Pediatric transverse myelitis

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. Neurology ® 2016;87 (Suppl 2):S46-S52 GLOSSARY ADS 5 acquired demyelinating syndrome; AFM 5 acute flaccid myelitis; AQP4 5 aquaporin-4; ASIA 5 American Spinal Injury Association; ATM 5 acute transverse myelitis; GBS 5 Guillain-Barré syndrome; IL 5 interleukin; IVIg 5 IV immunoglobulin; LETM 5 longitudinally extensive TM; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; PLEX 5 plasmapheresis; SLE 5 systemic lupus erythematosus; TMCWG 5 Transverse Myelitis Consortium Working Group.Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder classically described as demyelinating. ATM comprises a subgroup of the noncompressive transverse myelopathies.1,2 It is a potentially devastating condition with variable outcome. 3 ATM must be differentiated from other, rarer presentations of noncompressive myelopathy.4,5 ATM may be the first presentation of relapsing acquired demyelinating syndromes (ADS) such as neuromyelitis optica (NMO) or multiple sclerosis (MS). A PubMed search using the terms "pediatric or paediatric transverse myelitis" revealed that more than 200 articles have been published in English on the topic between 1976 and 2015. In this article we discuss current knowledge on clinical features, pathogenesis, and investigative and management ...

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