Nosology of Primary Progressive Aphasia and the Neuropathology of Language

Mesulam, Marek Marsel; Coventry, Christina; Bigio, Eileen H.; Geula, Changiz; Thompson, Cynthia K.; Bonakdarpour, Borna; Gefen, Tamar; Rogalskı, Emily; Weıntraub, Sandra

doi:10.1007/978-3-030-51140-1_3

Cited by 33 publications

(37 citation statements)

References 97 publications

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“…Information regarding the incidence and prevalence of svPPA is limited, though the available data indicates a prevalence of about 4-8 per 100,000 [38]. The svPPA entity is very close to the previously developed "semantic dementia" diagnostic category [11,39], and most patients will fulfill both sets of criteria; see Mesulam et al [40] for more discussion on the nuances between these concepts.…”

Section: Semantic Variant Primary Progressive Aphasiamentioning

confidence: 95%

Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology

et al. 2021

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Frontotemporal dementia encompasses a group of clinical syndromes defined pathologically by degeneration of the frontal and temporal lobes. Historically, these syndromes have been challenging to diagnose, with an average of about three years between the time of symptom onset and the initial evaluation and diagnosis. Research in the field of neuroimaging has revealed numerous biomarkers of the various frontotemporal dementia syndromes, which has provided clinicians with a method of narrowing the differential diagnosis and improving diagnostic accuracy. As such, neuroimaging is considered a core investigative tool in the evaluation of neurodegenerative disorders. Furthermore, patterns of neurodegeneration correlate with the underlying neuropathological substrates of the frontotemporal dementia syndromes, which can aid clinicians in determining the underlying etiology and improve prognostication. This review explores the advancements in neuroimaging and discusses the phenotypic and pathologic features of behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia, as seen on structural magnetic resonance imaging and positron emission tomography.

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Section: Semantic Variant Primary Progressive Aphasiamentioning

confidence: 95%

Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology

et al. 2021

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“… 5 PPA is associated with multiple neuropathologic entities such as all major forms of frontotemporal lobar degeneration as well as Alzheimer’s disease. 6 Especially, lvPPA is widely known as progressive fluent aphasia associated with Alzheimer’s disease. 5 , 7 Here, we use the term ‘fluent’ to characterize the following speech: apraxia of speech or agrammatism is absent; and prosody, grammar, syntax and phrase length are normal on spontaneous speech.…”

Section: Introductionmentioning

confidence: 99%

Unclassified fluent variants of primary progressive aphasia: distinction from semantic and logopenic variants

Watanabe

Hikida

Ikeda

et al. 2022

Brain Communications

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Primary progressive aphasia, a neurodegenerative syndrome, presents mainly with language impairment. Both semantic and logopenic variants are fluent variants of primary progressive aphasia. Before the research criteria of primary progressive aphasia were proposed, progressive fluent aphasias, such as progressive anomic aphasia, transcortical sensory aphasia, and Wernicke’s aphasia, were reported as classical progressive fluent aphasias seen in Alzheimer’s disease. However, since the research criteria of primary progressive aphasia were established, classical fluent variants (other than semantic and logopenic variants) have been neglected and have not been included in the current classification of primary progressive aphasia. This study aimed to determine whether unclassified fluent variants (other than semantic and logopenic variants) can be manifestations of primary progressive aphasia. This study also reconfirmed the characteristics of classical progressive fluent aphasia, such as progressive anomic aphasia, progressive transcortical sensory aphasia, and progressive Wernicke’s aphasia as unclassified fluent variants of primary progressive aphasia, using comparison with the current model of primary progressive aphasia. Twelve consecutive patients with an unclassified fluent variant other than semantic or logopenic variant underwent language, neurological, neuropsychological, and neuroimaging (MRI and single-photon emission computed tomography) testing. Based on comprehensive language tests, we redefined the diagnoses as primary progressive anomic aphasia (n = 8), primary progressive transcortical sensory aphasia (n = 3), and primary progressive Wernicke’s aphasia (n = 1). Anomic aphasia was characterised by anomia but preserved repetition and comprehension; transcortical sensory aphasia by relatively preserved repetition but poor word comprehension; and Wernicke’s aphasia by poor repetition and word comprehension. In patients with anomic aphasia, voxel-based morphometry of MRI data revealed cortical atrophy, which was most prominent in the temporoparietal lobes, with no obvious lateralisation; in 2/3 of patients with transcortical sensory aphasia and in one patient with Wernicke’s aphasia, it revealed atrophy, predominantly in the left temporoparietal lobe. Statistical analysis of single-photon emission computed tomography using three-dimensional stereotactic surface projections revealed patterns of left-sided hypoperfusion in the majority of patients. The temporal and parietal lobes were involved in all cases; the degree of hypoperfusion was higher in patients with transcortical sensory aphasia or Wernicke’s aphasia than in patients with anomic aphasia. The present study demonstrated the clinical and imaging features of 12 patients with an unclassified fluent variant of primary progressive aphasia, which we redefined as primary progressive anomic aphasia, primary progressive transcortical sensory aphasia, and primary progressive Wernicke’s aphasia. Classical fluent variants other than semantic and logopenic variants can be found in primary progressive aphasia.

