Profiles of language impairment in primary progressive aphasia

Neurocase ◽  
1995 ◽  
Vol 1 (1) ◽  
pp. 39j-54
Author(s):  
H. Karbe
Keyword(s):  
Language Impairment ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Contribution of the Cognitive Approach to Language Assessment to the Differential Diagnosis of Primary Progressive Aphasia

2021 ◽  
Vol 11 (6) ◽  
pp. 815
Author(s):  
Joël Macoir ◽  
Annie Légaré ◽  
Monica Lavoie
Keyword(s):  
Differential Diagnosis ◽  
Language Impairment ◽  
Primary Progressive Aphasia ◽  
Behavioral Management ◽  
Language Abilities ◽  
Cognitive Approach ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Assessment Approach ◽  
Cognitive Neuropsychological

Diagnosis of primary progressive aphasia (PPA) is essentially based on the identification of progressive impairment of language abilities while other cognitive functions are preserved. The three variants of PPA are characterized by core and supportive clinical features related to the presence or absence of language impairment in different linguistic domains. In this article, we review the cognitive neuropsychological approach to the assessment of PPA and its contribution to the differential diagnosis of the three variants. The main advantage of this assessment approach is that it goes beyond the mere description and classification of clinical syndromes and identifies impaired and preserved cognitive and linguistic components and processes. The article is structured according to the main language domains: spoken production, language comprehension, and written language. Each section includes a brief description of the cognitive processes involved in the assessment tasks, followed by a discussion of typical characteristics for each PPA variant and common pitfalls in the interpretation of the results. In addition, the clinical benefit of the cognitive neuropsychological approach for the behavioral management of PPA is briefly sketched out in the conclusion.

Primary Progressive Aphasia Presenting With Neuropsychiatric Symptoms

2021 ◽  
pp. 089198872110361
Author(s):  
Mirjam M. P. Mulder-Heijstra ◽  
Regina R. Jokel ◽  
Howard H. Chertkow ◽  
David D. K. Conn ◽  
Linda L. Mah
Keyword(s):  
Language Impairment ◽  
Neurodegenerative Disorder ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Primary Progressive Aphasia ◽  
Motor Disorder ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Speech Language Pathologist ◽  
Insidious Onset

We describe a case of primary progressive aphasia (PPA) with an underlying neurodegenerative motor disorder (possible ALS or PSP), presenting with symptoms of irritability and frustration, that were misdiagnosed and treated as a primary psychiatric disorder, i.e. depression. PPA is a rare neurodegenerative disorder characterized by insidious onset and gradual progression of speech and language impairment. We emphasize that PPA can initially masquerade as or be accompanied by neuropsychiatric symptoms potentially leading to misdiagnosis. Most prevalent neuropsychiatric symptoms reported in the PPA literature are agitation, depression, anxiety, apathy, irritability, abnormal appetite and disinhibition. To ensure early diagnosis of PPA, if a patient presents with new psychiatric symptoms accompanied by new onset speech and/or language impairment, referral to a specialist (i.e., neurologist and/or speech-language pathologist) is recommended.

Clinical Characteristic in Primary Progressive Aphasia in Relation to Alzheimer’s Disease Biomarkers

2021 ◽  
pp. 1-13
Author(s):  
Sung Hoon Kang ◽  
Hanna Cho ◽  
Jiho Shin ◽  
Hang-Rai Kim ◽  
Young Noh ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Language Impairment ◽  
Amyloid Β ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Clinical Feature ◽  
Progressive Aphasia ◽  
Disease Biomarkers ◽  
Primary Progressive

Background: Primary progressive aphasia (PPA) is associated with amyloid-β (Aβ) pathology. However, clinical feature of PPA based on Aβ positivity remains unclear. Objective: We aimed to assess the prevalence of Aβ positivity in patients with PPA and compare the clinical characteristics of patients with Aβ-positive (A+) and Aβ-negative (A–) PPA. Further, we applied Aβ and tau classification system (AT system) in patients with PPA for whom additional information of in vivo tau biomarker was available. Methods: We recruited 110 patients with PPA (41 semantic [svPPA], 27 non-fluent [nfvPPA], 32 logopenic [lvPPA], and 10 unclassified [ucPPA]) who underwent Aβ-PET imaging at multi centers. The extent of language impairment and cortical atrophy were compared between the A+ and A–PPA subgroups using general linear models. Results: The prevalence of Aβ positivity was highest in patients with lvPPA (81.3%), followed by ucPPA (60.0%), nfvPPA (18.5%), and svPPA (9.8%). The A+ PPA subgroup manifested cortical atrophy mainly in the left superior temporal/inferior parietal regions and had lower repetition scores compared to the A–PPA subgroup. Further, we observed that more than 90%(13/14) of the patients with A+ PPA had tau deposition. Conclusion: Our findings will help clinicians understand the patterns of language impairment and cortical atrophy in patients with PPA based on Aβ deposition. Considering that most of the A+ PPA patents are tau positive, understanding the influence of Alzheimer’s disease biomarkers on PPA might provide an opportunity for these patients to participate in clinical trials aimed for treating atypical Alzheimer’s disease.

