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Published on: May 18, 2010
Pediatric transverse myelitis
Michael Absoud1, Benjamin M Greenberg2, Ming Lim2
1From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France. michael.absoud@gstt.nhs.uk.
Insights
Pediatric acute transverse myelitis (ATM) is an immune disorder affecting the CNS in children. While outcomes are generally better than in adults, more research is needed for optimal treatment and standardized care.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Pediatric acute transverse myelitis (ATM) is an immune-mediated central nervous system (CNS) disorder.
- It accounts for 20% of acquired demyelinating syndromes (ADS) in children.
- ATM requires differentiation from other myelopathies and can be an initial presentation of relapsing ADS like NMO or MS.
Purpose of the Study:
- To review current knowledge on clinical features, diagnostics, pathogenesis, and management of pediatric ATM.
- To highlight the need for standardized protocols for clinical assessment and investigation.
- To suggest future research directions for understanding prognostic factors and optimizing treatment.
Main Methods:
- Review of existing literature on pediatric acute transverse myelitis.
- Analysis of diagnostic criteria and their application in children.
- Examination of MRI findings, including lesion characteristics and location.
- Discussion of the role of antibodies like MOG in ATM pathogenesis and treatment.
Main Results:
- Diagnostic criteria for ATM can be applied to children, though sensory levels may be less evident.
- MRI often shows centrally located lesions involving gray and white matter, with longitudinally extensive ATM being common.
- Asymptomatic brain MRI lesions are present in over one-third of cases and predict MS or NMO.
- Children generally experience better outcomes than adults, with 50% recovering fully within 2 years.
Conclusions:
- Pediatric ATM is a significant condition with variable outcomes and substantial healthcare demands.
- Further research, including standardized international collaborative studies, is essential to identify prognostic factors for disability and relapse.
- There is a lack of robust controlled trials to guide optimal treatment strategies for ATM in both children and adults.
Abstract:
Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be differentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the Transverse Myelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some. MRI lesions are often centrally located with high T2 signal intensity involving gray and neighboring white matter. Longitudinally extensive ATM occurs in the majority. Asymptomatic lesions on brain MRI are seen in more than one-third and predict MS or NMO. The role of antibodies such as myelin oligodendrocyte glycoprotein in monophasic and relapsing ATM and their significance in therapeutic approaches remain unclear. ATM is a potentially devastating condition with variable outcome and presents significant cumulative demands on health and social care resources. Children generally have a better outcome than adults, with one-half making a complete recovery by 2 years. There is need for standardization of clinical assessment and investigation protocols to enable international collaborative studies to delineate prognostic factors for disability and relapse. There are no robust controlled trials in children or adults to inform optimal treatment of ATM, with one study currently open to recruitment. This review provides an overview of current knowledge of clinical features, investigative workup, pathogenesis, and management of ATM and suggests future directions.
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