Heterogeneity of Baló’s concentric sclerosis: a study of eight cases with different therapeutic concepts

Abstract

Background Baló’s Concentric Sclerosis (BCS) is a rare heterogeneous demyelinating disease with a variety of phenotypes on Magnetic Resonance Imaging (MRI). Existing literature lacks data especially on the therapeutic approach of the disease which we intended to elucidate by means of suggesting a new possible BCS classification and introducing different therapeutic concepts based on each BCS-subgroup characteristics. Methods We present a retrospective study of eight treated patients with BCS-type lesions, emphasizing on MRI characteristics and differences on therapeutic maneuvers. Results Data analysis showed: at disease onset the BCS-type lesion was tumefactive (size ≥2 cm) in 6 patients, with a mean size of 2.7 cm (± 0.80 SD); a coexistence of MS-like plaques on brain MRI was identified in 7 patients of our cohort. The mean age was 26.3 years (±7.3 SD) at disease onset and the mean follow-up period was 56.8 months (range 9–132 months). According to radiological characteristics and response to therapies, we further categorized them into 3 subgroups: a) Group-1; BCS with or without coexisting nonspecific white matter lesions; poor response to intravenous methylprednisolone (IVMP); treated with high doses of immunosuppressive agents (4 patients), b) Group-2; BCS with typical MS lesions; good response to IVMP; treated with MS-disease modifying therapies (2 patients), c) Group-3; BCS with typical MS lesions; poor response to IVMP; treated with rituximab (2 patients). Conclusions Our study introduces a new insight regarding the categorization of BCS into three subgroups depending on radiological features at onset and during the course of the disease, in combination with the response to different immunotherapies. Immunosuppressive agents such as cyclophosphamide are usually effective in BCS. However, therapeutic alternatives like anti-CD20 monoclonal antibodies or more classical disease-modifying MS therapies can be considered when BCS has also mixed lesions similar to MS. Future studies with a larger sample size are necessary to further establish these findings, thus leading to better treatment algorithms and improved clinical outcomes.

Clinico-radiologic features and therapeutic strategies in tumefactive demyelination: a retrospective analysis of 50 consecutive cases

“…confirmed MS) were also included. We excluded patients with Balo-like lesions, as they represent a distinct clinical and radiological disease entity described by our research team, 19 as well as pediatric MS patients, and patients with acute demyelinating encephalomyelitis and neuromyelitis optica (NMO). Other exclusion criteria were the presence of peripheral nervous system involvement, other autoimmune comorbidities or systemic diseases, prior exposure to radiation, and radiological or clinical data indicative of an ensuing neoplastic or paraneoplastic process.…”

Current and Future Developments in Imaging and Treatment of White Matter Disease: A Systematic Review

“…Very uncommon demyelinating disease Baló's concentric sclerosis (BCS) might show up on MRI with a broad variety of symptoms. Separated into three groups, BCS was systematically based on radiological characteristics and treatment outcomes [ 28 ]. At the beginning of the disease, six patients showed 2.7 cm in diameter tumefactive BCS lesions, and seven patients had concurrent MS-like plaques on brain MRI.…”

Immunopathology of Tumefactive Demyelinating Lesions-From Idiopathic to Drug-Related Cases

“…Hardy et al, presented an interesting case with coexisting Baló lesion and a tumefactive demyelinating lesion without evidence of other more typical demyelinating lesions ( 67 ). BCS can occur in patients with otherwise typical MS ( 68 ). Clinical work from research group recently pointed to clinical heterogeneity subgrouping patients in those that share common features with MS (OCBs, MS-type lesions, relapses, humoral responses) and those with a more aggressive disease requiring higher immunosuppression that might be characterized by a different underlying pathology ( 68 ).…”