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。     

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。     

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。     

后部失语的鉴别诊断

失语为高级皮质言语中枢损害而引起的表达，理解等方面的障碍，以大脑外侧裂为界，将此以前的病变引起的言语障碍称为“前部失语”在此以后病变所致的言语障碍称“后部失语”。临床上后部失语均有命名障碍，流利性言语，理解障碍及不同程度的复述障碍，在作“感觉性失语”“命名性失语”及“传导性失语”时常易混淆，本文将以具体病例对上述问题进行讨论。     

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。     

几种主要失语综合征的病灶部位和言语障碍特征

类别病灶部位自发言语言语理解口语复述能力物品(人物)命名阅读理解能力书写能力运动性失语优势额下回后部皮质或皮质下不流利、费力部分障碍差部分障碍到完全障碍朗读困难、理解好中等度障碍感觉性失语优势侧颞上回后1/3区域及其周围部分流利、言语错乱完全障碍差部分障碍到完全障碍朗读困难、理解差差,空洞传导性失语优势侧颞叶峡部岛叶皮质下的弓状束和联络纤维流利、言语错乱接近正常很差严重障碍朗读困难、理解差中等度障碍命名性失语优势侧颞、枕、顶结合区流利、空洞正常正常完全障碍稍差或正常轻度障碍经皮质运动性失语优势侧额叶内…     

丘脑出血所致的失语(附8例分析)

本文报道8例左侧丘脑出血所致的失语,对其语言行为障碍进行了描述,对其可能的发病机制进行了讨论。作者指出,丘脑性失语具有“超皮质”的特性,即保留有复述的功能,可命名为超皮质性丘脑失语,作为一种独立的失语类型,可归之于无复述障碍的失语类型项下。     

原发进行性失语的病理特点

原发进行性失语(PPA)系一临床综合征,1982年由Mesulam报道,至今已有60多例,但尚缺乏病理资料。作者报告1例临床诊断为Pick氏病的PPA患者的脑部病理特点。 患者男,59岁。因进行性流利性失语9年就诊。系列CT扫描显示双侧外侧裂增宽,左侧明显,MRI显示双侧颞叶严重萎缩,EEG正常。病程中患者出     

语义性痴呆

语义性痴呆(SD)是痴呆综合征之一,与颞前叶的非阿尔茨海默病性退行性变化有关。SD患者两侧颞前叶的萎缩不对称,以左颞前叶的萎缩更明显。患者通常表现出进行性加重的命名不能和语义理解障碍,但其自发言语表达流利,没有发音和语法错误。其他认知功能如情景记忆相对保留。对于SD的神经心理学、病理学和影像学研究有助于解释不同的语言和记忆成分在大脑内的结构定位及其相互间的关系。     

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。     

Semantic dementia and primary progressive aphasia: a problem of categorization?

The relationship between semantic dementia (SD) and primary progressive aphasia (PPA) has been the subject of debate ever since the syndromes were first described, in converging streams of research from the neuropsychological and neurologic communities. The most salient clinical features of SD are anomia with circumlocution and semantic paraphasia, single-word comprehension deficit, and reduced category fluency. Of critical importance is the fact that patients also show deficits on non-verbal tasks using visual, auditory, and other modalities, suggesting that the key impairment in SD is a breakdown in conceptual knowledge rather than a specific problem with language. The finding of item consistency between the various tests supports this view. The order in which the features appear can be explained by the variable degree of redundancy in access to semantic knowledge from the different perceptual modalities. Atrophy is seen in the anterior and inferior temporal lobe rather than in classic language areas, further distancing SD from aphasic syndromes. Semantic dementia and progressive non-fluent aphasia (PNFA) share some clinical and pathologic characteristics with frontal variant frontotemporal dementia, but there are also clear differences between the three syndromes. We believe that many patients described as having fluent primary progressive aphasia in fact have early SD. Semantic dementia is a well-defined syndrome, distinct from PNFA but related to it within the spectrum of frontotemporal lobar degeneration syndromes.     

