Global Perspectives on the Management of Primary Progressive Aphasia.

Speech-language therapists/pathologists (SLT/Ps) are key professionals in the management and treatment of primary progressive aphasia (PPA), however, there are gaps in education and training within the discipline, with implications for skills, confidence, and clinical decision-making. This survey aimed to explore the areas of need amongst SLT/Ps working with people living with PPA (PwPPA) internationally to upskill the current and future workforce working with progressive communication disorders. One hundred eighty-five SLT/Ps from 27 countries who work with PwPPA participated in an anonymous online survey about their educational and clinical experiences, clinical decision-making, and self-reported areas of need when working with this population. Best practice principles for SLT/Ps working with PwPPA were used to frame the latter two sections of this survey. Only 40.7% of respondents indicated that their university education prepared them for their current work with PwPPA. Competency areas of "Knowing people deeply," "Practical issues," "Connectedness," and "Preventing disasters" were identified as the basic areas of priority and need. Respondents identified instructional online courses (92.5%), sample tools and activities for interventions (64.8%), and concrete training on providing care for advanced stages and end of life (58.3%) as central areas of need in their current work. This is the first international survey to comprehensively explore the perspectives of SLT/Ps working with PwPPA. Based on survey outcomes, there is a pressing need to enhance current educational and ongoing training opportunities to better promote the well-being of PwPPA and their families, and to ensure appropriate preparation of the current and future SLT/P workforce.

Utility of visual rating scales in primary progressive aphasia.

INTRODUCTION: Differential diagnosis among subjects with Primary Progressive Aphasia (PPA) can be challenging. Structural MRI can support the clinical profile. Visual rating scales are a simple and reliable tool to assess brain atrophy in the clinical setting. The aims of the study were to establish to what extent the visual rating scales could be useful in the differential diagnosis of PPA, to compare the clinical diagnostic impressions derived from routine MRI interpretations with those obtained using the visual rating scale and to correlate results of the scales in a voxel-based morphometry (VBM) analysis. METHOD: Patients diagnosed with primary progressive aphasia (PPA) according to current criteria from two centers-Ospedale Maggiore Policlinico of Milan and Hospital Clínic de Barcelona-were included in the study. Two blinded clinicians evaluated the subjects MRIs for cortical atrophy and white matter hyperintensities using two protocols: routine readings and the visual rating scale. The diagnostic accuracy between patients and controls and within PPA subgroups were compared between the two protocols. RESULTS: One hundred fifty Subjects were studied. All the scales showed a good to excellent intra and inter-rater agreement. The left anterior temporal scale could differentiate between semantic PPA and all other variants. The rater impression after the protocol can increase the accuracy just for the logopenic PPA. In the VBM analysis, the scores of visual rating scales correlate with the corresponding area of brain atrophy. CONCLUSION: The Left anterior temporal rating scale can distinguish semantic PPA from other variants. The rater impression after structured view improved the diagnostic accuracy of logopenic PPA compared to normal readings. The unstructured view of the MRI was reliable for identifying semantic PPA and controls. Neither the structured nor the unstructured view could identify the nonfluent and undetermined variants.

Multifocal Transcranial Direct Current Stimulation in Primary Progressive Aphasia Does Not Provide a Clinical Benefit Over Speech Therapy.

BACKGROUND: Primary progressive aphasia (PPA) is a group of neurodegenerative disorders including Alzheimer's disease and frontotemporal dementia characterized by language deterioration. Transcranial direct current stimulation (tDCS) is a non-invasive intervention for brain dysfunction. OBJECTIVE: To evaluate the tolerability and efficacy of tDCS combined with speech therapy in the three variants of PPA. We evaluate changes in fMRI activity in a subset of patients. METHODS: Double-blinded, randomized, cross-over, and sham-controlled tDCS study. 15 patients with PPA were included. Each patient underwent two interventions: a) speech therapy + active tDCS and b) speech therapy + sham tDCS stimulation. A multifocal strategy with anodes placed in the left frontal and parietal regions was used to stimulate the entire language network. Efficacy was evaluated by comparing the results of two independent sets of neuropsychological assessments administered at baseline, immediately after the intervention, and at 1 month and 3 months after the intervention. In a subsample, fMRI scanning was performed before and after each intervention. RESULTS: The interventions were well tolerated. Participants in both arms showed clinical improvement, but no differences were found between active and sham tDCS interventions in any of the evaluations. There were trends toward better outcomes in the active tDCS group for semantic association and reading skills. fMRI identified an activity increase in the right frontal medial cortex and the bilateral paracingulate gyrus after the active tDCS intervention. CONCLUSION: We did not find differences between active and sham tDCS stimulation in clinical scores of language function in PPA patients.

