Preview

Clinical Medicine (Russian Journal)

Advanced search
Open Access Open Access  Restricted Access Subscription Access

Neurosarcoidosis

https://doi.org/10.30629/0023-2149-2025-103-12-865-872

Abstract

Neurosarcoidosis is a rare manifestation of systemic sarcoidosis characterized by involvement of both the central and peripheral nervous systems. The clinical presentation is highly variable and depends on the localization of granulomatous inflammation, which complicates the diagnostic process. This article reviews current understanding of the pathogenesis, clinical phenotypes, and differential diagnosis of neurosarcoidosis. It also describes methods of neuroimaging, laboratory diagnostics, and histological verification of the disease. Special attention is given to therapeutic strategies, including glucocorticoid and immunosuppressive therapy. Prognostic considerations and potential directions for future research are discussed.

About the Authors

S. A. Terpigorev
National Medical and Surgical Center named after N.I. Pirogov of the Ministry of Healthcare of the Russia, Institute of Advanced Medical Training of Doctors
Russian Federation

Stanislav A. Terpigorev — Doctor of Medical Sciences, Head of the Department of Internal Medicine

Moscow 



R. R. Bogdanov
National Medical and Surgical Center named after N.I. Pirogov of the Ministry of Healthcare of the Russia, Institute of Advanced Medical Training of Doctors
Russian Federation

Rinat R. Bogdanov — Candidate of Medical Sciences, Associate Professor, Head of the Department of Neurology

Moscow 



A. V. Dimertsev
National Medical and Surgical Center named after N.I. Pirogov of the Ministry of Healthcare of the Russia, Institute of Advanced Medical Training of Doctors
Russian Federation

Alexey V. Dimertsev — Candidate of Medical Sciences, Neurosurgeon, Department of Neurosurgery

Moscow 



References

1. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am. J. Respir. Crit. Care Med. 1999;160(2):736–55.

2. Stern B., Royal W 3rd., Gelfand J., Clifford D., Tavee J., Pawate S. et al. Definition and consensus diagnostic criteria for neurosarcoidosis: from the neurosarcoidosis Consortium Consensus Group. JAMA Neurol. 2018;75(12):1546–1553.

3. Fritz D., van de Beek D., Brouwer M. Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis. BMC Neurology. 2016;16(1):220.

4. Kidd D.P. “Neurosarcoidosis: clinical manifestations, investigation and treatment”. Practical Neurology. 2020;20(3):199-212.

5. Chuchalin A.G., Avdeev S.N., Aisanov Z.R., Baranova O.P., Borisov S.E., Geppe N.A. et al. Sarcoidosis: federal clinical guidelines for diagnosis and treatment. Pulmonology. 2022;32(6):806–833. (In Russian).

6. Stern B., Krumholz A., Johns C., Scott P., Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol. 1985;42(9):909–917. DOI: 10.1001/archneur.1985.04060080095022

7. Berker T. K., Isikay A., Gurlek A. A. Panhypopituitarism as the first manifestation of sarcoidosis: case report and review of the literature. Pituitary. 2004;7(2):73 –78.

8. Tavee J.O., Karwa K., Ahmed Z., Thompson N., Parambil J., Culver D.A. Sarcoidosis- associated small fiber neuropathy in a large cohort: clinical aspects and response to IVIG and anti-TNF alpha treatment. Respir. Med. 2017;126:135–138.

9. Ginat D.T., Dhillon G., Almast J. Magnetic resonance imaging of neurosarcoidosis. J. Clin. Imaging Sci. 2011;1:15. DOI: 10.4103/2156-7514.76693. PMID: 21977388; PMCID: PMC3173834

10. Imsirovic B., Guso E., Omerhodzic I., Tawil AA., Merhemic Z. Neurosarcoidosis — the Role of Magnetic Resonance Imaging in Diagnostics. Acta Inform. Med. 2023;31(1):73–75. DOI: 10.5455/aim.2023.31.73-75. PMID: 37038484; PMCID: PMC10082661

11. Perillo T.; Capasso R.; Pinto A. Neuroimaging of the Most Common Meningitis and Encephalitis of Adults: A Narrative Review. Diagnostics. 2024;14:1064. DOI: 10.3390/diagnostics14111064

12. Flanagan E.P., Kaufmann T.J., Krecke K.N. et al. Discriminating long myelitis of neuromyelitis optica from sarcoidosis. Ann. Neurol. 2016;79:437.

13. Zajicek J.P., Scolding N.J., Foster O. et al. Central nervous system sarcoidosis — diagnosis and management. QJM. 1999;92:103.

14. Murphy O.C., Salazar-Camelo A., Jimenez J.A., et al. Clinical and MRI phenotypes of sarcoidosis-associated myelopathy. Neurol. Neuroimmunol. Neuroinflamm. 2020;7(4).

15. Petereit HF, Reske D, Tumani H, et al. Soluble CSF interleukin 2 receptor as indicator of neurosarcoidosis. J. Neurol. 2010; 257:1855.

16. Stewar R.D., Auffret M.D., Warr A., Wiser A.H. et al. Assembly of 913 microbial genomes from metagenomic sequencing of the cow rumen. Nature Communications. 2018;9(1):870.

