Myelitis and Other Autoimmune Myelopathies

Sebastian Lopez Chiriboga, MD; Eoin P. Flanagan, MBBCh Spinal Cord Disorders p. 62-92 February 2021, Vol.27, No.1 doi: 10.1212/CON.0000000000000900
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PURPOSE OF REVIEW This article provides an update on the clinical diagnosis and management of immune-mediated myelopathies, including the relevance of imaging, ancillary testing with an emphasis on autoantibody biomarkers, recognition of myelitis mimics, and therapeutic approach.

RECENT FINDINGS The imaging characterization of immune-mediated myelopathies and the discovery of neural autoantibodies have been crucial in improving our ability to accurately diagnose myelitis. The identification of autoantibodies directed against specific central nervous system targets has led to major improvements in our understanding of the mechanisms underlying inflammation in myelitis. It has also allowed distinction of these myelopathy etiologies from noninflammatory etiologies of myelopathy and from multiple sclerosis and provided insight into their risk of recurrence, treatment response, and long-term clinical outcomes. Prompt recognition and appropriate testing in the setting of acute and subacute myelopathies is critical as timely administration of immunotherapy can help improve symptoms and prevent permanent neurologic disability. A patient should not be classified as having “idiopathic transverse myelitis” without a comprehensive evaluation for a more specific etiology. Achieving the correct diagnosis and learning to recognize noninflammatory myelitis mimics is crucial as they have therapeutic and prognostic implications.

SUMMARY Identifying the clinical and radiographic features of immune-mediated myelitis and recognizing mimics and pitfalls will help clinicians treat confirmed autoimmune myelitis appropriately.

Address correspondence to Dr Eoin P. Flanagan, 200 First Street SW, Rochester, Minnesota 55905, [email protected].

RELATIONSHIP DISCLOSURE: Dr Lopez Chiriboga reports no disclosure. Dr Flanagan has received research/grant support from MedImmune/VielaBio.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Lopez Chiriboga and Flanagan discuss the unlabeled/investigational use of azathioprine, corticosteroids, cyclophosphamide, IV immunoglobulin, methotrexate, mycophenolate mofetil, plasma exchange, and rituximab for the treatment of myelitis and other autoimmune myelopathies.

© 2021 American Academy of Neurology.