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

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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First Symptoms of Primary Progressive Aphasia and Alzheimer's Disease in Brazilian Individuals

Frontiers in Neurology ◽

10.3389/fneur.2021.628406 ◽

2021 ◽

Vol 12 ◽

Author(s):

Talita Gallas dos Reis ◽

Thais Helena Machado ◽

Paulo Caramelli ◽

Francisco Scornavacca ◽

Liana Lisboa Fernandez ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Differential Diagnosis ◽

Latin American ◽

Primary Progressive Aphasia ◽

Motor Speech ◽

Progressive Aphasia ◽

Primary Progressive ◽

Initial Symptoms ◽

Semantic Variant

Background: Primary Progressive Aphasia (PPA) is characterized by progressive language impairment due to focal degeneration of brain areas related to linguistic processing. The detection and differential diagnosis of PPA can be difficult with clinical features that may overlap with features of other neurological conditions, such as Alzheimer's disease (AD). The scientific production on PPA in Latin American patients is still scarce. This study investigated the first symptoms in a Brazilian sample of patients with PPA in comparison with AD patients.Method: We compared the first symptoms reported by caregivers of people with PPA (n = 20; semantic variant n = 8, non-fluent variant n = 7, logopenic variant n = 3, and unclassified cases n = 2) and AD (n = 16). Data were collected through the application of a structured questionnaire that was presented in an interview format to the caregiver who knew the patient best.Results: Anomia, paraphasias and motor speech difficulties were the first symptoms capable of differentiating patients with PPA from those with AD, while memory was exclusive of AD. Among the PPA variants, anomia was the initial symptom associated with the semantic variant, while motor speech difficulties were associated with the non-fluent variant. The results are discussed considering the unique cultural and sociodemographic characteristics of this studied population.Conclusion: This study demonstrated that some of the initial symptoms of PPA patients may be unique to clinical variants of PPA and of AD, and their investigation may be useful for the early and differential diagnosis of this population.

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Supplemental Material for The Concept of Regularization: Resolving the Problem of Surface Dyslexia in Semantic Variant Primary Progressive Aphasia Across Different Languages

Neuropsychology ◽

10.1037/neu0000611.supp ◽

2019 ◽

Keyword(s):

Primary Progressive Aphasia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Semantic Variant ◽

Surface Dyslexia

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Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

Current Neurology and Neuroscience Reports ◽

10.1007/s11910-021-01097-z ◽

2021 ◽

Vol 21 (3) ◽

Author(s):

Justina Ruksenaite ◽

Anna Volkmer ◽

Jessica Jiang ◽

Jeremy CS Johnson ◽

Charles R Marshall ◽

...

Keyword(s):

Care Management ◽

Primary Progressive Aphasia ◽

Disease Stage ◽

Major Step ◽

Progressive Aphasia ◽

Primary Progressive ◽

New Findings ◽

Critical Issues ◽

Semantic Variant ◽

Auditory Impairments

Abstract Purpose of Review The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers. Recent Findings Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering ‘fragmentary’ syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed. Summary New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.

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Breakdowns in Informativeness of Naturalistic Speech Production in Primary Progressive Aphasia

Brain Sciences ◽

10.3390/brainsci11020130 ◽

2021 ◽

Vol 11 (2) ◽

pp. 130

Author(s):

Jeanne Gallée ◽

Claire Cordella ◽

Evelina Fedorenko ◽

Daisy Hochberg ◽

Alexandra Touroutoglou ◽

...

Keyword(s):

Primary Progressive Aphasia ◽

Speech Analysis ◽

Clinical Settings ◽

Functional Communication ◽

Or Efficiency ◽

Progressive Aphasia ◽

Primary Progressive ◽

Communication Impairment ◽

Semantic Variant ◽

Lower Production

“Functional communication” refers to an individual’s ability to communicate effectively in his or her everyday environment, and thus is a paramount skill to monitor and target therapeutically in people with aphasia. However, traditional controlled-paradigm assessments commonly used in both research and clinical settings often fail to adequately capture this ability. In the current study, facets of functional communication were measured from picture-elicited speech samples from 70 individuals with mild primary progressive aphasia (PPA), including the three variants, and 31 age-matched controls. Building upon methods recently used by Berube et al. (2019), we measured the informativeness of speech by quantifying the content of each patient’s description that was relevant to a picture relative to the total amount of speech they produced. Importantly, form-based errors, such as mispronunciations of words, unusual word choices, or grammatical mistakes are not penalized in this approach. We found that the relative informativeness, or efficiency, of speech was preserved in non-fluent variant PPA patients as compared with controls, whereas the logopenic and semantic variant PPA patients produced significantly less informative output. Furthermore, reduced informativeness in the semantic variant is attributable to a lower production of content units and a propensity for self-referential tangents, whereas for the logopenic variant, a lower production of content units and relatively ”empty” speech and false starts contribute to this reduction. These findings demonstrate that functional communication impairment does not uniformly affect all the PPA variants and highlight the utility of naturalistic speech analysis for measuring the breakdown of functional communication in PPA.

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