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.     

原发性进行性失语综合征

一、概述缓慢进展的失语最早于１８世纪由Ｐｉｃｋ以及Ｒｏｓｅｎｆｅｌｄ先后描述,病人常表现为命名障碍的语言障碍,常见于Ａｌｚｈｅｉｍｅｒ病（ＡＤ）、Ｐｉｃｋ病（ＰｉＤ）［１］。１９８２年Ｍｅｓｕｌａｍ［２］报道６例缓慢进展的失语病人,无智能和行为障碍,并首先提出缓慢进展的失语（ｓｌｏｗｌｙｐｒｏｇｒｅｓｓｉｖｅａｐｈａｓｉａ）这一概念,意指无痴呆的进行性失语,老年前期多见,病程迁延多年,晚期可出现痴呆,病变部位主要位于左侧半球外侧裂周围。１９８７年Ｍｅｓｕｌａｍ［３］进一步充实了ＰＰＡ这一概念,并…     

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。     

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.     

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。     

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。     

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。     

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。     

Primary progressive aphasia: a case report

We report a 69-year-old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal dominance. Single photon emission tomography (SPECT) showed hypoperfusion in the frontotemporoparietal region, positron emission tomography (PET) demonstrated a global cortical reduction of glucose utilization with a lesser decrement in the occipital lobes. The clinical symptoms and the neuropsychological findings fit the diagnosis of primary progressive aphasia.     

Primary progressive aphasia in a bilingual speaker: a single-case study

We report on the case of an elderly bilingual woman presenting with a diagnosis of primary progressive aphasia. The participant's native language was Friulian (L1), a predominantly oral Romance language, and her second language was Italian (L2), formally learned at primary school in oral and written forms. We investigated her linguistic abilities by means of the Bilingual Aphasia Test ( . The assessment of bilingual aphasia. Hillsdale, NJ: Lawrence Erlbaum Associates), which is specifically devised for studying language levels and skills in bilingual/polyglot individuals with aphasia. Specifically, we focused on different tasks extracted from the Bilingual Aphasia Test, targeting phonology, morphology, syntax and lexical semantics. Results show that both languages were affected to a clinically significant degree, but with different profiles in terms of linguistic levels, suggesting the presence of greater phonological, morphological, grammatical and syntactic impairments in L2. Results are discussed in terms of possible dissociations both within the language system of each language and between languages, within the Procedural/Declarative theoretical framework of language acquisition in bilinguals.     

Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: A neuropsychological and neuroimaging analysis

There is currently considerable interest in the clinical spectrum of progressive nonfluent aphasia (PNFA) and progressive supranuclear palsy (PSP) and the intersection of these two entities. Here, we undertook a detailed prospective clinical, neuropsychological, and neuroimaging analysis of 14 consecutive patients presenting with PNFA to identify cases meeting clinical criteria for PSP. These patients had further detailed assessment of extrapyramidal and oculomotor functions. All patients had high‐resolution MR brain volumetry and a cortical thickness analysis was undertaken on the brain images. Four patients presenting with PNFA subsequently developed features of a PSP syndrome, including a typical oculomotor palsy. The neuropsychological profile in these cases was similar to other patients with PNFA, however, with more marked reduction in propositional speech, fewer speech errors, less marked impairment of literacy skills but more severe associated deficits of episodic memory and praxis. These PSP‐PNFA cases had less prominent midbrain atrophy but more marked prefrontal atrophy than a comparison group of five patients with pathologically confirmed PSP without PNFA and more prominent midbrain atrophy but less marked perisylvian atrophy than other PNFA cases. In summary, although the PSP‐PNFA syndrome overlaps with PNFA without PSP, certain neuropsychological and neuroanatomical differences may help predict the development of a PSP syndrome. © 2010 Movement Disorder Society     

Telerehabilitation of anomia in primary progressive aphasia

Background: The efficacy of telerehabilitation-based treatment for anomia has been demonstrated in post-stroke aphasia, but the efficacy of this method of anomia treatment delivery has not been established within the context of degenerative illness.

Aims: The current study evaluated the feasibility and efficacy of a telerehabilitation-based approach to anomia treatment within the three subtypes of primary progressive aphasia (PPA).

Methods & Procedures: Each of the three telerehabilitation participants represented a distinct subtype of PPA. Following a baseline evaluation of language and cognition, a phonological treatment and an orthographic treatment were administered to all participants over the course of 6 months. One month after the end of treatment, a post-treatment evaluation began. All treatment sessions and the majority of the evaluation sessions were administered via telerehabilitation. Treatment effects were examined within each subject, and treatment effects were also compared between each telerehabilitation participant and a group of in-person participants who had the same subtype of PPA.

Outcomes & Results: All three telerehabilitation participants exhibited positive treatment effects. CGR (nonfluent/agrammatic variant PPA) and WCH (logopenic variant PPA) showed maintenance of naming for prophylaxis items under both treatment conditions, while ACR (semantic variant PPA) demonstrated increased naming of remediation items under the phonological treatment condition. Compared to in-person participants with the same subtype of PPA, each of the telerehabilitation participants typically showed effects that were either within the expected range or larger than expected.

Conclusions: Telerehabilitation-based anomia treatment is feasible and effective in all three subtypes of PPA.     

