Sunghye Cho, Naomi Nevler, Sharon Ash, Sanjana Shellikeri, David J Irwin, Lauren Massimo, Katya Rascovsky, Christopher Olm, Murray Grossman, Mark Liberman
We implemented an automated analysis of lexical aspects of semi-structured speech produced by healthy elderly controls (n=37) and three patient groups with frontotemporal degeneration (FTD): behavioral variant FTD (n=74), semantic variant primary progressive aphasia (svPPA, n=42), and nonfluent/agrammatic PPA (naPPA, n=22). Based on previous findings, we hypothesized that the three patient groups and controls would differ in the counts of part-of-speech (POS) categories and several lexical measures. With a natural language processing program, we automatically tagged POS categories of all words produced during a picture description task...
November 4, 2020: medRxiv: the preprint server for health sciences
Yu A Shpilyukova, E Yu Fedotova, E S Berdnikovich, R N Konovalov, M N Zakharova, D A Grishina, N N Yakhno, S N Illarioshkin
OBJECTIVE: To evaluate the frequency of C9orf72 -associated frontotemporal dementia (FTD) in the Russian population and to study clinical features of GGGGCC-repeat expansion carriers. MATERIAL AND METHODS: Twenty-eight patients with FTD are included in the study: 15 with a behavioral variant of FTD (bvFTD) and 13 with a agrammatic/non-fluent variant of primary progressive aphasia (avPPA). The mean age was 62 years (34-80), the mean disease duration was 4 years (1-10)...
2020: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Salvatore Mazzeo, Cristina Polito, Sonia Padiglioni, Valentina Berti, Silvia Bagnoli, Gemma Lombardi, Irene Piaceri, Marco Carraro, Maria Teresa De Cristofaro, Alessandro Passeri, Camilla Ferrari, Benedetta Nacmias, Sandro Sorbi, Valentina Bessi
We aimed to detail language profiles, brain metabolic patterns and proportion of Alzheimer's disease biomarkers in a cohort of patients with mixed primary progressive aphasia (mPPA). We considered 58 patients with PPA: 10 with non-fluent/agrammatic variant (nfvPPA), 16 with semantic variant (svPPA), 21 with logopenic variant (lvPPA) and 9 with mPPA. Patients with mPPA were further classified as 4 nf/lvPPA (with prevailing features for nfvPPA and lvPPA) and 5 s/lvPPA (with prevailing features for svPPA and lvPPA)...
September 8, 2020: Neurobiology of Aging
Jennifer L Whitwell, Nirubol Tosakulwong, Christopher C Schwarz, Matthew L Senjem, Anthony J Spychalla, Joseph R Duffy, Jon Graff-Radford, Mary M Machulda, Bradley F Boeve, David S Knopman, Ronald C Petersen, Val J Lowe, Clifford R Jack, Dennis W Dickson, Joseph E Parisi, Keith A Josephs
OBJECTIVE: To characterize longitudinal MRI and PET abnormalities in autopsy confirmed Pick's disease (PiD) and determine how patterns of neurodegeneration differ with respect to clinical syndrome. METHODS: Seventeen patients with PiD were identified who had antemortem MRI (8 with behavioral variant frontotemporal dementia [bvFTD-PiD], 6 with non-fluent/agrammatic primary progressive aphasia [naPPA-PiD], one with semantic primary progressive aphasia, one with unclassified primary progressive aphasia, and one with corticobasal syndrome)...
September 28, 2020: Neurology
Massimiliano Ruggeri, Clelia Biagioli, Monica Ricci, Carmela Gerace, Carlo Blundo
BACKGROUND: Despite initial underreporting of language dysfunctions in corticobasal syndrome (CBS), aphasia is now recognized as a frequent feature of this disease. Aphasia in CBS seems clinically overlying to a non-fluent/agrammatic primary progressive aphasia (nfaPPA), which is also a clinical phenotype associated with corticobasal degeneration (CBD) pathology. However, the clinical features of aphasia in CBS still remain poorly delineated, resulting in misjudgements in the differential diagnosis from a PPA presentation of the disease...
