Assessment of Individuals with Primary Progressive Aphasia

AbstractSpeech–language pathologists play a crucial role in the assessment and treatment of individuals with primary progressive aphasia (PPA). The speech–language evaluation is a critical aspect of the diagnostic and rehabilitative process, informing differential diagnosis as well as intervention planning and monitoring of cognitive-linguistic status over time. The evaluation should include a thorough case history and interview and a detailed assessment of speech–language and cognitive functions, with tasks designed to detect core and associated deficits outlined in current diagnostic criteria. In this paper, we review assessments that can be utilized to examine communication and cognition in PPA, including general aphasia batteries designed for stroke and/or progressive aphasia as well as tests of specific cognitive-linguistic functions, including naming, object/person knowledge, single-word and sentence comprehension, repetition, spontaneous speech/language production, motor speech, written language, and nonlinguistic cognitive domains. The comprehensive evaluation can inform diagnostic decision making and facilitate planning of interventions that are tailored to the patient's current status and likely progression of deficits. As such, the speech–language evaluation allows the medical team to provide individuals with PPA and their families with appropriate recommendations for the present and the future.

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A Case of a Monolingual Spanish-Speaking Latina with Semantic Variant Primary Progressive Aphasia (svPPA)

Archives of Clinical Neuropsychology ◽

10.1093/arclin/acz029.57 ◽

2019 ◽

Vol 34 (7) ◽

pp. 1290-1290

Author(s):

L Perez

Keyword(s):

Working Memory ◽

Language Production ◽

Primary Progressive Aphasia ◽

Motor Speech ◽

Problem Solving Skills ◽

Progressive Aphasia ◽

Primary Progressive ◽

Spanish Speaking ◽

Word Finding ◽

Semantic Variant

Abstract Objective Often, individuals with lower educational attainment and limited proficiency in the English language get misdiagnosed and/or undertreated, which can impact their quality of life and other outcomes. The present case study intends to review and discuss the presentation of a monolingual, Spanish-speaking woman with Semantic Variant Primary Progressive Aphasia (svPPA), who was originally referred for a neuropsychological evaluation to determine the severity of her existing Alzheimer’s Disease (AD) diagnosis. Case Description Ms. X, is a 64-year-old, right-handed Hispanic woman with 6 years of education. Symptoms included forgetfulness, restlessness, and insomnia. Her family reported that she was repeating her ideas frequently during conversations and failing to recognize previously acquainted people, including her own relatives. A recent MRI of the brain showed anterior temporal lobe atrophy. Diagnostic Impressions and Outcomes Overall, she showed naming deficits (anomia), impaired verbal fluency, surface dyslexia, and significant problems with comprehension. Executive functioning, sentence repetition, working memory, and attention were generally intact. Qualitatively, her speech was apparently fluent and automatic, yet clearly empty in meaning. In Ms. X’s case, collateral reports of word-finding difficulties, tendency to repeat her thoughts incessantly, associative agnosia and prosopagnosia, and spared repetition and motor speech are strongly indicative of svPPA. Discussion svPPA primarily impacts language production and comprehension, and is characterized by severe anomia, word-finding difficulties, impaired single word comprehension, and in some cases, defective recognition of familiar faces. On testing, impairments can be observed in confrontation naming, with motor speech and repetition, working memory, episodic memory, visuospatial skills, and problem-solving skills relatively intact. Language symptoms are thought to stem from deficits of the semantic system.

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An Overview on Primary Progressive Aphasia and Its Variants

Behavioural Neurology ◽

10.1155/2006/260734 ◽

2006 ◽

Vol 17 (2) ◽

pp. 77-87 ◽

Cited By ~ 40

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.

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Quantitative Analysis of Agrammatism in Agrammatic Primary Progressive Aphasia and Dominant Apraxia of Speech

Journal of Speech Language and Hearing Research ◽

10.1044/2018_jslhr-l-17-0474 ◽

2018 ◽

Vol 61 (9) ◽

pp. 2337-2346 ◽

Cited By ~ 3

Author(s):

Katerina A. Tetzloff ◽

Rene L. Utianski ◽

Joseph R. Duffy ◽

Heather M. Clark ◽

Edythe A. Strand ◽

...

