Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study

Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration.info:eu-repo/semantics/publishedVersio

Propuesta de inclusión de capacidades predictivas en un teclado virtual

En este trabajo se propone la implementación de capacidades predictivas en un teclado virtual. Estas capacidades han sido diseñadas pensando específicamente en personas con algún tipo de discapacidad motriz que les impida utilizar un teclado convencional. Se propone un sistema de predicción con capacidad de aprendizaje de nuevas palabras y del estilo de escritura del usuario. Se propone realizar la predicción de la palabra actual a partir de las teclas pulsadas y de la palabra siguiente una vez completada la palabra actual. Todo ello con el ob jetivo de minimizar el número de pulsaciones de teclas necesario para escribir un texto mediante un teclado virtual. Los resultados obtenidos muestran que un 21% de las palabras se pueden escribir con una única pulsación y que dos pulsaciones son suficientes para escribir otro 37% ; lo que permite ahorrar hasta un 42% de pulsaciones al escribir un texto en estilo literario

Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study

Background: Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration. Methods: In this international cross-sectional study, patients were examined at the referral centers following a standardized protocol. The motor examination was filmed, allowing 3 independent specialists in movement disorders to analyze 28 patients for interrater reliability assessment. The scale included 34 items (maximal score, 135) encompassing 6 subscales for cognition, behavior, disability, parkinsonism, dystonia, and other neurological signs. Results: Forty-seven genetically confirmed patients (30 ± 17 years; range, 6-77 years) were examined with the scale (mean score, 62 ± 21; range, 20-106). Dystonia with prominent cranial involvement and atypical parkinsonian features were present in all patients. Other common signs were cognitive impairment, psychiatric features, and slow and hypometric saccades. Dystonia, parkinsonism, and other neurological features had a moderate to strong correlation with disability. The scale showed good internal consistency for the total scale (Cronbach's α = 0.87). On interrater analysis, weighted kappa values (0.30-0.93) showed substantial or excellent agreement in 85% of the items. The scale also discriminated a subgroup of homozygous c.1583C>T patients with lower scores, supporting construct validity for the scale. Conclusions: The proposed scale seems to be a reliable and valid instrument for the assessment of pediatric and adult patients with pantothenate kinase-associated neurodegeneration. Additional validation studies with a larger sample size will be required to confirm the present results and to complete the scale validation testing. © 2017 International Parkinson and Movement Disorder Society. © 2017 International Parkinson and Movement Disorder Societ