Heterogeneity of myelopathy in patients with systemic sarcoidosis

Myelopathy occurs in 1% of patients with sarcoidosis and is usually caused by the underlying disease. Comorbidity as its possible cause should be excluded, especially in a case of atypical neurosarcoidosis.

Objective: to analyze the features of myelopathy in patients with systemic sarcoidosis

Patients and methods. Twelve patients (7 women and 5 men) aged 41.5 [32.5; 45.3] years with systemic sarcoidosis and myelopathy were examined. The clinical and radiographic features of spinal cord (SC) injury and the nature of changes in laboratory parameters were analyzed.

Results and discussion. The cause of myelopathy was sarcoidosis (neurosarcoidosis (NS)) in 7 (58%) patients (Group 1) and multiple sclerosis in 4 (33%) (Group 2). One more patient developed myopathy due to extradural lipomatosis (this case is described in the clinical observation section). In Group 1, myelopathy was the first manifestation of sarcoidosis in 4 (57%) of the 7 patients. Six (86%) patients were observed to have incomplete regression of symptoms; 5 (71%) showed a progressive course. Magnetic resonance imaging (MRI) revealed the signs of a lesion in the thoracic SC in 4 (57%) patients with NS, as well as damage to three or more of its segments in 5 (71%) and a radiological pattern of sarcoidosis-induced SC lesion in 6 (86%). MRI findings showed that all the 4 (100%) patients in Group 2 had cervical SC injury, no patterns typical of NS, as well as the signs of meningeal contrast agent accumulation. None of them displayed pleocytosis and low glucose levels in the cerebrospinal fluid. Extradural lipomatosis-induced myelopathy was compressive with positive changes after discontinuation of glucocorticoids.

Conclusion. It is necessary to take into account the possibility of comorbidity as a cause of myelopathy in patients with sarcoidosis and the likelihood of SC lesion as a complication of therapy for the underlying disease.

1. Ibitoye RT, Wilkins A, Scolding NJ. Neurosarcoidosis: a clinical approach to diagnosis and management. J Neurol.2017 May;264(5):1023-28. doi: 10.1007/s00415-016-8336-4. Epub 2016 Nov 22.

2. Munakomi S. Case Report: Case report on multiple extradural thoracic lesions with myelopathy as the clinical presentation in a systemic sarcoidosis - another tale of a lurking entity F1000Res. 2018 Jan 3;7:6. doi: 10.12688/f1000research.13553.1. eCollection 2018.

3. Duhon BS, Shah L, Schmidt MH. Isolated intramedullary neurosarcoidosis of the thoracic spine: case report and review of the literature. Eur Spine J. 2012 Jun;21 Suppl 4:S390-5. doi: 10.1007/s00586-011-1842-2. Epub 2011 May 20.

4. MacLean HJ, Abdoli M. Neurosarcoidosis as an MS Mimic: The trials and tribulations of making a diagnosis. Mult Scler Relat Disord. 2015 Sep;4(5):414-29. doi: 10.1016/j.msard.2015.06.012. Epub 2015 Jun 24.

5. Zajicek JP, Scolding NJ, Foster O, et al. Central nervous system sarcoidosis - diagnosis and management. Mult Scler Relat Disord. 2015 Sep;4(5):414-29. doi: 10.1016/j.msard.2015.06.012. Epub 2015 Jun 24.

6. Stern BJ, Royal W 3rd, Gelfand JM, et al. Definition and consensus diagnostic criteria for neurosarcoidosis. JAMA Neurol. 2018 Dec 1; 75(12):1546-53. doi: 10.1001/jamaneurol.2018.2295.

7. Durel CA, Marignier R, Maucort-Boulch D, et al. Clinical features and prognostic factors of spinal cord sarcoidosis:a multicenter observational study of 20 biopsy-proven patients. J Neurol. 2016 May;263(5):981-90. doi: 10.1007/s00415-016-8092-5. Epub 2016 Mar 23.

