Estudio clinico terapéutico de la demencia en las personas con síndrome de down y eficacia del donepecilo en esta población / Clinical-therapeutic study of dementia in people with down syndrome and the effectiveness of donepezil in this population

Introducción. Las personas con síndrome de Down envejecen precozmente, sobre todo a partir de los 40 años, mientras un porcentaje significativo inicia, a partir de esta edad, un deterioro progresivo de sus capacidades cognitivas y funcionales previas, secundario todo ello a un proceso degenerativo primario de tipo enfermedad de Alzheimer. Estudios clínicos piloto en esta población, en los que se ha valorado la repuesta al tratamiento con donepecilo, han demostrado beneficios terapéuticos. Objetivo. Evaluar la eficacia y seguridad del tratamiento farmacológico con donepecilo sobre los trastornos cognitivos y conductuales en población con síndrome de Down mayor de 40 años, en la que la familia y educadores de referencia han observado cambios cognitivos y conductuales respecto a su nivel previo de discapacidad. Pacientes y métodos. Los pacientes han sido seleccionados a través de diferentes instituciones filiadas por la Fundació Catalana de Síndrome de Down y por la Federación Catalana Pro Personas con Disminución Psíquica. Se han utilizado escalas de valoración de deterioro, conducta y funcionalismo adaptadas a esta población. Resultados. Los resultados del estudio han demostrado que el donepecilo ralentiza la progresión de la disfunción cognitiva, especialmente durante los primeros tres meses de tratamiento. Esto sucede tanto en las escalas cognitivas como sociales. Conclusiones. El donepecilo parece ser efectivo en el tratamiento de los trastornos cognitivos y de la conducta en la demencia progresiva del síndrome de Down. Todas las muestras publicadas en diferentes estudios han sido pequeñas, lo que hace necesario poner en marcha estudios multicéntricos para validar la respuesta de estos sujetos a la terapia anticolinesterásica (AU)

Introduction. People with Down syndrome have an early aging process, especially form their 40s. There is a significant average of them who initiate, from that age on, a progressive decline of their cognitive and functional abilities, due to a primary degenerative process Alzheimer’s disease type. When assessing response to treatment with, pilot clinical trials on this population have demonstrated real benefits therapeutically. Aims. To assess the efficacy and safety of a pharmacological treatment with donepezil over cognitive and behavioral disturbances on patients with Down syndrome older than 40 years, areas where family and professional educators of reference have observed cognitive and behavioral changes in comparison with their previous level of disability. Patients and methods. Patients have been selected from different institutions affiliated at the Catalan Foundation for the Down syndrome and by the Catalan Federation Pro Persons with Psychic Disability. Several deterioration, behavioral and functional assessment scales have been used, all of them validated into this population. Results. The results of study demonstrated that donepezil slowed the progression of the cognitive dysfunction, especially during the first three months of treatment. This occurred for both cognitive and social-behavioral outcomes. Conclusions. Donepezil appears to be effective in the treatment of cognitive and behavioural disturbances associated with the progressive dementia syndrome in Down’s. However, the sample sizes used in this, and all published studies are small and this emphasizes the need for a larger, multi-center trial to fully evaluate the nature and extent of the response of Down's syndrome patients to anticolinesterase therapy (AU)

[Clinical-therapeutic study of dementia in people with Down syndrome and the effectiveness of donepezil in this population]. / Estudio clinicoterapéutico de la demencia en las personas con síndrome de Down y eficacia del donepecilo en esta población.

INTRODUCTION: People with Down syndrome have an early aging process, especially form their 40s. There is a significant average of them who initiate, from that age on, a progressive decline of their cognitive and functional abilities, due to a primary degenerative process Alzheimer's disease type. When assessing response to treatment with, pilot clinical trials on this population have demonstrated real benefits therapeutically. AIMS: To assess the efficacy and safety of a pharmacological treatment with donepezil over cognitive and behavioral disturbances on patients with Down syndrome older than 40 years, areas where family and professional educators of reference have observed cognitive and behavioral changes in comparison with their previous level of disability. PATIENTS AND METHODS: Patients have been selected from different institutions affiliated at the Catalan Foundation for the Down syndrome and by the Catalan Federation Pro Persons with Psychic Disability. Several deterioration, behavioral and functional assessment scales have been used, all of them validated into this population. RESULTS: The results of study demonstrated that donepezil slowed the progression of the cognitive dysfunction, especially during the first three months of treatment. This occurred for both cognitive and social-behavioral outcomes. CONCLUSIONS: Donepezil appears to be effective in the treatment of cognitive and behavioural disturbances associated with the progressive dementia syndrome in Down's. However, the sample sizes used in this, and all published studies are small and this emphasizes the need for a larger, multi-center trial to fully evaluate the nature and extent of the response of Down's syndrome patients to anticolinesterase therapy.