Primary Progressive Aphasia: Sharpening the Focus on a Clinical Syndrome

1992 ◽  
pp. 43-66 ◽  
Author(s):  
M.-M. Mesulam ◽  
S. Weintraub
Keyword(s):  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Primary progressive aphasia: Classification of variants in 100 consecutive Brazilian cases

2013 ◽  
Vol 7 (1) ◽  
pp. 110-121 ◽  
Author(s):  
Mirna Lie Hosogi Senaha ◽  
Paulo Caramelli ◽  
Sonia M.D. Brucki ◽  
Jerusa Smid ◽  
Leonel T. Takada ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Language Impairment ◽  
Demographic Data ◽  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Diagnosis And Classification ◽  
Consensus Classification

ABSTRACT Primary progressive aphasia (PPA) is a neurodegenerative clinical syndrome characterized primarily by progressive language impairment. Recently, consensus diagnostic criteria were published for the diagnosis and classification of variants of PPA. The currently recognized variants are nonfluent/agrammatic (PPA-G), logopenic (PPA-L) and semantic (PPA-S). Objective: To analyze the demographic data and the clinical classification of 100 PPA cases. Methods: Data from 100 PPA patients who were consecutively evaluated between 1999 and 2012 were analyzed. The patients underwent neurological, cognitive and language evaluation. The cases were classified according to the proposed variants, using predominantly the guidelines proposed in the consensus diagnostic criteria from 2011. Results: The sample consisted of 57 women and 43 men, aged at onset 67.2±8.1 years (range of between 53 and 83 years). Thirty-five patients presented PPA-S, 29 PPA-G and 16 PPA-L. It was not possible to classify 20% of the cases into any one of the proposed variants. Conclusion: It was possible to classify 80% of the sample into one of the three PPA variants proposed. Perhaps the consensus classification requires some adjustments to accommodate cases that do not fit into any of the variants and to avoid overlap where cases fit more than one variant. Nonetheless, the established current guidelines are a useful tool to address the classification and diagnosis of PPA and are also of great value in standardizing terminologies to improve consistency across studies from different research centers.

scholarly journals Primary progressive aphasia: A dementia of the language network

2013 ◽  
Vol 7 (1) ◽  
pp. 2-9 ◽  
Author(s):  
Marsel Mesulam
Keyword(s):  
Language Impairment ◽  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Medical Attention ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Clinical Variants ◽  
And Behavior ◽  
Work Up ◽  
Language Network

ABSTRACT Primary progressive aphasia (PPA) is a clinical syndrome diagnosed when three core criteria are met. First, there should be a language impairment (i.e., aphasia) that interferes with the usage or comprehension of words. Second, the neurological work-up should determine that the disease is neurodegenerative, and therefore progressive. Third, the aphasia should arise in relative isolation, without equivalent deficits of comportment or episodic memory. The language impairment can be fluent or non-fluent and may or may not interfere with word comprehension. Memory for recent events is preserved although memory scores obtained in verbally mediated tests may be abnormal. Minor changes in personality and behavior may be present but are not the leading factors that bring the patient to medical attention or that limit daily living activities. This distinctive clinical pattern is most conspicuous in the initial stages of the disease, and reflects a relatively selective atrophy of the language network, usually located in the left hemisphere. There are different clinical variants of PPA, each with a characteristic pattern of atrophy. The underlying neuropathological diseases are heterogeneous and can include Alzheimer's disease as well as frontotemporal lobar degeneration. The clinician's task is to recognize PPA and differentiate it from other neurodegenerative phenotypes, use biomarkers to surmise the nature of the underlying neuropathology, and institute the most fitting multimodal interventions.

scholarly journals Neural dynamics of semantic categorization in semantic variant of Primary Progressive Aphasia

2020 ◽  
Author(s):  
V. Borghesani ◽  
C. L. Dale ◽  
S. Lukic ◽  
L. B. N. Hinkley ◽  
M. Lauricella ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Superior Temporal Gyrus ◽  
Semantic Knowledge ◽  
Clinical Syndrome ◽  
Perceptual Processing ◽  
Semantic Representations ◽  
Semantic Categorization ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

