Pediatric Acute Transverse Myelitis Overview and Differential Diagnosis

Abstract

Acute transverse myelitis is a clinical syndrome affecting the spinal cord, which is characterized by acute onset of motor, sensory, and autonomic dysfunction. Approximately 20% of cases of acute transverse myelitis occur in children. This review summarizes the current published literature on acute transverse myelitis, including epidemiology, diagnostic criteria, pathogenesis, clinical presentation, clinical evaluation, and differential diagnosis. The article also summarizes the neuroimaging features, acute and chronic complications, treatments, and prognosis of acute transverse myelitis in the pediatric population. The initial evaluation centers on differentiation from other causes of myelopathy, and cases are further divided into idiopathic or disease-associated acute transverse myelitis. Correct diagnosis is important for treatment and prognosis. Treatment begins with intensive surveillance for acute life-threatening respiratory or autonomic complications. Immunomodulating therapy is recommended for noninfectious causes, using high-dose intravenous corticosteroids or plasma exchange. Other therapeutic options are also discussed. Prognosis depends on a number of factors, and evidence suggests that the majority of children have a good outcome. A small percentage of children diagnosed with acute transverse myelitis later are diagnosed with other demyelinating diseases, especially neuromyelitis optica, or multiple sclerosis. The most common long-term complications of acute transverse myelitis are urinary, motor, or sensory dysfunction.

MRI Findings in Children with Acute Flaccid Paralysis and Cranial Nerve Dysfunction Occurring during the 2014 Enterovirus D68 Outbreak

“…35 The restriction of involvement to the central gray matter in our series is not typical of the imaging or clinical findings of idiopathic acute transverse myelitis (admittedly a diagnosis of exclusion), which is typically described as central, extensive spinal cord T2 hyperintensity, swelling, and lesion enhancement. 36,37 Nerve root enhancement is also not typical of either acute disseminated encephalomyelitis or transverse myelitis. While Guillain-Barre syndrome is characterized by nerve root enhancement, cord and brain stem lesions are not typical features.…”

A case of pediatric paraparesis secondary to an idiopathic acute transverse myelitis

“…They greatly vary because of the topographical variability (the level, extension and localization) of the disease and, whenever secondary, of the pathology of the underlying cause. As so, clinical onset can be rapidly progressive or more slowly-evolving over a few weeks, and symptoms can affect all limbs, only the lower ones, with or without symmetrical impairment, and predominantly affect one of the three abovementioned motor, sensory and autonomic systems [2]. MRI is the fundamental study to carry in an emergency setting so to exclude compressive lesions.…”

“…The first line therapy is methylprednisolone for 5-7 days, followed by oral prednisolone (1 mg/kg/day) for 3-4 weeks. Nonpharmacological treatment includes intermittent bladder catheterization and physiotherapy [2,9].…”

“…Regarding the last etiological group idiopathic spontaneous dorsal spinal cord infarction is another possible unusual etiology of acute paraparesis in children. When it is possible to exclude all of these causes, ATM is then classified as idiopathic [1,2,6].…”

“…It is very rare, with an estimated incidence of 1-5 cases per million per year [1]. Of these, only 1/5 occur in children, mainly before the age of two (a bimodal incidence can be seen, with a low number of cases between two and five years) [1,2].…”