语言学评定量表,本文就原发性进行性失语的语言学量表检测进行综述。     

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.     

进行性非流利性失语一例及文献复习

目的分析早期进行性非流利性失语患者的临床、影像、语言等特点,以提高临床对该类疾病的认识。方法报告1例早期进行性非流利性失语患者的临床、影像学、实验室检查等特点,并结合文献进行复习。结果早期进行性非流利性失语患者口语表达能力受损较重,主要表现在信息量、流利性及系列语言表达方面,而复述命名表达、听理解及阅读书写能力相对受损不明显。记忆及人格无明显改变。结论汉语文化背景的进行性非流利性失语患者早期可只表现为语言表达功能障碍,而其他高级神经活动受影响不明显。     

Functional disability in primary progressive aphasia

Background: In primary progressive aphasia (PPA), assessment of language predominates over assessment of functional impairment in activities of daily living (ADLs) in clinical and research environments. Most of the knowledge on functional disability in PPA relies largely on anecdotal experience and limited numbers of studies published to date.

Aims: (1) To describe the different patterns of ADL functional disability in the main PPA variants: semantic variant, nonfluent aphasia, and the more recently defined logopenic variant; (2) to draw relations between functional disability, cognitive, and behavioural symptoms in the PPAs; (3) to examine the impact of functional disability on carer burden, and (4) to provide specific strategies to address the described problems.

Main Contribution: Profiles of disease progression are described from a functional perspective, as well as the relationship (or lack thereof) between functional disability and cognitive and behavioural symptoms. Dementia-management strategies for carers and professionals in overcoming day-to-day difficulties are provided, and the impact of functional deficits on those around the patient, including their spouses and children, are discussed.

Conclusions: Patterns of ADL functional disability and their progression vary between PPA subtypes. Understanding these different profiles of impairment is critical to the development of tailored interventions. There is a range of therapeutic strategies which can be trialled to promote improved ADL functioning, which in turn may also help in reducing levels of carer burden in PPA.     

Agrammatic primary progressive aphasia in two dextral patients with right hemispheric involvement

Agrammatic primary progressive aphasia (PPA-G) has been known to be associated with focal brain atrophy involving the left posterior frontal and anterior insular regions. However, aphasia can also rarely result from right hemispheric lesions in right-handed patients, so-called crossed aphasia in dextrals (CAD). We report two right-handed patients with PPA-G whose 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) showed hypometabolism predominantly in the right hemisphere, implicating “crossed PPA-G.”     

Distinguishing logopenic from semantic & nonfluent variant primary progressive aphasia: Patterns of linguistic and behavioral correlations

While language characteristics of logopenic variant primary progressive aphasia (lvPPA) are well-defined, behavioral characteristics are less understood. We investigated correlations between language and behavioral scores across three variants of primary progressive aphasia (PPA) and found language performance and behavioral disturbances are correlated in lvPPA, but not other PPA subtypes. Results suggest that unlike other PPA variants, patients diagnosed with lvPPA do not develop negative behaviors until language deficits are severe. This is consistent with the underlying neuropathology of lvPPA, Alzheimer's Disease. Such findings are crucial to clinical prognosis, especially when considering the progressive nature of this disease.     

Validating new diagnostic imaging criteria for primary progressive aphasia via anatomical likelihood estimation meta‐analyses

Recently, diagnostic clinical and imaging criteria for primary progressive aphasia (PPA) have been revised by an international consortium (Gorno‐Tempini et al. Neurology 2011;76:1006‐14). The aim of this study was to validate the specificity of the new imaging criteria and investigate whether different imaging modalities [magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography (FDG‐PET)] require different diagnostic subtype‐specific imaging criteria. Anatomical likelihood estimation meta‐analyses were conducted for PPA subtypes across a large cohort of 396 patients: firstly, across MRI studies for each of the three PPA subtypes followed by conjunction and subtraction analyses to investigate the specificity, and, secondly, by comparing results across MRI vs. FDG‐PET studies in semantic dementia and progressive nonfluent aphasia. Semantic dementia showed atrophy in temporal, fusiform, parahippocampal gyri, hippocampus, and amygdala, progressive nonfluent aphasia in left putamen, insula, middle/superior temporal, precentral, and frontal gyri, logopenic progressive aphasia in middle/superior temporal, supramarginal, and dorsal posterior cingulate gyri. Results of the disease‐specific meta‐analyses across MRI studies were disjunct. Similarly, atrophic and hypometabolic brain networks were regionally dissociated in both semantic dementia and progressive nonfluent aphasia. In conclusion, meta‐analyses support the specificity of new diagnostic imaging criteria for PPA and suggest that they should be specified for each imaging modality separately.     

Whole‐brain white matter disruption in semantic and nonfluent variants of primary progressive aphasia

Semantic (svPPA) and nonfluent (nfPPA) variants of primary progressive aphasia are associated with distinct patterns of cortical atrophy and underlying pathology. Little is known, however, about their contrasting spread of white matter disruption and how this relates to grey matter (GM) loss. We undertook a structural MRI study to investigate this relationship. We used diffusion tensor imaging, tract‐based spatial statistics, and voxel‐based morphometry to examine fractional anisotropy (FA) and directional diffusivities in nine patients with svPPA and nine patients with nfPPA, and compared them to 16 matched controls after accounting for global GM atrophy. Significant differences in topography of white matter changes were found, with more ventral involvement in svPPA patients and more widespread frontal involvement in nfPPA individuals. However, each group had both ventral and dorsal tract changes, and both showed spread of diffusion abnormalities beyond sites of local atrophy. There was a clear dissociation in sensitivity of diffusion tensor imaging measures between groups. SvPPA patients showed widespread changes in FA and radial diffusivity, whereas changes in axial diffusivity were more restricted and proximal to sites of GM atrophy. NfPPA patients showed isolated changes in FA, but widespread axial and radial diffusivity changes. These findings reveal the extent of white matter disruption in these variants of PPA after accounting for GM loss. Further, they suggest that differences in the relative sensitivity of diffusion metrics may reflect differences in the nature of underlying white matter pathology in these two subtypes. Hum Brain Mapp, 2013. © 2011 Wiley Periodicals, Inc.     