A case of developmental deep dyslexia: What's left is right

Primary progressive aphasia (PPA) is a progressive neurodegenerative disorder characterized by the deterioration of language functions. The Han language bears some unique features from the Latin languages; however, the features of PPA in the Han language-speaking population are not well understood. In this study, we performed a 3-year follow-up on a Han language-speaking PPA patient with corresponding changes in magnetic resonance imaging (MRI). During the early stage, linguistic analysis revealed several symptoms including difficulty with auditory comprehension, right–left disorientation, reading disorders, and agraphia, specifically the execution of serial oral instructions. This Chinese PPA patient presented with a reading disorder, but his word comprehension ability remained intact. There are two different possible modalities of incorrect writing in this case. The patient also presented with noun–verb double dissociation. The early-stage MRI showed atrophy of the left frontal lobe, which was most severe in the inferior frontal gyrus. Three years later, the patient was found to have progressive atrophy in the parietal, frontal, and temporal lobes, among which the frontal lobe remained the most severely affected region. The brain imaging of the Chinese-speaking PPA patient showed changes similar to those of a Latin language-speaking PPA patient. The prominent change was asymmetrical atrophy in the frontal and temporal lobes. This is the first report of noun–verb double dissociation existing in a Chinese-language speaking PPA patient. The dissociation may be related to an impaired function of the inferior frontal gyrus, which is likely associated with verb-naming in Chinese-speaking people. Several unique features were observed in this case, including impairment in writing ability.     

Primary progressive aphasia: a comparative study of progressive nonfluent aphasia and semantic dementia

Primary progressive aphasia (PPA), a degenerative disorder, is often misdiagnosed as Alzheimer's disease. Its subtypes, semantic dementia (SD), and progressive nonfluent aphasia (PNFA), are often difficult to differentiate from each other. Our objective was to highlight the differences in the language profiles of patients with SD and PNFA. To bring out these differences, we report two patients with PPA, one with SD and the other with PNFA. They were administered the Western aphasia battery (WAB) and a semantic battery, which assesses semantic memory. The profiles of language impairment on the WAB indicated that the patient with PNFA had syntactic errors in expressive speech but relatively preserved semantics and comprehension, whereas the patient with SD had preserved syntax but made semantic errors in expressive speech, and had impaired comprehension. There were differences in their performance on the semantic battery too. The patient with SD made relatively less errors on confrontation naming, although on the pointing task he failed to point to those line drawings, which he was unable to name on confrontation. In contrast, the finding of the PNFA patient was the reverse of this. Supplementing conventional neuropsychological tests with formal tests for assessment of language functions is useful in the early diagnosis of PPA. The performance of PPA patients on a detailed assessment of language that includes use of formal tests such as the semantic battery helps to differentiate PNFA from SD.     

Confrontation naming and morphometric analyses of structural MRI in frontotemporal dementia

We studied the neural basis for confrontation naming difficulty in 29 patients with frontotemporal dementia (FTD) by correlating naming with voxel-based morphometric analyses of gray matter volume in structural MRI. We found that naming is significantly impaired in FTD, including patients with semantic dementia (SD), progressive nonfluent aphasia (PNFA), and nonaphasic patients (NON-APH) with a disorder of social and executive functioning. Significant cortical atrophy was found in the left anterior temporal cortex in all three FTD subgroups relative to healthy seniors. We also found significant cortical atrophy in unique anatomic distributions in each FTD subgroup. This included: lateral, ventral, and parahippocampal regions of the left temporal lobe in SD; inferior, orbital, dorsolateral, and premotor regions of the left frontal lobe in PNFA, and bilateral frontal regions in NON-APH. Direct correlations between confrontation naming and gray matter volume revealed distinct patterns in each FTD subgroup. SD patients showed a significant correlation in the left lateral temporal cortex, PNFA patients in several left frontal regions, and NON-APH patients in the right dorsolateral prefrontal cortex. These findings suggest that confrontation naming is supported by a large-scale neural network, and that naming is compromised in FTD due to interruption of the network in several different ways.     

Primary Progressive Aphasia and Alzheimer’s Disease: Brief History, Recent Evidence

Primary progressive aphasia (PPA) has been recognized as a syndrome distinct from the usual pattern of language deterioration in Alzheimer??s disease and typically more related to the pathology of frontotemporal dementia (FTD). In recent years, however, the syndromes of primary progressive aphasia have become more complex, divided into the three subtypes of progressive nonfluent aphasia (PNFA), semantic dementia (SD), and logopenic/phonological progressive aphasia (LPA). These syndromes have not only made the linguistic analysis more complex, but the associated pathologies have also become more variable. In particular, PNFA is usually, but not always, associated with FTD pathology and often evidence of a tau mutation, but rarely AD; SD is usually associated with FTD of the ubiquitin staining or progranulin (TAR-DNA) mutation type, but, again, occasionally AD; LPA is typically associated with AD pathology. Patterns of atrophy on magnetic resonance imaging (MRI) generally conform to these subtypes, with PNFA associated with left frontal and insular atophy, SD associated with bilateral temporal atrophy, and LPA associated with L superior-posterior temporal and parietal atrophy. These patterns can also be seen on positron emission (PET) scanning with fluorodeoxyglucose. The newer amyloid binding ligand PET technologies are less useful for detecting regional atrophy patterns but more useful for indication of the underlying pathology. We can thus speak of syndromes of PPA or underlying pathological bases of PPA.     

