Vici syndrome is a rare, genetically unresolved congenital multisystem disorder comprising agenesis of the corpus callosum, cataracts, immunodeficiency, cardiomyopathy, and hypopigmentation. An associated neuromuscular phenotype has not previously been described in detail. We report on an infant with clinical features suggestive of Vici syndrome and additional sensorineural hearing loss. Muscle biopsy revealed several changes including markedly increased variability in fiber size, increased internal nuclei, and abnormalities on Gomori trichrome and oxidative stains, raising a wide differential diagnosis including neurogenic atrophy, centronuclear myopathy (CNM) or a metabolic (mitochondrial) cytopathy. Respiratory chain enzyme studies, however, were normal and sequencing of common CNM-associated genes did not reveal any mutations. This case expands the clinical spectrum of Vici syndrome and indicates that muscle biopsy ought to be considered in infants presenting with suggestive clinical features. In addition, we suggest that Vici syndrome is considered in the differential diagnosis of infants presenting with congenital callosal agenesis and that additional investigation has to address the possibility of associated ocular, auditory, cardiac, and immunologic involvement when this radiologic finding is present.

The most common mechanism of cerebellar infarctions was arterial occlusion as a result of intracranial vertebral artery dissection (40%), mainly with PICA involvement. Embolism from a cardiac source resulted primarily from patent foramen ovale and rheumatic valvular disease. Hematologic disturbances and migraine were responsible for a few cases.

ObjectiveTo estimate the effects on homicide rates of the gang truce that was brokered in El Salvador in 2012.MethodsMathematical models based on municipal-level census, crime and gang-intelligence data were used to estimate the effect of the truce on homicide rates. One model estimated the overall effect after accounting for the linear trend and seasonality in the homicide rate. In a moderated-effect model, we investigated the relationship between the truce effect and the numbers of MS13 (Mara Salvatrucha 13) and Eighteenth-Street gang members imprisoned per 100 000 population. We then ran each of these two models with additional control variables. We compared values before the truce – 1 January 2010 to 29 February 2012 – with those after the truce – 1 March 2012 to 31 December 2013.FindingsThe overall-effect models with and without additional control variables indicated a homicide rate after the truce that was significantly lower than the value before the truce, giving rate ratios of 0.55 (95% confidence interval, CI: 0.49–0.63) and 0.61 (95% CI: 0.54–0.69), respectively. For any given municipality, the effectiveness of the truce appeared to increase as the number of MS13 gang members imprisoned per 100 000 population increased. We did not observe the same significant relationship for imprisoned Eighteenth-Street gang members.ConclusionIn the 22 months following the establishment of a national gang truce, the homicide rate was about 40% lower than in the preceding 26 months. The truce’s impact appeared particularly strong in municipalities with relatively high numbers of imprisoned MS13 gang members per 100 000 population.

ResumenEste artículo tiene por objetivo general examinar y analizar preliminarmente, el proceso a través del cual se dividió la pandilla Barrio 18 en "los Sureños" y "los Revolucionarios". La metodología empleada se ha basado en la aplicación de las técnicas de revisión documental y entrevistas en profundidad. Este trabajo arranca de un esfuerzo previo, en el que, con una visión holística, se entiende a las maras o pandillas a partir de cuatro criterios: 1) Su forma organizacional; 2) Su composición; 3) La naturaleza de sus acciones; y 4) Su conjunto de valores y normas socioculturales. La definición de pandilla como categoría en clave de identidad resulta esencial. La necesidad de construir una definición holística de pandilla tiene el objetivo de evitar "suavizar" el accionar delictivo de los pandilleros, así como "sobre-criminalizar" a las pandillas. Palabras claveBarrio 18, maras y pandillas, escisiones criminales, sur-sureños, revolucionarios. Abstract:This article's general objective is to provide a prelimary examination and analysis of the process through which Barrio 18 split into "los Sureños" and "los Revolucionarios". The methodology this study was based on, used specialized techniques such as document review and in-depth interviews. This work starts from a previous effort, in which, through a holistic vision, gangs or maras are understood from four different perspectives: 1) Their organizational structure; 2) Their composition; 3) The nature of their actions; and 4) Their set of values and socio-cultural norms. The definition of gang as a key category as far as their identity is essential. The need to build a holistic definition of gang is meant to "tone down" gang member's criminal activities, as well as to "over-criminalize" gangs.

El objetivo de este trabajo es acercarse a las vías de “retiro” de pandilleros en el país, a fin de mostrar el panorama general de las opciones existentes y describir sus características esenciales. La metodología se basa en la aplicación de dos técnicas de investigación: la revisión documental y las entrevistas en profundidad. Se exponen categorías de análisis importantes con respecto a los expandilleros, como por ejemplo las de “calmado”, “retirado” y “peseta”, cada una de las cuales plantea escenarios y prospectivas diferentes. Además, se explora el papel del Gobierno, el rol de la sociedad civil (incluyendo las iglesias católica y evangélica; en especial, las pentecostales), la participación del sector privado y ciertos mecanismos de las pandillas mismas. En definitiva, a todo el estudio le subyace una reflexión de carácter “identitario”, por cuanto concierne a la construcción, deconstrucción y eventual reconstrucción de la identidad, a nivel personal y grupal, de los miembros y exmiembros de maras o pandillas.