Hemosiderosis of Central Nervous System: a case report of a rare and underdiagnosed disease

Authors

DOI:

https://doi.org/10.33448/rsd-v10i11.19579

Keywords:

Superficial Siderosis; Hemosiderin Deposition; Hemorrhage; Cerebellar Ataxia; Deafness.

Abstract

Superficial Siderosis (SS) of Central Nervous System is a rare disease characterized by the deposit of hemosiderin in the brain and spinal cord. Clinically, it is characterized by progressive sensorineural ataxia and deafness associated with injury of superior motor neuron. The diagnosis is made by magnetic resonance imaging (MRI) of the encephalon and spinal cord. The objective of the study is to report the case of a patient with characteristic elements of the syndrome, accompanied in a private medical clinic.

References

Anderson, N. E., Sheffield, S., & Hope, J. K. A. (1999). Superficial siderosis of the central nervous system: A late complication of cerebellar tumors. Neurology, 52(1), 163–163. https://doi.org/10.1212/WNL.52.1.163

Cerqueira, A. C. R. de., Nardi, A. E., & Bezerra, J. M. F. (2010). Superficial siderosis of the central nervous system: an unusual cause of sensorineural hearing loss. Arquivos de Neuro-Psiquiatria, 68(3), 469–471. https://doi.org/10.1590/S0004-282X2010000300028

Charidimou, A. (2016). Cortical Superficial Siderosis Presumed due to Cerebral Amyloid Angiopathy: Minimum Standards for Rating and Reporting. American Journal of Neuroradiology, 37(5), E43–E44. https://doi.org/10.3174/ajnr.A4748

Chen, H., Raza, H. K., Jing, J., Ye, X., Zhang, Z., Hua, F., & Cui, G. (2019). Superficial siderosis of central nervous system with unknown cause: report of 2 cases and review of the literature. British Journal of Neurosurgery, 33(3), 305–308. https://doi.org/10.1080/02688697.2017.1406452

Fearnley, J. M., Stevens, J. M., & Rudge, P. (1995). Superficial siderosis of the central nervous system. Brain, 118(4), 1051–1066. https://doi.org/10.1093/brain/118.4.1051

Fragoso, Y. D., Adoni, T., Brooks, J. B. B., Gomes, S., Goncalves, M. V. M., Jovem, C. L., Matta, A. P. da C., Oliveira, J. F., Siquinelli, F., Tauil, C. B., Troiani, G. N., & Wille, P. R. (2017). Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder. Arquivos de Neuro-Psiquiatria, 75(2), 92–95. https://doi.org/10.1590/0004-282x20170001

Hiraka, T., Kanoto, M., Toyoguchi, Y., Igari, R., Kato, T., & Hosoya, T. (2018). Superficial Siderosis Associated with a Spinal Dural Defect. Magnetic Resonance in Medical Sciences, 17(3), 189–190. https://doi.org/10.2463/mrms.ci.2017-0043

Koeppen, A. H., Michael, S. C., Li, D., Chen, Z., Cusack, M. J., Gibson, W. M., Petrocine, S. V., & Qian, J. (2008). The pathology of superficial siderosis of the central nervous system. Acta Neuropathologica, 116(4), 371–382. https://doi.org/10.1007/s00401-008-0421-z

Kumar, N. (2007). Superficial Siderosis. Archives of Neurology, 64(4), 491. https://doi.org/10.1001/archneur.64.4.491

Lee, S.-Y., Lee, D.-H., Bae, Y. J., Song, J.-J., Kim, J. S., & Koo, J.-W. (2018). Bilateral Vestibulopathy in Superficial Siderosis. Frontiers in Neurology, 9. https://doi.org/10.3389/fneur.2018.00422

Offenbacher, H., Fazekas, F., Schmidt, R., Kapeller, P., & Fazekas, G. (1996). Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology, 38(S1), S51–S56. https://doi.org/10.1007/BF02278119

Pichler, M., Vemuri, P., Rabinstein, A. A., Aakre, J., Flemming, K. D., Brown, R. D., Kumar, N., Kantarci, K., Kremers, W., Mielke, M. M., Knopman, D. S., Jack, C. R., Petersen, R. C., Lowe, V., & Graff-Radford, J. (2017). Prevalence and Natural History of Superficial Siderosis. Stroke, 48(12), 3210–3214. https://doi.org/10.1161/STROKEAHA.117.018974

Ribeiro, D. C., Nunes, J., Ribeiro, A. C., Maricato, F., & Ribeiro, C. (2013). Superficial siderosis of the central nervous system: an usual cause of sensorineural hearing loss. Brazilian Journal of Otorhinolaryngology, 79(2), 257. https://doi.org/10.5935/1808-8694.20130044

River, Y., Honigman, S., Gomori, J. M., & Reches, A. (1994). Superficial hemosiderosis of the central nervous system. Movement Disorders, 9(5), 559–562. https://doi.org/10.1002/mds.870090509

Ryu, S. M., Kim, E.-S., Kim, S.-K., Lee, S.-H., & Eoh, W. (2016). Superficial Siderosis of the Central Nervous System Originating from the Thoracic Spine: A Case Report. Korean Journal of Spine, 13(2), 83. https://doi.org/10.14245/kjs.2016.13.2.83

Sighary, M., Cohen-Addad, D., & Linden, C. (2018). Superficial siderosis: Chronic sequelae following brain hemorrhage. Radiology Case Reports, 13(3), 624–626. https://doi.org/10.1016/j.radcr.2018.03.001

Tuñón Gómez, M., Brea Álvarez, B., & Piazza Dobarganes, A. (2015). Hipoacusia neurosensorial: hemosiderosis superficial del sistema nervioso central. Acta Otorrinolaringológica Española, 66(6), 362–363. https://doi.org/10.1016/j.otorri.2014.01.001

Vilaça, C. D. O., Magella, C. B., Afonso, C., Py, M. O., Santos, A. S., Novellino, P., & Orsini, M. (2015). Superficial siderosis of the central nervous system: case report. Medicina (Ribeirao Preto. Online), 48(4), 412. https://doi.org/10.11606/issn.2176-7262.v48i4p412-415

Wilson, D., Chatterjee, F., Farmer, S. F., Rudge, P., McCarron, M. O., Cowley, P., & Werring, D. J. (2017). Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway. Annals of Neurology, 81(3), 333–343. https://doi.org/10.1002/ana.24850

Yin, R. K. (2015). O estudo de caso: Bookman.

Downloads

Published

31/08/2021

How to Cite

PERLIN, C. M. .; COLOMBO, L. A. .; GROTO, A. D. .; RIGON, B. G. da S. . Hemosiderosis of Central Nervous System: a case report of a rare and underdiagnosed disease. Research, Society and Development, [S. l.], v. 10, n. 11, p. e270101119579, 2021. DOI: 10.33448/rsd-v10i11.19579. Disponível em: https://www.rsdjournal.org/index.php/rsd/article/view/19579. Acesso em: 19 apr. 2024.

Issue

Section

Health Sciences