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Objectives To determine to what extent acute demyelinating episodes versus chronic degenerative phenomena drive retinal neuroaxonal damage in pediatric acquired demyelinating syndromes (ADS). Methods We acquired optical coherence tomography (OCT) data (follow-up range: 2 weeks - 5 years, at variable intervals from presentation) in pediatric participants who had multiple sclerosis (MS), monophasic ADS, or were healthy. Multivariable mixed effects models were used to assess the association of the number of demyelinating episodes (either optic neuritis [ON], or non-ON relapses) with changes in retinal nerve fiber layer (RNFL) or ganglion cell layer-inner plexiform layer (GCIPL) thickness. Results 64 OCT sans from 23 MS, and 33 scans from 12 monophasic ADS participants were compared with 68 scans from 62 healthy participants. The first ON episode had the biggest impact on RNFL or GCIPL thickness in monophasic ADS (RNFL: -7.9 µm, CI=5.5, p = 0.0056; GCIPL: -8.4 µm, CI=4.4, p = 0.0002) and MS (RNFL: -16 µm, CI = 3.7, p < 10-6; GCIPL: -15 µm, CI = 2.6, p < 10-6). Non-ON relapses were also associated with small but significant retinal thickness reductions in MS (RNFL: -2.6 µm/relapse, CI = 1.4, p = 0.0003; GCIPL: -2.8 µm/relapse, CI = 0.89, p < 10-6). MS participants showed progressive GCIPL thinning independent of acute demyelinating episodes (-2.7 µm/year, CI = 1.9, p = 0.0058). Conclusions We showed a prominent impact of early ON episodes on OCT measures of neuroaxonal structure in patients with ADS. We also demonstrated negative effects of non-ON relapses, and the presence of chronic retinal neurodegenerative changes, in youth with MS.
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Esclerosis Múltiple , Neuritis Óptica , Enfermedades de la Retina , Adolescente , Niño , Humanos , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/diagnóstico por imagen , Fibras Nerviosas , Neuritis Óptica/complicaciones , Neuritis Óptica/diagnóstico por imagen , Recurrencia , Retina/diagnóstico por imagen , Células Ganglionares de la Retina , Tomografía de Coherencia Óptica/métodosRESUMEN
Background: This study quantified and compared weekday and weekend patterns of device-measured physical activity (PA) and sedentary behavior between youth with pediatric multiple sclerosis (MS) and controls for the purpose of informing future PA behavior change interventions. Methods: Participant data were obtained from 3 ongoing observational studies, and the sample included 40 participants with pediatric MS and 41 controls. Light PA (LPA), moderate to vigorous PA (MVPA), and sedentary behavior data were collected using activity monitors (ActiGraph LLC) over 1 week. The main analysis involved a 2-way mixed factor analysis of variance with group as a between-subjects factor (pediatric MS vs control) and day as a within-subjects factor (weekday vs weekend day). Results: There was no group by day interaction from the analysis of variance for percentage of activity monitor wear time spent in LPA, MVPA, or sedentary behavior. There was no effect of group for LPA, MVPA, or sedentary behavior. There was an effect of day of week on percentage of day spent in LPA, MVPA, and sedentary behavior. Conclusions: These results suggest that youth with pediatric MS and controls were less physically active and more sedentary on weekends than on weekdays, but there were no differences between groups in PA and sedentary behavior overall or by day of the week. Physical activity interventions may be more successful by initially targeting weekend day activity.
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BACKGROUND: Adherence to disease-modifying therapies (DMTs) in pediatric multiple sclerosis (MS) is not well understood. We examined the prevalence and risk factors for poor adherence in pediatric MS. METHODS: This cross-sectional study recruited youth with MS from 12 North American pediatric MS clinics. In addition to pharmacy-refill data, patients and parents completed self-report measures of adherence and quality of life. Additionally, patients completed measures of self-efficacy and well-being. Factor analysis and linear regression methods were used. RESULTS: A total of 66 youth (mean age, 15.7 years) received MS DMTs (33% oral, 66% injectable). Estimates of poor adherence (i.e. missing >20% of doses) varied by source: pharmacy 7%, parent 14%, and patient 41%. Factor analysis yielded two composites: adherence summary and parental involvement in adherence. Regressions revealed that patients with better self-reported physical functioning were more adherent. Parents were more likely to be involved in adherence when their child had worse parent-reported PedsQL School Functioning and lower MS Self-Efficacy Control. Oral DMTs were associated with lesser parental involvement in adherence. CONCLUSION: Rates of non-adherence varied by information source. Better self-reported physical functioning was the strongest predictor of adherence. Parental involvement in adherence was associated with worse PedsQL School Functioning and lower MS Self-Efficacy-measured confidence in controlling MS.
