Almost everyone will have met someone with eyes that seemingly move around of their own accord, often with a repetitive to and fro motion in a rhythmical fashion. In many instances, these people will have congenital nystagmus. Approximately 1 in 500 people have congenital nystagmus, and while they do not perceive a shaky image, their eyesight tends to be poor.
Until now, and despite many decades of research, the underlying mechanism of congenital nystagmus has remained elusive but its location was widely believed to reside in the brain stem as this area controls eye movements. However, a group of scientists from the Netherlands Institute for Neuroscience and the Erasmus MC, together with colleagues from the United States and Japan, suspected that they had to look elsewhere for the source of this disorder. In this study, they show that electrical oscillations in retinal neurons cause congenital nystagmus.
Years - Huib - Simonsz - Ophthalmologist - Sophia
Twenty years ago, Huib Simonsz, a pediatric ophthalmologist at the Sophia Children's Hospital in Rotterdam, discovered a group of patients who presented with different types of congenital night blindness and the same type of congenital nystagmus. "A defect in two proteins causes these types of night blindness. The two faulty proteins sit on either side of the nerve junction, a synapse, connecting the light-sensitive rods to a retinal interneuron. This impairs the signaling between the two cell types, which in turn causes retinal cells downstream from the interneuron to...
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