Adhesión al tratamiento farmacológico y descripción de sus factores asociados en pacientes con miastenia grave / Pharmacological treatment compliance and a description of its associated factors in patients with myasthenia gravis

Introducción. La adhesión al tratamiento farmacológico es un problema de salud pública, pero no se ha estudiado en pacientes con miastenia grave. Objetivo. Determinar la adhesión al tratamiento farmacológico en pacientes con miastenia grave y describir al grupo de pacientes no adherentes al tratamiento. Pacientes y métodos. Estudio de corte transversal, en pacientes con miastenia grave, en control y abandono del tratamiento farmacológico en el Hospital Padre Hurtado, Santiago de Chile. Los pacientes fueron invitados a participar de forma voluntaria y anónima. Se evaluó la adhesión al tratamiento con la escala Morisky-Green-Levine de cuatro ítems. Además, se evaluó la gravedad de la miastenia grave con el test manual de fuerza muscular (MMT); la calidad de vida asociada a la miastenia grave, con el 15-Item Quality Of Life Instrument for Myasthenia Gravis (MG-QOL15), y el riesgo de depresión, con el cuestionario de salud general de 12 ítems (GHQ-12). Resultados. Participaron 26 pacientes, de los cuales 15 (57,7%) eran mujeres. Sólo 10 (38,5%) mostraron adhesión al tratamiento. Los no adherentes presentaron mayor debilidad (MMT; p = 0,06) y peor calidad de vida (MG-QOL15; p = 0,008), y tomaban más fármacos para la miastenia grave (p = 0,003). Además, presentaron mayor riesgo de depresión (GHQ-12; p = 0,03) comparados con el grupo de adhesión al tratamiento. Conclusión. Tres de cada cinco pacientes con miastenia grave abandonaron el tratamiento, lo que se asoció con mayor debilidad, peor calidad de vida y mayor riesgo de depresión. Por lo tanto, debería evaluarse la adhesión al tratamiento en los pacientes con miastenia grave (AU)

Introduction. Medication adherence is a public health problem and this has not been previously studied in myasthenia gravis patients. Aim. To determine if patients with myasthenia gravis are adherent to treatment and to describe the clinical factors of patients who are non-adherent to treatment. Patients and methods. Cross-sectional study of patients with myasthenia gravis followed at Padre Hurtado Hospital, Santiago de Chile, who received their medication through the hospital and therefore were on the pharmacy's list. Patients'. participation was voluntary and anonymous. Medication adherence was assessed with the Morisky-Green-Levine survey (4 items). Patients were assessed for myasthenia gravis severity with the Manual Muscle Test, and myasthenia gravisrelated quality of life with the MG-QOL15. Finally, patients were screened for depression with the 12-Item General Health Questionnaire. Results. 26 patients were enrolled and 15 (57.7%) were women. Only 10 (38.5%) of patients were adherent to treatment. Patients who were not adherent to medication had more weakness (p = 0.06), worse quality of life (p = 0.008), were taking a greater number of myasthenia gravis drugs (p = 0.003) and had a higher risk of depression (p = 0.03). Conclusions. In this cohort of myasthenia gravis patients, three out of five patients were not adherent to treatment. These patients tended to have more weakness, worse quality of life and higher risk of depression. Medication adherence should be assessed routinely in patients with myasthenia gravis (AU)

Pure autonomic failure with cold induced sweating.

Pure autonomic failure (PAF) is a progressive autonomic neurodegenerative disorder. Cold induced sweating occurred in syndromes with mutations in CRLF1 and CLCF1 genes and in a case of cervical dissection. A patient with PAF developed sweating induced by cool ambient temperatures. He had severe orthostatic hypotension, abnormal cardiovagal reflexes, and paradoxical sweating in the upper trunk at a room temperature of 18°C. Skin biopsy showed involvement of somatic epidermal unmyelinated nerve fibers. Quantitative sensory testing showed abnormal thresholds to all thermal modalities. Possible mechanisms include cold induced noradrenaline release in remaining autonomic innervation and a supersensitive sudomotor response.