Andrew Sheridan1, Kush Kapur2, Ferne Pinard2, Fabienne Dietrich Alber3, Susana Camposano2, Mike G Pike4, Andrea Klein5,6,7, Mark P Gorman2. 1. School of Psychological Science, The University of Western Australia, Perth, Australia. 2. Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. 3. Department of Neurology/Neuropsychology, University Children's Hospital Zürich, University of Zürich, Zürich, Switzerland. 4. Children's Hospital, Oxford, UK. 5. Division of Child Neurology, University Children's Hospital Bern Inselspital, University of Bern, Bern, Switzerland. 6. Department of Pediatric Neurology, University Children's Hospital Basel, Basel, Switzerland. 7. Department of Pediatric, Neurology University Children's Hospital Lausanne, Lausanne, Switzerland.
Abstract
AIM: To determine predictors of full-scale IQ (FSIQ) in an international pediatric opsoclonus myoclonus syndrome (OMS) cohort. METHOD: In this retrospective and prospective cohort study at three academic medical centers (2006-2013), the primary outcome measure, FSIQ, was categorized based on z-score: above average (≥+1), average (+1 to -1), mildly impaired (-1 to -2), and impaired (<-2). Univariate analysis and multivariable linear regression modeling using stepwise selection with Akaike's information criterion was performed to understand the relationship between exposures and FSIQ. RESULTS: Of 81 participants, 37 with sufficient data had mean FSIQ 84.38 (SD 20.55) and median 90 (40-114) at latest available evaluation (mean age 8y 5mo). Twenty (54%), nine (24.3%), and eight (21.6%) had normal, mildly impaired, and impaired FSIQ respectively. The final multivariable linear regression model included 34 participants with evaluable data: number of relapses occurring before neuropsychological testing (p<0.001) and OMS severity score at last follow-up (p<0.001) predicted FSIQ (adjusted R2 =0.64). There was a mean decrease of 2.4 FSIQ points per OMS relapse. INTERPRETATION: Number of relapses negatively correlates with FSIQ in pediatric OMS. Demographic and clinical measures available at OMS onset did not predict FSIQ. Strategies to reduce OMS relapses may improve intellectual outcomes.
AIM: To determine predictors of full-scale IQ (FSIQ) in an international pediatric opsoclonus myoclonus syndrome (OMS) cohort. METHOD: In this retrospective and prospective cohort study at three academic medical centers (2006-2013), the primary outcome measure, FSIQ, was categorized based on z-score: above average (≥+1), average (+1 to -1), mildly impaired (-1 to -2), and impaired (<-2). Univariate analysis and multivariable linear regression modeling using stepwise selection with Akaike's information criterion was performed to understand the relationship between exposures and FSIQ. RESULTS: Of 81 participants, 37 with sufficient data had mean FSIQ 84.38 (SD 20.55) and median 90 (40-114) at latest available evaluation (mean age 8y 5mo). Twenty (54%), nine (24.3%), and eight (21.6%) had normal, mildly impaired, and impaired FSIQ respectively. The final multivariable linear regression model included 34 participants with evaluable data: number of relapses occurring before neuropsychological testing (p<0.001) and OMS severity score at last follow-up (p<0.001) predicted FSIQ (adjusted R2 =0.64). There was a mean decrease of 2.4 FSIQ points per OMS relapse. INTERPRETATION: Number of relapses negatively correlates with FSIQ in pediatric OMS. Demographic and clinical measures available at OMS onset did not predict FSIQ. Strategies to reduce OMS relapses may improve intellectual outcomes.
Authors: Thomas Rossor; E Ann Yeh; Yasmin Khakoo; Paola Angelini; Cheryl Hemingway; Sarosh R Irani; Gudrun Schleiermacher; Paramala Santosh; Tim Lotze; Russell C Dale; Kumaran Deiva; Barbara Hero; Andrea Klein; Pedro de Alarcon; Mark P Gorman; Wendy G Mitchell; Ming Lim Journal: Neurol Neuroimmunol Neuroinflamm Date: 2022-03-08