AbstractAwake humans constantly extract conceptual information from a flow of perceptual inputs. Category membership (e.g., is it an animate or inanimate thing?) is a critical semantic feature used to determine the appropriate response to a stimulus. Semantic representations are thought to be processed along a posterior-to-anterior gradient reflecting a shift from perceptual (e.g., it has eight legs) to conceptual (e.g., venomous spiders are rare) information. One critical region is the anterior temporal lobe (ATL): patients with semantic variant primary progressive aphasia (svPPA), a clinical syndrome associated with ATL neurodegeneration, manifest a deep loss of semantic knowledge.Here, we test the hypothesis that svPPA patients, in the absence of an intact ATL, perform semantic tasks by over-recruiting areas implicated in perceptual processing. We acquired MEG recordings of 18 svPPA patients and 18 healthy controls during a semantic categorization task. While behavioral performance did not differ, svPPA patients showed greater activation over bilateral occipital cortices and superior temporal gyrus, and inconsistent engagement of frontal regions.These findings indicate a pervasive reorganization of brain networks in response to ATL neurodegeneration: the loss of this critical hub leads to a dysregulated (semantic) control system, and defective semantic representations are compensated via enhanced perceptual processing.

Ethical and Practical Challenges of the Communication and Behavioral Manifestations of Primary Progressive Aphasia

2020 ◽  
Vol 41 (03) ◽  
pp. 249-256
Author(s):  
Donna C. Tippett ◽  
Argye E. Hillis
Keyword(s):  
Social Interactions ◽  
Primary Progressive Aphasia ◽  
Treatment Decisions ◽  
Clinical Syndrome ◽  
Self Determination ◽  
Family Education ◽  
Treatment Interventions ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

AbstractThe communication and behavioral manifestations of primary progressive aphasia (PPA) present ethical and practical challenges for individuals with this clinical syndrome as well as for individuals who are involved closely in their care. In this article, cases representing all three PPA variants (logopenic variant, nonfluent agrammatic, semantic variant) are presented to illustrate commonly encountered situations in which self-determination is at risk in decisions about housing, driving, social interactions, finances, and treatment interventions. Potential approaches, including patient/family education, implementation of safeguards, redirection to meaningful activities, and protections against vulnerability in treatment decisions, are described to preserve autonomy in patients with this neurodegenerative clinical syndrome.

scholarly journals [18F]AV-1451 binding in vivo mirrors the expected distribution of TDP-43 pathology in the semantic variant of primary progressive aphasia

2017 ◽  
Vol 89 (10) ◽  
pp. 1032-1037 ◽  
Author(s):  
W R Bevan-Jones ◽  
Thomas E Cope ◽  
P Simon Jones ◽  
Luca Passamonti ◽  
Young T Hong ◽  
...  
Keyword(s):  
Frontotemporal Lobar Degeneration ◽  
Primary Progressive Aphasia ◽  
Hierarchical Cluster ◽  
Clinical Syndrome ◽  
Semantic Dementia ◽  
Binding Potential ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

IntroductionSemantic dementia, including the semantic variant of primary progressive aphasia (svPPA), is strongly associated with TAR-DNA binding protein 43 (TDP-43) type C pathology. It provides a useful model in which to test the specificity of in vivo binding of the putative tau ligand [18F]AV-1451, which is elevated in frontotemporal lobar degeneration tauopathies.Methods and resultsSeven patients (five with svPPA and two with ‘right’ semantic dementia) and 12 healthy controls underwent positron emission tomography brain imaging with [18F]AV-1451. Two independent preprocessing methods were used. For both methods, all patients had clearly elevated binding potential (BPND (non-displaceable binding potential)) in temporal lobes, lateralising according to their clinical syndrome and evident in raw images. Region of interest analyses confirmed that BPND was significantly increased in temporal regions, insula and fusiform gyrus, consistent with those areas known to be most affected in semantic dementia. Hierarchical cluster analysis, based on the distribution of [18F]AV-1451 binding potential, separated semantic dementia from controls with 86% sensitivity and 100% specificity.Conclusions[18F]AV-1451 binds in vivo regions that are likely to contain TDP-43 and not significant tau pathology. While this suggests a non-tau target for [18F]AV-1451, the pathological regions in semantic dementia do not normally contain significant levels of recently proposed ‘off target’ binding sites for [18F]AV-1451, such as neuronal monoamine oxidase or neuromelanin. Postmortem and longitudinal data will be useful to assess the utility of [18F]AV-1451 to differentiate and track different types of frontotemporal lobar degeneration.

scholarly journals The Right Temporal Variant of Frontotemporal Dementia is Not Genetically Sporadic: A Case Series

2021 ◽  
pp. 1-7
Author(s):  
Hulya Ulugut Erkoyun ◽  
Sven J. van der Lee ◽  
Bas Nijmeijer ◽  
Rosalina van Spaendonk ◽  
Anne Nelissen ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Genetic Variant ◽  
Autosomal Dominant ◽  
Case Series ◽  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Memory Clinics ◽  
The Right

