Jeffrey Lambe1, Alissa Rothman1, Jerry Prince1, Shiv Saidha1, Peter A Calabresi1, Scott D Newsome2. 1. From the Departments of Neurology (J.L., A.R., S.S., P.A.C., S.D.N.) and Electrical and Computer Engineering (J.P.), Johns Hopkins University School of Medicine, Baltimore, MD. 2. From the Departments of Neurology (J.L., A.R., S.S., P.A.C., S.D.N.) and Electrical and Computer Engineering (J.P.), Johns Hopkins University School of Medicine, Baltimore, MD. snewsom2@jhmi.edu.
Abstract
OBJECTIVE: To evaluate whether structural and functional changes occur in the afferent visual system of patients with stiff-person syndrome (SPS) and whether these changes correlate with disease burden, given the high concentration of γ-aminobutyric acid receptors, which are generally thought to be involved in SPS pathogenesis, in the retina. METHODS: In this single-center, cross-sectional study, patients with SPS and healthy controls (HCs) underwent optical coherence tomography (OCT), with a subset undergoing high- and low-contrast visual acuity (VA) assessments. Burden of disease was assessed via the number of body regions affected. Individuals with uncontrolled hypertension or comorbid neurologic or ophthalmologic disorders were excluded. Statistical analyses were performed using mixed-effects linear regression models. RESULTS: Thirty-five patients with SPS and 40 age- and sex-matched HCs underwent OCT. A subset of 23 patients with SPS and 28 HCs underwent VA assessments. Relative to HCs, patients with SPS had lower ganglion cell + inner plexiform layer (GCIPL) thicknesses (SPS: 74.36 µm [SD 5.7]; HCs: 76.33 µm [SD 4.2]; p = 0.005), inner nuclear layer thicknesses (SPS: 44.37 µm [SD 2.7]; HCs: 45.18 µm [SD 2.2]; p = 0.042), and 100% (SPS: 53 [SD 9.6]; HCs: 57.5 [SD 6.1]; p = 0.005), 2.5% (SPS: 24.35 [SD 10.1]; HCs: 30.16 [SD 7.7]; p = 0.006), and 1.25% contrast (SPS: 16.41 [SD 10.6]; HCs: 20.84 [SD 8.6]; p = 0.034) letter acuity scores. GCIPL thicknesses correlated with the number of body regions affected in SPS (decrease of 1.25 µm [95% confidence interval, -2.2 to -0.3 µm; p = 0.008] per additional body region affected). CONCLUSIONS: Retinal neuronal pathology can occur in SPS. OCT may have utility as a biomarker of disease burden in SPS.
OBJECTIVE: To evaluate whether structural and functional changes occur in the afferent visual system of patients with stiff-person syndrome (SPS) and whether these changes correlate with disease burden, given the high concentration of γ-aminobutyric acid receptors, which are generally thought to be involved in SPS pathogenesis, in the retina. METHODS: In this single-center, cross-sectional study, patients with SPS and healthy controls (HCs) underwent optical coherence tomography (OCT), with a subset undergoing high- and low-contrast visual acuity (VA) assessments. Burden of disease was assessed via the number of body regions affected. Individuals with uncontrolled hypertension or comorbid neurologic or ophthalmologic disorders were excluded. Statistical analyses were performed using mixed-effects linear regression models. RESULTS: Thirty-five patients with SPS and 40 age- and sex-matched HCs underwent OCT. A subset of 23 patients with SPS and 28 HCs underwent VA assessments. Relative to HCs, patients with SPS had lower ganglion cell + inner plexiform layer (GCIPL) thicknesses (SPS: 74.36 µm [SD 5.7]; HCs: 76.33 µm [SD 4.2]; p = 0.005), inner nuclear layer thicknesses (SPS: 44.37 µm [SD 2.7]; HCs: 45.18 µm [SD 2.2]; p = 0.042), and 100% (SPS: 53 [SD 9.6]; HCs: 57.5 [SD 6.1]; p = 0.005), 2.5% (SPS: 24.35 [SD 10.1]; HCs: 30.16 [SD 7.7]; p = 0.006), and 1.25% contrast (SPS: 16.41 [SD 10.6]; HCs: 20.84 [SD 8.6]; p = 0.034) letter acuity scores. GCIPL thicknesses correlated with the number of body regions affected in SPS (decrease of 1.25 µm [95% confidence interval, -2.2 to -0.3 µm; p = 0.008] per additional body region affected). CONCLUSIONS: Retinal neuronal pathology can occur in SPS. OCT may have utility as a biomarker of disease burden in SPS.
Authors: Elliot M Frohman; James G Fujimoto; Teresa C Frohman; Peter A Calabresi; Gary Cutter; Laura J Balcer Journal: Nat Clin Pract Neurol Date: 2008-12
Authors: H Steffen; N Menger; W Richter; B Nölle; H Krastel; C Stayer; G H Kolling; H Wässle; H M Meinck Journal: Graefes Arch Clin Exp Ophthalmol Date: 1999-03 Impact factor: 3.117
Authors: John N Ratchford; Shiv Saidha; Elias S Sotirchos; Jiwon A Oh; Michaela A Seigo; Christopher Eckstein; Mary K Durbin; Jonathan D Oakley; Scott A Meyer; Amy Conger; Teresa C Frohman; Scott D Newsome; Laura J Balcer; Elliot M Frohman; Peter A Calabresi Journal: Neurology Date: 2013-01-01 Impact factor: 9.910
Authors: Prejaas Tewarie; Lisanne Balk; Fiona Costello; Ari Green; Roland Martin; Sven Schippling; Axel Petzold Journal: PLoS One Date: 2012-04-19 Impact factor: 3.240
Authors: Andrew Lang; Aaron Carass; Matthew Hauser; Elias S Sotirchos; Peter A Calabresi; Howard S Ying; Jerry L Prince Journal: Biomed Opt Express Date: 2013-06-14 Impact factor: 3.732