Errorless Learning Therapy in Semantic Variant of Primary Progressive Aphasia.

BACKGROUND: The semantic variant of primary progressive aphasia (svPPA) is characterized by a progressive loss of semantic knowledge impairing the ability to name and to recognize the meaning of words. OBJECTIVE: We aimed to evaluate the immediate and short-term effect of errorless learning speech therapy on the naming and recognition of commonly used words in patients with svPPA. METHODS: Eight participants diagnosed with svPPA received 16 sessions of intensive errorless learning speech therapy. Naming and word comprehension tasks were evaluated at baseline, immediately postintervention, and at follow-up after 1, 3, and 6 months. These evaluations were performed using two item sets (a trained list and an untrained list). RESULTS: In the naming tasks, patients showed a significant improvement in trained items immediately after the intervention, but that improvement decayed progressively when therapy ended. No improvements were found either in trained comprehension or in untrained tasks. CONCLUSION: Errorless learning therapy could improve naming ability in patients with svPPA. This effect may be due to the relative preservation of episodic memory, but the benefit is not maintained over time, presumably because there is no consolidation.

La disfagia en la enfermedad de Huntington: propuesta de intervención logopédica / Dysphagia in Huntington disease: a proposal for speech therapy

Introducción. La enfermedad de Huntington (EH) es una enfermedad neurodegenerativa autosómica dominante caracterizada por alteraciones motoras, cognitivas y conductuales. La disfagia es un síntoma común en la enfermedad y puede conllevar un cuadro de pérdida de peso y complicaciones respiratorias por broncoaspiración. Nuestro objetivo fue estudiar la disfunción de la deglución y presentar un programa específico de intervención para mejorar o mantener la eficacia deglutoria. Materiales y métodos. Se incluyeron 10 pacientes ambulatorios con EH entre 46 y 79 años de edad, en un estadio moderado de la enfermedad, que fueron comparados con 10 sujetos sanos ajustados en edad y género. Se evaluaron la eficacia y la seguridad de la fase oral y faríngea tanto cualitativa como cuantitativamente a través del método de exploración clínica con diferentes volúmenes y viscosidades. Resultados. Se observó cierre labial ineficaz en 6 pacientes, residuos orales en 4 y deglución fraccionada en 10; residuos faríngeos en 7, tos en 3, cambios de voz en 6 y una bajada de 2 puntos en la saturación de oxígeno en sangre en 3. El grupo control obtuvo una puntuación entre 60 y 63, mientras que los pacientes puntuaron entre 19 y 53. Conclusiones. Las fases oral y faríngea de la deglución ya están comprometidas en los estadios moderados de la enfermedad. Nuestra hipótesis es que un programa específico de rehabilitación puede mejorar las dificultades deglutorias de estos pacientes y contribuir a mantener su estado nutricional. Proponemos, por tanto, un programa de rehabilitación que incluya las praxias orolinguomandibulares, así como el entrenamiento de estrategias y cambios posturales (AU)

Introduction. Huntington disease (HD) is a progressive neurodegenerative autosomal dominant disease characterized by motor, behavioral and cognitive disturbances. Dysphagia is a common and life-threatening symptom of the disease leading to nutritional deficiencies and a high risk of bronchoaspiration. Our objective was to study swallowing dysfunction and to present a specific intervention to improve or maintain swallowing efficiency. Materials and methods. We recruited 10 ambulatory HD patients aged between 46 and 79 years old, with a moderate stage of the disease, who were compared with 10 age- and gender-matched healthy controls. The safety and effectiveness of the oral and pharyngeal phases was assessed qualitatively and quantitatively through the volume-viscosity swallow test. Results. We observed ineffective lip closure in 6 patients, oral residue in 4, repetitive swallows in 10, pharyngeal residue in 7, cough in 3, voice changes in 6, and a drop of 2 points in blood oxygen saturation in a further 3. Controls scored between 60 and 63 points, while patients scored between 19 and 53 points. Conclusions. The oral and pharyngeal swallowing phases are already compromised in moderate stages of HD. We hypothesize that a specific rehabilitation program for dysphagia could improve swallowing difficulties and contribute to maintaining nutritional status in affected individuals. Consequently, we propose a rehabilitation program including orolingual praxis and specific muscular postural strategies (AU)

[Relearning vocabulary. A comparative analysis between a case of dementia and Alzheimer's disease with predominant compromise of language]. / Reaprendizaje de vocabulario. Análisis comparativo entre un caso de demencia semántica y enfermedad de Alzheimer con afectación predominante del lenguaje.

