Baló’s concentric sclerosis in the pediatric population: literature review and analysis of clinical cases in the context of follow up observation

Baló’s concentric sclerosis is a rare variant of atypical pseudotumor demyelination, having a pathogenetic similarity with multiple sclerosis and a characteristic neuroimaging pattern with relative clinical heterogeneity. In children and adolescents, Baló’s concentric sclerosis even rarer than in adults, is represented in the scientific literature by single descriptions of clinical cases with a polymorphic clinical phenotype and various anamnestic prerequisites (for example, infectious). Moreover, the published cases do not involve the analysis of a long-term clinical following up in children and adolescents with this pathology, possibly due to their transition to adult neurological services as they grow up. This sample does not allow the analysis of cases of Baló’s concentric sclerosis with pediatric debuts and answer questions about the clinical features and therapeutic needs for such patients. Especially a lot of questions have accumulated concerning approaches to the treatment of patients with the classical, mono-focal form of Baló’s concentric sclerosis. The lack of scientific and clinical information hinders the adoption of administrative decisions on prescribing drugs from the PITRS group (drugs that change the course of multiple sclerosis) to such patients. To replenish the literature base of pediatric cases of Baló’s concentric sclerosis, we analyzed the Russian experience of long-term (from 2 to 6 years) curation of three juvenile patients with acute non-infectious demyelinating episode and monofocal concentric lesion of the white matter of the brain. Russian data demonstrate the long-term efficacy and tolerability of drugs from the PITRS group among pediatric patients with Baló’s concentric sclerosis.

Contribution:
Bykova O.V. — writing the text;
Kudryavtseva O.I. — text editing;
Sarzhina M.N. — writing the text;
Batysheva T.T. — writing the text; final approval for the publication of the manuscript.
All co-authors are responsible for the integrity of all parts of the manuscript and approval of its final version.

Acknowledgements. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 16, 2023
Accepted: March 3, 2023
Published: April 20, 2023

1. Moore G.R., Neumann P.E., Suzuki K., Lijtmaer H.N., Traugott U., Raine C.S. Balo’s concentric sclerosis: new observations on lesion development. Ann. Neurol. 1985; 17(6): 604–11. https://doi.org/10.1002/ana.410170614

2. Xie J.S., Jeeva-Patel T., Margolin E. Baló’s concentric sclerosis – A rare entity within the spectrum of demyelinating diseases. J. Neurol. Sci. 2021; 428: 117570. https://doi.org/10.1016/j.jns.2021.117570

3. Pohl D., Rostasy K., Krone B., Hanefeld F. Baló’s concentric sclerosis associated with primary human herpesvirus 6 infection. J. Neurol. Neurosurg. Psychiatry. 2005; 76(12): 1723–5. https://doi.org/10.1136/jnnp.2004.062331

4. Hardy T.A., Miller D.H. Balo’s concentric sclerosis. Lancet Neurol. 2014; 13(7): 740–6. https://doi.org/10.1016/s1474-4422(14)70052-3

5. Garbern J., Spence A.M., Alvord E.C.Jr. Balo’s concentric demyelination diagnosed premortem. Neurology. 1986; 36(12): 1610–4. https://doi.org/10.1212/wnl.36.12.1610

6. Jarius S., Würthwein C., Behrens J.R., Wanner J., Haas J., Paul F., et al. Baló’s concentric sclerosis is immunologically distinct from multiple sclerosis: results from retrospective analysis of almost 150 lumbar punctures. J. Neuroinflammation. 2018; 15(1): 22. https://doi.org/10.1186/s12974-017-1043-y

7. Nader Kawachi J.A., Andrade Magdaleno Mde L., Peñaherrera C.A., Fernández De Lara Y., Lavenant Borja M.I. Early diagnosis of Balo’s concentric sclerosis by diffusion tensor tractography: a case report and literature review. Medwave. 2016; 16(2): e6402. https://doi.org/10.5867/medwave.2016.02.6402

8. Charil A., Yousry T.A., Rovaris M., Barkhof F., De Stefano N., Fazekas F., et al. MRI and the diagnosis of multiple sclerosis: expanding the concept of “no better explanation”. Lancet Neurol. 2006; 5(10): 841–52. https://doi.org/10.1016/S1474-4422(06)70572-5

9. Kavanagh E.C., Heran M.K., Fenton D.M., Lapointe J.S., Nugent R.A., Graeb D.A. Diffusion-weighted imaging findings in Balo concentric sclerosis. Br. J. Radiol. 2006; 79(943): e28–31. https://doi.org/10.1259/bjr/36636301

10. Spaander F.H., Vink T., Natté R., de Bruijn S.F. Onion-shaped white matter disease? Clin. Neurol. Neurosurg. 2014; 126: 123–5. https://doi.org/10.1016/j.clineuro.2014.08.024

