Compartmental Differences in Macular Retinal Ganglion Cell Function

The purpose of this study was to investigate local differences of macular retinal ganglion cell (RGC) function by means of the steady-state pattern electroretinogram (SS-PERG). SS-PERGs were recorded in healthy subjects (n = 43) in response to gratings (1.6 c/deg, 15.63 reversals/s, and 98% contrast) presented on an LED display (800 cd/m2, 12.5 degrees eccentricity at 30 cm viewing distance) partitioned in triangular sectors (inferior [I]; nasal [N]; superior [S]; and temporal [T]) or concentric regions (central [C] and annulus [A]). For each partition, response amplitude (nV), amplitude adaptation (% change over recording time), phase/latency (deg/ms), and oscillatory potentials (OPs) amplitude (root mean square [RMS] nV) were measured. Data were analyzed with Generalized Estimating Equation (GEE) statistics. Amplitude differed (P T, I) as well as concentrically (C: 684 nV; A: 323 nV; and C>A). Latency did not differ between sectors (range = 53-54 ms, P = 0.45) or concentrically (range = 51-51 ms, P = 0.7). Adaptation did not differ (P = 0.66) concentrically (C: -19% and A: -22%) but differed (P = 0.004) between sectors (I: +25% and S: -29%). The OP amplitude did not differ (P = 0.5) between sectors (range = 63-73 nV) as well as concentrically (range = 82-90 nV, P = 0.3). Amplitude profiles paralleled RGC densities from histological studies. Adaptation profile suggested greater autoregulatory challenge in the inferior retina. Latency profile may reflect axonal conduction time to the optic nerve head assuming a direct relationship between axon length and its size/velocity. Location-independent OPs may reflect preganglionic activity. Normal macular RGC function displays local differences that may be related to local vulnerability in optic nerve disorders

Erdheim-Chester Disease Presenting with Diplopia; A Challenging Diagnosis and Effective Treatment

Erdheim-Chester Disease (ECD) is an infiltrative disease which can involve the CNS and presents with different symptoms. Herein, we present a patient with ECD who presented with sixth nerve palsy, with a challenging course of diagnosis and successful treatment with two specific protein-targeting agents