INTRODUCTION: Semantic dementia is characterised by a progressive loss of semantic content that initially affects the capacity to name things, and is associated with asymmetric atrophy of the anterior temporal lobes. In Alzheimer's disease (AD) with predominant compromise of language, anomia is also the main symptom. The study examined the capacity to relearn vocabulary of two patients, each exhibiting one of these two forms of degenerative anomia. CASE REPORTS: The two cases presented similar ages, gender, levels of schooling and degree of compromise. Their capacity to name a list of 40 pictures was evaluated at baseline, following 20 sessions of relearning, at one month and at six months. The patient with semantic dementia named 25/40 objects at baseline, 40/40 after relearning, 35/40 at one month and 27/40 at six months. The patient with AD named 29/40 at baseline, 30/40 after relearning, 29/40 at one month and 32/40 at six months. No intrusions were observed following relearning. CONCLUSIONS: The patient with semantic dementia was able to relearn all the vocabulary she was shown, even though she lost everything she had acquired after treatment was interrupted. The AD patient did not improve her naming capacity with therapy. These differences suggest that the learning and consolidation circuits are affected in different ways. Subjects with semantic dementia, but not those with AD, could benefit from word relearning strategies with this method.

Reaprendizaje de vocabulario. Análisis comparativo entre un caso de demencia semántica y enfermedad de Alzheimer con afectación predominante del lenguaje / Relearning vocabulary. A comparative analysis between a case of dementia and Alzheimer’s disease with predominant compromise of language

Introducción. La demencia semántica se caracteriza por una pérdida progresiva del contenido semántico que afecta inicialmente la capacidad para nominar, asociándose a atrofia temporal anterior asimétrica. En la enfermedad de Alzheimer (EA) con afectación predominante del lenguaje, la anomia es también el síntoma principal. Se estudió la capacidad de reaprendizaje de vocabulario en un paciente de cada una de estas dos formas de anomia degenerativa. Casos clínicos. Ambos casos presentaban edad, género, escolarización y grado de afectación similares. La capacidad de nominar una lista de 40 imágenes se evaluó basalmente, tras 20 sesiones de reaprendizaje, al mes y a los seis meses. La paciente con demencia semántica basalmente nominaba 25/40 objetos, tras el reaprendizaje 40/40, al mes 35/40 y a los 6 meses 27/40. La paciente con EA basalmente nominaba 29/40, tras el reaprendizaje 30/40, al mes 29/40, a los 6 meses 32/40. No se objetivaron intrusiones tras el reaprendizaje.Conclusiones. La paciente con demencia semántica fue capaz de reaprender todo el vocabulario presentado, si bien tras interrumpir el tratamiento perdió lo adquirido. La EA no mejoró su capacidad de nominación con la terapia. Estas diferencias sugieren una afectación diferencial en los circuitos de aprendizaje y consolidación. Los sujetos con demencia semántica, pero no los sujetos con EA, podrían beneficiarse de estrategias de reaprendizaje de palabras con esta metodología (AU)

Introduction. Semantic dementia is characterised by a progressive loss of semantic content that initially affects the capacity to name things, and is associated with asymmetric atrophy of the anterior temporal lobes. In Alzheimer’s disease (AD) with predominant compromise of language, anomia is also the main symptom. The study examined the capacity to relearn vocabulary of two patients, each exhibiting one of these two forms of degenerative anomia. Case reports. The two cases presented similar ages, gender, levels of schooling and degree of compromise. Their capacity to name a list of 40 pictures was evaluated at baseline, following 20 sessions of relearning, at one month and at six months. The patient with semantic dementia named 25/40 objects at baseline, 40/40 after relearning, 35/40 at one month and 27/40 at six months. The patient with AD named 29/40 at baseline, 30/40 after relearning, 29/40 at one month and 2/40 at six months. No intrusions were observed following relearning. Conclusions. The patient with semantic dementia was able to relearn all the vocabulary she was shown, even though she lost everything she had acquired after treatment was interrupted. The AD patient did not improve her naming capacity with therapy. These differences suggest that the learning and consolidation circuits are affected in different ways. Subjects with semantic dementia, but not those with AD, could benefit from word relearning strategies with this method (AU)