Kristin M Schaffer, William S Evans, Christina D Dutcher, Christina Philburn, Maya L Henry
Purpose This study sought to determine the initial feasibility and benefit of a novel intervention that combines speech-language treatment with counseling treatment for an individual with the nonfluent/agrammatic variant of primary progressive aphasia (PPA). Method Using a single-case experimental design, we evaluated the utility of modified script training paired with aphasia-modified cognitive behavioral therapy. The study employed a multiple baseline design across scripts for the primary linguistic outcome measure and a mixed methods approach for analyzing counseling outcomes...
July 27, 2021: American Journal of Speech-language Pathology
Eun-Joo Kim, Seong-Ho Koh, Jungsoon Ha, Duk L Na, Sang Won Seo, Hee-Jin Kim, Kyung Won Park, Jae-Hong Lee, Jee Hoon Roh, Jay C Kwon, Soo Jin Yoon, Na-Yeon Jung, Jee H Jeong, Jae-Won Jang, Hee-Jin Kim, Kee Hyung Park, Seong Hye Choi, SangYun Kim, Young Ho Park, Byeong C Kim, Young-Eun Kim, Hyuk Sung Kwon, Hyun-Hee Park, Jeong-Hwa Jin
BACKGROUND: Telomeres are repetitive DNA sequences of TTAGGG at the ends of chromosomes. Many studies have shown that telomere shortening is associated with aging-related diseases, such as cardiovascular diseases, hypertension, diabetes, cancer, and various neurodegenerative diseases, including Alzheimer's disease, vascular dementia, Parkinson's disease, and dementia with Lewy bodies. However, changes in telomere length (TL) in patients with frontotemporal dementia (FTD) syndrome are unclear...
July 3, 2021: Journal of the Neurological Sciences
Harri Sivasathiaseelan, Charles R Marshall, Elia Benhamou, Janneke E P van Leeuwen, Rebecca L Bond, Lucy L Russell, Caroline Greaves, Katrina M Moore, Chris J D Hardy, Chris Frost, Jonathan D Rohrer, Sophie K Scott, Jason D Warren
Laughter is a fundamental communicative signal in our relations with other people and is used to convey a diverse repertoire of social and emotional information. It is therefore potentially a useful probe of impaired socio-emotional signal processing in neurodegenerative diseases. Here we investigated the cognitive and affective processing of laughter in forty-seven patients representing all major syndromes of frontotemporal dementia, a disease spectrum characterised by severe socio-emotional dysfunction (twenty-two with behavioural variant frontotemporal dementia, twelve with semantic variant primary progressive aphasia, thirteen with nonfluent-agrammatic variant primary progressive aphasia), in relation to fifteen patients with typical amnestic Alzheimer's disease and twenty healthy age-matched individuals...
June 23, 2021: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Ilaria Pagnoni, Elena Gobbi, Enrico Premi, Barbara Borroni, Giuliano Binetti, Maria Cotelli, Rosa Manenti
BACKGROUND: Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by a gradual, insidious and progressive loss of language abilities, with naming difficulties being an early and persistent impairment common to all three variants. In the absence of effective pharmacological treatments and given the progressive nature of the disorder, in the past few decades, many studies have investigated the effectiveness of language training to minimize the functional impact of word-finding difficulties in daily life...
July 16, 2021: Translational Neurodegeneration
Joseph R Duffy, Rene L Utianski, Keith A Josephs
Background: Apraxia of speech (AOS) can be caused by neurodegenerative disease and sometimes is its presenting sign (i.e., primary progressive apraxia of speech, PPAOS). During the last several decades our understanding of PPAOS has evolved from clinical recognition to a fuller understanding of its core and associated clinical features, its distinction from but relationship with primary progressive aphasia, its temporal course and eventual progression to include other neurological deficits, and its neuroimaging correlates and underlying pathology...
2021: Aphasiology
Sladjana Lukic, Valentina Borghesani, Elizabeth Weis, Ariane Welch, Rian Bogley, John Neuhaus, Jessica Deleon, Zachary A Miller, Joel H Kramer, Bruce L Miller, Nina F Dronkers, Maria L Gorno-Tempini
Naming of nouns and verbs can be selectively impaired in neurological disorders, but the specificity of the neural and cognitive correlates of such dissociation remains unclear. Functional imaging and stroke research sought to identify cortical regions selectively recruited for nouns versus verbs, yet findings are inconsistent. The present study investigated this issue in neurodegenerative diseases known to selectively affect different brain networks, thus providing new critical evidence of network specificity...
