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Primary progressive aphasia

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https://www.readbyqxmd.com/read/28915686/evaluation-on-efficacy-and-safety-of-the-addition-of-x-knife-therapy-to-gefitinib-in-nsclc-patients-with-symptomatic-brain-metastases
#1
Linbo Cai, Xiaoguang Qiu, Haihong Yang, Mingyao Lai, Changguo Shan, Weiping Hong, Juan Li, Longhui Luo, Ping Zhang, Lichao Wang
BACKGROUND: Stereotactic radiosurgery (SRS) is a widely used therapy for brain metastases(BMs) in Non-small cell lung cancer(NSCLC). However, its role in symptomatic patients with EGFR mutation remains unclear. We have retrospectively reviewed the clinical data of patients with symptomatic BMs whom received SRS as a salvage approach and concurrent gifitinib therapy. METHODS: Seven patients with primary NSCLC, symptomatic BMs, and EGFR mutation were identified in a retrospective review of patients treated with SRS using X-knife at Guangdong 999 Brain Hospital between 1 January 2012 and 31 August 2014...
August 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28912300/-18-f-av-1451-binding-in-vivo-mirrors-the-expected-distribution-of-tdp-43-pathology-in-the-semantic-variant-of-primary-progressive-aphasia
#2
W R Bevan-Jones, Thomas E Cope, P Simon Jones, Luca Passamonti, Young T Hong, Tim D Fryer, Robert Arnold, Kieren S J Allinson, Jonathan P Coles, Franklin I Aigbirhio, Karalyn Patterson, John T O'Brien, James B Rowe
INTRODUCTION: Semantic dementia, including the semantic variant of primary progressive aphasia (svPPA), is strongly associated with TAR-DNA binding protein 43 (TDP-43) type C pathology. It provides a useful model in which to test the specificity of in vivo binding of the putative tau ligand [(18)F]AV-1451, which is elevated in frontotemporal lobar degeneration tauopathies. METHODS AND RESULTS: Seven patients (five with svPPA and two with 'right' semantic dementia) and 12 healthy controls underwent positron emission tomography brain imaging with [(18)F]AV-1451...
September 14, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28879086/emotion-detection-deficits-and-changes-in-personality-traits-linked-to-loss-of-white-matter-integrity-in-primary-progressive-aphasia
#3
Namita Multani, Sebastiano Galantucci, Stephen M Wilson, Tal Shany-Ur, Pardis Poorzand, Matthew E Growdon, Jung Yun Jang, Joel H Kramer, Bruce L Miller, Katherine P Rankin, Maria Luisa Gorno-Tempini, Maria Carmela Tartaglia
Non-cognitive features including personality changes are increasingly recognized in the three PPA variants (semantic-svPPA, non fluent-nfvPPA, and logopenic-lvPPA). However, differences in emotion processing among the PPA variants and its association with white matter tracts are unknown. We compared emotion detection across the three PPA variants and healthy controls (HC), and related them to white matter tract integrity and cortical degeneration. Personality traits in the PPA group were also examined in relation to white matter tracts...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28872040/structural-magnetic-resonance-imaging-in-frontotemporal-lobar-dementia
#4
Anne Bertrand, Sebastian Stroër, Isabelle Le Ber, Marc Teichmann, Didier Dormont
Frontotemporal lobar dementia (FTLD) is a heterogeneous group of neurodegenerative diseases. FTLD encompass: 1) behavioral forms, sometimes associated with amyotrophic lateral sclerosis; 2) linguistic forms (semantic and non-fluent primary progressive aphasia); 3) atypical parkinsonian syndromes (progressive supranuclear palsy and corticobasal syndrome). Standard brain MRI allows for strengthening the clinical suspicion of FTLD, by showing a pattern of atrophy in relation with the patient's clinical symptoms: frontotemporal anterior atrophy in behavioral forms; temporopolar or inferior left frontal atrophy in the linguistic forms; mesencephalic or corticosubcortical hemispheric atrophy in forms with atypical pakinsonism...