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“…Salient clinical symptoms of PPA include declines in understanding and expressing language through symbolic representations (e.g., words, sounds, letters) [ 15 ]. Language impairments remain the persistent and salient feature of PPA over the disease course [ 16 – 19 ]. With time, other cognitive and behavioral deficits can appear as disease spreads throughout the brain beyond the language network [ 19 ].…”

Section: Introductionmentioning

confidence: 99%

“…Language impairments remain the persistent and salient feature of PPA over the disease course [ 16 – 19 ]. With time, other cognitive and behavioral deficits can appear as disease spreads throughout the brain beyond the language network [ 19 ]. An estimated 60% of PPA is associated with a form of frontotemporal lobar degeneration and the remaining 40% with the neuropathology of Alzheimer’s disease [ 19 , 20 ].…”

Section: Introductionmentioning

confidence: 99%

Communication Bridge™-2 (CB2): an NIH Stage 2 randomized control trial of a speech-language intervention for communication impairments in individuals with mild to moderate primary progressive aphasia

Roberts

Rademaker

Salley

et al. 2022

Trials

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Background Primary progressive aphasia (PPA) is a clinical dementia syndrome. Impairments in language (speaking, reading, writing, and understanding) are the primary and persistent symptoms. These impairments progress insidiously and devastate communication confidence, participation, and quality of life for persons living with PPA. Currently, there are no effective disease modifying treatments for PPA. Speech-language interventions hold promise for mitigating communication challenges and language symptoms. However, evidence regarding their efficacy in PPA is of low quality and there are currently no rigorous randomized trials. Method Communication Bridge™-2 (CB2) is a Stage 2, superiority, single-blind, randomized, parallel group, active-control, behavioral clinical trial delivered virtually within a telehealth service delivery model to individuals with PPA. Ninety carefully characterized participants with clinically confirmed PPA will be randomized to one of two speech-language intervention arms: (1) Communication Bridge™ a dyadic intervention based in communication participation therapy models that incorporates salient training stimuli or (2) the control intervention a non-dyadic intervention based in impairment therapy models addressing word retrieval and language production that incorporates fixed stimuli. The superiority of Communication Bridge™ over the Control arm will be evaluated using primary outcomes of communication confidence and participation. Other outcomes include accuracy for trained words and scripts. Participants complete two therapy blocks over a 12-month period. Outcomes will be measured at baseline, at each therapy block, and at 12 months post enrollment. Discussion The CB2 trial will supply Level 2 evidence regarding the efficacy of the Communication Bridge™ intervention delivered in a telehealth service delivery model for individuals with mild to moderate PPA. An important by-product of the CB2 trial is that these data can be used to evaluate the efficacy of speech-language interventions delivered in both trial arms for persons with PPA. The impact of these data should not be overlooked as they will yield important insights examining why interventions work and for whom, which will advance effectiveness trials for speech-language interventions in PPA. Trial Registration ClinicalTrials.govNCT03371706. Registered prospectively on December 13, 2017.

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Nosology of Primary Progressive Aphasia and the Neuropathology of Language

Cited by 33 publications

References 97 publications

Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology

Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology

Unclassified fluent variants of primary progressive aphasia: distinction from semantic and logopenic variants

Communication Bridge™-2 (CB2): an NIH Stage 2 randomized control trial of a speech-language intervention for communication impairments in individuals with mild to moderate primary progressive aphasia

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