scholarly journals Primary Progressive Aphasia and the Left Hemisphere Language Network

2016 ◽  
Vol 15 (4) ◽  
pp. 93 ◽  
Author(s):  
M. Marsel Mesulam
Keyword(s):  
Left Hemisphere ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Network

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 Verbal and Nonverbal Memory in Primary Progressive Aphasia: The Three Words-Three Shapes Test

2013 ◽  
Vol 26 (1-2) ◽  
pp. 67-76 ◽  
Author(s):  
Sandra Weintraub ◽  
Emily Rogalski ◽  
Emily Shaw ◽  
Sabrina Sawlani ◽  
Alfred Rademaker ◽  
...  
Keyword(s):  
Left Hemisphere ◽  
Test Performance ◽  
Memory Loss ◽  
Primary Progressive Aphasia ◽  
Signs And Symptoms ◽  
Published Data ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
On Line ◽  
Memory Network

Objectives:To investigate cognitive components and mechanisms of learning and memory in primary progressive aphasia (PPA) using a simple clinical measure, the Three Words Three Shapes Test (3W3S).Background:PPA patients can complain of memory loss and may perform poorly in standard tests of memory. The extent to which these signs and symptoms reflect dysfunction of the left hemisphere language versus limbic memory network remains unknown.Methods:3W3S data from 26 patients with a clinical diagnosis of PPA were compared with previously published data from patients with typical dementia of the Alzheimer type (DAT) and cognitively healthy elders.Results:PPA patients showed two bottlenecks in new learning. First, they were impaired in the effortless (but not effortful) on-line encoding of verbal (but not non-verbal) items. Second, they were impaired in the retrieval (but not retention) of verbal (but not non-verbal) items. In contrast, DAT patients had impairments also in effortful on-line encoding and retention of verbal and nonverbal items.Conclusions:PPA selectively interferes with spontaneous on-line encoding and subsequent retrieval of verbal information. This combination may underlie poor memory test performance and is likely to reflect the dysfunction of the left hemisphere language rather than medial temporal memory network.

scholarly journals Cortical cholinergic denervation in primary progressive aphasia with Alzheimer pathology

Neurology ◽  
2019 ◽  
Vol 92 (14) ◽  
pp. e1580-e1588 ◽  
Author(s):  
M.-Marsel Mesulam ◽  
Nava Lalehzari ◽  
Farzan Rahmani ◽  
Daniel Ohm ◽  
Ryan Shahidehpour ◽  
...  
Keyword(s):  
Detailed Analysis ◽  
Left Hemisphere ◽  
Basal Forebrain ◽  
Cholinergic Neurons ◽  
Primary Progressive Aphasia ◽  
Neurotrophin Receptor ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Cortical Areas ◽  
The Status

ObjectiveTo investigate the status of the basal forebrain cholinergic system in primary progressive aphasia (PPA) as justification for cholinergic therapy.MethodsA cohort of 36 brains from PPA participants with the neuropathology of Alzheimer disease (PPA-AD, n = 14) or frontotemporal lobar degeneration (PPA-tau, n = 12; PPA-TDP, n = 10) were used for semiquantitative rating of degeneration and gliosis of basal forebrain cholinergic neurons (BFCN). A subpopulation of 5 PPA-AD and 7 control brains underwent detailed analysis of BFCN pathology and cortical cholinergic axonal loss employing immunohistochemical and histochemical methods and stereologic analysis.ResultsSemiquantitatively, 11 (∼80%) PPA-AD participants were rated as having moderate/severe BFCN loss and gliosis, whereas none of the PPA-tau and only 1 (10%) PPA-TDP participant received such a rating. Detailed analysis in the subpopulation of PPA-AD participants revealed substantial tangle formation, loss of BFCN, and degeneration of cortical cholinergic axons. Compared to controls, loss of p75 low affinity neurotrophin receptor-positive BFCN was detected in the PPA-AD participants (p < 0.01). Acetylcholinesterase-positive cholinergic axons in all cortical areas studied displayed loss in PPA-AD (p < 0.005–0.0001). The loss was more severe in the language-dominant left hemisphere and, within the left hemisphere, in language-affiliated cortical areas.ConclusionsOur results demonstrate prominent depletion of BFCN and cortical cholinergic axons in PPA-AD when compared with normal control or other neuropathologic variants of PPA. The demonstration of cholinergic denervation with an anatomy that fits the clinical picture suggests that cholinergic treatment is justified in patients with PPA who have positive AD biomarkers.

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
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