September 1, 2017: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
https://www.readbyqxmd.com/read/28867555/lexical-access-in-semantic-variant-ppa-evidence-for-a-post-semantic-contribution-to-naming-deficits
#5
Stephen M Wilson, Charlotte Dehollain, Sophie Ferrieux, Laura Hook, Marc Teichmann
The most salient clinical symptom of semantic variant primary progressive aphasia (PPA) is a profound and pervasive anomia. These patients' naming impairments have been shown to reflect in large part a domain-general deterioration of conceptual knowledge that impacts both linguistic and non-linguistic processing. However, it is possible that post-semantic stages of lexical access may also contribute to naming deficits. To clarify the stages at which lexical access breaks down in semantic variant PPA, eleven French-speaking patients were asked to name objects, and were then queried for semantic, lexical-syntactic, and word form information pertaining to the items they could not name...
August 31, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28853339/tdq-60-a-color-picture-naming-test-for-adults-and-elderly-people-validation-and-normalization-data
#6
Joël Macoir, Catherine Beaudoin, Josée Bluteau, Olivier Potvin, Maximiliano A Wilson
Word-finding difficulties are usually assessed with picture-naming tests. In this article, we present the TDQ-60, a new test designed to assess acquired lexical access deficits, taking into account semantics and psycholinguistic variables. The article includes three studies. Study 1 describes the development phase of the TDQ-60.  In study 2, healthy control participants and individuals with a diagnosis of the semantic variant of primary progressive aphasia were assessed to establish the convergent and discriminant validity of the TDQ-60...
August 30, 2017: Neuropsychology, Development, and Cognition. Section B, Aging, Neuropsychology and Cognition
https://www.readbyqxmd.com/read/28843341/artificial-grammar-learning-in-vascular-and-progressive-non-fluent-aphasias
#7
Thomas E Cope, Benjamin Wilson, Holly Robson, Rebecca Drinkall, Lauren Dean, Manon Grube, P Simon Jones, Karalyn Patterson, Timothy D Griffiths, James B Rowe, Christopher I Petkov
Patients with non-fluent aphasias display impairments of expressive and receptive grammar. This has been attributed to deficits in processing configurational and hierarchical sequencing relationships. This hypothesis had not been formally tested. It was also controversial whether impairments are specific to language, or reflect domain general deficits in processing structured auditory sequences. Here we used an artificial grammar learning paradigm to compare the abilities of controls to participants with agrammatic aphasia of two different aetiologies: stroke and frontotemporal dementia...
August 24, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28838910/assessment-of-iron-deposition-in-the-brain-in-frontotemporal-dementia-and-its-correlation-with-behavioral-traits
#8
R Sheelakumari, C Kesavadas, T Varghese, R M Sreedharan, B Thomas, J Verghese, P S Mathuranath
BACKGROUND AND PURPOSE: Brain iron deposition has been implicated as a major culprit in the pathophysiology of neurodegeneration. However, the quantitative assessment of iron in behavioral variant frontotemporal dementia and primary progressive aphasia brains has not been performed, to our knowledge. The aim of our study was to investigate the characteristic iron levels in the frontotemporal dementia subtypes using susceptibility-weighted imaging and report its association with behavioral profiles...
August 24, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28813486/differentiating-between-subtypes-of-primary-progressive-aphasia-and-mild-cognitive-impairment-on-a-modified-version-of-the-frontal-behavioral-inventory
#9
Donna C Tippett, Carol B Thompson, Cornelia Demsky, Rajani Sebastian, Amy Wright, Argye E Hillis
Behavioral assessment has been investigated in frontotemporal lobar degeneration and Alzheimer's disease, but has not been explored extensively in subtypes of primary progressive aphasia (PPA). We explored the ability of a modified version of the Frontal Behavioral Inventory (FBI-mod) to discriminate between patients with distinct subtypes of PPA and patients with mild cognitive impairment (MCI). We hypothesized that individuals with nonfluent agrammatic PPA (nfaPPA) would have higher negative behavior scores than other groups and that individuals with semantic variant PPA (svPPA) would have higher disinhibition scores than other groups...
2017: PloS One
https://www.readbyqxmd.com/read/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#10
REVIEW
Angela B Deutschländer, Owen A Ross, Dennis W Dickson, Zbigniew K Wszolek
The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here we review clinical, imaging, neuropathologic and genetic features of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and frontotemporal lobar degeneration (FTLD). The terms CBD and FTLD refer to pathologically confirmed cases of corticobasal syndrome (CBS) and frontotemporal dementia (FTD)...