June 2, 2021: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Jonathan Sikora, Colin Stein, Delaney Ubellacker, Alexandra Walker, Donna C Tippett
The premise of this study is that spoken word recognition and object knowledge are impaired in semantic variant primary progressive aphasia (PPA) (svPPA) and are spared in logopenic variant (lvPPA) and nonfluent agrammatic primary progressive aphasia (nfaPPA) at disease onset. Over time, however, there may be heterogeneity in these abilities in lvPPA and nfaPPA. We hypothesized that individuals with svPPA would demonstrate poorer performance on baseline spoken word recognition and object knowledge than those with lvPPA and nfaPPA) as documented in the literature, but that rates of decline over time on spoken word recognition and object knowledge would be similar in all 3 PPA variants because these become less distinguishable with disease progression...
June 4, 2021: Medicine (Baltimore)
Masahiko Takaya, Kazunari Ishii, Kazumasa Saigoh, Osamu Shirakawa
BACKGROUND: Alzheimer's disease is a neurodegenerative disease involving the deposition of pathologic amyloid-β and tau protein in the cerebral cortex. Alzheimer's disease is commonly characterized by progressive impairment of recent memory. Primary progressive aphasia is also often observed in patients with Alzheimer's disease. Moreover, language-associated symptoms, such as primary progressive aphasia, are diverse and varied in Alzheimer's disease. However, nonfluent/agrammatic variant primary progressive aphasia is not generally considered a symptom of Alzheimer's disease...
May 26, 2021: Journal of Medical Case Reports
Dario Saracino, Sophie Ferrieux, Marie Noguès-Lassiaille, Marion Houot, Aurélie Funkiewiez, Leila Sellami, Vincent Deramecourt, Florence Pasquier, Philippe Couratier, Jérémie Pariente, Amandine Géraudie, Stéphane Epelbaum, David Wallon, Didier Hannequin, Olivier Martinaud, Fabienne Clot, Agnès Camuzat, Simona Bottani, Daisy Rinaldi, Sophie Auriacombe, Marie Sarazin, Mira Didic, Claire Boutoleau-Bretonnière, Christel Thauvin-Robinet, Julien Lagarde, Carole Roué-Jagot, François Sellal, Audrey Gabelle, Frédérique Etcharry-Bouyx, Alexandre Morin, Cinzia Coppola, Richard Levy, Bruno Dubois, Alexis Brice, Olivier Colliot, Maria Luisa Gorno-Tempini, Marc Teichmann, Raffaella Migliaccio, Isabelle Le Ber
OBJECTIVE: To determine relative frequencies and linguistic profiles of primary progressive aphasia (PPA) variants associated with GRN (progranulin) mutations and to study their neuroanatomic correlates. METHODS: Patients with PPA carrying GRN mutations (PPA- GRN ) were selected among a national prospective research cohort of 1,696 patients with frontotemporal dementia, including 235 patients with PPA. All patients with amyloid-positive CSF biomarkers were excluded...
July 6, 2021: Neurology
Yi-Jing Bai, Xiao-Wei Liu, Wei-Dong Le
No abstract text is available yet for this article.
February 22, 2021: Chinese Medical Journal
Adam M Staffaroni, Sandra Weintraub, Katya Rascovsky, Katherine P Rankin, Jack Taylor, Julie A Fields, Kaitlin B Casaletto, Argye E Hillis, Sladjana Lukic, Maria Luisa Gorno-Tempini, Hilary Heuer, Merilee A Teylan, Walter A Kukull, Bruce L Miller, Bradley F Boeve, Howard J Rosen, Adam L Boxer, Joel H Kramer
Introduction: The Frontotemporal Lobar Degeneration Module (FTLD-MOD) includes a neuropsychological battery designed to assess the clinical features of FTLD, although much is unknown about its utility. We investigated FTLD-MOD and Uniform Data Set 3.0 (UDS) language tests for differential diagnosis and disease monitoring. Methods: Linear regressions compared baseline performances in 1655 National Alzheimer's Coordinating Center participants (behavioral variant frontotemporal dementia (bvFTD, n = 612), semantic variant primary progressive aphasia (svPPA, n = 168), non-fluent/agrammatic variant PPA (nfvPPA, n = 168), logopenic variant PPA (lvPPA, n = 109), and controls (n = 581))...
2021: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
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...
February 6, 2021: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Katarina L Haley, Adam Jacks, Jordan Jarrett, Taylor Ray, Kevin T Cunningham, Maria Luisa Gorno-Tempini, Maya L Henry
Purpose Of the three currently recognized variants of primary progressive aphasia, behavioral differentiation between the nonfluent/agrammatic (nfvPPA) and logopenic (lvPPA) variants is particularly difficult. The challenge includes uncertainty regarding diagnosis of apraxia of speech, which is subsumed within criteria for variant classification. The purpose of this study was to determine the extent to which a variety of speech articulation and prosody metrics for apraxia of speech differentiate between nfvPPA and lvPPA across diverse speech samples...
