Post-infectious Acute Transverse Myelitis

Abstract

Acute transverse myelitis (TM) is a disease of the spinal cord defined by (1) sensory, motor, or autonomic dysfunction attributable to the spinal cord; (2) bilateral signs or symptoms; (3) clearly defined sensory level; (4) no evidence of compressive cord lesion; (5) inflammation defined by CSF pleocytosis, CSF elevated IgG index, and/or gadolinium enhancement on MRI; and (6) progression to symptom nadir between 4 h and 21 days. In up to 50% of cases of idiopathic cases, an ongoing or preceding systemic infection is documented, but its causal role in the pathophysiology of this disease is unclear. Post-infectious acute TM is thought to be an autoimmune process triggered at least in part by post-infectious inflammation. Urgent neuroimaging followed by comprehensive evaluation with lumbar puncture and autoimmune serology is required to confirm an inflammatory etiology and to rule out mimics such as infection, infarction, vascular malformation, tumor, or disease-related TM (from a systemic autoimmune disorder or an acquired demyelinating disease). After evidence for an inflammatory etiology is gathered, treatment should be initiated first with high-dose intravenous corticosteroids. Should initial presentation be severe or should patients not significantly improve with corticosteroids, plasma exchange, IVIg, or pulse cyclophosphamide may be considered. Other important aspects of treatment include symptomatic management, physical therapy, and patient/family psychosocial support. Prognosis for children with post-infectious acute TM is quite variable. While the majority of children do have full or good recovery, many are left with residual neurologic deficits including persistent ambulatory difficulties, sensory disturbances, and/or abnormal bladder function.