Evaluación neurocognitivo-emocional en la enfermedad de Pelizaeus-Merzbacher (PMD)

María López-Sánchez

doi:10.14198/DCN.2018.5.1.02

Autores/as

María López-Sánchez Asociación Española contra la Leucodistrofia – ELA España, (Madrid), España

DOI:

https://doi.org/10.14198/DCN.2018.5.1.02

Palabras clave:

Pelizaeus-Merzbacher, PMD, Neuropsicología, Atención, Memoria, Funciones Ejecutivas

Resumen

Pelizaeus-Merzbacher (en adelante PMD) es una enfermedad crónica, minoritaria y neurodegenerativa que pertenece a las leucodistrofias hipomielinizanes. El objetivo de este estudio es proporcionar una base científica de los aspectos cognitivos, ya que en la actualidad se carece de bibliografía enfocada en esto, y así enfatizar en la necesidad de intervención desde la estimulación y la neuropsicología. Los resultados del estudio sugieren que un mayor número síntomas iniciales, fundamentalmente el bajo tono muscular y los problemas de motricidad, correlaciona con una mayor gravedad a nivel motriz en el momento presente. Además, el tiempo medio para llegar a un diagnóstico (4 años y 2 meses), correlaciona positivamente con el número de terapias que reciben los cuidadores, si bien es cierto que parece que este tiempo se está acortando en los últimos años. El estado emocional de los cuidadores muestra una leve sintomatología ansioso-depresiva, correlacionando positivamente la Ansiedad Estado con el número de manifestaciones iniciales y negativamente con las habilidades motrices de la persona con PMD. Entre las terapias de elección para personas con PMD destaca la fisioterapia y la logopedia, no encontrándose la rehabilitación neuropsicológica entre las terapias que reciben este colectivo. Por último, existe una correlación negativa entre la Salud física y las Habilidades del lenguaje.

Citas

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