The long-term outcomes of the Anderson-Kestenbaum procedure.

Introduction: Nystagmus is an involuntary, conjugated, rhythmic movement of the eye that can be idiopathic or secondary to ocular or neurologic pathologies. Patients with nystagmus often have a position of gaze in which their symptoms are dampened or absent, referred to as the "null zone." The Anderson-Kestenbaum procedure is a bilateral recess-resect procedure of the four horizontal rectus muscles which aims to bring the null position into the primary gaze.

A visually guided swim assay for mouse models of human retinal disease recapitulates the multi-luminance mobility test in humans.

Purpose: The purpose of this study was to develop a visually guided swim assay (VGSA) for measuring vision in mouse retinal disease models comparable to the multi-luminance mobility test (MLMT) utilized in human clinical trials.

Methods: Three mouse retinal disease models were studied: Bardet-Biedl syndrome type 1 (), = 5; Bardet-Biedl syndrome type 10 (), = 11; and X linked retinoschisis (retinoschisin knockout; KO), = 5. Controls were normally-sighted mice, = 10.

Progressive retinal degeneration of rods and cones in a Bardet-Biedl syndrome type 10 mouse model.

Bardet-Biedl syndrome (BBS) is a multi-organ autosomal-recessive disorder caused by mutations in at least 22 different genes. A constant feature is early-onset retinal degeneration leading to blindness. Among the most common forms is BBS type 10 (BBS10), which is caused by mutations in a gene encoding a chaperonin-like protein.