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 An Overview on Primary Progressive Aphasia and Its Variants

2006 ◽  
Vol 17 (2) ◽  
pp. 77-87 ◽  
Author(s):  
Serena Amici ◽  
Maria Luisa Gorno-Tempini ◽  
Jennifer M. Ogar ◽  
Nina F. Dronkers ◽  
Bruce L. Miller
Keyword(s):  
Language Impairment ◽  
Sentence Comprehension ◽  
Primary Progressive Aphasia ◽  
Cognitive Domains ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Word Finding ◽  
Degenerative Disorder ◽  
Language Output ◽  
Progressive Nonfluent Aphasia

We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profiles of 42 PPA patients. Mesulam originally defined PPA as a progressive degenerative disorder characterized by isolated language impairment for at least two years. The most common variants of PPA are: (1) Progressive nonfluent aphasia (PNFA), (2) semantic dementia (SD), (3) logopenic progressive aphasia (LPA). PNFA is characterized by labored speech, agrammatism in production, and/or comprehension. In some cases the syndrome begins with isolated deficits in speech. SD patients typically present with loss of word and object meaning and surface dyslexia. LPA patients have word-finding difficulties, syntactically simple but accurate language output and impaired sentence comprehension. The neuropsychological data demonstrated that SD patients show the most characteristic pattern of impairment, while PNFA and LPA overlap within many cognitive domains. The neuroimaging analysis showed left perisylvian region involvement. A comprehensive cognitive, neuroimaging and pathological approach is necessary to identify the clinical and pathogenetic features of different PPA variants.

P2-347: Progression of language impairment in primary progressive aphasia: Cognitive and structural MRI predictors

2006 ◽  
Vol 2 ◽  
pp. S343-S343
Author(s):  
Mathieu Vandenbulcke ◽  
Ronald Peeters ◽  
Kathleen Porke ◽  
Paul Van Hecke ◽  
Rik Vandenberghe
Keyword(s):  
Language Impairment ◽  
Primary Progressive Aphasia ◽  
Structural Mri ◽  
Progressive Aphasia ◽  
Primary Progressive

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 Cognitive and Affective Empathy Disruption in Non-Fluent Primary Progressive Aphasia Syndromes

2016 ◽  
Vol 18 (1) ◽  
pp. 117-129 ◽  
Author(s):  
Jessica L. Hazelton ◽  
Muireann Irish ◽  
John R. Hodges ◽  
Olivier Piguet ◽  
Fiona Kumfor
Keyword(s):  
Language Impairment ◽  
Disease Onset ◽  
Primary Progressive Aphasia ◽  
Cognitive Empathy ◽  
Reactivity Index ◽  
Affective Empathy ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Patient Groups ◽  
Social Cognitive Deficits

Empathy involves being able to understand and respond to others’ emotional experiences. Whilst deficits in empathy have been observed in frontotemporal dementia, the extent to which empathy is disrupted in dementia syndromes with predominant language impairment remains unclear. The current study investigated cognitive and affective empathy in the two non-fluent primary progressive aphasia syndromes: progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA). Informants of 23 PNFA and 16 LPA patients completed the Interpersonal Reactivity Index (IRI), regarding patients’ capacity for empathy pre- and post-disease onset. Twenty-four healthy control participants completed the self-rated IRI for comparison of post-disease empathy capabilities. Within-group analyses revealed reduced cognitive empathy and increased personal distress in both patient groups. In addition, lowered affective empathy was reported in PNFA, with a similar trend observed in LPA. Interestingly, reduced affective empathy was associated with greater carer burden in LPA. Between-group analyses revealed reduced cognitive empathy in both patient groups relative to controls. The current study is the first to document empathy changes in PNFA and LPA, offering insight into the social cognitive deficits experienced in these syndromes. Future neuroimaging studies are needed to identify the underlying neural correlates and mechanisms driving empathy deficits in PNFA and LPA.

scholarly journals Non-primary progressive language impairment in neurodegenerative conditions: protocol for a scoping review

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Sharon A. Savage ◽  
Aida Suárez-González ◽  
Alice Cassani ◽  
Ragaviveka Gopalan ◽  
Joshua Stott
Keyword(s):  
Scoping Review ◽  
Language Impairment ◽  
Primary Progressive Aphasia ◽  
Systematic Investigation ◽  
Motor Deficits ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Cognitive Behaviour ◽  
Language Difficulties ◽  
The Impact

Abstract Background Progressive language difficulties arise in many neurodegenerative conditions, causing significant impact upon patients and families. This occurs most obviously in primary progressive aphasia (PPA) but can also occur within other forms of progressive disease. In these cases, language decline may be significant, but as they are not the presenting or dominant symptom, may be overlooked in favour of more prominent cognitive, behaviour or motor deficits. To date, there has been no systematic investigation into non-primary progressive aphasia. This scoping review aims to describe the currently reported language impairments found in non-language-led dementias and identify their clinical relevance, defined as the impact on everyday living. It also seeks to identify the reported interventions for language impairment in this patient group to-date. Method We will conduct a scoping review of published studies that have assessed and/or treated aphasia in people diagnosed with a neurodegenerative condition other than primary progressive aphasia. The systematic search will include the electronic databases PubMed, MEDLINE, OVID-EMBASE, PsycINFO, and speechBITE, using search terms for specific non-language-led dementia subtypes. Findings will be mapped and described according to the type of language difficulties identified and rehabilitation approaches employed. Intervention studies will be evaluated for their methodological rigour using validated scales. Discussion This scoping review will provide an overview of the types of aphasia found in neurodegenerative conditions where language dysfunction is not the primary focus. Current treatment approaches (and gaps in the provision of treatment) will be identified.

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