July 29, 2020: International Journal of Language & Communication Disorders
Hiroyuki Watanabe, Manabu Ikeda, Etsuro Mori
Cortical neurodegeneration-induced non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) is a clinical syndrome characterized by non-fluent speech, such as apraxia of speech or agrammatism. We describe the case of an 80-year-old right-handed woman who exhibited nfvPPA. Atypically, our patient also presented with generalized auditory agnosia. Brain magnetic resonance imaging revealed left-sided predominant atrophy of the bilateral perisylvian area, including the inferior frontal and superior temporal lobes...
2020: Frontiers in Neurology
Naomi Nevler, Sharon Ash, Corey McMillan, Lauren Elman, Leo McCluskey, David J Irwin, Sunghye Cho, Mark Liberman, Murray Grossman
OBJECTIVE: We implemented automated methods to analyze speech and evaluate the hypothesis that cognitive and motor factors impair prosody in partially distinct ways in patients with amyotrophic lateral sclerosis (ALS). METHODS: We recruited 213 participants, including 67 with ALS (44 with motor ALS, 23 with ALS-frontotemporal degeneration (ALS-FTD)), 33 healthy controls, and neurodegenerative reference groups with behavioral variant FTD (n=90) and non-fluent/agrammatic primary progressive aphasia (n=23)...
July 16, 2020: Neurology
M Torso, M Bozzali, M Cercignani, M Jenkinson, S A Chance
Fronto-temporal dementia (FTD) is a common type of presenile dementia, characterized by a heterogeneous clinical presentation that includes three main subtypes: behavioural-variant FTD, non-fluent/agrammatic variant primary progressive aphasia and semantic variant PPA. To better understand the FTD subtypes and develop more specific treatments, correct diagnosis is essential. This study aimed to test the discrimination power of a novel set of cortical Diffusion Tensor Imaging measures (DTI), on FTD subtypes...
July 8, 2020: Scientific Reports
Alexandre Bejanin, Gautam Tammewar, Gabe Marx, Yann Cobigo, Leonardo Iaccarino, John Kornak, Adam M Staffaroni, Bradford C Dickerson, Bradley F Boeve, David S Knopman, Marilu Gorno-Tempini, Bruce L Miller, William J Jagust, Adam L Boxer, Howard J Rosen, Gil D Rabinovici
OBJECTIVE: To compare the sensitivity of structural MRI and 18 F-fludeoxyglucose PET (18 FDG-PET) to detect longitudinal changes in frontotemporal dementia (FTD). METHODS: Thirty patients with behavioral variant FTD (bvFTD), 7 with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA), 16 with semantic variant primary progressive aphasia (svPPA), and 43 cognitively normal controls underwent 2-4 MRI and 18 FDG-PET scans (total scans/visit = 270) as part of the Frontotemporal Lobar Degeneration Neuroimaging Initiative study...
July 14, 2020: Neurology
Donna C Tippett, Argye E Hillis
The communication and behavioral manifestations of primary progressive aphasia (PPA) present ethical and practical challenges for individuals with this clinical syndrome as well as for individuals who are involved closely in their care. In this article, cases representing all three PPA variants (logopenic variant, nonfluent agrammatic, semantic variant) are presented to illustrate commonly encountered situations in which self-determination is at risk in decisions about housing, driving, social interactions, finances, and treatment interventions...
June 2020: Seminars in Speech and Language
Jeremy C S Johnson, Jessica Jiang, Rebecca L Bond, Elia Benhamou, Maï-Carmen Requena-Komuro, Lucy L Russell, Caroline Greaves, Annabel Nelson, Harri Sivasathiaseelan, Charles R Marshall, Anna P Volkmer, Jonathan D Rohrer, Jason D Warren, Chris J D Hardy
Logopenic variant primary progressive aphasia (lvPPA) is the least well defined of the major primary progressive aphasia (PPA) syndromes. We assessed phoneme discrimination in patients with PPA (semantic, nonfluent/agrammatic, and logopenic variants) and typical Alzheimer's disease, relative to healthy age-matched participants. The lvPPA group performed significantly worse than all other groups apart from tAD, after adjusting for auditory verbal working memory. In the combined PPA cohort, voxel-based morphometry correlated phonemic discrimination score with grey matter in left angular gyrus...
