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OBJECTIVES: To explore the challenges in diagnosing acute flaccid myelitis (AFM) and evaluate clinical features and treatment paradigms associated with under recognition. STUDY DESIGN: This was a retrospective multicenter study of pediatric patients (≤18 years) who were diagnosed with AFM from 2014 to 2018 using the Centers for Disease Control and Prevention's case definition. RESULTS: In 72% of the cases (126 of 175), AFM was not considered in the initial differential diagnosis (n = 108; 61.7%) and/or the patient was not referred for acute care (n = 90; 51.4%) at the initial clinical encounter, and this did not improve over time. Although many features of the presentation were similar in those initially diagnosed with AFM and those who were not; preceding illness, constipation, and reflexes differed significantly between the 2 groups. Patients with a non-AFM initial diagnosis more often required ventilatory support (26.2% vs 12.2%; OR, 0.4; 95% CI, 0.2-1.0; P = .05). These patients received immunomodulatory treatment later (3 days vs 2 days after neurologic symptom onset; 95% CI, -2 to 0; P = .05), particularly intravenous immunoglobulin (5 days vs 2 days; 95% CI, -4 to -2; P < .001). CONCLUSIONS: Delayed recognition of AFM is concerning because of the risk for respiratory decompensation and need for intensive care monitoring. A non-AFM initial diagnosis was associated with delayed treatment that could have a clinical impact, particularly as new treatment options emerge.
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Viroses do Sistema Nervoso Central , Infecções por Enterovirus , Mielite , Doenças Neuromusculares , Criança , Humanos , Mielite/diagnóstico , Mielite/terapia , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/terapia , Viroses do Sistema Nervoso Central/diagnóstico , Viroses do Sistema Nervoso Central/terapia , Estudos Retrospectivos , Infecções por Enterovirus/diagnóstico , Infecções por Enterovirus/terapiaRESUMO
Anti-myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies are associated clinically with either a monophasic or relapsing disease course. We investigated the frequency and clinical importance of acquired asymptomatic brain magnetic resonance imaging (MRI) lesions in a prospective incident cohort of 74 MOG-IgG positive children with serial MRI scans over a median of 5 years from presentation. Silent new lesions were detected in 14% of MOG-IgG positive participants, most commonly within the first months post-onset, with a positive predictive value for clinically relapsing disease of only 20%. Detection of asymptomatic lesions alone need not prompt initiation of chronic immunotherapy. ANN NEUROL 2021;89:408-413.
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Doenças Assintomáticas , Autoanticorpos/imunologia , Encéfalo/diagnóstico por imagem , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/diagnóstico por imagem , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Esclerose Múltipla/diagnóstico por imagem , Glicoproteína Mielina-Oligodendrócito/imunologia , Adolescente , Encéfalo/fisiopatologia , Criança , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/imunologia , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/fisiopatologia , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/terapia , Encefalomielite Aguda Disseminada/imunologia , Encefalomielite Aguda Disseminada/fisiopatologia , Encefalomielite Aguda Disseminada/terapia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulina G , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Imunoterapia , Imageamento por Ressonância Magnética , Masculino , Esclerose Múltipla/imunologia , Esclerose Múltipla/fisiopatologia , Esclerose Múltipla/terapia , Bandas Oligoclonais/líquido cefalorraquidiano , Troca Plasmática , RecidivaRESUMO
Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory demyelinating disease of the central nervous system (CNS) primarily affecting the optic nerves and spinal cord, but also involving other regions of the CNS including the area postrema, periaqueductal gray matter, and hypothalamus. Knowledge related to pediatric manifestations of NMOSD has grown in recent years, particularly in light of newer information regarding the importance of not only antibodies to aquaporin 4 (AQP4-IgG) but also myelin oligodendrocyte glycoprotein (MOG-IgG) in children manifesting clinically with this syndrome. In this review, we describe the current state of the knowledge related to clinical manifestations, diagnosis, and chronic therapies for children with NMOSD, with emphasis on literature that has been published in the last 5 years. Following the review, we propose recommendations for the assessment/follow up clinical care, and treatment of this population.
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[This corrects the article DOI: 10.3389/fped.2020.00339.].
