scholarly journals MODULARITY and granularity ACROSS the language network-A primary progressive aphasia perspective

Cortex ◽  
2021 ◽  
Author(s):  
M.-Marsel Mesulam ◽  
Christina A. Coventry ◽  
Benjamin M. Rader ◽  
Alan Kuang ◽  
Jaiashre Sridhar ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network

scholarly journals Primary progressive aphasia and the evolving neurology of the language network

2014 ◽  
Vol 10 (10) ◽  
pp. 554-569 ◽  
Author(s):  
M.-Marsel Mesulam ◽  
Emily J. Rogalski ◽  
Christina Wieneke ◽  
Robert S. Hurley ◽  
Changiz Geula ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network

scholarly journals Primary progressive aphasia: A dementia of the language network

2013 ◽  
Vol 7 (1) ◽  
pp. 2-9 ◽  
Author(s):  
Marsel Mesulam
Keyword(s):  
Language Impairment ◽  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Medical Attention ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Clinical Variants ◽  
And Behavior ◽  
Work Up ◽  
Language Network

ABSTRACT Primary progressive aphasia (PPA) is a clinical syndrome diagnosed when three core criteria are met. First, there should be a language impairment (i.e., aphasia) that interferes with the usage or comprehension of words. Second, the neurological work-up should determine that the disease is neurodegenerative, and therefore progressive. Third, the aphasia should arise in relative isolation, without equivalent deficits of comportment or episodic memory. The language impairment can be fluent or non-fluent and may or may not interfere with word comprehension. Memory for recent events is preserved although memory scores obtained in verbally mediated tests may be abnormal. Minor changes in personality and behavior may be present but are not the leading factors that bring the patient to medical attention or that limit daily living activities. This distinctive clinical pattern is most conspicuous in the initial stages of the disease, and reflects a relatively selective atrophy of the language network, usually located in the left hemisphere. There are different clinical variants of PPA, each with a characteristic pattern of atrophy. The underlying neuropathological diseases are heterogeneous and can include Alzheimer's disease as well as frontotemporal lobar degeneration. The clinician's task is to recognize PPA and differentiate it from other neurodegenerative phenotypes, use biomarkers to surmise the nature of the underlying neuropathology, and institute the most fitting multimodal interventions.

scholarly journals Perturbations of language network connectivity in primary progressive aphasia

Cortex ◽  
2019 ◽  
Vol 121 ◽  
pp. 468-480
Author(s):  
Borna Bonakdarpour ◽  
Robert S. Hurley ◽  
Allan R. Wang ◽  
Hernando R. Fereira ◽  
Anisha Basu ◽  
...  
Keyword(s):  
Network Connectivity ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network

Neuroimaging in Primary Progressive Aphasia

2014 ◽  
Vol 24 (4) ◽  
pp. 145-156 ◽  
Author(s):  
Borna Bonakdarpour
Keyword(s):  
Computed Tomography ◽  
Diffusion Tensor ◽  
Functional Neuroimaging ◽  
Photon Emission ◽  
Primary Progressive Aphasia ◽  
Emission Computed Tomography ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Diagnostic Certainty ◽  
Language Network

Neuroimaging is a standard part of a primary progressive aphasia (PPA) diagnostic work-up and an important component of research investigating changes in the speech-language network in patients with PPA. In this paper, structural neuroimaging, including computed tomography (CT), magnetic resonance imaging (MRI), and diffusion tensor imaging (DTI), as well as functional neuroimaging, including single photon emission computed tomography (SPECT), positron emission tomography (PET), and functional MRI (fMRI), are discussed. Neuroimaging, in conjunction with meticulous clinical and neuropsychological evaluation, can increase diagnostic certainty for PPA subtyping and identification of underlying pathology, which is important for justification of potential pharmacological treatments, such as cholinesterase inhibitors. MRI and, more recently, DTI, have expanded our knowledge of structural brain changes in PPA, including gray matter abnormalities as well as alterations in neuronal tracts. SPECT and PET provide information regarding brain regional blood perfusion (SPECT) or metabolism (PET). Recently, thanks to PET ligands that bind to amyloid protein, it has become possible to diagnose or rule out Alzheimer pathology as a cause for PPA and tau imaging may be forthcoming. Finally, fMRI provides a unique window into brain-behavior relations for language as well as reorganization of the language network in disease. fMRI has also been used to gauge the effects of therapeutic interventions, including language treatment, and can be used for implementation of neuromodulatory mediations, such as repetitive transcranial magnetic stimulation (TMS).

