RESUMEN
OBJECTIVES: To examine the clinical trajectories and neural correlates of cognitive and emotion processing changes in the non-fluent/agrammatic (nfvPPA) and the logopenic (lvPPA) variants of primary progressive aphasia (PPA). DESIGN: Observational case-control longitudinal cohort study. SETTING: Research clinic of frontotemporal dementia. PARTICIPANTS: This study recruited 29 non-semantic PPA patients (15 nfvPPA and 14 lvPPA) and compared them with 15 Alzheimer's disease (AD) patients and 14 healthy controls. MEASUREMENTS: Participants completed an annual assessment (median = 2 years; range = 1-5 years) of general cognition, emotion processing and structural MRI. Linear mixed effects models investigated clinical and imaging trajectories between groups. RESULTS: Over time, lvPPA showed the greatest cognitive deterioration. In contrast, nfvPPA showed significant decline in emotion recognition, whereas AD showed preserved emotion recognition, even with disease progression. Importantly, lvPPA also developed emotion processing impairments, with disease progression. Both nfvPPA and lvPPA showed continuing cortical atrophy in hallmark language-processing regions associated with these syndromes, together with progressive involvement of the right hemisphere regions, mirroring left hemisphere atrophy patterns at presentation. Decline in emotion processing was associated with bilateral frontal atrophy in nfvPPA and right temporal atrophy in lvPPA. CONCLUSIONS: Our results show divergent clinical courses in nfvPPA and lvPPA, with rapid cognitive and neural deterioration in lvPPA and emotion processing decline in both groups and support the concurrent assessment of cognition and emotion processing in the clinic to inform diagnosis and monitoring in the non-semantic variants of PPA.
Asunto(s)
Enfermedad de Alzheimer , Afasia Progresiva Primaria , Afasia Progresiva Primaria no Fluente , Humanos , Enfermedad de Alzheimer/psicología , Afasia Progresiva Primaria/diagnóstico por imagen , Afasia Progresiva Primaria/complicaciones , Afasia Progresiva Primaria/psicología , Atrofia , Progresión de la Enfermedad , Emociones , Estudios Longitudinales , Afasia Progresiva Primaria no Fluente/complicaciones , Estudios de Casos y ControlesRESUMEN
Amygdala atrophy has been found in frontotemporal dementia (FTD), yet the specific changes of its subregions across different FTD phenotypes remain unclear. The aim of this study was to investigate the volumetric alterations of the amygdala subregions in FTD phenotypes and how they evolve with disease progression. Patients clinically diagnosed with behavioral variant FTD (bvFTD) (n = 20), semantic dementia (SD) (n = 20), primary nonfluent aphasia (PNFA) (n = 20), Alzheimer's disease (AD) (n = 20), and 20 matched healthy controls underwent whole brain structural MRI. The patient groups were followed up annually for up to 3.5 years. Amygdala nuclei were segmented using FreeSurfer, corrected by total intracranial volumes, and grouped into the basolateral, superficial, and centromedial subregions. Linear mixed effects models were applied to identify changes in amygdala subregional volumes over time. At baseline, bvFTD, SD, and AD displayed global amygdala volume reduction, whereas amygdala volume appeared to be preserved in PNFA. Asymmetrical amygdala atrophy (left > right) was most pronounced in SD. Longitudinally, SD and PNFA showed greater rates of annual decline in the right basolateral and superficial subregions compared to bvFTD and AD. The findings provide comprehensive insights into the differential impact of FTD pathology on amygdala subregions, revealing distinct atrophy patterns that evolve over disease progression. The characterization of amygdala subregional involvement in FTD and their potential role as biomarkers carry substantial clinical implications.
