RESUMEN El objetivo del artículo es describir y analizar la trayectoria de vida y las contribuciones humanitarias del Dr. Zambrano, médico egresado de la Escuela de Medicina de San Fernando, quién fue presidente del Centro de Estudiantes de Medicina en una época políticamente álgida en la educación universitaria pública. Emigró a los Estados Unidos en 1970, bajo el contexto de crisis sociopolítica económica en el Perú y en el mundo, donde se convirtió en un renombrado médico internista y cardiólogo del St. Luke's Hospital. A pesar de su lejanía, mantuvo un lazo estrecho con el Perú organizando múltiples misiones médicas para el beneficio de poblaciones vulnerables, capacitación al personal médico e implementación tecnológica del Hospital Regional de Cajamarca. En 2011 se inauguró el Centro Médico Educativo en Chincha, siendo Zambrano uno de los líderes para su construcción. A pesar de padecer una enfermedad invalidante dedicó hasta los últimos días de su vida a brindar ayuda a los más necesitados. Su trayectoria de vida nos muestra un ejemplo de compromiso con el Perú y de un ejercicio de la medicina comprometida con la solidaridad y el desarrollo de la medicina en su país de origen.

ABSTRACT The objective of the article is to describe and analyze the life trajectory and the humanitarian contributions of Dr. Zambrano, a physician who graduated from San Fernando Medical school, and was president of the Center for Medical Students at a politically critical time in public university education. He emigrated to the United States in 1970, in the context of sociopolitical crisis in Peru and the world, where he became a renowned internist and cardiologist at St. Luke's Hospital. Despite the distance, he maintained a close relationship with Peru, organizing multiple medical missions for underserved populations, training medical personnel, and providing technological implementation to Cajamarca Regional Hospital. In 2011, the Educational Medical Center was inaugurated in Chincha, with Zambrano being one of the leaders for its construction. Despite suffering from a disabling illness, he dedicated until the last days of his life, providing help for those most in need. His life trajectory shows us an example of commitment to Peru and practice of Medicine committed to solidarity and the development of Medicine in his country of origin.

Idiopathic inflammatory myopathies (MII) are a group of autoimmune diseases that mainly affect the proximal muscles. The most common types are Dermatomyositis (DM), Polymyositis (PM), Necrotizing autoimmune myopathy and Inclusion body myositis. Unique forms are identified in their clinical presentation consisting of muscular and extramuscular manifestations, their myopathic alterations in the electromyogram and the elevation of muscle enzymes. However, muscle biopsy remains the gold standard for diagnosis. These disorders are tratable with a proper. The goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity and improve quality of life.This review aims at a basic diagnostic approach in patients with suspicion of MMI through its main clinical, laboratory and histopathological findings.

Las miopatías inflamatorias idiopáticas (MII) son un grupo de enfermedades autoinmunes crónicas que afectan principalmente a los músculos proximales. Los tipos más comunes son dermatomiositis (DM), polimiositis (PM), miopatía autoinmune necrotizante y miositis por cuerpos de inclusión. Se identifican de forma única por su presentación clínica que consiste en manifestaciones musculares y extramusculares, sus alteraciones miopáticas en el electromiograma y la elevación de las enzimas musculares. Sin embargo, la biopsia muscular sigue siendo el gold estándar para el diagnóstico. Estos trastornos son potencialmente tratables con un diagnóstico adecuado. Los objetivos del tratamiento son eliminar la inflamación, restaurar el rendimiento muscular, reducir la morbilidad y mejorar la calidad de vida. Esta revisión tiene como objetivo proporcionar un enfoque de diagnóstico básico a los pacientes con sospecha de MMI a través de sus principales hallazgos clínicos, de laboratorio e histopatológicos.

São relatados dois casos de distrofia miotônica acompanhada de sonolência excessiva diurna. A avaliação polissonográfica de noite inteira revelou grande número de apnéias do sono tipo central deflagrando despertares freqüentes. As apnéias dos tipos obstrutivo e misto ocorreram em níveis normais e não se constatou hipoxia. Houve diminuição da eficiência do sono e redução dos estágios 3, 4 e REM. A apnéia central e a sonolência diurna que acarreta representariam manifestações precoces do comprometimento do sistema nervoso central na distrofia miotônica.

Two cases of myotonic dystrophy with excessive daytime somnolence are described. All-night polysomnographic studies were performed revealing high number of central sleep apnea which triggered micro-arousals and awakenings leading to decrease of sleep efficiency as well as of stage 3, 4 and REM. Obstructive and mixed apneas were found in the normal range. Hipoxia was not present in both recordings. Central sleep apneas and its secondary excessive daytime sleepinesse may indicate early signs of the central nervous system impairment related to myotonic dystrophy, as a multi-organ disease.

Este trabalho apresenta o estudo de quatro membros de uma família com degeneração espinocerebelar e movimentos sacádicos oculares lentos. Estudos neurofisiológicos detalhados mostraram anormalidades em tratos do sistema nervoso central que não eram aparentes ao exame clínico. Além dos movimentos oculares sacádicos lentos também foi possível demonstrar durante o registro poligráfico do sono a ausência completa dos movimentos oculares rápidos do sono REM apesar dos outros parâmetros do sono REM estarem normais. Desta maneira o estudo permite que se tire as seguintes conclusões: os movimentos sacádicos oculares lentos e os movimentos oculares rápidos do sono REM são mediados por mecanismo central comum possivelmente através da formação reticular paramediana da ponte; outras características fisiológicas do sono REM, particularmente a hipotonia muscular, não são necessariamente mediadas pelo mesmo sistema neuronal.

Four members of a family with spinocerebellar degeneration and slow saccadic eye movements are described. Detailed electrophysiological studies revealed abnormalities of neurological pathways not apparent clinically. The patients had slow saccades as mesasured electrophysiologically, as well as absence of rapid eye movements (REM) despite REM stages of sleep. These studies suggest that although saccadic eye movement and REM are mediated through the pontine paramedian reticular formation, other characteristics of REM sleep are not necessarily mediated through the same neurons.