June 18, 2020: Annals of Clinical and Translational Neurology
Nikhil Seniaray, Ritu Verma, Rajeev Ranjan, Ethel Belho, Vanshika Gupta, Harsh Mahajan
FTLD (Frontotemporal lobar degeneration) is a clinically and pathologically heterogeneous group of degenerative disorders, characterized by predominantly asymmetric degeneration of frontal and temporal lobes with selective neuronal loss and gliosis. The disease presents with variable degrees of impairment in behavior, language, executive control, and motor symptoms with progressive loss of cognition. On the basis of presenting clinical symptoms, FTLD is further divided into behavioral variant, nonfluent/agrammatic variant primary progressive aphasia (PPA), semantic variant PPA, logopenic variant PPA, progressive supranuclear palsy, and corticobasal ganglionic degeneration...
July 2020: Clinical Nuclear Medicine
Yasmeen Faroqi-Shah, Ashlyn Treanor, Nan Bernstein Ratner, Bronte Ficek, Kimberly Webster, Kyrana Tsapkini
PURPOSE: Language decline has been associated with healthy aging and with various neurodegenerative conditions, making it challenging to differentiate among these conditions. This study examined the utility of linguistic measures derived from a short narrative language sample for 1) identifying language characteristics and cut-off scores to differentiate between healthy aging, Primary Progressive Aphasia (PPA), Mild Cognitive Impairment (MCI), and Alzheimer's dementia (AD); and 2) differentiating among PPA variants in which language is the primary impairment...
May 2020: Journal of Communication Disorders
Joanne T Douglas
Primary progressive aphasia is a major clinical presentation of frontotemporal lobar degeneration and is a young-onset disorder characterized by deteriorating language skills. There is currently no cure for primary progressive aphasia, nor is it possible to slow the course of the underlying progressive brain degeneration. Hence the chief goal of treatment is palliative. Although the inability to employ language at one's previous level represents a significant functional impairment for those affected, there is a dearth of information about how to make meaningful improvements to the quality of life of people in the early stages of primary progressive aphasia...
April 10, 2020: Dementia
Vankadari Kousik, Bhagwant Rai Mittal, Rajender Kumar, Harmandeep Singh, Manoj Goyal
Primary progressive aphasia is a neurodegenerative variant of frontotemporal lobe degeneration presenting with isolated selective impairment of language domain, not secondarily due to stroke. We present a case of middle-aged female patient who underwent F-FDG PET of the brain for evaluating progressively declining speaking ability associated with altered fluency of speech and occasional mutism. F-FDG PET revealed asymmetric hypometabolism involving the left inferior frontal gyrus along with left anterior cingulate gyrus suggestive of Broca's aphasia...
March 23, 2020: Clinical Nuclear Medicine
Olivier Piguet, Fiona Kumfor
PURPOSE OF REVIEW: Frontotemporal dementia (FTD) is a rare dementia, that accounts for about 15% of all dementia cases. Despite consensus diagnostic criteria, FTD remains difficult to diagnose in life because of its complex and variable clinical phenomenology and heterogeneous disorders. This review provides an update on the current knowledge of the main FTD syndromes -- the behavioural variant, semantic variant, and nonfluent/agrammatic variant-- their brain abnormalities and genetic profiles...
April 2020: Current Opinion in Neurology
Donna C Tippett
Primary progressive aphasia (PPA) is classified into three variants, logopenic variant PPA (lvPPA), nonfluent agrammatic PPA (nfaPPA), and semantic variant PPA (svPPA), based on clinical (syndromic) characteristics with support from neuroimaging and/or underlying neuropathology. Classification of PPA variants provides information valuable to disease management. International consensus criteria are widely employed to identify PPA subtypes; however, classification is complex, and some individuals do not fit neatly into the subtyping scheme...
