Considerations in Subtyping and Monitoring of Symptom Progression in Primary Progressive Aphasia

2014 ◽  
Vol 24 (4) ◽  
pp. 122-127
Author(s):  
Daisy Sapolsky ◽  
Kimiko Domoto-Reilly ◽  
Bradford C. Dickerson
Keyword(s):  
Critical Role ◽  
Primary Progressive Aphasia ◽  
Family Counseling ◽  
Language Abilities ◽  
Language Preservation ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Speech Language Pathologist ◽  
Initial Symptoms ◽  
Symptom Progression

A speech-language pathologist (SLP) may be one of the initial clinical providers for a patient with primary progressive aphasia (PPA), a group of neurodegenerative diseases involving the selective, progressive deterioration of speech and/or language abilities. While the three primary subtypes of PPA have distinct profiles of language preservation and impairment, the process of identifying the subtype can be challenging for many reasons, including subtle initial symptoms which can be difficult to detect on standard testing batteries. Early and accurate subtyping is important for clinical and research applications, which we will discuss here. The SLP plays a critical role in the initial subtyping process, as well as in helping to confirm that the course and presentation are consistent with a root diagnosis of PPA, as opposed to normal aging or other etiologies. The involvement of the SLP over time then focuses on monitoring symptom severity and progression, which is a particularly relevant issue for this population. The SLP can apply this information to inform treatment planning and patient/family counseling, and to assess potential benefits from interventions including conventional and novel speech therapies, and pharmaceutical treatments as they become available.

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.

scholarly journals Thinking Outside the Stroke: Treating Primary Progressive Aphasia (PPA)

2012 ◽  
Vol 17 (2) ◽  
pp. 37-49 ◽  
Author(s):  
Becky Khayum ◽  
Christina Wieneke ◽  
Emily Rogalski ◽  
Jaimie Robinson ◽  
Mary O’Hara
Keyword(s):  
Quality Of Life ◽  
Brain Injury ◽  
Cognitive Abilities ◽  
Speech Therapy ◽  
Primary Progressive Aphasia ◽  
Supportive Services ◽  
Language Abilities ◽  
Progressive Aphasia ◽  
Primary Progressive

In this article, we explore the symptoms, cause, treatment potential, and supportive services for individuals diagnosed with Primary Progressive Aphasia (PPA). Although it is possible to regain certain cognitive abilities with stroke or brain injury, in PPA, language abilities worsen and other symptoms emerge with time, shortening the lifespan. The goal of speech therapy for PPA is not to regain lost language, but rather to maximize communication for as long as possible. In this article, we offer information and tools for speech-language pathologists to help people living with PPA achieve these goals and improve overall quality of life.

scholarly journals Phonological Processing in Primary Progressive Aphasia

2016 ◽  
Vol 28 (2) ◽  
pp. 210-222 ◽  
Author(s):  
Maya L. Henry ◽  
Stephen M. Wilson ◽  
Miranda C. Babiak ◽  
Maria Luisa Mandelli ◽  
Pelagie M. Beeson ◽  
...  
Keyword(s):  
Language Processing ◽  
Phonological Processing ◽  
Critical Role ◽  
Dorsal Stream ◽  
Primary Progressive Aphasia ◽  
Binary Logistic Regression ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Dorsal Pathway ◽  
High Degree

