1887
Volume 2022, Issue 3
  • ISSN: 0253-8253
  • EISSN: 2227-0426

Abstract

Systemic lupus erythematosus is a chronic autoimmune connective tissue disorder that can affect all the neuroaxes in the central and peripheral nervous systems. Myelopathy in systemic lupus erythematosus is one of the least common neuropsychiatric syndromes accounting for 1%–2% of cases. Myelopathy has long been diagnosed based on clinical findings, laboratory tests, and gold-standard gadolinium-enhanced magnetic resonance imaging (MRI). MRI-negative myelopathy is a recently described subset of myelopathies. Here, we report the case of a young woman from rural West Bengal, India, who presented with overlapping features of white-matter and gray-matter myelopathy associated with peripheral neuropathy and bilateral asymmetric lower motor neuron-type facial paresis. The historical analysis yielded clues toward an etiological diagnosis of systemic lupus erythematosus, further substantiated by seropositivity of lupus-specific autoantibodies. Her neurological disabilities responded poorly to oral administration of hydroxychloroquine, bolus intravenous administration of methylprednisolone, and high-dose cyclophosphamide therapy but eventually responded remarkably well to cyclical rituximab therapy. This case adds to the tally of cases of MRI-negative lupus myelopathy. MRI-negative myelopathy in systemic lupus erythematosus can be easily missed if not meticulous attention is paid during clinical history taking and examinations.

Loading

Article metrics loading...

/content/journals/10.5339/qmj.2022.42
2022-09-12
2024-04-18
Loading full text...

Full text loading...

/deliver/fulltext/qmj/2022/3/qmj.2022.42.html?itemId=/content/journals/10.5339/qmj.2022.42&mimeType=html&fmt=ahah

References

  1. Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al.2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019; 71;:(9):1400–1412.
    [Google Scholar]
  2. Chiganer EH, Hryb JP, Carnero Contentti E. Myelitis and lupus: clinical manifestations, diagnosis and treatment. Review. Reumatol Clin. 2017; 13;:(6):344–348.
    [Google Scholar]
  3. Das S, Ray BK, Pandit A, Dubey S. MRI negative myeloradiculoneuropathy unmasking systemic lupus erythematosus. Rheumatology. 2021; 60;:(9):e318–e320.
    [Google Scholar]
  4. Hryb JP, Chiganer E, Contentti EC, Di Pace JL, Lessa C, Perassolo MB. Myelitis in systemic lupus erythematosus: clinical features, immunological profile and magnetic resonance imaging of five cases. Spinal Cord Ser Cases. 2016; 2;:(1):1–5.
    [Google Scholar]
  5. Kovacs B, Lafferty TL, Brent LH, DeHoratius RJ. Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature. Ann Rheum Dis. 2000; 59;:(2):120–124.
    [Google Scholar]
  6. Sechi E, Krecke KN, Pittock SJ, Dubey D, Lopez-Chiriboga AS, Kunchok A, et al. Frequency and characteristics of MRI-negative myelitis associated with MOG autoantibodies. Mult Scler. 2021; 27;:(2):303–308.
    [Google Scholar]
  7. Saison J, Costedoat-Chalumeau N, Maucort-Boulch D, Iwaz J, Marignier R, Cacoub P, et al. Systemic lupus erythematosus-associated acute transverse myelitis: manifestations, treatments, outcomes, and prognostic factors in 20 patients. Lupus. 2015; 24;:(1):74–81.
    [Google Scholar]
  8. Das S, Dubey S, Pandit A, Ray BK. Myeloradiculoneuropathy due to vitamin B12 deficiency: an unusual clinical and radiological presentation. BMJ Case Rep. 2021;25; 14:(1):e239415.
    [Google Scholar]
  9. Oiwa H, Kuriyama A, Matsubara T, Sugiyama E. Clinical value of autoantibodies for lupus myelitis and its subtypes: a systematic review. Semin Arthritis Rheum. 2018; 48;:(2):214–220.
    [Google Scholar]
  10. Wong SH, Boggild M, Enevoldson TP, Fletcher NA. Myelopathy but normal MRI: where next? Pract Neurol. 2008; 8;:(2):90–102.
    [Google Scholar]
  11. Bag-Ozbek A, Hui-Yuen JS. Emerging B-cell therapies in systemic lupus erythematosus. Ther Clin Risk Manag. 2021;:17:39–54.
    [Google Scholar]
  12. Shipman WD, Dasoveanu DC, Lu TT. Tertiary lymphoid organs in systemic autoimmune diseases: pathogenic or protective? F1000Res. 2017;6.
    [Google Scholar]
  13. Peters A, Pitcher LA, Sullivan JM, Mitsdoerffer M, Acton SE, Franz B, et al. Th17 cells induce ectopic lymphoid follicles in central nervous system tissue inflammation. Immunity. 2011; 35;:(6):986–996.
    [Google Scholar]
  14. Bortoluzzi A, Silvagni E, Furini F, Piga M, Govoni M. Peripheral nervous system involvement in systemic lupus erythematosus: a review of the evidence. Clin Exp Rheumatol. 2019; 28;:(4):465–474.
    [Google Scholar]
  15. Costallat BL, Ferreira DM, Costallat LT, Appenzeller S. Myelopathy in systemic lupus erythematosus: clinical, laboratory, radiological and progression findings in a cohort of 1,193 patients. Rev Bras Reumatol Engl Ed. 2016; 56;:(3):240–251.
    [Google Scholar]
  16. Birnbaum J, Petri M, Thompson R, Izbudak I, Kerr D. Distinct subtypes of myelitis in systemic lupus erythematosus. Arthritis Rheum. 2009; 60:(11):3378–3387.
    [Google Scholar]
  17. Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, et al. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs. Ann Rheum Dis. 2010; 69:(12):2074–2082.
    [Google Scholar]
  18. Shipa M, Embleton-Thirsk A, Parvaz M, Santos LR, Muller P, Chowdhury K, et al. Effectiveness of Belimumab after rituximab in systemic lupus erythematosus: a randomized controlled trial. Ann Intern Med. 2021; 174:(12):1647–1657.
    [Google Scholar]
  19. Ghosh R, De K, Roy D, Mandal A, Biswas S, et al. A case of area postrema variant of neuromyelitis optica spectrum disorder following SARS-CoV-2 infection. J Neuroimmunol. 2020;350:577439.
    [Google Scholar]
  20. Gupta DK, Atam V, Chaudhary SC. Recurrent lower motor neuron type facial palsy: an unusual manifestation of SLE. BMJ Case Rep. 2011;bcr1220103564.
    [Google Scholar]
  21. Kazzaz NM, El-Rifai R. Unusual aetiology of isolated lower motor neuron facial palsy: systemic lupus erythematosus presenting with cranial nerve palsy and nephritis. BMJ Case Rep. 2013;bcr2013200378.
    [Google Scholar]
http://instance.metastore.ingenta.com/content/journals/10.5339/qmj.2022.42
Loading
/content/journals/10.5339/qmj.2022.42
Loading

Data & Media loading...

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error