Dentro de su programa de fomento del talento y difusión e intercambio de conocimiento, IDIVAL pone en marcha el programa Valdecilla Progress Reports. En este programa de seminarios impartidos por investigadores jóvenes del ámbito clínico y de los laboratorios IDIVAL, se expondrán los avances científicos que están desarrollando dentro de los proyectos de investigación en los que participan. Está previsto que intervengan jóvenes con contratos predoctorales, investigadores Post-MIR Valdecilla López Albo, Río Hortega y becas Inn-Val y que sus correspondientes directores de proyecto estén presentes durante los Progress Reports.
El programa comienza el día 2 de Noviembre, miércoles, en Sesiones que tendrán lugar a las 14:30 en el aula 4-5 del Pabellón 16 del Hospital Universitario Marqués de Valdecilla. Las sesiones se impartirán en Inglés y tendrán una duración de 30 minutos.
La primera Sesión del programa será impartida por Ana Lara Pelayo Negro, Especialista del Servicio de Neurología del Hospital Universitario Marqués de Valdecilla, Ex-contrato Post-MIR Wenceslao López Albo y se titulará: EVOLUTION OF CHARCOT–MARIE–TOOTH DISEASE TYPE 1A DUPLICATION: A 2-YEAR CLINICO-ELECTROPHYSIOLOGICAL AND LOWER-LIMB MUSCLE MRI LONGITUDINAL STUDY. La Dra Ana Lara Pelayo pertenece al Grupo de Enfermedades Neurodegenerativas de IDIVAL.
El resumen de la sesión es el siguiente: The objective of this study was to analyze Charcot–Marie–Tooth disease type 1A (CMT1A) evolution. We conducted a 2-year longitudinal study in 14 CMT1A patients and 14 age- and sex-matched controls. In the patients, we performed neurological examination with hand-held dynamometry, electrophysiology, and lowerlimb muscle MRI, both at baseline and 2 years later, while controls were examined at baseline only. Patients’ ages ranged from 12 to 51 years. Outstanding manifestations on initial evaluation included pes cavus, areflexia, lower-limb weakness, and foot hypopallesthesia. In evaluating muscle power, good correlation was observed between manual testing and dynamometry. Compared to controls, Lunge, 10-Meter-Walking, and 9-Hole-Peg tests were impaired. Their CMT neuropathy score and functional disability scale showed that patients exhibited mild phenotype and at most slight walking difficulty. Electrophysiology revealed marked nerve conduction slowing and variable compound muscle action potential amplitude reduction. On lowerlimb muscle MRI, there was distally accentuated fatty infiltration accompanied by edema in calf muscles. All these clinico-electrophysiological and imaging findings remained almost unaltered during monitoring. Using multivariate analysis, no significant predictors of progression associated to the disease were obtained. We conclude that in the 2-year period of study, CMT1A patients showed mild progression with good concordance between clinicoelectrophysiological and imaging findings.