Therapy for naming deficits in two variants of primary progressive aphasia

Background: Primary progressive aphasia (PPA) refers to a progressive and selective decline in language due to neurodegenerative disease. There are three variants of PPA, progressive nonfluent aphasia (PNFA), semantic dementia (SD), and logopaenic progressive aphasia (LPA). All variants include impaired object naming, but distinct underlying deficits might interfere with naming. Therefore, individuals with different types of PPA may respond differently to naming therapy.

Aims: To identify differences in patterns of success and generalisation in response to the same treatment in patient with LPA and a patient with SD. Furthermore, we wished to identify whether the treatment effect was item specific (trained words) or generalised to untrained words in trained or untrained categories.

Methods & Procedures: Participants included an individual with LPA and one with SD. An assessment of lexical processing was administered before and after a naming treatment to assess underlying deficits and generalisation effects. Therapy consisted of a cueing hierarchy treatment. Treatment items consisted of pictured objects in the categories of fruits/vegetables and clothing.

Outcomes & Results: Two different patterns of performance were observed. The LPA participant improved in naming of treated items and untreated items in both treated and untreated categories. The participant with SD improved in naming treated items only, but showed less deterioration in untreated items in treated than untreated categories.

Conclusions: Individuals with PPA can show improved naming (at least temporarily) with therapy, but generalisation to untrained items may depend on the underlying cause of the naming deficit, which may differ across subtypes.     

Transcortical sensory aphasia due to extensive infarction of left cerebral hemisphere]

We report a case of transcortical sensory aphasia occurred after extensive infarction of left cerebral hemisphere. A 68-year-old, right-handed man with atrial fibrillation suddenly developed cerebral embolism of left middle cerebral artery. He was treated conservatively, and the right hemiplegia, aphasia, apraxia in a slight degree and right hemispatial neglect in a slight degree consequently existed. MRI showed a large cortical and subcortical infarct lesion including the left Broca's area, central region, perisylvian area with Wernicke's area and temporal lobe. In contrast, neuropsychological evaluation using the Western Aphasia Battery (WAB) demonstrated transcortical sensory aphasia, e.g., fluency 8, auditory comprehension 1. repetition 10 and object naming 2.4. In addition to preserved repetition, both linguistic prosody and affective prosody were well preserved. Most cases with transcortical sensory aphasia are known to occur with the lesion including temporo-parieto-occipital junction of dominant hemisphere. Our patient and a few other reported cases of transcortical sensory aphasia had a lesion in perisylvian area including Wernicke's area. Therefore, it is possible that their minor hemisphere worked selectively for repetition. Furthermore, we suggest that this patient presented dissociative aphasia that all the process of repetition and the function of linguistic and emotional prosody were represented in the right hemisphere and the other functions including comprehension of word meanings were existed in the left hemisphere. We believe that our case of transcortical sensory aphasia with dissociative aphasia gives a suggestion about the mechanism and localization of repetition and prosody in the whole system of language.     

Transcortical sensory aphasia following left frontal infarction

Two right-handed patients who exhibited language disability after left frontal infarction are described. The patients spoke fluently and exhibited excellent repetition ability from the onset of infarction without exhibiting any oral apraxia, but had deficits in auditory comprehension, naming, reading and writing. In both patients, brain magnetic resonance imaging (MRI) revealed infarction in the left inferior frontal gyrus, the middle frontal gyrus and the anterior part of the lower precentral gyrus. Single photon emission computed tomography (SPECT) revealed decreased blood flow in the same regions as those shown to be infarcted by MRI. The MRI and SPECT findings and the symptoms of these patients suggest that left frontal lesions that encompass Broca’s area produce fluent aphasia if the posterior part of the left precentral gyrus or motor cortex remains intact and that lesions anterior to Broca’s area and the middle frontal gyrus produce a deficit in auditory comprehension of single words as well as sentences. Received: 14 March 1997 Received in revised form: 29 September 1997 Accepted: 15 October 1997