From the Research
Longitudinal Extensive Transverse Myelitis (LETM) is a severe inflammatory condition affecting three or more contiguous vertebral segments of the spinal cord, requiring prompt diagnosis and treatment to prevent permanent disability, as highlighted in a recent study 1. The significance of LETM lies in its potential to cause significant morbidity and mortality if left untreated or undertreated. LETM can be associated with various underlying conditions, including neuromyelitis optica spectrum disorder (NMOSD), multiple sclerosis, and autoimmune disorders such as Sjögren's syndrome, as discussed in studies 2, 3. Key features of LETM include:
- A spinal cord lesion extending over three or more vertebrae, as seen on MRI of the spine
- Clinical presentation of paraparesis or tetraparesis, sensory disturbances, and gait, bladder, bowel, and/or sexual dysfunction
- Variable etiologies, including autoimmune, infectious, and neoplastic disorders Treatment of LETM typically involves:
- High-dose intravenous methylprednisolone (1000 mg daily for 3-5 days), followed by an oral prednisone taper starting at 1 mg/kg/day and gradually decreasing over 2-3 months
- Plasma exchange therapy (5-7 exchanges over 10-14 days) if there's inadequate response to steroids within 3-5 days
- Long-term immunosuppression for patients with antibody-positive conditions like NMOSD, commonly with rituximab, mycophenolate mofetil, or azathioprine, as recommended in studies 4, 5. Supportive care is essential, including physical therapy, management of neurogenic bladder/bowel, and prevention of pressure ulcers and deep vein thrombosis. Identifying underlying causes of LETM is crucial for appropriate long-term management, as emphasized in a study 1.