UZUN SPİNAL KORD LEZYONU OLAN HASTADA BİYOPSİ İLE NÖROMİYELİTİS OPTİKA TANISI: OLGU SUNUMU

Abstract

Nöromyelitis Optika Spektrum Hastalıkları (NMOSH) tekrarlayan optik nörit ve longitudinal uzun segment transvers miyelit atakları ile seyreden, Aquaporin-4 antikor pozitifliği ile karakterize inflamatuar demiyelinizan bir hastalıktır. Uzun segment longitudinal transvers miyelit, NMOSH dışında; sistemik otoimmun hastalıklarda, spinal kord tümörlerinde, spinal vasküler patolojilerde de saptanabilmektedir. NMOSH, genç populasyonda ağır disabiliteye neden olduğu için erken teşhis ve tedavi çok önemlidir. Bu sunumda, akut parapleji ve idrar inkontinansı şikayeti ile polikliniğimize başvuran yirmi üç yaşındaki kadın hasta tartışılmıştır. Spinal Manyetik Rezonans Görüntüleme’de C7-T7 arasında kord içinde kontrast tutan longitudinal uzun ödemli lezyon saptandı. Astrositom olarak raporlanması nedeniyle lezyondan biyopsi yapıldı ve tümör düşünülmedi. NMOSH tanısı sonradan doğrulanan Aquaporin-4 pozitifliği ile kesinleşti. Uzun segment spinal lezyonlar, NMOSH’da nadir olmasa da lezyonun ödematöz ve beklenenden uzun olması nedeniyle bu olguyu sunmak istedik.

Keywords

• nöromiyelitis optika,Aquaporin-4,transvers miyelit

The Diagnosis of Neuromyelitis Optica by Biopsy in a Patient with Long Spinal Cord Lesion: A Case Report

Abstract

Neuromyelitis Optica Spectrum Diseases (NMOSD) is an inflammatory demyelinating disease, which is characterized by recurrent optic neuritis, longitudinal long-segment transverse myelitis attacks and Aquaporin-4 antibody positivity. Except NMOSD, long-segment longitudinal transverse myelitis can be also detected in systemic autoimmune diseases, spinal cord tumors, spinal vascular pathologies, Early detection and treatment of NMOSD is very important as the disease may cause severe disability in young population. A 23-year-old female patient was admitted to our clinic with acute paraplegia and urine incontinence. Spinal Magnetic Resonance Imaging showed a densly edematose, contrast enhanced lesion longitudinally extending between C7 and T7 in the spinal cord. As the lesion was reported to be an astrocytoma, a biopsy was performed. However, pathological stainings revelaed no tumor. NMOSD was verified later with Aquaporin-4 positivity. Although longitudinally extending spinal cord lesions are not rare in NMOSD, our patient’s lesion was very edematose and longer than expected that deserved reporting.

Keywords

• Aquaporin-4,neuromyelitis optica,transverse myelitis

References

1. 1. Bruscolini A, Sacchetti M, La Cava M, Gharbiya M, Ralli M, Lambiase A et al. Diagnosis and management of neuromyelitis optica spectrum disorders-an update. Autoimmun Rev. 2018;17(3):195-200.
2. 2. Tuncel D, Özay E, Yücesan C, Aydın N. Nöromyelitis Optika: Olgu Sunumu. Türk Nöroloji Dergisi. 2008;14(3):200-3.
3. 3. Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-89.
4. 4. Dumrikarnlert C, Siritho S, Chulapimphan P, Ngamsombat C, Satukijchai C, Prayoonwiwat N. The characteristics of spinal imaging in different types of demyelinating diseases. J Neurol Sci. 2017;372:138-43. Doi: 10.1016/j.jns.2016.11.035.
5. 5. Flanagan EP, Kaufmann TJ, Krecke KN, Aksamit AJ, Pittock SJ, Keegan BM et al. Discriminating long myelitis of neuromyelitis optica from sarcoidosis. Ann Neurol. 2016;79(3):437-47.
6. 6. Zalewski NL, Morris PP, Weinshenker BG, Lucchinetti CF, Guo Y, Pittock SJ, Krecke KN et al. Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders. J Neurol Neurosurg Psychiatry. 2017;88(3):218-25.
7. 7. Pekcevik Y, Mitchell CH, Mealy MA, Orman G, Lee IH, Newsome SD et al. Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging. Mult Scler. 2016;22(3):302-11.
8. 8. Flanagan EP, Weinshenker BG. Neuromyelitis optica spectrum disorders. Curr Neurol Neurosci Rep. 2014;14(9):483.

9. 9. Pittock SJ, Lennon VA, De Seze J, Vermersch P, Homburger HA, Wingerchuk DM et al. Neuromyelitis optica and non–organ-specific autoimmunity. Arch Neurol. 2008;65(1):78-83.
10. 10. Pittock SJ, Lennon VA, Bakshi N, Shen L, McKeon A, Quach H et al. Seroprevalence of aquaporin-4–IgG in a northern California population representative cohort of multiple sclerosis. JAMA Neurol. 2014;71(11):1433-6.
11. 11. Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006;59(3):566-9.
12. 12. Kitley J, Woodhall M, Waters P, Leite MI, Devenney E, Craig J et al. Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype. Neurology. 2012;79(12):1273-7.
13. 13. Flanagan EP. Autoimmune myelopathies. In: Pittock S, Vincent A, eds. Handbook of Clinical Neurology. 3rd ed. 133: 327 Elsevier. 2016:327-51.
14. 14. Bourre B, Lefaucheur R, Girault C. Treatment of NMO relapse in the elderly: rituximab when plasma exchange fails? Acta Neurol Belg. 2013;113(3):335-6.
15. 15. Ringelstein M, Metz I, Ruprecht K, Koch A, Rappold J, Ingwersen J et al. Contribution of spinal cord biopsy to diagnosis of aquaporin-4 antibody positive neuromyelitis optica spectrum disorder. Mult Scler. 2014;20(7):882-8.