ABSTRACT
La displasia septo-óptica (DSO)[MIM182230] es una entidad heterogénea poco frecuente, caracterizada por la tríada clásica: hipoplasia del nervio óptico, anomalías de las hormonas hipofisarias y defectos de la línea media cerebral (incluyendo agenesia del septumpellucidumy/o del cuerpo calloso; también se han descrito malformaciones corticales asociadas, citado como síndrome DSO plus). Referimos el primer caso clínico conocido, en el que el signo de diagnóstico inicial de DSO fue una midriasis bilateral, como manifestación de hipoplasia de ambos nervios ópticos, hipoplasia hipofisaria y disgenesia cerebral con alteración de migración neuronal. Discutimos el diagnóstico diferencial de la midriasis congénita (AU)
Septo-optic dysplasia (SOD)[MIM182230] consisting of a heterogeneous and uncommon condition characterised by the classic triad: optic nerve hypoplasia, abnormalities of pituitary hormone, and defects of the brain midline (including agenesis of the septum pellucidum and/or the corpus callosum; it has also been described associated cortical malformations, it was referred to as SOD plus syndrome).We report the first known case in which the initial diagnostic sign of SOD was a bilateral mydriasis as a manifestation of hypoplasia of both optic nerves, pituitary hypoplasia andcerebral dysgenesis with neuronal migration disorder. We discuss the differential diagnosis of congenital mydriasis (AU)
Subject(s)
Humans , Septo-Optic Dysplasia/diagnosis , Mydriasis/congenital , Neural Conduction/physiology , Optic Nerve/physiopathologyABSTRACT
Septo-optic dysplasia (SOD)[MIM182230] consisting of a heterogeneous and uncommon condition characterised by the classictriad: optic nerve hypoplasia, abnormalities of pituitary hormone, and defects of thebrain midline (including agenesis of the septum pellucidum and/or the corpus callosum; ithas also been described associated cortical malformations, it was referred to as SOD plus syndrome).We report the first known case in which the initial diagnostic sign of SOD was a bilateralmydriasis as a manifestation ofhypoplasia of both optic nerves, pituitary hypoplasia andcerebral dysgenesis with neuronal migration disorder.We discuss thedifferential diagnosis of congenital mydriasis.
Subject(s)
Abnormalities, Multiple/diagnosis , Mydriasis/congenital , Septo-Optic Dysplasia/diagnosis , Atrophy , Blindness/etiology , Cell Movement , Cerebral Ventricles/abnormalities , Diagnosis, Differential , Early Diagnosis , Humans , Infant, Newborn , Intellectual Disability/etiology , Male , Mydriasis/etiology , Optic Nerve/pathology , Pituitary Gland/abnormalities , Septum Pellucidum/abnormalitiesSubject(s)
Ferritins/blood , Zellweger Syndrome/blood , Female , Humans , Infant , Infant, Newborn , Peroxisomal Disorders/bloodABSTRACT
No disponible
Subject(s)
Humans , Female , Infant, Newborn , Zellweger Syndrome/diagnosis , Peroxisomal Disorders/diagnosis , Ferritins/bloodABSTRACT
We report on the switching of an all-optical flip-flop consisting of a semiconductor optical amplifier (SOA) and a distributed feedback laser diode (DFB), bidirectionally coupled to each other. Both simulation and experimental results are presented. Switching times as low as 50ps, minimal required switch pulse energies below 1pJ and a repetition rate of 1.25GHz have been measured. Contrast ratios over 25dB have been obtained. The dependence on the pulse length and CW input power of the minimal required switch energy is investigated.