Individuals with primary progressive aphasia (PPA) show selective breakdown in regions within the proposed dorsal (articulatory–phonological) and ventral (lexical–semantic) pathways involved in language processing. Phonological STM impairment, which has been attributed to selective damage to dorsal pathway structures, is considered to be a distinctive feature of the logopenic variant of PPA. By contrast, phonological abilities are considered to be relatively spared in the semantic variant and are largely unexplored in the nonfluent/agrammatic variant. Comprehensive assessment of phonological ability in the three variants of PPA has not been undertaken. We investigated phonological processing skills in a group of participants with PPA as well as healthy controls, with the goal of identifying whether patterns of performance support the dorsal versus ventral functional–anatomical framework and to discern whether phonological ability differs among PPA subtypes. We also explored the neural bases of phonological performance using voxel-based morphometry. Phonological performance was impaired in patients with damage to dorsal pathway structures (nonfluent/agrammatic and logopenic variants), with logopenic participants demonstrating particular difficulty on tasks involving nonwords. Binary logistic regression revealed that select phonological tasks predicted diagnostic group membership in the less fluent variants of PPA with a high degree of accuracy, particularly in conjunction with a motor speech measure. Brain–behavior correlations indicated a significant association between the integrity of gray matter in frontal and temporoparietal regions of the left hemisphere and phonological skill. Findings confirm the critical role of dorsal stream structures in phonological processing and demonstrate unique patterns of impaired phonological processing in logopenic and nonfluent/agrammatic variants of PPA.

scholarly journals Language training for oral and written naming impairment in primary progressive aphasia: a review

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Ilaria Pagnoni ◽  
Elena Gobbi ◽  
Enrico Premi ◽  
Barbara Borroni ◽  
Giuliano Binetti ◽  
...  
Keyword(s):  
Semantic Processing ◽  
Neurodegenerative Disorder ◽  
Processing System ◽  
Primary Progressive Aphasia ◽  
Main Body ◽  
Language Training ◽  
Language Abilities ◽  
Large Variability ◽  
Progressive Aphasia ◽  
Primary Progressive

Abstract Background Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by a gradual, insidious and progressive loss of language abilities, with naming difficulties being an early and persistent impairment common to all three variants. In the absence of effective pharmacological treatments and given the progressive nature of the disorder, in the past few decades, many studies have investigated the effectiveness of language training to minimize the functional impact of word-finding difficulties in daily life. Main body We review language treatments most commonly used in clinical practice among patients with different variants of PPA, with a focus on the enhancement of spoken and written naming abilities. Generalization of gains to the ability to name untrained stimuli or to other language abilities and the maintenance of these results over time are also discussed. Forty-eight studies were included in this literature review, identifying four main types of language treatment: a) lexical retrieval treatment, b) phonological and/or orthographic treatment, c) semantic treatment, and d) a multimodality approach treatment. Overall, language training is able to induce immediate improvements of naming abilities in all variants of PPA. Moreover, despite the large variability among results, generalization and long-term effects can be recorded after the training. The reviewed studies also suggest that one factor that determines the choice of a particular approach is the compromised components of the lexical/semantic processing system. Conclusion The majority of studies have demonstrated improvements of naming abilities following language treatments. Given the progressive nature of PPA, it is essential to apply language treatment in the early stages of the disease.

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

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.

scholarly journals Sleep talking and primary progressive aphasia: case study and autopsy findings in a patient with logopenic primary progressive aphasia and dementia with Lewy bodies

2019 ◽  
Vol 12 (5) ◽  
pp. e228938 ◽  
Author(s):  
Alexandra Clemans Apple ◽  
Qinwen Mao ◽  
Eileen Bigio ◽  
Borna Bonakdarpour
Keyword(s):  
Right Hemisphere ◽  
Lewy Bodies ◽  
Primary Progressive Aphasia ◽  
Language Abilities ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Speech Output ◽  
Sleep Talking ◽  
The Right

This case study highlights the parasomnia behaviours of an individual with primary progressive aphasia, a type of dementia known for decline in language abilities. Despite a paucity of speech during the day, this individual had concurrent sleep talking at night; a combination which, to our knowledge, has never been reported before. Post-mortem pathology confirmed clinical suspicion of both Alzheimer and Lewy body diseases, both asymmetric to the left side. Given this rare left-sided asymmetrical pathology, we hypothesise that the relatively preserved right hemisphere may have allowed for access to intact overlearned phrases which usually originate from the right hemisphere to appear while asleep. A second hypothesis is also presented which postulates that bottom-up processing may have overridden top-down apathy during sleep and allowed for speech output in this case.

scholarly journals Neural Correlates of Letter and Semantic Fluency in Primary Progressive Aphasia

