An Overview on Primary Progressive Aphasia and Its Variants

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

Brain Sciences ◽

10.3390/brainsci11060815 ◽

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.

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Primary Progressive Aphasia Presenting With Neuropsychiatric Symptoms

Journal of Geriatric Psychiatry and Neurology ◽

10.1177/08919887211036189 ◽

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.

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

Journal of Alzheimer s Disease ◽

10.3233/jad-210392 ◽

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.

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Stealing Cookies in the Twenty-First Century: Measures of Spoken Narrative in Healthy Versus Speakers With Aphasia

American Journal of Speech-Language Pathology ◽

10.1044/2018_ajslp-17-0131 ◽

2019 ◽

Vol 28 (1S) ◽

pp. 321-329 ◽

Cited By ~ 1

Author(s):

Shauna Berube ◽

Jodi Nonnemacher ◽

Cornelia Demsky ◽

Shenly Glenn ◽

Sadhvi Saxena ◽

...

Keyword(s):

Preliminary Data ◽

Primary Progressive Aphasia ◽

Total Content ◽

First Century ◽

Healthy Controls ◽

Progressive Aphasia ◽

Primary Progressive ◽

Link Type ◽

The Future ◽

Language Output

Purpose Our goal was to evaluate an updated version of the “Cookie Theft” picture by obtaining norms based on picture descriptions by healthy controls for total content units (CUs), syllables per CU, and the ratio of left–right CUs. In addition, we aimed to compare these measures from healthy controls to picture descriptions obtained from individuals with poststroke aphasia and primary progressive aphasia (PPA) to assess whether these measures can capture impairments in content and efficiency of communication. Method Using an updated version of this picture, we analyzed descriptions from 50 healthy controls to develop norms for numbers of syllables, total CUs, syllables per CU, and left–right CU. We provide preliminary data from 44 individuals with aphasia (19 with poststroke aphasia and 25 with PPA). Results A total of 96 CUs were established based on the written transcriptions of spoken picture descriptions of the 50 control participants. There was a significant effect of group on total CUs, syllables, syllables per CU, and left–right CUs. The poststroke participants produced significantly fewer total CU and syllables than those with PPA. Each aphasic group produced significantly fewer total CUs, fewer syllables, more syllables per CU, and lower left–right CUs (indicating a right-sided bias) compared to controls. Conclusions Results show that the measures of numbers of syllables, total CUs, syllables per CU, and left–right CUs can distinguish language output of individuals with aphasia from controls and capture impairments in content and efficiency of communication. A limitation of this study is that we evaluated only 44 individuals with aphasia. In the future, we will evaluate other measures, such as CUs per minute, lexical variability, grammaticality, and ratio of nouns to verbs. Supplemental Material https://doi.org/10.23641/asha.7015223

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

Neurology India ◽

10.4103/0028-3886.16398 ◽

2005 ◽

Vol 53 (2) ◽

pp. 162 ◽

Cited By ~ 7

Author(s):

PS Mathuranath ◽

Annamma George

Keyword(s):

Comparative Study ◽

Primary Progressive Aphasia ◽

Semantic Dementia ◽

Nonfluent Aphasia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Progressive Nonfluent Aphasia

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

Dementia & Neuropsychologia ◽

10.1590/s1980-57642013dn70100017 ◽

2013 ◽

Vol 7 (1) ◽

pp. 110-121 ◽

Cited By ~ 6

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.

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