From the Research
The initial treatment for unilateral neurological deficits due to Acute Transverse Myelitis (ATM) is high-dose intravenous corticosteroids, typically methylprednisolone 1000 mg daily for 3-5 days. This should be initiated as soon as possible after diagnosis, ideally within the first few hours to days of symptom onset. Following the IV course, patients are typically transitioned to oral prednisone (1 mg/kg/day) with a gradual taper over 2-4 weeks. If there is no clinical improvement after 3-5 days of IV steroids, plasma exchange therapy (PLEX) should be considered as second-line treatment, usually administered as 5-7 exchanges over 10-14 days. Corticosteroids work by reducing inflammation and edema in the spinal cord, which helps limit the immune-mediated damage to myelin and axons. This rapid anti-inflammatory action is crucial in ATM as the condition can progress quickly, potentially leading to permanent neurological damage. Supportive care is also essential, including physical therapy, pain management, and addressing complications such as urinary retention or constipation. Early rehabilitation should be initiated to maximize functional recovery.
The use of corticosteroids as the initial treatment is supported by the fact that ATM is an immune-mediated disorder, and reducing inflammation is key to limiting damage to the spinal cord 1. While other treatments such as plasma exchange therapy may be considered in cases where there is no improvement with corticosteroids, the evidence suggests that corticosteroids should be the first line of treatment 2, 3. Additionally, supportive care and early rehabilitation are crucial in maximizing functional recovery and improving outcomes for patients with ATM.
Some studies have investigated the use of other treatments, such as intravenous immunoglobulin (IVIG) and cyclophosphamide, but the evidence is limited, and these treatments are not currently recommended as first-line therapy 4, 5. The most recent and highest quality study on this topic is from 2021, which reported on the use of plasma exchange therapy in patients with severe attacks of neuromyelitis optica spectrum disorder (NMOSD) and long extensive transverse myelitis (LETM) 3. However, this study did not investigate the use of corticosteroids as initial treatment, and therefore, does not change the current recommendation.
In terms of predictive factors for good outcomes, a baseline Expanded Disability Status Scale (EDSS) score ≤6 before the attack was associated with significant improvement at 6 months 3. However, this does not change the initial treatment recommendation, and all patients with unilateral neurological deficits due to ATM should be treated with high-dose intravenous corticosteroids as soon as possible after diagnosis.
Key points to consider in the treatment of ATM include:
- Initiation of high-dose intravenous corticosteroids as soon as possible after diagnosis
- Transition to oral prednisone with a gradual taper over 2-4 weeks
- Consideration of plasma exchange therapy as second-line treatment in cases where there is no improvement with corticosteroids
- Supportive care, including physical therapy, pain management, and addressing complications such as urinary retention or constipation
- Early rehabilitation to maximize functional recovery.