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Case Reports
. 2015 Jan;38(1):111-4.
doi: 10.1179/2045772314Y.0000000209. Epub 2014 Mar 28.

Recurrent longitudinal extensive transverse myelitis in a neuro-Behçet syndrome treated with infliximab

Uğur Uygunoğlu, Maarya Pasha, Sabahattin Saip, Aksel Siva
Case Reports

Recurrent longitudinal extensive transverse myelitis in a neuro-Behçet syndrome treated with infliximab

Uğur Uygunoğlu et al. J Spinal Cord Med. 2015 Jan.

Abstract

Background: Spinal cord involvement is not common, but can be seen in neuro-Behçet's syndrome (NBS). The major site of involvement is the cervical spinal cord with the myelitis-like inflammatory lesions continuing more than two segments, and extending to the brainstem.

Case: A 30-year-old male patient who has been followed with a diagnosis of Behçet's syndrome admitted to our neurology department clinically and radiologically suggestive of recurrent and extensive longitudinal myelitis. His anti-aquaporine antibody was negative. Because of insufficient effect of azathioprine, cyclophosphamide, and corticosteroids, infliximab was started. His clinical and radiological status is stationary for 3 years under infliximab treatment.

Discussion: Myelitis such as that occurring in our patient may have a similar presentation like neuromyelitis optica (NMO), which should therefore be included in differential diagnosis. Myelitis observed in both NMO and NBS shows spinal cord lesions longer than three or more vertebrae. Anti-aquaporine antibody must be evaluated in all patients presenting with longitudinal myelitis. Anti-tumor necrosis factor agent infliximab might be an alternative treatment in severe form of NBS such as myelitis.

Conclusion: In our case, successful treatment of recurrent and extensive longitudinal transverse myelitis in NBS with infliximab was demonstrated.

Keywords: Neuro-Behçet syndrome; Neuromyelitis optica.

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Figures

Figure 1
Figure 1
Cervical and upper thoracic spinal MRI: sagittal T2-weighted images show an extensive longitudinal lesion from C4 to T4 without gadolinium enhancement.
Figure 2
Figure 2
Cervical and upper thoracic spinal MRI: sagittal T2-weighted images reveal a longitudinal lesion from T1 to T7, which shows gadolinium enhancement at the level of T5.
Figure 3
Figure 3
Cervical and upper thoracic spinal MRI: sagittal T2-weighted images reveal an extensive longitudinal lesion from the level of T1 to T7, with gadolinium enhancement at the levels of T3–T4 and T6–T7.
See this image and copyright information in PMC

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