Primary progressive aphasia : a case report.

Primary progressive aphasia is due to focal left perisylvian degeneration and manifests with progressive decline in language function for two or more years. There is preservation of cognitive functions and activities of daily living continue to be normal. We report a case of progressive aphasia in a 65 year old lady.     

Primary progressive non-fluent aphasia: a case study

A case study of a 65 year old man is described with an eight-year history of progressive primary non-fluent aphasia accompanied by agrammatism, phonemic paraphasias and mild spelling dysgraphia. His naming ability, however, has remained at an exceptionally high level and there has been no evidence of impairment of word or sentence comprehension. Non-verbal skills and memory functions have also been preserved within the range of his very high premorbid level of abilities. Single photon emission computed tomography was consistent with bifrontal hypoperfusion. We argue that the selective language deficits in this patient are characteristic of dynamic aphasia and of other speech disturbances which are also known to be associated with left frontal lesions. The possible underlying pathology is discussed in the context of known degenerative disorders.     

Patterns of breakdown in spelling in primary progressive aphasia

Introduction

The objective of this study is to determine which cognitive processes underlying spelling are most affected in the three variants of primary progressive aphasia (PPA): Logopenic variant primary progressive aphasia (lvPPA), Semantic variant primary progressive aphasia (svPPA), and Nonfluent variant primary progressive aphasia (nfvPPA).

Methods

23 PPA patients were administered The Johns Hopkins Dysgraphia Battery to assess spelling. Subtests evaluate for effects of word frequency, concreteness, word length, grammatical word class, lexicality (words vs pseudowords), and “regularity” by controlling for the other variables. Significant effects of each variable were identified with chi square tests. Responses on all spelling to dictation tests were scored by error type. 16 of the 23 subjects also had a high resolution MRI brain scan to identify areas of atrophy.

Results

We identified 4 patterns of spelling that could be explained by damage to one or more cognitive processes underlying spelling. Nine patients (3 unclassifiable, 4 with lvPPA, 2 with svPPA) had dysgraphia explicable by impaired access to lexical representations, with reliance on sublexical phonology-to-orthography conversion (POC). Two patients (with nfvPPA) showed dysgraphia explicable by impaired access to lexical representations and complete disruption of sublexical POC. Seven patients (4 with lvPPA, 1 with svPPA, 2 unclassifiable) showed dysgraphia explicable by impaired access to lexical-semantic representations and/or lexical representations with partially spared sublexical POC mechanisms. Five patients (1 with nfvPPA, 2 with svPPA, 1 with lvPPA, and 1 unclassifiable) showed dysgraphia explicable by impairment of the graphemic buffer.

Conclusions

Any cognitive process underlying spelling can be affected in PPA. Predominance of phonologically plausible errors, more accurate spelling of regular words than irregular words, and more accurate spelling of pseudowords than words (indicating spared POC mechanisms) may indicate a low probability of progression to nfvPPA.     

Primary progressive aphasia and Pick complex

Ten autopsied patients from a prospectively followed, clinically defined, neuropsychologically and radiologically documented cohort with primary progressive aphasia were histologically characterized. All were variants of frontotemporal degeneration (Pick complex): Pick body dementia, n=3, corticobasals degeneration (CBD), n=4, and tau and synuclein negative ubiquitinated inclusions of the motor neuron disease type, n=3. All shared superficial cortical spongiosis, neuronal loss, and gliosis. Although most patients had fluent anomic aphasia at onset, all progressed to a nonfluent or mute state. Comprehension, episodic memory, and activities of daily living were initially preserved. Three cases with Pick body dementia had verbal apraxia and stuttering at onset. Two of the patients with CBD pathology were older than the average primary progressive aphasia (PPA). All patients developed secondary syndromes either of frontotemporal dementia (FTD) and/or extrapyramidal-apraxic manifestations (CBD). By the time autopsy was obtained, the pathology appeared outside the language areas. Progressive aphasias secondary to Alzheimer's disease (AD) were excluded on the basis of early loss of memory and comprehension.Rather than the previously emphasized histological heterogeneity, clinically probable PPA has a predictive value of a group of related pathologies, collectively named frontotemporal degeneration, or Pick complex. This series of autopsied cases provides evidence for the clinical and pathological overlap of PPA with FTD and CBD, and contributes to the diagnostic and neuropsychological definition of PPA.     

Primary progressive aphasia: a patient with stress assignment impairment in reading aloud

Surface dyslexia is a pattern of reading impairment which has been seldom described in Italian native speakers. We report the case of a female Italian patient, RM, suffering from primary progressive aphasia (PPA) of the fluent type, who presented stress assignment errors in reading aloud. In Italian these errors are considered to be strongly suggestive of surface dyslexia. We studied RM's reading performance in light of existing cognitive models on reading. Since the first assessment, she presented multi-level impairment involving pre-semantic, lexical-semantic and post-semantic stages. Her stress assignment errors have been interpreted as a generalisation of the most frequent tendency in Italian language: namely to assign stress to the penultimate skyllable. In agreement with previous studies, our case suggests that surface dyslexia in PPA is not a monolithic entity but, on the contrary, that it may arise from impairment at various stages of the reading process. Received: 22 March 1999 / Accepted in revised form: 14 January 2000     

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.