2021 ◽  
Vol 12 (1) ◽  
pp. 1
Author(s):  
Marianna Riello ◽  
Constantine E. Frangakis ◽  
Bronte Ficek ◽  
Kimberly T. Webster ◽  
John E. Desmond ◽  
...  
Keyword(s):  
Cognitive Task ◽  
Critical Role ◽  
Demographic Factors ◽  
Primary Progressive Aphasia ◽  
Volumetric Analysis ◽  
Clinical Severity ◽  
Semantic Fluency ◽  
Brain Areas ◽  
Progressive Aphasia ◽  
Primary Progressive

Verbal fluency (VF) is an informative cognitive task. Lesion and functional imaging studies implicate distinct cerebral areas that support letter versus semantic fluency and the understanding of neural and cognitive mechanisms underlying task performance. Most lesion studies include chronic stroke patients. People with primary progressive aphasia (PPA) provide complementary evidence for lesion-deficit associations, as different brain areas are affected in stroke versus PPA. In the present study we sought to determine imaging, clinical and demographic correlates of VF in PPA. Thirty-five patients with PPA underwent an assessment with letter and category VF tasks, evaluation of clinical features and an MRI scan for volumetric analysis. We used stepwise regression models to determine which brain areas are associated with VF performance while acknowledging the independent contribution of clinical and demographic factors. Letter fluency was predominantly associated with language severity (R2 = 38%), and correlated with the volume of the left superior temporal regions (R2 = 12%) and the right dorsolateral prefrontal area (R2 = 5%). Semantic fluency was predominantly associated with dementia severity (R2 = 47%) and correlated with the volume of the left inferior temporal gyrus (R2 = 7%). No other variables were significantly associated with performance in the two VF tasks. We concluded that, independently of disease severity, letter fluency is significantly associated with the volume of frontal and temporal areas whereas semantic fluency is associated mainly with the volume of temporal areas. Furthermore, our findings indicated that clinical severity plays a critical role in explaining VF performance in PPA, compared to the other clinical and demographic factors.

scholarly journals Efficacy of Noninvasive Brain Stimulation (tDCS or TMS) Paired with Language Therapy in the Treatment of Primary Progressive Aphasia: An Exploratory Meta-Analysis

2020 ◽  
Vol 10 (9) ◽  
pp. 597
Author(s):  
Nicole R. Nissim ◽  
Paul J. Moberg ◽  
Roy H. Hamilton
Keyword(s):  
Brain Stimulation ◽  
Meta Analysis ◽  
Random Effect ◽  
Primary Progressive Aphasia ◽  
Language Outcomes ◽  
Language Therapy ◽  
Language Abilities ◽  
Noninvasive Brain Stimulation ◽  
Progressive Aphasia ◽  
Primary Progressive

Noninvasive brain stimulation techniques, such as transcranial direct current stimulation (tDCS) and transcranial magnetic stimulation (TMS), paired with behavioral language therapy, have demonstrated the capacity to enhance language abilities in primary progressive aphasia (PPA), a debilitating degenerative neurological syndrome that leads to declines in communication abilities. The aim of this meta-analysis is to systematically evaluate the efficacy of tDCS and TMS in improving language outcomes in PPA, explore the magnitude of effects between stimulation modalities, and examine potential moderators that may influence treatment effects. Standard mean differences for change in performance from baseline to post-stimulation on language-related tasks were evaluated. Six tDCS studies and two repetitive TMS studies met inclusion criteria and provided 22 effects in the analysis. Random effect models revealed a significant, heterogeneous, and moderate effect size for tDCS and TMS in the enhancement of language outcomes. Findings demonstrate that naming ability significantly improves due to brain stimulation, an effect found to be largely driven by tDCS. Future randomized controlled trials are needed to determine long-term effectiveness of noninvasive brain stimulation techniques on language abilities, further delineate the efficacy of tDCS and TMS, and identify optimal parameters to enable the greatest gains for persons with PPA.

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