11. Purohit B., Ganewatte E., Schreiner B., Kollias S. Balo’s concentric sclerosis with acute presentation and co-existing multiple sclerosis-typical lesions on MRI. Case. Rep. Neurol. 2015; 7(1): 44–50. https://doi.org/10.1159/000380813

12. Hardy T.A. Pseudotumoral demyelinating lesions: diagnostic approach and long-term outcome. Curr. Opin. Neurol. 2019; 32(3): 467–74. https://doi.org/10.1097/WCO.0000000000000683

13. Pique J., Bonneville F., Brassat D., Peaureaux D., Benaiteau M., Dumas H., et al. Peripheral late reactivation of a previously typical monofocal Baló’s concentric sclerosis lesion. Mult. Scler. 2015; 21(8): 1080–3. https://doi.org/10.1177/1352458515586087

14. Tzanetakos D., Vakrakou A.G., Tzartos J.S., Velonakis G., Evangelopoulos M.E., Anagnostouli M., et al. Heterogeneity of Baló’s concentric sclerosis: a study of eight cases with different therapeutic concepts. BMC Neurol. 2020; 20(1): 400. https://doi.org/10.1186/s12883-020-01971-2

15. Linnoila J., Chitnis T. Balo concentric sclerosis in children: a case series. J. Child. Neurol. 2014; 29(5): 603–7. https://doi.org/10.1177/0883073813517294

16. Kamari C., Galanakis E., Raissaki M., Briassoulis G., Vlachaki G., Vorgia P. Pediatric tumefactive multiple sclerosis case (with baló-like lesions), diagnostic and treatment challenges. Neurol. Sci. 2023; 44(1): 343–5. https://doi.org/10.1007/s10072-022-06396-y

17. Mowry E.M., Woo J.H., Ances B.M. Baló’s concentric sclerosis presenting as a stroke-like syndrome. Nat. Clin. Pract. Neurol. 2007; 3(6): 349–54. https://doi.org/10.1038/ncpneuro0522

18. Bhoi S.K., Naik S., Kalita J., Misra U.K. Multifocal Balo’s concentric sclerosis in children: report of a case and review of literature. J. Neurosci. Rural. Pract. 2017; 8(Suppl 1): S136–8. https://doi.org/10.4103/jnrp.jnrp_504_16

19. Karaarslan E., Altintas A., Senol U., Yeni N., Dincer A., Bayindir C., et al. Baló’s concentric sclerosis: clinical and radiologic features of five cases. AJNR Am. J. Neuroradiol. 2001; 22(7): 1362–7.

20. Losch H., Renneberg A., Pfleiderer S. Multifocal Balo’s concentric sclerosis in a 10-year-old German boy. Neuropediatrics. 2012; 43: PS17–09. https://doi.org/10.1055/s-0032-1307135

21. Dreha-Kulaczewski S.F., Helms G., Dechent P., Hofer S., Gärtner J., Frahm J. Serial proton MR spectroscopy and diffusion tensor imaging in infantile Balo’s concentric sclerosis. Neuroradiology. 2009; 51(2): 113–21. https://doi.org/10.1007/s00234-008-0470-y

22. Murakami Y., Matsuishi T., Shimizu T., Yamashita Y., Nagamitsu S., Kojima K., et al. Baló’s concentric sclerosis in a 4-year-old Japanese infant. Brain Dev. 1998; 20(4): 250–2. https://doi.org/10.1016/s0387-7604(98)00025-4

23. Popova E.V., Bryukhov V.V., Boyko A.N., Krotenkova M.V., Konovalova O.E., Sharanova S.N. Atypical multiple sclerosis – Balo’s concentric sclerosis: two case-reports and a review. Zhurnal nevrologii i psikhiatrii im. C.C. Korsakova. 2017; 117(2): 50–3. https://doi.org/10.17116/jnevro20171172250-53 EDN: https://elibrary.ru/yphwyh

24. Boyko A.N., Bykov O.V., Sivertseva S.A. Multiple Sclerosis in Children and Adolescents. Clinic, Diagnosis and Treatment [Rasseyannyy skleroz u detey i podrostkov. Klinika, diagnostika i lechenie]. Moscow: MIA; 2016: 113–20. (in Russian)

25. Zelada-Ríos L., Pacheco-Barrios K., Galecio-Castillo M., Yamunaqué-Chunga C., Álvarez-Toledo K., Otiniano-Sifuentes R. Acute disseminated encephalomyelitis and COVID-19: A systematic synthesis of worldwide cases. J. Neuroimmunol. 2021; 359: 577674. https://doi.org/10.1016/j.jneuroim.2021.577674

26. Krupp L.B., Tardieu M., Amato M.P., Banwell B., Chitnis T., Dale R.C., et al. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult. Scler. 2013; 19(10): 1261–7. https://doi.org/10.1177/1352458513484547