Asunto(s)
Amígdala del Cerebelo , Demencia Frontotemporal , Anciano , Femenino , Persona de Mediana Edad , Enfermedad de Alzheimer/patología , Amígdala del Cerebelo/diagnóstico por imagen , Amígdala del Cerebelo/patología , Atrofia/diagnóstico por imagen , Atrofia/patología , Estudios Transversales , Progresión de la Enfermedad , Demencia Frontotemporal/clasificación , Demencia Frontotemporal/diagnóstico por imagen , Demencia Frontotemporal/patología , Demencia Frontotemporal/fisiopatología , Estudios Longitudinales , Imagen por Resonancia Magnética , Tamaño de los Órganos , Afasia Progresiva Primaria no Fluente/patología , Factores de Tiempo , Humanos , MasculinoRESUMEN
BACKGROUND: The behavioral variant of frontotemporal dementia (bvFTD) is very heterogeneous in pathology, genetics, and disease course. Unlike Alzheimer's disease, reliable biomarkers are lacking and sporadic bvFTD is often misdiagnosed as a primary psychiatric disorder (PPD) due to overlapping clinical features. Current efforts to characterize and improve diagnostics are centered on the minority of genetic cases. OBJECTIVE: The multi-center study DIPPA-FTD aims to develop diagnostic and prognostic algorithms to help distinguish sporadic bvFTD from late-onset PPD in its earliest stages. METHODS: The prospective DIPPA-FTD study recruits participants with late-life behavioral changes, suspect for bvFTD or late-onset PPD diagnosis with a negative family history for FTD and/or amyotrophic lateral sclerosis. Subjects are invited to participate after diagnostic screening at participating memory clinics or recruited by referrals from psychiatric departments. At baseline visit, participants undergo neurological and psychiatric examination, questionnaires, neuropsychological tests, and brain imaging. Blood is obtained to investigate biomarkers. Patients are informed about brain donation programs. Follow-up takes place 10-14 months after baseline visit where all examinations are repeated. Results from the DIPPA-FTD study will be integrated in a data-driven approach to develop diagnostic and prognostic models. CONCLUSIONS: DIPPA-FTD will make an important contribution to early sporadic bvFTD identification. By recruiting subjects with ambiguous or prodromal diagnoses, our research strategy will allow the characterization of early disease stages that are not covered in current sporadic FTD research. Results will hopefully increase the ability to diagnose sporadic bvFTD in the early stage and predict progression rate, which is pivotal for patient stratification and trial design.
Asunto(s)
Acetamidas , Demencia Frontotemporal , Isotiocianatos , Humanos , Demencia Frontotemporal/genética , Estudios Prospectivos , Pronóstico , Pruebas Neuropsicológicas , BiomarcadoresRESUMEN
El tratamiento de desensibilización y reprocesamiento por movimiento ocular (EMDR de sus siglas en inglés: eye movement desensitization and reprocessing) es una terapia relativamente novedosa que de forma progresiva ha ido ganando popularidad en el tratamiento del trastorno por estrés postraumático. El objetivo de este trabajo es introducir el protocolo estándar EMDR, ofrecer una revisión de las hipótesis actuales sobre su mecanismo de acción y analizar la evidencia científica disponible sobre su eficacia clínica en pacientes adultos con diagnóstico de trastorno por estrés postraumático. Se realizó una revisión sistemática de la literatura publicada en las bases de datos PubMed y PsycINFO con los términos «eye movement desensitization and reprocessing» y «posttraumatic stress disorder» y sus contracciones en inglés «EMDR» y «PTSD». Se obtuvieron como resultado 15 ensayos controlados aleatorizados de elevada calidad metodológica que compararon EMDR con tratamientos no específicos, lista de espera y con tratamientos específicos. Los resultados de estos estudios permiten concluir que EMDR es una herramienta útil y basada en evidencia científica, tal y como refleja su reciente recomendación como tratamiento de elección en el trastorno por estrés postraumático por parte de distintas organizaciones internacionales de salud
Eye movement desensitization and reprocessing (EMDR) is a relatively new psychotherapy that has gradually gained popularity for the treatment of post-traumatic stress disorder. In the present work, the standardised EMDR protocol is introduced, along with current hypotheses of its mechanism of action, as well as a critical review of the available literature on its clinical effectiveness in adult post-traumatic stress disorder. A systematic review of the published literature was performed using PubMed and PsycINFO databases with the keywords «eye movement desensitization and reprocessing» and «post-traumatic stress disorder» and its abbreviations «EMDR» and «PTSD». Fifteen randomised controlled trials of good methodological quality were selected. These studies compared EMDR with unspecific interventions, waiting lists, or specific therapies. Overall, the results of these studies suggest that EMDR is a useful, evidence-based tool for the treatment of post-traumatic stress disorder, in line with recent recommendations from different international health organizations