8. Maksimova MYu. Neurosarcoidosis. Nevrolo-gicheskii vestnik. 2009; 3 (1): 35-42 (In Russ.).

9. Shmidt TE, Gracheva OM, Kazantsev KYu, et al. Spinal cord damage in sarcoidosis. Nevrologicheskii zhurnal. 2015;20(5):40-7. (In Russ.).

10. Scott AM, Yinh J, McAlindon T, et al. Two cases of sarcoidosis presenting as longitudinally extensive transverse myelitis. Clin Rheumatol. 2018 Oct;37(10):2899-905. doi: 10.1007/s10067-018-4144-9. Epub 2018 May 17.

11. Kranz PG, Amrhein TJ. Imaging approach to myelopathy acute, subacute, chronic. Radiol Clin North Am. 2019 Mar;57(2):257-79. doi: 10.1016/j.rcl.2018.09.006. Epub 2018 Dec 5.

12. Weidauer S, Wagner M, Nichtweie M. Magnetic resonance imaging and clinical features in acute and subacute myelopathies. Clin Neuroradiol. 2017 Dec;27(4):417-33. doi: 10.1007/s00062-017-0604-x. Epub 2017 Jun 30.

13. Nesbit GM, Miller GM, Baker HL Jr, et al. Spinal cord sarcoidosis a new finding at MR imaging with Gd-DTPA enhancement. Radiology. 1989 Dec;173(3):839-43. doi: 10.1148/radiology.173.3.2813795.

14. Tavee JO, Stern BJ. Neurosarcoidosis. Continuum (Minneap Minn). 2014 Jun;20(3 Neurology of Systemic Disease):545-59. doi: 10.1212/01.CON.0000450965.30710.e9.

15. Tyshkov C, Siddharama P, Bradshaw MJ, et al. Multiple sclerosis and sarcoidosis. A case for coexistence. Neurol Clin Pract. 2019 Jun; 9(3):218-227. doi: 10.1212/CPJ.00000000 00000629.

16. Wildstein MS, Martin SM, Glaser JA. Cryptococcal osteomyelitis in a 20-year-old male with sarcoidosis. Spine J. 2005 Jul-Aug; 5(4):467-70.

17. Asamoto S, Sugiyama H, D o i H, et al. A case of thoracic vertebral tuberculosis associated with pulmonary sarcoidosis. No Shinkei Geka. 2001 Sep;29(9):879-83.

18. Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002 Aug 27;59(4):499-505. doi: 10.1212/wnl.59.4.499.

19. Soni N, Bathla G, Maheshwarappa RP. Imaging findings in spinal sarcoidosis: a report of 18 cases and review of the current literature. Neuroradiol J.2019 Feb;32(1):17-28. doi: 10.1177/1971400918806634. Epub 2018 Oct 12.

20. Sohn M, Nozaki K, Culver DA, et al. Spinal cord sarcoidosis. Am J Med Sci. 2014 Mar;347(3):195-8. doi: 10.1097/MAJ.0b013e3182808781.

21. Zalewski NL, Krecke KN, Weinshenker BG, et al. Central canal enhancement and the trident sign in spinal cord sarcoidosis. Neurology. 2016 Aug 16;87(7):743-4. doi: 10.1212/WNL.0000000000002992.

22. Beh SC, Greenberg BM, Frohman T, et al. Transverse myelitis. Neurol Clin.2013 Feb; 31(1):79-138. doi: 10.1016/j.ncl.2012.09.008.

23. Artner J. Spinale epidurale Lipomatose. Orthopade. 2012 Nov;41(11):889-93. doi: 10.1007/s00132-012-1966-z.

24. Burkhardt N, Hamann GF. Extradural lipomatosis after long-term treatment with steroids. Nervenarzt. 2006 Dec;77(12):1477-9.

25. Godeau P, Brunet P, Wechsler B, et al. Compressive extradural lipomatosis as an unusual complication of corticosteroid therapy: one case (author's transl). Nouv Presse Med. 1979 Dec 3;8(47):3889-91.