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| IMAGES IN RHEUMATOLOGY |
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| Year : 2018 | Volume
: 13
| Issue : 2 | Page : 133-134 |
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Long segment extensive transverse myelitis in a patient of ankylosing spondylitis
Vijay Sardana, Sunil Kumar Sharma, Dilip Maheshwari, Bharat Bhushan
Department of Neurology, Government Medical College, Kota, Rajasthan, India
| Date of Web Publication | 24-May-2018 |
Correspondence Address:
Dr. Sunil Kumar Sharma
Flat No. 405 Chambal Residency, Nayapura Kota, Rajasthan
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/injr.injr_118_17
Keywords: Ankylosing spondylitis, antiaquaporin-4 antibody, HLA b27, long segment extensive transverse myelitis, myelopathy
How to cite this article:
Sardana V, Sharma SK, Maheshwari D, Bhushan B. Long segment extensive transverse myelitis in a patient of ankylosing spondylitis. Indian J Rheumatol 2018;13:133-4 |
Ankylosing spondylitis (AS) is a chronic inflammatory rheumatic disease that primarily affects the peripheral and axial joints of the body.[1] Neurological complications are uncommon in patients with AS.[1],[2] Most of the neurological complications are due to AS-associated joint stiffness, bony deformity or bony destruction leading to nerve root compression, spinal canal stenosis, cauda equina syndrome, and very rarely compressive/noncompressive myelopathy such as multiple sclerosis.
Our patient who had established AS (HLA B 27 positive) presented with acute-onset quadriparesis with urinary retention without a definite sensory level and visual symptoms. His neurological examination revealed normal higher mental function test and cranial nerve examination. He had weakness of all four limbs (power 4/5) with brisk deep-tendon reflexes and bilateral extensor plantar response. Magnetic resonance imaging (MRI) of the spine revealed long segment extensive spinal cord hyperintensity from C5-D1 to D3-D12 with minimal cord expansion in lower cervical and mid-lower dorsal spinal cord [Figure 1] and [Figure 2]. On biochemical evaluation, Erythrocyte sedimentation rate was 20 mm/h, and Antinuclear antibody (ANA), venereal disease research laboratory (VDRL), Human immunodeficiency virus (HIV), Hepatitis B surface antigen (HBsAg), Anti-hepatitis C virus (HCV) antibody were negative. Cerebrospinal fluid (CSF) showed the presence of 32 cells (lymphocytes-65% and polymorphs-35%) with normal protein (39% mg), sugar (52% mg), and ADA (4.2 U/L). Endemic-CSF viral markers were negative. CSF antiaquaporin-4 antibody (antineuromyelitis optica (NMO) antibody) was negative. The patient was managed conservatively and responded to short-term high-dose intravenous methyl Prednisolone and was given long-term maintenance immunosuppressive therapy with Azathioprine. Limb power at the time of discharge was 4+/5 and was 5/5 on follow-up after 1, 3, and 6 months. | Figure 1: Magnetic resonance imaging spine T2-weighted showing cord hyperintensity in cervical and dorsal region extending from C5-D1 and D3-D12 with minimal expansion of lower cervical and mid-lower dorsal spinal cord
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 | Figure 2: Magnetic resonance imaging spinal cord axial section – mid-dorsal region showing hyperintensity involving anterior and central portion of cord
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One case of noncompressive myelopathy associated with AS was reported in 2001 by Oh et al. without radiological confirmation.[3] Chen et al. reported three cases of noncompressive myelopathy characterized by cervico-dorsal spinal cord atrophy.[4] Thomas and Kendall reported two cases of MS among 45 patients with AS.[5] Khan and Kushner studied 196 patients with long-standing AS and reported two cases of MS as well, which showed widespread central nervous system involvement and classical transient attacks, but without laboratory or radiological confirmation.[6] However, in this case, such an extensive cord involvement is less likely to be associated with MS. CSF showed lymphocytic pleocytosis suggestive of myelitis. There was no antecedent fever or vaccination, and none of the clinical symptom was suggestive of infective pathology. VEP and BAER were normal, CSF antiaquaporin-4 antibody was negative, so NMO spectrum disorder was unlikely. VDRL, Viral serology including HIV, HBsAg, anti-HCV, and CSF-endemic viral markers were negative. Spinal cord vasculitis has been reported in few cases of rheumatoid arthritis and systemic lupus erythematosus. In AS, bony impingement of spinal artery supplying the cord is suggested to play a major role in the pathophysiology of vascular events, but it is less likely in this case as there is no radiological evidence of significant compression of the spinal cord in MRI. We suggest the inflammation of spinal cord due to AS per se as the pathogenesis of the longitudinally extensive transverse myelitis (LETM) in this case.
Only a few cases of noncompressive myelopathy associated with AS have been reported in the world literature, and none reported from Indian literature. To the best of our knowledge, LETM in a patient with AS has not been previously reported.
From this case presented above it appears that there may be some causal relation between AS and LETM and it may be included as a differential in a case of seronegative LETM.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | |  |
| 1. | Graham W, Ogryzlo MA. Ankylosing (Marie-strümpell) spondylitis; An analysis of 100 cases. Can Med Assoc J 1947;57:16-21.  |
| 2. | Wilkinson M, Bywaters EG. Clinical features and course of AS. Ann Rheum Dis 1958;17:209-28. |
| 3. | Oh DH, Jun JB, Kim HT, Lee SW, Jung SS, Lee IH, et al. Transverse myelitis in a patient with long-standing ankylosing spondylitis. Clin Exp Rheumatol 2001;19:195-6. |
| 4. | Chen WH, Yin HL, Lin HS, Chen CJ. Symptomatic noncompressive motoromyelopathy presents as early manifestation in ankylosing spondylitis. Rheumatol Int 2011;31:945-50. |
| 5. | Thomas DJ, Kendall MJ, Whitfield AG: Nervous system involvement in ankylosing spondylitis. BMJ 1974;1:148-50. |
| 6. | Khan MA, Kushner I. Ankylosing spondylitis and multiple sclerosis: A possible association. Arthritis Rheum 1979;22:784-6. |
[Figure 1], [Figure 2]
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