RESUMEN
BACKGROUND: Primary progressive aphasia (PPA) is a neurodegenerative syndrome characterized by speech and/or language impairment with relatively spared cognition. Research investigating behavioral speech-language intervention and methods for cognitive-linguistic assessment in PPA has predominantly centered around monolingual speakers. This gap hinders the widespread adoption of evidence-based approaches and exacerbates the inequities faced by culturally and linguistically diverse populations living with PPA. OBJECTIVE: This scoping review synthesizes the current evidence for assessment and treatment practices in bilingual PPA as well as the operationalization of bilingualism in PPA. METHODS: Arksey & O'Malley's scoping review methodology was utilized. Information was extracted from each study and entered into a data-charting template designed to capture information regarding operationalization of bilingualism in PPA and assessment and treatment practices. RESULTS: Of the 16 identified studies, 14 reported the results of assessments conducted in both languages. Three studies reported positive naming treatment outcomes. Thirteen studies included English-speaking participants, revealing linguistic bias. Most studies reported age of acquisition, proficiency, and patterns of language use rather than providing an operational definition for bilingualism. CONCLUSIONS: Neither formal assessment measures nor clear guidelines for assessment of bilingual PPA currently exist; however, language-specific measures are emerging. Speech-language intervention in bilingual PPA has been relatively unexplored, representing a significant gap in the literature. In order to improve diagnostic and treatment options for bilingual PPA, targeted efforts to increase representation of bilinguals from various sociocultural contexts, as well as those who speak a variety of language pairs, is necessary.
Asunto(s)
Afasia Progresiva Primaria , Multilingüismo , Humanos , Lenguaje , Lingüística , Habla , Afasia Progresiva Primaria/diagnóstico , Afasia Progresiva Primaria/terapiaRESUMEN
Oxidative stress-induced damage is a major mechanism in the pathophysiology of amyotrophic lateral sclerosis (ALS). A recent human clinical trial showed that the combination of nicotinamide riboside (NR) and pterostilbene (PT), molecules with potential to interfere in that mechanism, was efficacious in ALS patients. We examined the effect of these molecules in SOD1G93A transgenic mice, a well-stablished model of ALS. Assessment of neuromotor activity and coordination was correlated with histopathology, and measurement of proinflammatory cytokines in the cerebrospinal fluid. Cell death, Nrf2- and redox-dependent enzymes and metabolites, and sirtuin activities were studied in isolated motor neurons. NR and PT increased survival and ameliorated ALS-associated loss of neuromotor functions in SOD1G93A transgenic mice. NR and PT also decreased the microgliosis and astrogliosis associated with ALS progression. Increased levels of proinflammatory cytokines were observed in the cerebrospinal fluid of mice and humans with ALS. NR and PT ameliorated TNFα-induced oxidative stress and motor neuron death in vitro. Our results support the involvement of oxidative stress, specific Nrf2-dependent antioxidant defenses, and sirtuins in the pathophysiology of ALS. NR and PT interfere with the mechanisms leading to the release of proapoptotic molecular signals by mitochondria, and also promote mitophagy.
Asunto(s)
Esclerosis Amiotrófica Lateral/patología , Neuronas Motoras/patología , Mutación/genética , Niacinamida/análogos & derivados , Compuestos de Piridinio/farmacología , Estilbenos/farmacología , Superóxido Dismutasa-1/genética , Acetilcisteína/farmacología , Esclerosis Amiotrófica Lateral/líquido cefalorraquídeo , Animales , Antioxidantes/farmacología , Apoptosis/efectos de los fármacos , Citocinas/líquido cefalorraquídeo , Femenino , Masculino , Metaboloma , Ratones Endogámicos C57BL , Ratones Transgénicos , Mitocondrias/efectos de los fármacos , Mitocondrias/metabolismo , Actividad Motora/efectos de los fármacos , Neuronas Motoras/efectos de los fármacos , NAD/sangre , Factor 2 Relacionado con NF-E2/metabolismo , Degeneración Nerviosa/patología , Niacinamida/farmacología , Oxidación-Reducción , Especies Reactivas de Oxígeno/metabolismo , Sirtuina 1/metabolismo , Sirtuina 3/metabolismo , Médula Espinal/patología , Estilbenos/sangre , Análisis de SupervivenciaRESUMEN
Multivariate screening methods are increasingly being implemented but there is no worldwide harmonized criterion for their validation. This study contributes to establish protocols for validating these methodologies. We propose the following strategy: (1) Establish the multivariate classification model and use receiver operating characteristic (ROC) curves to optimize the significance level (α) for setting the model's boundaries. (2) Evaluate the performance parameter from the contingency table results and performance characteristic curves (PCC curves). The adulteration of hazelnut paste with almond paste and chickpea flour has been used as a case study. Samples were analyzed by infrared (IR) spectroscopy and the multivariate classification technique used was soft independent modeling of class analogies (SIMCA). The ROC study showed that the optimal α value for setting the SIMCA boundaries was 0.03 in both cases. The sensitivity value was 93%, specificity 100% for almond and 98% for chickpea, and efficiency 97% for almond and 93% for chickpea.