ABSTRACT
INTRODUCTION: Spinal muscular atrophy 5q (SMA) is a genetic neurodegenerative disease that affects alpha motor neurons producing progressive weakness. New outcome measures are currently required to accurately characterise the disease progression and the efficacy of new available treatments. The objective of this work is to preliminarily validate a new intelligent keyboard (Neuromyotype) measuring typing strength and speed in patients with SMA. MATERIAL AND METHODS: Twenty two SMA patients older than 15 years, and 26 healthy controls were included. Three measurements were obtained with the keyboard (maximum strength, execution time of a random typing task, execution time of a sequential typing task) together with the time to complete the Nine-Hole Peg Test (9HPT). Patients were also administered motor (Hammersmith Functional Motor Scale Expanded, HFMSE; Revised Upper Limb module, RULM), and functional scales (Egen Klassification, EK2; and the revised version of Amyotrophic Lateral Sclerosis Functional Rating Scale, ALSFRS-R). The viability and construct validity of the Neuromyotype were analysed, measuring the discriminative power between patients and controls (using ROC curves and the Bangdiwala's B statistic), between the different functional types of SMA (walker, sitter and non-sitter) and their correlation with the rest of motor scales. RESULTS: Neuromyotype measurements could be performed in all patients, unlike the rest of the scales. Its administration was quick and easy. The 3 variables on the keyboard discriminated very well between patients and controls, with strength (ROC = 0.963) being the one that best differentiates from the 3, equaling 9HPT (ROC = 0.966). They also showed a good ability to differentiate by functional type (especially non-sitters from sitters and walkers), with sequential time (B = 0.83) being the tool that best discriminates between the three groups above the rest of motor scales. All motor and functional scales showed strong or very strong correlations with each other (rs = 0.71-0.99), with strength correlating better with motor scales and timed variables with functional scales. CONCLUSION: This study shows the feasibility and validity of Neuromyotype for the evaluation of adolescent and adult patients with SMA. Data obtained with this tool could be of great clinical relevance, saving time and resources compared to the rest of the scales.
ABSTRACT
La planta del pie está sujeta a importantes presiones que están influenciadas por factores dinámicos (locomoción, velocidad, etc) y por parámetros relativos al sujeto (peso corporal, edad, posibles patologías, etc). El estudio de estas presiones permite la localización y cuantificación de las zonas de hiperpresión, aspecto de vital importancia en pacientes diabéticos. La disponibilidad y uso de sistemas de medida de presión (plataformas de fuerza dentro del zapato), ha hecho posible la comprensión de una parte fundamental de los problemas de los pies. El efecto acumulativo de presiónde bajo nivel no se ha tenido en cuenta ni se ha evaluado suficientemente, principalmente debido a que la mayoría de los dispositivos de medida de presión funcionan con precisión únicamente durante unas horas. Algunos trabajos recientes, sugieren que la medida combinada de presiones plantares y actividad, evaluada como número de pasos reales frente al tiempo, pueden apoyar la hipótesis de que la destrucción del tejido en la planta del pie, que precede a la formación de una úlcera, es el resultado de la combinación al menos del estrés repetitivo a presiones bajas y de la acción de picos de presiones localizadas. Los nuevos sistemas de podobarografía deberían permitir el seguimiento de los pacientes durante su vida diaria, siempre y cuando la cantidad y calidad de los datos sea de utilidad clínica práctica
There are important pressures applying under the foot, which are influenced by many dynamic factors (such as locomotion, speed, etc) and parameters related to the subject (such as body weight, age, other diseases, etc. The study of foot pressures allow the localisation and quantification of the hyperpressure areas, which is very important for diabetic patients. The availability and use of footpressure measuring systems (force platforms and in-shoe systems) has made possible the understanding of an essential part of foot related problems. The cumulative effect of low pressure has not been sufficiently considered and evaluated, mainly due to the fact that most of the pressure measuring devices work accurately for only a few hours. Some recent studies suggest that the combined measurement of plantar pressures and activity, assessed as the actual number of steps vs. time, may support the hypothesis that the destruction of foot sole tissue prior to the ulcer formation results from the combination at least of repetitive stress at low pressures and the action of localized peak pressures. Therefore, it the new systems of baropodography should permit the monitoring of patients on a daily basis, provided that the quantity and quality of gathered data might be useful in the clinical practice
