

Infratentorial superficial hemosiderosis
https://doi.org/10.30629/2658-7947-2024-29-3-58-65
Abstract
Introduction. Superficial hemosiderosis of the central nervous system is a chronic, progressive disease caused by continuous low-volume bleeding into the subarachnoid space and, as a result, subpial deposition of hemoglobin de-
tritus. Infratentorial superfi cial hemosiderosis (ISS) is characterized by hemosiderin accumulation primarily on the surface of the cerebellum, brainstem and spinal cord. Early detection of ISS may prevent severe disability and raise the chance of successful medical treatment.
The purpose of this study was to analyze clinical, radiological, instrumental, and laboratory findings, based on a series of ISS cases diagnosed at the Research Center of Neurology.
Material and methods. Seven patients diagnosed with ISS were included in this study (4 men, 3 women). Evaluation of the clinical findings, disease history, brain and spinal cord MRI, MR-angiography, brainstem auditory evoked potentials, audiometry, abdominal ultrasound, and laboratory iron metabolism tests was performed.
Results. The median age of subjects was 63 years, and the median duration of disease was 3 years. We identified the cause of ISS in four patients. The most common clinical symptoms were cerebellar ataxia and sensorineural hearing loss. All patients had a typical ISS pattern on brain MRI images.
Conclusion. With the development of neuroimaging, iSS is becoming a more frequent finding. Clinicians should be aware of its causes and optimal management strategy. Further studies of possible iSS treatments are needed to reduce free iron neurotoxicity and minimize clinical manifestations of the disease.
Keywords
About the Authors
E. P. NuzhnyiRussian Federation
Moscow
L. R. Zaripova
Russian Federation
Moscow
E. Yu. Fedotova
Russian Federation
Moscow
R. N. Konovalov
Russian Federation
Moscow
A. S. Filatov
Russian Federation
Moscow
A. N. Moskalenko
Russian Federation
Moscow
S. N. Illarioshkin
Russian Federation
Moscow
References
1. Wilson D., Chatterjee F., Farmer S.F., Rudge P., McCarron M.O., Cowley P., Werring D.J. Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway. Ann Neurol. 2017;81(3):333–343. doi: 10.1002/ana.24850
2. Makotrova T.A., Levin O.S., Arablinskii A.V. Cerebral amyloid angiopathy. S.S. Korsakov Journal of Neurology and Psychiatry (special issues) (Zhurnal Nevrologii i Psikhiatrii imeni S.S. Korsakova). 2014;114(6–2):87–93. (In Russ.).
3. Kumar N. Superfi cial Siderosis: A Clinical Review. Ann Neurol. 2021;89(6):1068–1079. doi: 10.1002/ana.26083
4. Meshkat S., Ebrahimi P., Tafakhori A., Taghiloo A., Shafiee S., Salimi A., Aghamollaii V. Idiopathic superficial siderosis of the central nervous system. Cerebellum Ataxias. 2021;8(1):9. doi: 10.1186/s40673-021-00133-5
5. Kumar N., Cohen-Gadol A.A., Wright R.A., Miller G.M., Piepgras D.G., Ahlskog J.E. Superfi cial siderosis. Neurology. 2006;66(8):1144–52. doi: 10.1212/01.wnl.0000208510.76323.5b
6. Hemptinne C., Coche A., Duprez T., Demaerel P., Raftopoulos C., Boschi A. Optic Neuropathy Revealing Severe Superficial Siderosis in the Setting of Long-standing Low-grade Intracranial Neoplasm. Neuroophthalmology. 2021;46(3):171–177. doi: 10.1080/01658107.2021.1958870
7. Kumar N. Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol. 2010;31(1):5–14. doi: 10.3174/ajnr.A1628
8. Ohira M., Takao M. Nationwide epidemiological survey of superficial hemosiderosis in Japan. J Neurol Sci. 2019;404:106–111. doi: 10.1016/j.jns.2019.07.019
9. [Suslin A.S., Kremneva E.I., Krotenkova I.A., Dreval M.V., Konovalov R.N., Sergeeva A.N., et al. Superficial siderosis of the central nervous system: case report. REJR. 2018;8(4):242–247. (In Russ.)]. doi: 10.21569/2222-7415-2018-8-4-242-247
10. Klocheva E.G., Goldobin V.V., Timonina O.I. A clinical case of the idiopathic superficial siderosis of the central nervous system. Neurological Bulletin (Nevrologicheskiy vestnik). 2015;47(2):77–81. (In Russ.)