August 12, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28803212/data-driven-classification-of-patients-with-primary-progressive-aphasia
#11
Paul Hoffman, Seyed Ahmad Sajjadi, Karalyn Patterson, Peter J Nestor
Current diagnostic criteria classify primary progressive aphasia into three variants-semantic (sv), nonfluent (nfv) and logopenic (lv) PPA-though the adequacy of this scheme is debated. This study took a data-driven approach, applying k-means clustering to data from 43 PPA patients. The algorithm grouped patients based on similarities in language, semantic and non-linguistic cognitive scores. The optimum solution consisted of three groups. One group, almost exclusively those diagnosed as svPPA, displayed a selective semantic impairment...
August 10, 2017: Brain and Language
https://www.readbyqxmd.com/read/28757671/slowed-articulation-rate-is-a-sensitive-diagnostic-marker-for-identifying-non-fluent-primary-progressive-aphasia
#12
Claire Cordella, Bradford C Dickerson, Megan Quimby, Yana Yunusova, Jordan R Green
BACKGROUND: Primary progressive aphasia (PPA) is a neurodegenerative aphasic syndrome with three distinct clinical variants: non-fluent (nfvPPA), logopenic (lvPPA), and semantic (svPPA). Speech (non-) fluency is a key diagnostic marker used to aid identification of the clinical variants, and researchers have been actively developing diagnostic tools to assess speech fluency. Current approaches reveal coarse differences in fluency between subgroups, but often fail to clearly differentiate nfvPPA from the variably fluent lvPPA...
2017: Aphasiology
https://www.readbyqxmd.com/read/28750682/behavioural-and-neuroanatomical-correlates-of-auditory-speech-analysis-in-primary-progressive-aphasias
#13
Chris J D Hardy, Jennifer L Agustus, Charles R Marshall, Camilla N Clark, Lucy L Russell, Rebecca L Bond, Emilie V Brotherhood, David L Thomas, Sebastian J Crutch, Jonathan D Rohrer, Jason D Warren
BACKGROUND: Non-verbal auditory impairment is increasingly recognised in the primary progressive aphasias (PPAs) but its relationship to speech processing and brain substrates has not been defined. Here we addressed these issues in patients representing the non-fluent variant (nfvPPA) and semantic variant (svPPA) syndromes of PPA. METHODS: We studied 19 patients with PPA in relation to 19 healthy older individuals. We manipulated three key auditory parameters-temporal regularity, phonemic spectral structure and prosodic predictability (an index of fundamental information content, or entropy)-in sequences of spoken syllables...
July 27, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28747523/-18-f-flortaucipir-pet-mri-correlations-in-non-amnestic-and-amnestic-variants-of-alzheimer-disease
#14
Ilya M Nasrallah, Yin Jie Chen, Meng-Kang Hsieh, Jeffrey S Philips, Kylie Ternes, Grace Stockbower, Yvette Sheline, Corey T McMillan, Murray Grossman, David A Wolk
Non-amnestic Alzheimer disease (AD) variants, including posterior cortical atrophy (PCA) and logopenic variant of primary progressive aphasia (lvPPA), differ in distributions of tau aggregates and neurodegeneration compared to amnestic AD. We evaluated whether (18)F-flortaucipir (also called (18)F-AV-1451) Positron Emission Tomography (PET), targeting tau aggregates, detects these differences and compared this to magnetic resonance imaging (MRI) measures of gray matter (GM) atrophy. Methods: 5 PCA, 4 lvPPA, 6 age-matched AD, and 6 control subjects underwent (18)F-flortaucipir PET and MRI...