Trouble and repair during conversations of people with primary progressive aphasia

Background: Primary progressive aphasia (PPA) affects a range of language domains that impact on communication. Little is known about the nature of conversation breakdown in PPA. The identification of trouble in conversation, its repair and the success of repairs has been used effectively to examine conversation breakdown in neurogenic language disorders such as dementia of the Alzheimer type (DAT) and acute onset aphasia. This study investigated trouble and repair in the conversations of people with PPA.

Aims: The first aim of this study is to describe the contributions of individuals with PPA and their conversation partner to conversation. The second aim is to describe the trouble that occurs in dyadic conversations between three individuals with PPA and their communication partner. The third aim is to describe the repair behaviours used by the individuals with PPA and their communication partners.

Methods & Procedures: Dyadic conversations about everyday activities between three individuals with PPA and their partners and three control dyads were video recorded and transcribed. Number of words, number of turns and length of turns were measured and trouble-indicating behaviours (TIBs) and repair behaviours were categorised.

Outcomes & Results: Individuals with PPA had reduced mean length of turn but maintained their share of turn-taking. They demonstrated a variety of TIBs that differed from the noninteractive repairs, which do not require a response from the partner in the conversation and which have been observed in studies of conversation in DAT. Their partners bore the greater burden of highlighting trouble and need for repair using collaborative, interactive, TIBs. Three different conversational profiles were observed in the three PPA dyads, reflecting different patterns of language and cognitive impairment.

Conclusions: Individuals with PPA were active participants in conversation effectively indicating and responding to trouble. Understanding trouble and repair in the conversations of individuals with PPA has the potential to enhance assessment and inform clinical practice.     

Lexicality effects in word and nonword recall of semantic dementia and progressive nonfluent aphasia

Background: Verbal working memory is an essential component of many language functions, including sentence comprehension and word learning. As such, working memory has emerged as a domain of intense research interest both in aphasiology and in the broader field of cognitive neuroscience. The integrity of verbal working memory encoding relies on a fluid interaction between semantic and phonological processes. That is, we encode verbal detail using many cues related to both the sound and meaning of words. Lesion models can provide an effective means of parsing the contributions of phonological or semantic impairment to recall performance.

Methods & Procedures: We employed the lesion model approach here by contrasting the nature of lexicality errors incurred during recall of word and nonword sequences by three individuals with progressive nonfluent aphasia (a phonological dominant impairment) compared to that of two individuals with semantic dementia (a semantic dominant impairment). We focused on psycholinguistic attributes of correctly recalled stimuli relative to those that elicited a lexicality error (i.e., nonword → word OR word → nonword).

Outcomes & Results: Patients with semantic dementia showed greater sensitivity to phonological attributes (e.g., phoneme length, wordlikeness) of the target items relative to semantic attributes (e.g., familiarity). Patients with PNFA showed the opposite pattern, marked by sensitivity to word frequency, age of acquisition, familiarity, and imageability.

Conclusions: We interpret these results in favour of a processing strategy such that in the context of a focal phonological impairment patients revert to an over-reliance on preserved semantic processing abilities. In contrast, a focal semantic impairment forces both reliance on and hypersensitivity to phonological attributes of target words. We relate this interpretation to previous hypotheses about the nature of verbal short-term memory in progressive aphasia.     

Patterns of decline in naming and semantic knowledge in primary progressive aphasia

Background: Individuals with primary progressive aphasia (PPA) and their caregivers want to know what to expect so that they can plan support appropriately. The ability to predict decline in naming and semantic knowledge, and advise individuals with PPA and their caregivers regarding future planning, would be invaluable clinically.

Aims: The aims of this study were to investigate patterns of decline in naming and semantic knowledge in each of the clinical variants of PPA (logopenic variant PPA, lvPPA; nonfluent agrammatic PPA, nfaPPA; and semantic variant PPA, svPPA) and to examine the effects of other variables on rate of decline. We hypothesized that speech-language rehabilitation, higher education, and higher baseline test scores would be associated with slower decline, and older age with faster decline.

Methods and Procedures: A total of 94 participants with PPA underwent language testing, including 36 participants with lvPPA, 31 participants with nfaPPA, and 27 participants with svPPA. All participant groups were similar in age and education. We focused on decline on three tests: the short form of the Boston Naming Test (BNT), the Hopkins Assessment of Naming Actions (HANA), and the short form of the Pyramids and Palm Trees Test (PPTT).

Outcome and Results: Across language tests, the most precipitous rates of decline (loss of points per month) occurred in nfaPPA, followed by svPPA, then lvPPA. Female sex, longer symptom duration, higher baseline test score, and speech-language rehabilitation were associated with slower decline.

Conclusions: PPA variants were distinguishable by rapidity of decline, with nfaPPA having the most precipitous decline. As hypothesized, higher baseline test scores and speech-language rehabilitation were associated with slower decline. Surprisingly, age and education were not important prognostically for individuals in this study. Further study of prognostically-relevant variables in PPA is indicated in this population.