11. Popova Yu.L., Volkova L.I. Idiopathic superfi cial siderosis of the central nervous system. Ural Medical Journal. 2015;10(133):64–65. (In Russ.)
12. Artukarslan E., Matin F., Donnerstag F., Gärtner L., Lenarz T., Lesinski-Schiedat A. Cochlea implantation in patients with superficial hemosiderosis. Eur Arch Otorhinolaryngol. 2022;279(9):4363–4370. doi: 10.1007/s00405-021-07198-2
13. Bonomo G., Cusin A., Rubiu E., Iess G., Bonomo R., Boncoraglio G.B. et al. Diagnostic approach, therapeutic strategies, and surgical indications in intradural thoracic disc herniation associated with CSF leak, intracranial hypotension, and CNS superficial siderosis. Neurol Sci. 2022;43(7):4167–4173. doi: 10.1007/s10072-022-06059-y
14. Kumar N. Superficial siderosis: associations and therapeutic implications. Arch Neurol. 2007;64(4):491–6. doi: 10.1001/archneur.64.4.491
15. Kondziella D., Lindelof M., Haziri D., Larsen V.A., Kruse A. Diagnostic and therapeutic challenges in superficial CNS siderosis. Dan Med J. 2015;62(5):A5079.
16. Yoo A., Jou J., Klopfenstein J.D., Kattah J.C. Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System. Front Neurol. 2018;9:358. doi: 10.3389/fneur.2018.00358
17. Sampaio L., Aires A., Garrett M.C., Pinto M. Superficial siderosis of the central nervous system. BMJ Case Rep. 2015;2015:bcr2015211957. doi: 10.1136/bcr-2015-211957
18. Lee S.Y., Lee D.H., Bae Y.J., Song J.J., Kim J.S., Koo J.W. Bilateral Vestibulopathy in Superficial Siderosis. Front Neurol. 2018;9:422. doi: 10.3389/fneur.2018.00422
19. Friedauer L., Rezny-Kasprzak B., Steinmetz H., du Mesnil de Rochemont R., Foerch C. Spinal dural leaks in patients with infratentorial superfi cial siderosis of the central nervous system — Refi nement of a diagnostic algorithm. Eur J Neurol. 2022;29(4):1136–1144. doi: 10.1111/ene.14611
20. Kessler R.A., Li X., Schwartz K., Huang H., Mealy M.A., Levy M. Two-year observational study of deferiprone in superficial siderosis. CNS Neurosci Ther. 2018;24(3):187–192. doi: 10.1111/cns.12792
21. Sammaraiee Y., Banerjee G., Farmer S., Hylton B., Cowley P., Eleftheriou P., et al. Risks associated with oral deferiprone in the treatment of infratentorial superfi cial siderosis. J Neurol. 2020;267(1):239–243. doi: 10.1007/s00415-019-09577-6
22. Kuo P.H., Kuo S.H., Lo R.Y. Deferiprone Reduces Hemosiderin Deposition in Superficial Siderosis. Can J Neurol Sci. 2017;44(2):219–220. doi: 10.1017/cjn.2016.329
23. Levy M. Ten years of iron chelation in a patient with superficial siderosis. Neurol Sci. 2019;40(9):1947–1949. doi: 10.1007/s10072-019-03791-w
Review
For citations:
Nuzhnyi E.P., Zaripova L.R., Fedotova E.Yu., Konovalov R.N., Filatov A.S., Moskalenko A.N., Illarioshkin S.N. Infratentorial superficial hemosiderosis. Russian neurological journal. 2024;29(3):58-65. (In Russ.) https://doi.org/10.30629/2658-7947-2024-29-3-58-65