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Factores Inmunológicos/administración & dosificación , Cumplimiento de la Medicación/estadística & datos numéricos , Esclerosis Múltiple/tratamiento farmacológico , Adolescente , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Esclerosis Múltiple/psicología , América del Norte , Padres , Factores de Riesgo , Autoeficacia , AutoinformeRESUMEN
OBJECTIVES: The aim of this study was to describe cognitive, academic, and psychosocial outcomes after an incident demyelinating event (acquired demyelinating syndromes, ADS) in childhood and to investigate the contribution of brain lesions and confirmed MS diagnosis on outcome. METHODS: Thirty-six patients with ADS (mean age=12.2 years, SD=2.7, range: 7-16 years) underwent brain MRI scans at presentation and at 6-months follow-up. T2-weighted lesions on MRI were assessed using a binary classification. At 6-months follow-up, patients underwent neuropsychological evaluation and were compared with 42 healthy controls. RESULTS: Cognitive, academic, and behavioral outcomes did not differ between the patients with ADS and controls. Three of 36 patients (8.3%) were identified with cognitive impairment, as determined by performance falling ≤1.5 SD below normative values on more than four independent tests in the battery. Poor performance on a visuomotor integration task was most common, observed among 6/32 patients, but this did not differ significantly from controls. Twelve of 36 patients received a diagnosis of MS within 3 years post-ADS. Patients with MS did not differ from children with monophasic ADS in terms of cognitive performance at the 6-months follow-up. Fatigue symptoms were reported in 50% of patients, irrespective of MS diagnosis. Presence of brain lesions at onset and 6 months post-incident demyelinating event did not associate with cognitive outcome. CONCLUSIONS: Children with ADS experience a favorable short-term neurocognitive outcome, even those confirmed to have MS. Longitudinal evaluations of children with monophasic ADS and MS are required to determine the possibility of late-emerging sequelae and their time course. (JINS, 2016, 22, 1050-1060).
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Disfunción Cognitiva/diagnóstico , Enfermedades Desmielinizantes/diagnóstico , Esclerosis Múltiple/diagnóstico , Adolescente , Niño , Disfunción Cognitiva/diagnóstico por imagen , Disfunción Cognitiva/etiología , Disfunción Cognitiva/fisiopatología , Enfermedades Desmielinizantes/complicaciones , Enfermedades Desmielinizantes/diagnóstico por imagen , Enfermedades Desmielinizantes/fisiopatología , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/diagnóstico por imagen , Esclerosis Múltiple/fisiopatologíaRESUMEN
OBJECTIVE: We evaluated the relationship of optical coherence tomography (OCT)-measured ganglion cell layer (GCL) and retinal nerve fiber layer (RNFL) thickness to other functional measures of afferent visual pathway competence including high-contrast visual acuity (HCVA) and low-contrast visual acuity (LCVA), visual field sensitivity, and color vision perception in a pediatric population with demyelinating disorders. METHODS: This was a cross-sectional evaluation of 37 children, aged 8-18 years, with pediatric demyelinating disorders (n = 74 eyes), and 18 healthy controls (n = 36 eyes), who were recruited from the University of Toronto, Hospital for Sick Children and the University of Calgary, Alberta Children's Hospital, Canada. A standardized visual battery, including spectral-domain OCT, visual fields, LCVA, and HCVA, was performed in all subjects. RESULTS: Mean RNFL thickness was 26 µm (25.6%) lower in patients with demyelination (76.2 µm [3.7]) compared to controls (102.4 µm [2.1]) (p < 0.0001). Mean GCL thickness was 20% lower in patients as compared to controls (p < 0.0001). Mean GCL and RNFL thickness were strongly correlated (r = 0.89; p < 0.0001), yet in contrast to RNFL thickness, no differences in GCL thickness were noted between optic neuritis (ON) eyes and non-ON eyes of patients. HCVA and LCVA and visual field mean deviation scores decreased linearly with lower RNFL thickness. CONCLUSION: GCL thickness was decreased in patients regardless of history of ON. The retina may be a site of primary neuronal injury in pediatric demyelination.