Axonal injury within language network in primary progressive aphasia

2003 ◽  
Vol 53 (2) ◽  
pp. 242-247 ◽  
Author(s):  
Marco Catani ◽  
Massimo Piccirilli ◽  
Antonio Cherubini ◽  
Roberto Tarducci ◽  
Tiziana Sciarma ◽  
...  
Keyword(s):  
Axonal Injury ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network

scholarly journals Familial language network vulnerability in primary progressive aphasia

Neurology ◽  
2020 ◽  
Vol 95 (7) ◽  
pp. e847-e855 ◽  
Author(s):  
Sandra Weintraub ◽  
Benjamin Rader ◽  
Christina Coventry ◽  
Jaiashre Sridhar ◽  
Jessica Wood ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Structural Mri ◽  
Cognitive Test ◽  
Network Vulnerability ◽  
Total Brain Volume ◽  
Temporal Area ◽  
Course Of Illness ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network

ObjectiveTo investigate evidence of the potential role of early cortical vulnerability in the development of primary progressive aphasia (PPA).MethodA woman with a diagnosis of PPA and her 9 adult siblings, 7 with developmental language disabilities, underwent neuropsychological testing, structural MRI, and resting-state fMRI. Whole-exome sequencing was conducted for genes associated with dyslexia or with neurodegenerative dementia.ResultsThe siblings demonstrated lower verbal than nonverbal cognitive test scores in a developmental dyslexia pattern. On structural MRI, although the siblings did not differ from controls in total brain volume, the left hemisphere language area volume was significantly smaller than the right. Furthermore, cortical connectivity between the left superior temporal area, previously identified as the region of peak atrophy in the proband early in the course of illness, and adjacent language network components, including the planum temporale, was decreased in the siblings. No distinctive genetic signatures were identified.ConclusionThis report further supports the hypothesis that at least some cases of PPA may be based on a familial language network vulnerability that interferes with the acquisition of language in some members and that makes the language network a locus of least resistance to the effects of an independently late-arising neurodegenerative disease in others. This association offers a conceptual model to explain why identical neurodegenerative diseases may selectively target the language network in some individuals while targeting networks that regulate memory or behavior in others. The genetic basis for this vulnerability remains to be determined.

Primary Progressive Aphasia Associated With GRN Mutations: New Insights Into the Non-amyloid Logopenic Variant

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012174
Author(s):  
Dario Saracino ◽  
Sophie Ferrieux ◽  
Marie Noguès-Lassiaille ◽  
Marion Houot ◽  
Aurélie Funkiewiez ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Cross Sectional Study ◽  
Csf Biomarkers ◽  
Cognitive Profiles ◽  
Apraxia Of Speech ◽  
Cross Sectional ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Left Posterior ◽  
Language Network

Objective.To determine relative frequencies and linguistic profiles of primary progressive aphasia (PPA) variants associated with progranulin (GRN) mutations, and study their neuroanatomical correlates.Methods.PPA patients carrying GRN mutations (PPA-GRN) were selected amongst a national prospective research cohort of 1,696 frontotemporal dementia (FTD) patients, including 235 patients with PPA. All PPA patients with amyloid-positive CSF biomarkers were excluded. In this cross-sectional study, speech/language and cognitive profiles were characterized with standardized evaluations, and grey matter (GM) atrophy patterns using voxel-based morphometry. Comparisons were performed with controls, and sporadic PPA patients.Results.Among the overall population of 235 patients, 45 (19%) carried GRN mutations. We studied 32 of these and showed that logopenic PPA (lvPPA) was the most frequent linguistic variant (13, 41%), followed by non-fluent/agrammatic (nfvPPA: 9, 28%) and mixed forms (8, 25%). Semantic variant was rather rare (2, 6%). LvPPA patients, qualified as non-amyloid-lvPPA, presented canonical logopenic deficit. Seven out of 13 had a pure form, six showed subtle additional linguistic deficits not fitting criteria for mixed PPA, hence labelled as “logopenic-spectrum variant”. GM atrophy primarily involved left posterior temporal gyrus, mirroring neuroanatomical changes of amyloid-positive-lvPPA. NfvPPA patients presented agrammatism (89%) rather than apraxia of speech (11%).Conclusions.This study shows that most frequent PPA variant associated with GRN mutations is non-amyloid lvPPA, preceding nfvPPA and mixed forms, and illustrates that language network may be affected at different levels. GRN testing is indicated for PPA patients, whether familial or sporadic. This finding is important for upcoming GRN gene-specific therapies.

3Anatomical and functional analysis of language network changes in primary progressive aphasia

NeuroImage ◽  
2001 ◽  
Vol 13 (6) ◽  
pp. 608
Author(s):  
Sreepadma P. Sonty ◽  
M.-Marsel Mesulam ◽  
Cynthia Thompson ◽  
Nancy Johnson ◽  
Sandra Weintraub ◽  
...  
Keyword(s):  
Functional Analysis ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network
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