2020: F1000Research
Elisa Canu, Federica Agosta, Giovanni Battistella, Edoardo G Spinelli, Jessica DeLeon, Ariane E Welch, Maria Luisa Mandelli, H Isabel Hubbard, Andrea Moro, Giuseppe Magnani, Stefano F Cappa, Bruce L Miller, Massimo Filippi, Maria Luisa Gorno-Tempini
OBJECTIVE: To understand whether the clinical phenotype of nonfluent/agrammatic primary progressive aphasia (nfvPPA) could present differences depending on the patient's native language. METHODS: In this cross-sectional study, we analyzed connected speech samples in monolingual English (nfvPPA-E) and Italian speakers (nfvPPA-I) who were diagnosed with nfvPPA and matched for age, sex, and Mini-Mental State Examination scores. Patients also received a comprehensive neuropsychological battery...
March 10, 2020: Neurology
Toji Miyagawa, Danielle Brushaber, Jeremy Syrjanen, Walter Kremers, Julie Fields, Leah K Forsberg, Hilary W Heuer, David Knopman, John Kornak, Adam Boxer, Howard J Rosen, Bradley F Boeve, Brian Appleby, Yvette Bordelon, Jessica Bove, Patrick Brannelly, Christina Caso, Giovanni Coppola, Reilly Dever, Christina Dheel, Bradford Dickerson, Susan Dickinson, Sophia Dominguez, Kimiko Domoto-Reilly, Kelley Faber, Jessica Ferrell, Ann Fishman, Jamie Fong, Tatiana Foroud, Ralitza Gavrilova, Debra Gearhart, Behnaz Ghazanfari, Nupur Ghoshal, Jill S Goldman, Jonathan Graff-Radford, Neill Graff-Radford, Ian Grant, Murray Grossman, Dana Haley, Robin Hsiung, Edward Huey, David Irwin, David Jones, Lynne Jones, Kejal Kantarci, Anna Karydas, Daniel Kaufer, Diana Kerwin, Ruth Kraft, Joel Kramer, Walter Kukull, Irene Litvan, Diane Lucente, Codrin Lungu, Ian Mackenzie, Miranda Maldonado, Masood Manoochehri, Scott McGinnis, Emily McKinley, Mario F Mendez, Bruce Miller, Namita Multani, Chiadi Onyike, Jaya Padmanabhan, Alexander Pantelyat, Rodney Pearlman, Leonard Petrucelli, Madeline Potter, Rosa Rademakers, Eliana M Ramos, Kate Rankin, Katya Rascovsky, Erik D Roberson, Emily Rogalski, Pheth Sengdy, Leslie Shaw, Maria C Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Q Trojanowski, Ping Wang, Sandra Weintraub, Bonnie Wong, Zbigniew Wszolek
INTRODUCTION: We created global rating scoring rules for the CDR® plus NACC FTLD to detect and track early frontotemporal lobar degeneration (FTLD) and to conduct clinical trials in FTLD. METHODS: The CDR plus NACC FTLD rating was applied to 970 sporadic and familial participants from the baseline visit of Advancing Research and Treatment in Frontotemporal Lobar Degeneration (ARTFL)/Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects (LEFFTDS)...
January 2020: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
Giovanni Battistella, Valentina Borghesani, Maya Henry, Wendy Shwe, Michael Lauricella, Zachary Miller, Jessica Deleon, Bruce L Miller, Nina Dronkers, Simona M Brambati, William W Seeley, Maria Luisa Mandelli, Maria Luisa Gorno-Tempini
Intrinsic connectivity networks (ICNs) identified through task-free fMRI (tf-fMRI) offer the opportunity to investigate human brain circuits involved in language processes without requiring participants to perform challenging cognitive tasks. In this study, we assessed the ability of tf-fMRI to isolate reproducible networks critical for specific language functions and often damaged in primary progressive aphasia (PPA). First, we performed whole-brain seed-based correlation analyses on tf-fMRI data to identify ICNs anchored in regions known for articulatory, phonological, and semantic processes in healthy male and female controls (HCs)...
December 18, 2019: Journal of Neuroscience
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