This review defines current clinical criteria for diagnosis, differential diagnosis, and clinical evaluation of acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica, and summarizes principles of treatment.
Consensus criteria for transverse myelitis and acute disseminated encephalomyelitis have been proposed. A specific biomarker, aquaporin-4 autoantibody, has been discovered for neuromyelitis optica that allows for early and accurate diagnosis even in the absence of cardinal findings of optic neuritis and myelitis. The antibody is pathogenic and is facilitating an understanding of the pathophysiology of neuromyelitis optica and development of antigen-specific treatments.
Clinical and radiologic findings combined with serologic findings may permit classification of syndromes of transverse myelitis and acute disseminated encephalomyelitis in ways that may predict risk of relapse, type of relapse, and prognosis. Treatment, especially to prevent relapse, is dependent on the specific disease context in which syndromes such as transverse myelitis occur.
Address correspondence to Dr Brian G. Weinshenker, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, firstname.lastname@example.org.
Relationship Disclosure: Dr Wingerchuk receives research support from Alexion, Genentech, Genzyme Corporation, Guthy-Jackson Charitable Foundation, and Terumo BCT, Inc. Dr Weinshenker serves on the data and safety monitoring board of Biogen Idec and Novartis and serves as a consultant regarding neuromyelitis optica therapeutics for Asahi Kasei Medical Co, Ltd, Elan Corporation, and Novartis. Dr Weinshenker receives royalties for licensed technology for the diagnosis of neuromyelitis optica from RSR Limited, and receives research funding from Guthy-Jackson Charitable Foundation.
Unlabeled Use of Products/Investigational Use Disclosure: Drs Wingerchuk and Weinshenker discuss the unlabeled uses of corticosteroids and plasma exchange for the treatment of acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica; IV immunoglobulin for acute disseminated encephalomyelitis; and azathioprine, mycophenolate mofetil, rituximab, mitoxantrone, methotrexate, and eculizumab for neuromyelitis optica.