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A Multimodal Approach for Clinical Diagnosis and Treatment of Primary Progressive Aphasia (MAINSTREAM): A Study Protocol.

ABSTRACT: Primary Progressive Aphasia (PPA) is a syndrome due to different neurodegenerative disorders selectively disrupting language functions. PPA specialist care is underdeveloped. There are very few specialists (neurologists, psychiatrists, neuropsychologists, and speech therapists) and few hospital- or community-based services dedicated to the diagnosis and continuing care of people with PPA. Currently, healthcare systems struggle to provide adequate coverage of care that is too often fragmented, uncoordinated, and unresponsive to the needs of people with PPA and their families. Recently, attention has been gained by non-invasive brain stimulation techniques that allow a personalized treatment approach, such as transcranial Direct Current Stimulation (tDCS). The MAINSTREAM trial looks forward to introducing and evaluating therapeutic innovations such as tDCS coupled with language therapy in rehabilitation settings. A Multimodal Approach for Clinical Diagnosis and Treatment of Primary Progressive Aphasia, MAINSTREAM (ID: 3430931) was registered in the clinicaltrials.gov database (identifier: NCT05730023) on 15 February 2023.

SUBMITTER: Cotelli M

PROVIDER: S-EPMC10377301 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature

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A Multimodal Approach for Clinical Diagnosis and Treatment of Primary Progressive Aphasia (MAINSTREAM): A Study Protocol.

Cotelli Maria M Baglio Francesca F Manenti Rosa R Blasi Valeria V Galimberti Daniela D Gobbi Elena E Pagnoni Ilaria I Rossetto Federica F Rotondo Emanuela E Esposito Valentina V De Icco Roberto R Giudice Carla C Tassorelli Cristina C Catricalà Eleonora E Perini Giulia G Alaimo Cristina C Campana Elena E Benussi Luisa L Ghidoni Roberta R Binetti Giuliano G Carandini Tiziana T Cappa Stefano Francesco SF

Brain sciences 20230712 7

Primary Progressive Aphasia (PPA) is a syndrome due to different neurodegenerative disorders selectively disrupting language functions. PPA specialist care is underdeveloped. There are very few specialists (neurologists, psychiatrists, neuropsychologists, and speech therapists) and few hospital- or community-based services dedicated to the diagnosis and continuing care of people with PPA. Currently, healthcare systems struggle to provide adequate coverage of care that is too often fragmented, un ...[more]

PMID: 37508992

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Project description:BackgroundPrimary progressive aphasia is a language-led dementia, often associated with frontotemporal dementia. It presents as insidious deterioration of language skills (e.g. naming objects and understanding complex sentences), with relative sparing of cognitive skills initially. There is little research examining the effectiveness of communication skills training for primary progressive aphasia, yet speech and language therapists (SLTs) report regularly using this in clinical practice. 'Better Conversations with Primary Progressive Aphasia' has potential to reduce barriers and increase facilitators to conversation and consequently improve confidence in communication and quality of life for people living with primary progressive aphasia and their conversation partners. The aim of this pilot study is to examine the feasibility of running a trial of the 'Better Conversations with Primary Progressive Aphasia' intervention.MethodsA single blind, randomised controlled pilot study will recruit 42 participants with primary progressive aphasia and their conversation partners across seven UK National Health Service Trusts. Participants will be randomised on a 1:1 basis, stratified by site, to receive either the 'Better Conversations with Primary Progressive Aphasia' intervention (21 couples) or no speech and language therapy treatment (21 couples). Participants are recruited by SLTs who will conduct pre-intervention assessment (week 1) and deliver the intervention (weeks 2 to 5). Junior researchers, who are blinded to allocation, will complete post-intervention measures (week 6). SLTs complete 9 h of training to prepare them to deliver the intervention. The primary objective of the study is to establish for a phase III effectiveness study whether the program can be delivered as intended in a UK National Health Service setting. Specifically, it will establish (1) the acceptability of randomisation, (2) an assessment of treatment fidelity to determine necessary levels of SLT training, (3) the most appropriate primary outcome measure, (4) sample size requirements, (5) predicted patient recruitment and retention rates and (6) refined inclusion criteria.DiscussionInsights from this study will be of relevance to guide development of future research and in particular, trials of therapeutic interventions in PPA, as well as for clinical care for this population.Trial registrationRetrospectively registered 28/02/2018 ISRCTN10148247.