Background: Right temporal variant frontotemporal dementia (rtvFTD) has been generally considered as a right sided variant of semantic variant primary progressive aphasia (svPPA), which is a genetically sporadic disorder. Recently, we have shown that rtvFTD has a unique clinical syndrome compared to svPPA and behavioral variant frontotemporal dementia. Objective: We challenge the assumption that rtvFTD is a sporadic, non-familial variant of FTD by identifying potential autosomal dominant inheritance and related genes in rtvFTD. Methods: We collected all subjects with a diagnosis of FTD or primary progressive aphasia who had undergone genetic screening (n = 284) and subsequently who had a genetic variant (n = 48) with a diagnosis of rtvFTD (n = 6) in 2 specialized memory clinics. Results: Genetic variants in FTD related genes were found in 33% of genetically screened rtvFTD cases; including MAPT (n = 4), GRN (n = 1), and TARDBP (n = 1) genes, whereas only one svPPA case had a genetic variant in our combined cohorts. Additionally, 4 out of 6 rtvFTD subjects had a strong family history for dementia. Conclusion: Our results demonstrate that rtvFTD, unlike svPPA, is not a pure sporadic, but a heterogeneous potential genetic variant of FTD, and screening for genetic causes for FTD should be performed in patients with rtvFTD.

scholarly journals Neural dynamics of semantic categorization in semantic variant of Primary Progressive Aphasia

eLife ◽  
2021 ◽  
Vol 10 ◽  
Author(s):  
Valentina Borghesani ◽  
Corby L Dale ◽  
Sladjana Lukic ◽  
Leighton BN Hinkley ◽  
Michael Lauricella ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Superior Temporal Gyrus ◽  
Semantic Knowledge ◽  
Clinical Syndrome ◽  
Perceptual Processing ◽  
Behavioral Performance ◽  
Semantic Representations ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

Semantic representations are processed along a posterior-to-anterior gradient reflecting a shift from perceptual (e.g., it has eight legs) to conceptual (e.g., venomous spiders are rare) information. One critical region is the anterior temporal lobe (ATL): patients with semantic variant primary progressive aphasia (svPPA), a clinical syndrome associated with ATL neurodegeneration, manifest a deep loss of semantic knowledge. We test the hypothesis that svPPA patients perform semantic tasks by over-recruiting areas implicated in perceptual processing. We compared MEG recordings of svPPA patients and healthy controls during a categorization task. While behavioral performance did not differ, svPPA patients showed indications of greater activation over bilateral occipital cortices and superior temporal gyrus, and inconsistent engagement of frontal regions. These findings suggest a pervasive reorganization of brain networks in response to ATL neurodegeneration: the loss of this critical hub leads to a dysregulated (semantic) control system, and defective semantic representations are seemingly compensated via enhanced perceptual processing.

scholarly journals Rehabilitation in semantic dementia: Study of the effectiveness of lexical reacquisition in three patients

2010 ◽  
Vol 4 (4) ◽  
pp. 306-312 ◽  
Author(s):  
Mirna Lie Hosogi Senaha ◽  
Sonia Maria Dozzi Brucki ◽  
Ricardo Nitrini
Keyword(s):  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Semantic Dementia ◽  
Errorless Learning ◽  
Boston Naming Test ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Zero Items ◽  
Naming Performance ◽  
Naming Test

Abstract Although language rehabilitation in patients with primary progressive aphasia (PPA) is recommended, rehabilitation studies in this clinical syndrome are scarce. Specifically, in relation to semantic dementia (SD), few studies have shown the possibility of lexical relearning. Objective: To analyze the effectiveness of rehabilitation for lexical reacquisition in SD. Methods: Three SD patients were submitted to training for lexical reacquisition based on principles of errorless learning. Comparisons between naming performance of treated items (pre and post-training) and non-treated items of the Boston Naming Test (BNT) were made. Results: All patients improved their performance in naming treated words after intervention. However, decline in performance in naming of non-treated items was observed. Case 1 named zero items at baseline while her performance post-training was 29.4% correct responses without cueing, and 90.7% correct with and without cueing. Case 2 named 6.9% of items correctly at baseline and his performance in post-training was 52.9% without cueing and 87.3%, with and without cueing. Case 3 named zero items at baseline and his performance in post-training was 100% correct responses without cueing. Considering the performance in naming the non-treated items of the BNT, the percentages of correct responses in the first evaluation and in the re-evaluation, respectively were: 16.7% and 8.3% (case 1; 14 month-interval); 26.7% and 11.6% (case 2; 18 month-interval) and 11.6% and 8.3% (case 3; 6 month-interval). Conclusions: The reacquisition of lost vocabulary may be possible in SD despite progressive semantic deterioration.

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