From the Guidelines
High-dose glucocorticoids, such as intravenous methylprednisolone, should be given early in the treatment of transient myelitis, while awaiting MRI confirmation, and continued if infection has been ruled out. This approach is supported by the 2010 EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations, which suggest that high-dose glucocorticoids may be effective in reducing inflammation and improving neurological symptoms 1.
Key Considerations
- The involvement of more than three spinal cord segments may indicate longitudinal myelopathy, which can be further investigated with determination of serum NMO IgG (aquaporin) antibodies 1.
- Brain MRI should be performed when other neuropsychiatric symptoms or signs co-exist and in the differential diagnosis of demyelinating disorders 1.
- Mild-to-moderate CSF abnormalities are common but non-specific, and microbiological studies are important to exclude infectious myelitis 1.
Treatment Approach
- The combination of intravenous methylprednisolone and intravenous cyclophosphamide can be effective in SLE myelitis if used promptly, within the first few hours, and neurological response paralleled by MRI improvement occurs within a few days to 3 weeks 1.
- Plasma exchange therapy has been used in severe cases, and anticoagulation therapy in antiphospholipid-positive myelopathy with good results 1.
- Factors associated with severe neurological deficit include extensive spinal cord MRI lesions, reduced muscle strength or sphincter dysfunction at presentation, antiphospholipid antibodies, and delay (>2 weeks) in the initiation of therapy 1.
Monitoring and Supportive Care
- Close monitoring during recovery is important, and any worsening symptoms should prompt immediate medical attention to rule out progression to a more serious condition.
- Supportive care includes physical therapy to maintain muscle strength and range of motion, pain management with medications, and bladder/bowel management if needed.
From the Research
Definition and Symptoms of Transient Myelitis
- Transient myelitis is a rare neurological disorder of the spinal cord caused by inflammation, which can have devastating neurologic effects with up to two-thirds of patients having a moderate to severe degree of residual disability 2.
- Symptoms typically develop over hours or days and then worsen over a matter of days to weeks, and can include sensory alteration, weakness, and autonomic dysfunction including bowel and bladder problems, temperature dysregulation, or even bouts of hypertension 2.
- Clinical presentation of acute transverse myelitis (ATM) typically includes some combination of motor weakness, sensory symptoms, and bowel and bladder dysfunction 3.
Diagnosis and Evaluation
- Evaluation for compressive etiologies must be a priority as compressive myelopathy and transverse myelitis are often clinically indistinguishable and emergent surgical intervention is indicated in such cases 2.
- If neuroimaging and CSF studies indicate inflammation within the central nervous system, then a work-up for myelitis must include autoimmune, inflammatory, and infectious etiologies 2.
- Neuroimaging, CSF, and serological studies are useful in the management of patients with myelitis 2.
Treatment Options
- Acute therapeutic options for ATM include corticosteroids, plasma exchange, IV immunoglobulin, and chemotherapeutic agents such as cyclophosphamide 3.
- Some studies suggest that plasma exchange or cyclophosphamide may be beneficial in the treatment of idiopathic transverse myelitis 4.
- However, other studies have found that methylprednisolone may not be effective in the treatment of acute transverse myelitis 5.
- Therapeutic plasma exchange (TPE) may be more effective than corticosteroids in the treatment of certain conditions, such as anti-N-methyl-D-aspartate receptor antibody encephalitis 6.