Brain Volumes in Opsoclonus-Myoclonus Ataxia Syndrome: A Longitudinal Study
Montaha Almudhry1, Matthias W Wagner2,3, Giulia Longoni1,4
1Program in Neurosciences and Mental Health, SickKids Research Institute, The Hospital for Sick Children, Canada.
Insights
Children with opsoclonus-myoclonus ataxia syndrome show altered brain growth trajectories. This longitudinal study reveals significant differences in brain maturation compared to healthy children.
Area of Science:
- Pediatric Neurology
- Neurodevelopmental Disorders
- Neuroimaging
Background:
- Opsoclonus-myoclonus ataxia syndrome (OMAS) is a rare autoimmune disorder affecting children.
- Longitudinal brain development patterns in OMAS are not well understood.
Purpose of the Study:
- To investigate the longitudinal brain growth trajectories in pediatric OMAS patients.
- To compare brain volumes and maturation in OMAS patients versus healthy controls.
Main Methods:
- Longitudinal case-control study utilizing brain MRI scans from 2009-2020.
- Automatic brain volumetry performed using FreeSurfer software.
- Statistical analysis of growth trajectory curvature using paired t-tests with Bonferroni correction.
Main Results:
- 14 OMAS patients and 474 healthy controls were analyzed.
- Significant differences in growth trajectory curvature were observed in OMAS patients for cerebral white matter, cerebral gray matter, cerebellar white matter, cerebellar gray matter, and brainstem.
- P-values ranged from <.001 to .049, indicating significant deviations in brain maturation.
Conclusions:
- Children with OMAS exhibit abnormal brain maturation.
- Deviations are noted in supratentorial brain regions, brainstem, and cerebellum.
- Findings highlight the impact of OMAS on neurodevelopment.
Introduction:
Little is known about the longitudinal trajectory of brain growth in children with opsoclonus-myoclonus ataxia syndrome. We performed a longitudinal evaluation of brain volumes in pediatric opsoclonus-myoclonus ataxia syndrome patients compared with age- and sex-matched healthy children.
Patients And Methods:
This longitudinal case-control study included brain magnetic resonance imaging (MRI) scans from consecutive pediatric opsoclonus-myoclonus ataxia syndrome patients (2009-2020) and age- and sex-matched healthy control children. FreeSurfer analysis provided automatic volumetry of the brain. Paired t tests were performed on the curvature of growth trajectories, with Bonferroni correction.
Results:
A total of 14 opsoclonus-myoclonus ataxia syndrome patients (12 female) and 474 healthy control children (406 female) were included. Curvature of the growth trajectories of the cerebral white and gray matter, cerebellar white and gray matter, and brainstem differed significantly between opsoclonus-myoclonus ataxia syndrome patients and healthy control children (cerebral white matter, P = .01; cerebral gray matter, P = .01; cerebellar white matter, P < .001; cerebellar gray matter, P = .049; brainstem, P < .01).
Discussion/Conclusion:
We found abnormal brain maturation in the supratentorial brain, brainstem, and cerebellum in children with opsoclonus-myoclonus ataxia syndrome.
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