March 17, 2021: Journal of Speech, Language, and Hearing Research: JSLHR
M Teichmann
In the field of primary progressive aphasia (PPA), the most recent international consensus criteria of 2011 for diagnosis and variant classification have been shown not to capture accurately the whole range of PPA patients. Up to 30-40% of PPA patients appear not to satisfy the criteria of the three 'classical' PPA variants (non-fluent/agrammatic, logopenic, semantic) and are labelled either 'mixed PPA' or 'unclassifiable PPA'. Based on the PPA literature since 2011, this article discusses why patients might be under-diagnosed with respect to the three PPA variants, thus leading to the default concept of 'mixed/unclassifiable PPA' and, conversely, why the non-fluent/agrammatic variant appears to be over-diagnosed...
February 19, 2021: Revue Neurologique
Justina Ruksenaite, Anna Volkmer, Jessica Jiang, Jeremy Cs Johnson, Charles R Marshall, Jason D Warren, Chris Jd Hardy
PURPOSE OF REVIEW: The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers. RECENT FINDINGS: Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering 'fragmentary' syndromes...
February 4, 2021: Current Neurology and Neuroscience Reports
John A Duignan, Aoife Haughey, Justin A Kinsella, Ronan P Killeen
Dementia with Lewy bodies (DLB) and frontotemporal lobar degeneration (FTLD) are common causes of dementia. Early diagnosis of both conditions is challenging due to clinical and radiological overlap with other forms of dementia, particularly Alzheimer's disease (AD). Structural and functional imaging combined can aid differential diagnosis and help to discriminate DLB or FTLD from other forms of dementia. Imaging of DLB involves the use of 123 I-FP-CIT SPECT and 123 I-metaiodobenzylguanidine (123 I-MIBG), both of which have an established role distinguishing DLB from AD...
January 2, 2021: Seminars in Nuclear Medicine
M J Torres-Prioris, M L Berthier
One decade ago, the diagnostic criteria for three major forms of primary progressive aphasia (PPA) - nonfluent-agrammatic variant (nfvPPA), semantic variant (svPPA), and logopenic variant (lvPPA) - have been established based on expert consensus [1]. However, non-canonical forms of PPA (i.e., dynamic aphasia, pure apraxia of speech, pure anomia, dysprosodia) [2] and some features such as echolalia (repetition of what one has just heard) tend to be underestimated.
January 1, 2021: European Journal of Neurology
Cynthia K Thompson, Elena Barbieri, Jennifer E Mack, Aaron Wilkins, Kathy Y Xie
This study reports the results of a longitudinal study examining the effects of treatment for sentence processing deficits for a 70-year-old gentleman (DK) with the agrammatic variant of Primary Progressive Aphasia (PPA). On entry into the study, he presented with a 2-year history of impaired verb and sentence processing and concomitant neural atrophy in primarily subcortical regions. Spanning an 18-month period, treatment focused on improving comprehension and production of syntactically complex, passive and object cleft, structures, consecutively...
December 30, 2020: Neurocase
Shoko Ota, Shigenori Kanno, Ayumi Morita, Wataru Narita, Nobuko Kawakami, Kazuo Kakinuma, Yumiko Saito, Erena Kobayashi, Toru Baba, Osamu Iizuka, Yoshiyuki Nishio, Minoru Matsuda, Hayato Odagiri, Keiko Endo, Kentaro Takanami, Etsuro Mori, Kyoko Suzuki
OBJECTIVE: This study aimed to examine echolalia and its related symptoms and brain lesions in primary progressive aphasia (PPA). METHODS: Forty-five patients with PPA were included: 19 non-fluent/agrammatic variant PPA (nfvPPA), 5 semantic variant PPA, 7 logopenic variant PPA, and 14 unclassified PPA patients. We detected echolalia in unstructured conversations. An evaluation of language function and the presence of parkinsonism, grasp reflex, imitation behaviour, and disinhibition were assessed...
December 11, 2020: European Journal of Neurology
Jennifer E Mack, Elena Barbieri, Sandra Weintraub, M-Marsel Mesulam, Cynthia K Thompson
PURPOSE: This study examined grammatical production impairments in primary progressive aphasia (PPA), as measured by structured tests and narrative samples. We aimed to quantify the strength of the relationship between grammatical measures across tasks, and identify factors that condition it. Three grammatical domains were investigated: overall sentence production, verb morphology, and verb-argument structure. METHODS: 77 participants with PPA (34 PPA-G, 16 PPA-L, 15 PPA-S and 12 other) completed a battery of grammatical tests and a narrative language sample was obtained...
January 22, 2021: Neuropsychologia
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