This report presents a rare case of a possible isolated sarcoidosis-associated myelopathy (SAM) in a 50-year-old woman who exhibited sensory disturbances and gait instability. Cerebrospinal fluid (CSF) analysis revealed inflammatory characteristics without evidence of infectious disease. Computed tomography (CT) scans of the head, chest, abdomen, and pelvis showed no vascular, neoplastic, or systemic granulomatous abnormalities. Magnetic resonance imaging (MRI) of the spine with gadolinium enhancement demonstrated the characteristic trident sign on axial sequences, strongly suggestive of SAM. The patient underwent pulse therapy with methylprednisolone followed by a tapering course of glucocorticoids, resulting in gradual clinical improvement. Although a definitive diagnosis requires histopathological confirmation, this case underscores the importance of MRI recognition patterns in facilitating prompt diagnosis and guiding effective therapeutic management.
References
[1]
Arkema, E.V. and Cozier, Y.C. (2020) Sarcoidosis Epidemiology: Recent Estimates of Incidence, Prevalence and Risk Factors. Current Opinion in Pulmonary Medicine, 26, 527-534. https://doi.org/10.1097/mcp.0000000000000715
[2]
Barreras, P. and Stern, B.J. (2022) Clinical Features and Diagnosis of Neurosarcoidosis—Review Article. Journal of Neuroimmunology, 368, Article ID: 577871. https://doi.org/10.1016/j.jneuroim.2022.577871
[3]
Zalewski, N.L., Krecke, K.N., Weinshenker, B.G., Aksamit, A.J., Conway, B.L., McKeon, A., et al. (2016) Central Canal Enhancement and the Trident Sign in Spinal Cord Sarcoidosis. Neurology, 87, 743-744. https://doi.org/10.1212/wnl.0000000000002992
[4]
Fritz, D., van de Beek, D. and Brouwer, M.C. (2016) Clinical Features, Treatment and Outcome in Neurosarcoidosis: Systematic Review and Meta-Analysis. BMC Neurology, 16, Article No. 220. https://doi.org/10.1186/s12883-016-0741-x
Mustafa, R., Passe, T.J., Lopez-Chiriboga, A.S., Weinshenker, B.G., Krecke, K.N., Zalewski, N.L., et al. (2021) Utility of MRI Enhancement Pattern in Myelopathies with Longitudinally Extensive T2 Lesions. Neurology Clinical Practice, 11, e601-e611. https://doi.org/10.1212/cpj.0000000000001036
[7]
Flanagan, E.P., Kaufmann, T.J., Krecke, K.N., Aksamit, A.J., Pittock, S.J., Keegan, B.M., et al. (2016) Discriminating Long Myelitis of Neuromyelitis Optica from Sarcoidosis. Annals of Neurology, 79, 437-447. https://doi.org/10.1002/ana.24582
[8]
Jolliffe, E.A., Keegan, B.M. and Flanagan, E.P. (2018) Trident Sign Trumps Aquaporin-4-IgG ELISA in Diagnostic Value in a Case of Longitudinally Extensive Transverse Myelitis. Multiple Sclerosis and Related Disorders, 23, 7-8. https://doi.org/10.1016/j.msard.2018.04.012
[9]
Frohman, E.M. and Wingerchuk, D.M. (2010) Transverse Myelitis. New England Journal of Medicine, 363, 564-572. https://doi.org/10.1056/nejmcp1001112
[10]
Murphy, O.C., Salazar-Camelo, A., Jimenez, J.A., Barreras, P., Reyes, M.I., Garcia, M.A., et al. (2020) Clinical and MRI Phenotypes of Sarcoidosis-Associated Myelopathy. Neurology Neuroimmunology & Neuroinflammation, 7, e722. https://doi.org/10.1212/nxi.0000000000000722
[11]
Boban, J. and Thurnher, M.M. (2019) Ventral-Subpial Enhancement in Spinal Cord Sarcoidosis. Neurology, 92, 236-238. https://doi.org/10.1212/wnl.0000000000006857
[12]
Drent, M., Crouser, E.D. and Grunewald, J. (2021) Challenges of Sarcoidosis and Its Management. New England Journal of Medicine, 385, 1018-1032. https://doi.org/10.1056/nejmra2101555
[13]
Stern, B.J., Royal, W., Gelfand, J.M., Clifford, D.B., Tavee, J., Pawate, S., et al. (2018) Definition and Consensus Diagnostic Criteria for Neurosarcoidosis. JAMA Neurology, 75, 1546-1553. https://doi.org/10.1001/jamaneurol.2018.2295