Current perspectives on the diagnosis and management of acute transverse myelitis

ABSTRACT

Introduction

Acute transverse myelitis (ATM) is a term that encompasses a wide range of etiologies from immune-mediated to infectious. Management and prognosis differ for each specific etiology, and thus determining the disease-specific diagnosis of ATM is crucial.

Areas covered

The distinguishing clinical, radiologic, serologic, and cerebrospinal fluid features for common etiologies of ATM, such as multiple sclerosis, aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and spinal cord sarcoidosis, are covered. Acute flaccid myelitis variant of ATM is also explored. Red flags suggesting ATM mimics are briefly reviewed. Management of ATM in this review mainly focuses on treatment for immune-mediated causes and is divided into acute treatment, preventive treatment for certain etiologies, and supportive management. Although maintenance attack-prevention treatment for immune-mediated ATM is mainly guided by observational studies and expert opinion, clinical trials have been completed in AQP4+NMOSD and are underway in MOGAD to help provide solid evidence for treatment efficacy.

Expert opinion

The term ATM should be replaced by a disease-specific diagnosis to direct management. Discovery of disease-associated antibodies has changed the landscape of ATM diagnosis and allowed research on disease mechanisms. Translating our knowledge on pathophysiology into targeted therapy with monoclonal antibodies has provided new treatment options for patients.

KEYWORDS:

• AQP4
• autoimmune neurology
• biologics
• MOGAD
• monoclonal antibody
• myelopathy
• neuromyelitis optica
• sarcoidosis
• transverse myelitis
• treatment

Article highlights

• Acute transverse myelitis (ATM) should be replaced by a disease-specific diagnosis wherever possible

• Clinical, radiologic, serologic, and cerebrospinal fluid features can help distinguish specific etiologies and reveal red flags that suggest ATM mimics

• Immune-mediated ATM requires timely acute treatment with immunotherapy

• Myelitis in AQP4-IgG-positive NMOSD and some other immune-mediated myelopathies require maintenance immunotherapy for relapse prevention

• Currently, there are three FDA-approved biologics for AQP4-IgG-positive NMOSD, and clinical trials for MOGAD are underway

Declaration of Interest

EP Flanagan served on advisory boards for Alexion, Genentech, and Horizon Therapeutics. He has received speaker honoraria from Pharmacy Times and royalties from UpToDate for a topic on MOGAD. He was a site primary investigator in a randomized clinical trial on inebilizumab in neuromyelitis optica spectrum disorder run by Horizon Therapeutics. He is also the principal investigator on an RO1 Research Project on MOG-IgG disease.

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This manuscript has not been funded.