July 26, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28713256/baseline-performance-predicts-tdcs-mediated-improvements-in-language-symptoms-in-primary-progressive-aphasia
#15
Eric M McConathey, Nicole C White, Felix Gervits, Sherry Ash, H Branch Coslett, Murray Grossman, Roy H Hamilton
Primary Progressive Aphasia (PPA) is a neurodegenerative condition characterized by insidious irreversible loss of language abilities. Prior studies suggest that transcranial direct current stimulation (tDCS) directed toward language areas of the brain may help to ameliorate symptoms of PPA. In the present sham-controlled study, we examined whether tDCS could be used to enhance language abilities (e.g., picture naming) in individuals with PPA variants primarily characterized by difficulties with speech production (non-fluent and logopenic)...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28662598/comparing-the-effects-of-clinician-and-caregiver-administered-lexical-retrieval-training-for-progressive-anomia
#16
Stephanie M Grasso, Kaleigh M Shuster, Maya L Henry
There is a growing body of literature indicating that lexical retrieval training can result in improved naming ability in individuals with neurodegenerative disease. Traditionally, treatment is administered by a speech-language pathologist, with little involvement of caregivers or carry-over of practice into the home. This study examined the effects of a lexical retrieval training programme that was implemented first by a clinician and, subsequently, by a trained caregiver. Two dyads, each consisting of one individual with anomia caused by neurodegenerative disease (one with mild cognitive impairment and one with logopenic primary progressive aphasia) and their caregiver, participated in the study...
June 30, 2017: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/28659753/neural-correlates-of-verbal-episodic-memory-and-lexical-retrieval-in-logopenic-variant-primary-progressive-aphasia
#17
Khaing T Win, John Pluta, Paul Yushkevich, David J Irwin, Corey T McMillan, Katya Rascovsky, David Wolk, Murray Grossman
Objective: Logopenic variant primary progressive aphasia (lvPPA) is commonly associated with Alzheimer's disease (AD) pathology. But lvPPA patients display different cognitive and anatomical profile from the common clinical AD patients, whose verbal episodic memory is primarily affected. Reports of verbal episodic memory difficulty in lvPPA are inconsistent, and we hypothesized that their lexical retrieval impairment contributes to verbal episodic memory performance and is associated with left middle temporal gyrus atrophy...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28624827/qualitative-assessment-of-verbal-fluency-performance-in-frontotemporal-dementia
#18
Esther van den Berg, Lize C Jiskoot, Mariëlle J H Grosveld, John C van Swieten, Janne M Papma
BACKGROUND/AIMS: Verbal fluency is impaired in patients with frontotemporal dementia (FTD) and primary progressive aphasia (PPA). This study explored qualitative differences in verbal fluency (clustering of words, switching between strategies) between FTD and PPA variants. METHODS: Twenty-nine patients with behavioral variant FTD (bvFTD) and 50 with PPA (13 nonfluent/agrammatic, 14 semantic, and 23 logopenic) performed a semantic and letter fluency task. Clustering (number of multiword strings) and switching (number of transitions between clustered and nonclustered words) were recorded by two independent raters...
2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28611820/white-matter-disruption-and-connected-speech-in-non-fluent-and-semantic-variants-of-primary-progressive-aphasia
#19
Karine Marcotte, Naida L Graham, Kathleen C Fraser, Jed A Meltzer, David F Tang-Wai, Tiffany W Chow, Morris Freedman, Carol Leonard, Sandra E Black, Elizabeth Rochon
Differential patterns of white matter disruption have recently been reported in the non-fluent (nfvPPA) and semantic (svPPA) variants of primary progressive aphasia (PPA). No single measure is sufficient to distinguish between the PPA variants, but connected speech allows for the quantification of multiple measures. The aim of the present study was to further investigate the white matter correlates associated with connected speech features in PPA. We examined the relationship between white matter metrics and connected speech deficits using an automated analysis of transcriptions of connected speech and diffusion tensor imaging in language-related tracts...
January 2017: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/28594853/the-unexpected-co-occurrence-of-grn-and-mapt-p-a152t-in-basque-families-clinical-and-pathological-characteristics
#20
Fermin Moreno, Begoña Indakoetxea, Myriam Barandiaran, María Cristina Caballero, Ana Gorostidi, Francesc Calafell, Alazne Gabilondo, Mikel Tainta, Miren Zulaica, José F Martí Massó, Adolfo López de Munain, Pascual Sánchez-Juan, Suzee E Lee
BACKGROUND: The co-occurrence of the c.709-1G>A GRN mutation and the p.A152T MAPT variant has been identified in 18 Basque families affected by frontotemporal dementia (FTD). We aimed to investigate the influence of the p.A152T MAPT variant on the clinical and neuropathological features of these Basque GRN families. METHODS AND FINDINGS: We compared clinical characteristics of 14 patients who carried the c.709-1G>A GRN mutation (GRN+/A152T-) with 21 patients who carried both the c...
2017: PloS One
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