Project description:Crossed aphasia has been reported mainly as post-stroke aphasia resulting from brain damage ipsilateral to the dominant right hand. Here, we described a case of a crossed nonfluent/agrammatic primary progressive aphasia (nfvPPA), who developed a corticobasal syndrome (CBS). We collected clinical, cognitive, and neuroimaging data for four consecutive years from a 55-year-old right-handed lady (JV) presenting with speech disturbances. 18-fluorodeoxyglucose positron emission tomography ((18)F-FDG PET) and DaT-scan with (123)I-Ioflupane were obtained. Functional MRI (fMRI) during a verb naming task was acquired to characterize patterns of language lateralization. Diffusion tensor MRI was used to evaluate white matter damage within the language network. At onset, JV presented with prominent speech output impairment and right frontal atrophy. After 3 years, language deficits worsened, with the occurrence of a mild agrammatism. The patient also developed a left-sided mild extrapyramidal bradykinetic-rigid syndrome. The clinical picture was suggestive of nfvPPA with mild left-sided extrapyramidal syndrome. At this time, voxel-wise SPM analyses of (18)F-FDG PET and structural MRI showed right greater than left frontal hypometabolism and damage, which included the Broca's area. DaT-scan showed a reduced uptake in the right striatum. FMRI during naming task demonstrated bilateral language activations, and tractography showed right superior longitudinal fasciculus (SLF) involvement. Over the following year, JV became mute and developed frank left-sided motor signs and symptoms, evolving into a CBS clinical picture. Brain atrophy worsened in frontal areas bilaterally, and extended to temporo-parietal regions, still with a right-sided asymmetry. Tractography showed an extension of damage to the left SLF and right inferior longitudinal fasciculus. We report a case of crossed nfvPPA followed longitudinally and studied with advanced neuroimaging techniques. The results highlight a complex interaction between individual premorbid developmental differences and the clinical phenotype.

Project description:IntroductionPrimary progressive aphasia (PPA) is divided into three prototypical subtypes that are all characterized by their single core symptom of aphasia. Although later in their course, other cognitive, behavioral, and motor domains may become involved, little is known about the progression profile of each subtype relative to the other subtypes.MethodsIn this longitudinal retrospective cohort study, based on the recent biomarker-supported diagnostic criteria, 24 subjects diagnosed with semantic variant (svPPA), 22 with non-fluent variant (nfvPPA), and 18 with logopenic variant (lvPPA) were collected and followed up for 1-6 years. Symptom distribution, cognitive test and neuropsychiatric inventory scores, and progression into another syndrome were assessed.ResultsOver time, lvPPA progressed with broader language problems (PPA-extended) and nfvPPA progressed to mutism, whereas semantic impairment remained the major problem in svPPA. Apart from linguistic problems, svPPA developed pronounced behavioral disturbances, whereas lvPPA exhibited a greater cognitive decline. By contrast, in nfvPPA motor deficits were more common. Furthermore, within 5 years (IQR = 2.5) after clinical onset, 65.6% of the patients additionally fulfilled the clinical criteria for another neurodegenerative syndrome (PPA-plus). Fourteen out of 24 (58%) svPPA patients additionally met the diagnostic criteria of behavioral variant frontotemporal dementia (5.1 years, IQR = 1.1), whereas the clinical features of 15/18 (83%) lvPPA patients were consistent with Alzheimer disease dementia (4.5 years IQR = 3.4). Furthermore, 12/22 (54%) of the subjects with the nfvPPA progressed to meet the diagnostic criteria of corticobasal syndrome, progressive supranuclear palsy, or motor neuron disease (5.1 years IQR = 3.4).DiscussionDespite aphasia being the initial and unique hallmark of the syndrome, our longitudinal results showed that PPA is not a language limited disorder and progression differs widely for each subtype, both with respect to the nature of symptoms and disease duration.

Dataset Information

A Multimodal Approach for Clinical Diagnosis and Treatment of Primary Progressive Aphasia (MAINSTREAM): A Study Protocol.

Publications

A Multimodal Approach for Clinical Diagnosis and Treatment of Primary Progressive Aphasia (MAINSTREAM): A Study Protocol.

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