Typical and atypical pathology in primary progressive aphasia variants

  title={Typical and atypical pathology in primary progressive aphasia variants},
  author={Edoardo Gioele Spinelli and Maria Luisa Mandelli and Zachary A. Miller and Miguel Angel Santos-Santos and Stephen M. Wilson and Federica Agosta and Lea T. Grinberg and Eric J Huang and John Q. Trojanowski and Marita Meyer and Maya L. Henry and Giancarlo Comi and Gil D. Rabinovici and Howard J. Rosen and Massimo Filippi and Bruce L Miller and William W. Seeley and Maria Luisa Gorno-Tempini},
  journal={Annals of Neurology},
To characterize in vivo signatures of pathological diagnosis in a large cohort of patients with primary progressive aphasia (PPA) variants defined by current diagnostic classification. 

Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia

To estimate the prevalence of amyloid positivity, defined by positron emission tomography (PET)/cerebrospinal fluid (CSF) biomarkers and/or neuropathological examination, in primary progressive

Lewy Body Disease is a Contributor to Logopenic Progressive Aphasia Phenotype

Clinical features, metabolism and digital pathology in patients with logopenic progressive aphasia and pathologic diagnosis of diffuse Lewy body disease (DLBD) are described and compared to patients with LPA with other pathologies, as well as patients with classical features of probable dementia with Lewy bodies (pDLB).

Longitudinal naming and repetition relates to AD pathology and burden in autopsy‐confirmed primary progressive aphasia

In primary progressive aphasia (PPA) patients with autopsy‐confirmed Alzheimer's disease (AD) or frontotemporal lobar degeneration (FLTD), we tested how the core clinical features of logopenic

Treatment for Anomia in Bilingual Speakers with Progressive Aphasia

Anomia is an early and prominent feature of primary progressive aphasia (PPA) and other neurodegenerative disorders. Research investigating treatment for lexical retrieval impairment has focused

Graded, multidimensional intra- and intergroup variations in primary progressive aphasia and post-stroke aphasia

Language impairments caused by stroke and by neurodegeneration have overlapping symptomatology and nomenclature but have rarely been compared in detail, and Ingram et al. show that the two have considerable features in common and the variations of phenotype reflect four graded behavioural dimensions.

An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

The Movement Disorder Society clinical criteria for progressive supranuclear palsy (PSP) provide a framework for assessing the presence/severity of clinical symptoms and define a speech/language

Neuropsychiatric Aspects of Frontotemporal Dementia.

Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia

It is hypothesized that cognitive and neuroimaging findings consistent with damage to multiple brain networks, each associated with vulnerability to certain molecular disease subtypes, could indicate mixed pathology.

Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study

PPA cases could be more heterogeneous in their etiology than previously thought and underlying neurodegenerative comorbidities should be considered in routine practice, especially if the clinical presentation of PPA is atypical.



Clinical and pathological characterization of progressive aphasia

This study aimed to characterize a large group of progressive aphasic patients from a single center first clinically by case note review, and then pathologically.

Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia

To identify predictors of Alzheimer's disease (AD) versus frontotemporal lobar degeneration pathology in primary progressive aphasia (PPA), and determine whether the AD pathology is atypically

Primary progressive aphasia: clinicopathological correlations

  • M. Grossman
  • Psychology, Medicine
    Nature Reviews Neurology
  • 2010
Clinopathological correlations in PPA suggest an association between a specific PPA variant and an underlying pathology, although many cases of PPA are associated with an unexpected pathology.

Temporal Variant Frontotemporal Dementia Is Associated with Globular Glial Tauopathy

This research presents a novel probabilistic approach that allows us to assess the importance of knowing the carrier and removal status of canine coronavirus as a source of infection for other animals.

Aβ amyloid and glucose metabolism in three variants of primary progressive aphasia

The relationships between language presentation, Aβ amyloidosis, and glucose metabolism in three PPA variants using [11C]‐Pittsburgh compound B and [18F]‐labeled fluorodeoxyglucose positron emission tomography are studied.

Prediction of pathology in primary progressive language and speech disorders

The different anatomic distribution of the pathologic lesions could explain the different correlations between opercular and subcortical regions in tau pathologies with progressive anarthria and the left frontotemporal cortex in TDP-43-positive frontOTemporal lobar degeneration (FTLD-TDP).

Subtypes of progressive aphasia: application of the International Consensus Criteria and validation using β-amyloid imaging.

Insight is offered into the biological basis of clinical manifestations of Alzheimer's disease, which appear topographically independent of β-amyloid load and can be applied to the majority of cases with primary progressive aphasic using a simple speech and language assessment scale based upon four key variables.

In vivo signatures of nonfluent/agrammatic primary progressive aphasia caused by FTLD pathology

Clinical features in sporadic nfVPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks and it is proposed that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FT LD-TDP.

Asymmetry and heterogeneity of Alzheimer's and frontotemporal pathology in primary progressive aphasia.

Individual features of the aphasia were as informative of underlying pathology as more laborious subtype diagnoses and no clinical pattern was pathognomonic of a specific neuropathology type, highlighting the critical role of biomarkers for diagnosing the underlying disease.