Transverse Myelitis Diagnosis and Management

Introduction to Transverse Myelitis

A negative AQP4 antibody test does not exclude transverse myelitis, as most cases are AQP4-negative, according to the American Academy of Neurology 1
The presence of AQP4 antibodies is highly specific for neuromyelitis optica spectrum disorder (NMOSD) but is not required for a diagnosis of transverse myelitis, as stated by the National Institute of Neurological Disorders and Stroke 1

When evaluating a patient with transverse myelitis and negative AQP4 antibodies, testing for MOG-IgG antibodies using cell-based assays is recommended, as suggested by the American Academy of Neurology 2
The American College of Rheumatology recommends testing AQP4 and MOG antibodies in parallel if cost permits, or testing AQP4 first and then MOG if cost is limiting 1, 2
Only cell-based assays are acceptable for MOG testing, as ELISA has poor specificity, according to the National Institute of Neurological Disorders and Stroke 3

Longitudinally extensive spinal cord lesions (≥3 vertebral segments) suggest MOG-EM, idiopathic LETM, or seronegative NMOSD, as stated by the American Academy of Neurology 2, 4, 5
Short lesions (<2 segments) are more consistent with multiple sclerosis (MS) or other causes, according to the National Multiple Sclerosis Society 1

The absence of oligoclonal bands is seen in 87-88% of MOG-EM patients and distinguishes from MS, as reported by the American Academy of Neurology 2
Neutrophilic pleocytosis or WCC >50/μl suggests MOG-EM or NMOSD rather than MS, according to the National Institute of Neurological Disorders and Stroke 2

MOG-antibody associated disease represents a distinct disease entity from AQP4-positive NMOSD, as stated by the American Academy of Neurology 1, 4, 5
MOG-antibody associated disease commonly presents with LETM (80% at onset in pediatric cases) and is characterized by steroid-responsive disease with tendency to flare after steroid taper, according to the National Institute of Neurological Disorders and Stroke 2, 4, 5, 7

For acute episodes, high-dose intravenous corticosteroids are first-line treatment, as recommended by the American Academy of Neurology [1
For long-term management, if MOG-IgG positive, oral prednisone taper over weeks-to-months and consideration of rituximab for severe or refractory disease are recommended, as suggested by the American College of Rheumatology 3, 4