Keyword(s):

Language Production ◽

Primary Progressive Aphasia ◽

Broca’S Area ◽

Apraxia Of Speech ◽

Broca's Area ◽

Progressive Aphasia ◽

Primary Progressive ◽

Semantic Errors ◽

Description Task ◽

Mean Length Of Utterance

Purpose The aims of the study were to assess and compare grammatical deficits in written and spoken language production in subjects with agrammatic primary progressive aphasia (agPPA) and in subjects with agrammatism in the context of dominant apraxia of speech (DAOS) and to investigate neuroanatomical correlates. Method Eight agPPA and 21 DAOS subjects performed the picture description task of the Western Aphasia Battery (WAB) both in writing and orally. Responses were transcribed and coded for linguistic analysis. agPPA and DAOS were compared to 13 subjects with primary progressive apraxia of speech (PPAOS) who did not have agrammatism. Spearman correlations were performed between the written and spoken variables. Patterns of atrophy in each group were compared, and relationships between the different linguistic measures and integrity of Broca's area were assessed. Results agPPA and DAOS both showed lower mean length of utterance, fewer grammatical utterances, more nonutterances, more syntactic and semantic errors, and fewer complex utterances than PPAOS in writing and speech, as well as fewer correct verbs and nouns in speech. Only verb ratio and proportion of grammatical utterances correlated between modalities. agPPA and DAOS both showed greater involvement of Broca's area than PPAOS, and atrophy of Broca's area correlated with proportion of grammatical and ungrammatical utterances and semantic errors in writing and speech. Conclusions agPPA and DAOS subjects showed similar patterns of agrammatism, although subjects performed differently when speaking versus writing. Integrity of Broca's area correlates with agrammatism.

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Modality-Specific Deterioration in Naming Verbs in Nonfluent Primary Progressive Aphasia

Journal of Cognitive Neuroscience ◽

10.1162/089892902320474544 ◽

2002 ◽

Vol 14 (7) ◽

pp. 1099-1108 ◽

Cited By ~ 91

Author(s):

Argye E. Hillis ◽

Elizabeth Tuffiash ◽

Alfonso Caramazza

Keyword(s):

Language Production ◽

Primary Progressive Aphasia ◽

Neural Mechanisms ◽

Specific Nature ◽

Grammatical Class ◽

Progressive Aphasia ◽

Time Points ◽

Primary Progressive ◽

Lexical Representations ◽

Written Production

A longitudinal study of oral and written naming and comprehension of nouns and verbs in an individual (M. M. L.) with nonfluent primary progressive aphasia (PPA) is reported. M. M. L. showed progressive deterioration of oral naming of verbs well before deterioration of written naming of verbs and before deterioration of oral or written naming of nouns. Her comprehension of both nouns and verbs remained intact, at least relative to oral naming of verbs. Her performance is compared to that of two other individuals with nonfluent PPA, who were tested at two time points. These patients showed similar patterns with respect to grammatical word class (verbs more impaired than nouns) and modality (spoken production more impaired than written production), but somewhat different courses of deterioration. The modality-specific nature of the observed verb production deficits rules out a semantic locus for the grammatical class effects. The results provide a new source of evidence for the hypothesis that there are distinct neural mechanisms for accessing lexical representations of nouns and verbs in language production.

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Written Language Impairments in Primary Progressive Aphasia: A Reflection of Damage to Central Semantic and Phonological Processes

Journal of Cognitive Neuroscience ◽

10.1162/jocn_a_00153 ◽

2012 ◽

Vol 24 (2) ◽

pp. 261-275 ◽

Cited By ~ 30

Author(s):

Maya L. Henry ◽

Pélagie M. Beeson ◽

Gene E. Alexander ◽

Steven Z. Rapcsak

Keyword(s):

Phonological Processing ◽

Language Production ◽

Primary Progressive Aphasia ◽

Spoken Language ◽

Written Language ◽

Cortical Atrophy ◽

Language Performance ◽

Phonological Processes ◽

Progressive Aphasia ◽

Primary Progressive

Connectionist theories of language propose that written language deficits arise as a result of damage to semantic and phonological systems that also support spoken language production and comprehension, a view referred to as the “primary systems” hypothesis. The objective of the current study was to evaluate the primary systems account in a mixed group of individuals with primary progressive aphasia (PPA) by investigating the relation between measures of nonorthographic semantic and phonological processing and written language performance and by examining whether common patterns of cortical atrophy underlie impairments in spoken versus written language domains. Individuals with PPA and healthy controls were administered a language battery, including assessments of semantics, phonology, reading, and spelling. Voxel-based morphometry was used to examine the relation between gray matter volumes and language measures within brain regions previously implicated in semantic and phonological processing. In accordance with the primary systems account, our findings indicate that spoken language performance is strongly predictive of reading/spelling profile in individuals with PPA and suggest that common networks of critical left hemisphere regions support central semantic and phonological processes recruited for spoken and written language.

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