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Two Cases of Probable Neuro-Behçet's Disease with Longitudinally Extensive Transverse Myelitis.
Shunya Fujiwara1, Yasuhiro Manabe1, Yumiko Nakano1
1Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama, Japan.
Case Reports in Neurology
|March 12, 2021
Summary
Neuro-Behçet's disease (NBD) can cause longitudinally extensive transverse myelitis (LETM), presenting with severe neurological deficits. Early consideration of NBD in LETM cases may improve patient outcomes.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Neuro-Behçet's disease (NBD) is a rare systemic vasculitis affecting the central nervous system.
- Longitudinally extensive transverse myelitis (LETM) is characterized by spinal cord lesions spanning three or more vertebral segments.
Observation:
- Two patients with probable NBD presented with LETM, exhibiting paraplegia and sensory, bladder, and rectal dysfunction.
- MRI revealed extensive spinal cord lesions; one case showed continuous high signal intensity from the midbrain to the entire spinal cord on T2WI.
- The other patient had a lesion from Th2 to Th10, also centrally located on T2WI.
Findings:
- Both patients were treated with high-dose methylprednisolone.
- One patient demonstrated a unique, continuous midbrain-to-spinal cord lesion, previously unreported in NBD.
- The second patient showed a dramatic improvement with infliximab therapy.
Implications:
- These cases highlight the importance of considering NBD in the differential diagnosis of LETM.
- The findings suggest that NBD can manifest with extensive spinal cord involvement.
- Successful infliximab treatment in one patient indicates potential therapeutic avenues for NBD-associated LETM.