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OBJECTIVES: To examine the longitudinal relationship between physical activity and fatigue and depression among youth with demyelinating conditions. STUDY DESIGN: From September 2013 to March 2017, we performed a longitudinal study of consecutive youth diagnosed at their first visit with pediatric onset multiple sclerosis (POMS) or monophasic acquired demyelinating syndromes (mono-ADS) at a neuroinflammatory disorders clinic in a tertiary children's hospital. Fatigue was determined at each visit by the Pediatric Quality of Life Multidimensional Fatigue Scale, depressive symptoms by the Center of Epidemiologic Studies Depression Children Rating Scale, and physical activity level by the Godin Leisure Time Exercise Questionnaire. Mixed linear models were used to examine the associations of moderate-to-vigorous physical activity (MVPA) with fatigue and depression over time, adjusting for age, time from incident demyelination, sex, number of relapses, relapse within 30 days, and disability. RESULTS: In 182 patients (48 POMS, age 15 ± 1.7 years, 35 female; and 134 mono-ADS, age 12 ± 3.6 years 67 female) with 538 visits (mean follow-up 3.6 ± 2.7 years and 4.2 ± 3.3 years, respectively), a trajectory of increased fatigue over time was observed in POMS (2.28 points/year, P = .008) and mono-ADS (1.33 points/year, P = .007) patients. Youth with POMS had more depressive symptoms (estimate = 11.4 points, P < .002) than mono-ADS. Depressive symptoms increased over time in female patients with POMS (estimate = 1.4 points/year, P < .02). MVPA was associated with lower depression (-0.09, P < .001) and general fatigue (0.13, P = .02) over time in POMS. CONCLUSIONS: Youth with POMS who have higher levels of MVPA demonstrate lesser depressive symptoms and lower fatigue over time. Our results may inform future interventions to manage mood and fatigue in POMS.
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Depressão/etiologia , Depressão/prevenção & controle , Exercício Físico , Fadiga/etiologia , Fadiga/prevenção & controle , Esclerose Múltipla/complicações , Adolescente , Criança , Feminino , Humanos , Estudos Longitudinais , MasculinoRESUMO
OBJECTIVE: Central nervous system pathology in multiple sclerosis includes both focal inflammatory perivascular injury and injury to superficial structures, including the subpial region of the cortex, which reportedly exhibits a gradient of damage from the surface inward. We assessed how early in the multiple sclerosis course a "surface-in" process of injury suggesting progressive biology may begin. METHODS: We focused on the thalamus, which notably has both a cerebrospinal fluid (CSF) interface and a white matter interface. Thalamic volume trajectories were assessed in a prospectively followed cohort of children from initial presentation with either multiple sclerosis or monophasic acquired demyelination, and healthy controls. Voxelwise volume changes were calculated using deformation-based morphometry, and analyzed in relation to distance from the CSF interface by mixed effects modeling and semiparametric smoothing methods. RESULTS: Twenty-seven children with multiple sclerosis and 73 children with monophasic demyelination were prospectively followed with yearly brain scans (mean follow-up = 4.6 years, standard deviation = 1.9). A total of 282 healthy children with serial scans were included as controls. Relative to healthy controls, children with multiple sclerosis and children with monophasic demyelination demonstrated volume loss in thalamic regions adjacent to the white matter. However, only children with multiple sclerosis exhibited an additional surface-in gradient of thalamic injury on the ventricular side, which was already notable in the first year of clinical disease (asymptote estimate = 3.01, 95% confidence interval [CI] = 1.44-4.58, p = 0.0002) and worsened over time (asymptote:time estimate = 0.33, 95% CI = 0.12-0.54, p = 0.0021). INTERPRETATION: Our results suggest that a multiple sclerosis disease-specific surface-in process of damage can manifest at the earliest stages of the disease. ANN NEUROL 2019;85:340-351.
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Esclerose Múltipla/diagnóstico por imagem , Tálamo/diagnóstico por imagem , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/diagnóstico por imagem , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/patologia , Progressão da Doença , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla/patologia , Tamanho do Órgão , Tálamo/patologiaRESUMO
OBJECTIVES: To investigate physical activity levels in youth with multiple sclerosis and monophasic acquired demyelinating syndromes ([mono-ADS], ie, children without relapsing disease) compared with healthy controls and to determine factors that contribute to engagement in physical activity. We hypothesized that greater physical activity goal setting and physical activity self-efficacy would be associated with greater levels of vigorous physical activity in youth with multiple sclerosis. STUDY DESIGN: A total of 68 consecutive patients (27 multiple sclerosis, 41 mono-ADS) and 37 healthy controls completed fatigue, depression, Physical Activity Self-Efficacy Scale, perceived disability, Exercise Goal-Setting scale, and physical activity questionnaires, and wore an accelerometer for 7 days. All patients had no ambulatory limitations (Expanded Disability Status Scale, scores all <4). RESULTS: Youth with multiple sclerosis engaged in fewer minutes per day of vigorous (P = .009) and moderate and vigorous physical activity (P = .048) than did patients with mono-ADS and healthy controls. A lower proportion of the group with multiple sclerosis (63%) reported participating in any strenuous physical activity than the mono-ADS (85%) and healthy control (89%) groups (P = .020). When we adjusted for age and sex, the Physical Activity Self-Efficacy Scale and Exercise Goal-Setting scale were associated positively with vigorous physical activity in the group with multiple sclerosis. Fatigue and depression did not predict physical activity or accelerometry metrics. CONCLUSIONS: Youth with multiple sclerosis participate in less physical activity than their counterparts with mono-ADS and healthy controls. Physical activity self-efficacy and exercise goal setting serve as potentially modifiable correlates of physical activity, and are measures suited to future interventions aimed to increase physical activity in youth with multiple sclerosis.