17. Bilgin Topçuoğlu, Özgür & Kavas, Murat & O ztas, S. & Nc, Saeedudheen & Cetintas Afsar, Gulgun & Saraç, Sema & Midi, Ipek. (2016). EEG in Sarcoidosis Patients Without Neurological Findings. Clinical EEG and Neuroscience. 48(1). DOI: 10.1177/1550059416646651

18. Bradshaw MJ., Pawate S., Koth LL., Cho TA., Gelfand JM. Neurosarcoidosis: Pathophysiology, Diagnosis, and Treatment. Neurol. Neuroimmunol. Neuroinflamm. 2021;8(6):e1084.

19. Dudesek A., Rimmele F., Tesar S., Kolbaske S., Rommer PS., Benecke R., Zettl UK. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin. Exp. Immunol. 2014;175(3):385–396. DOI: 10.1111/cei.12204. PMID: 24028073; PMCID: PMC3927899

20. Salvarani C, Brown R.D. Jr, Calamia K.T. et al. Primary CNS vasculitis with spinal cord involvement. Neurology. 2008;70:2394.

21. Skripchenko N.V., Lobzin Yu.V., Ivanova G.P., Komantsev V.N., Alekseeva L.A., Ivanova M.V., Solnits A.A., Gorelik E.Yu., Skripchenko E.Yu. Neuroinfections in children. Childhood infections. 2014;13(1):8–18. (In Russian).

22. Tunkel A. R., Glaser C. A., Bloch K. C., Sejvar et al. The management of encephalitis: clinical practice guidelines by the Infectious Diseases Society of America. Clinical Infectious Diseases. 2008;47(3):303–327.

23. Barney J. Stern, Walter Royal III, Jeffrey M. Gelfand, MD, David B. Clifford, et al. JAMA Neurol. 2018;75(12):1546–1553. DOI: 10.1001/jamaneurol.2018.2295 Published online August 27, 2018.

24. Sobic-Saranovic D., Grozdic I., Videnovic-Ivanov J., Artiko V., Petrovic S., Saranovic D. et al. The clinical impact of 18F-FDG PET and PET/CT in patients with sarcoidosis. Journal of Nuclear Medicine. 2012;53(9):1543–1550

25. Baughman R.P., Valeyre D., Korsten P., Mathioudakis A.G., Wuyts W.A., Wells A., et al., ERS clinical practice guidelines on treatment of sarcoidosis, Eur. Respir. J. 2021. DOI: 10.1183/13993003.04079-2020. PubMed PMID: 34140301

26. Paramothayan N.S., Lasserson T.J., Jones P.W. Corticosteroids for pulmonary sarcoidosis. Epub 2005/04/23, Cochrane Database Syst. Rev. 2 (2005) CD001114, DOI: 10.1002/14651858.CD001114.pub2. PubMed PMID: 15846612; PubMed Central PMCID: PMCPMC6464973.

27. Rahaghi F.F., Baughman R.P., Saketkoo L.A., Sweiss N.J., Barney J.B., Birring S.S., et al., Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis. Eur. Respir. Rev. 2020;29(155):190146. DOI: 10.1183/16000617.0146-2019

28. Murayama K., Inoue A., Nakamura Y., Ochi M., Shigekawa S., Watanabe H., Kitazawa R., Kunieda T. A rare case of neurosarcoidosis occurred only in the medulla oblongata mimicking malignant brain tumor. Surgical neurology international. 2021;12:243.

29. Shields R., Sagan O., Roebke L., Vander Maten J., Shah S., Chang G., Ibrahim D., Naz S. Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review. Spinal cord series and cases. 2021;7(1):89.

30. Johns C., Johns C. A case of central diabetes insipidus secondary to neurosarcoidosis. Cureus. 2020;12(10):e11119. DOI: 10.7759/cureus.11119. PMID: 33240714; PMCID: PMC7682679)

31. Sponsler J., Werz M., Maciunas R., Cohen M. Neurosarcoidosis presenting with simple partial seizures and solitary enhancing mass: case reports and review of the literature. Epilepsy Behav. 2005;6(4):623– 30. DOI: 10.1016/j.yebeh.2005.02.016. PMID: 15907759)

32. Pandey A., Stoker T., Adamczyk L. A., & Stacpoole S. (2021). Aseptic meningitis and hydrocephalus secondary to neurosarcoidosis. BMJ case reports. 2021;14(8):e242312.

33. Joubert B., Chapelon-Abric C., Biard L. et al. Association of prognostic factors and immunosuppressive treatment with long-term outcomes in neurosarcoidosis. JAMA Neurol. 2017;74:1336–1344.

34. Affan M., Mahajan A., Rehman T. et al. The effect of race on clinical presentation and outcomes in neurosarcoidosis. J. Neurol. Sci. 2020;417:117073.

35. Thillai M., Atkins C.P., Crawshaw A., Hart S.P., Ho L.P., Kouranos V. et al. BTS Clinical Statement on pulmonary sarcoidosis, Thorax. 2021;76(1) :4–20, DOI: 10.1136/thoraxjnl-2019-214348. PubMed PMID: 33268456

36. Pawate S., Moses H., Sriram S. Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM. 2009;102:449.


Review

For citations:


Terpigorev S.A., Bogdanov R.R., Dimertsev A.V. Neurosarcoidosis. Clinical Medicine (Russian Journal). 2025;103(12):865-872. (In Russ.) https://doi.org/10.30629/0023-2149-2025-103-12-865-872

Views: 213

JATS XML

ISSN 0023-2149 (Print)
ISSN 2412-1339 (Online)