Abstract
BackgroundBaló’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.MethodsWe present a retrospective study of eight treated patients with BCS-type lesions, emphasizing on MRI characteristics and differences on therapeutic maneuvers.ResultsData 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).ConclusionsOur 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.
Highlights
Baló’s Concentric Sclerosis (BCS) is a rare heterogeneous demyelinating disease with a variety of phenotypes on Magnetic Resonance Imaging (MRI)
Baló’s concentric sclerosis (BCS) is a rare demyelinating disease, histopathologically characterized by large concentric lesions with circumferential rings of myelin loss alternating with rings of myelin preservation
Other typical radiological features include enhancement of the active layers of demyelination on contrast-enhanced T1-weighted images and diffusion restriction usually in the outermost rings on DWI [7]. These imaging findings are useful in the differential diagnosis of BCS from other tumefactive or atypical central nervous system (CNS) lesions e.g. neoplasms, vasculitis [8]; advanced MRI techniques such as Magnetic Resonance Spectroscopy, Diffusion Tensor Imaging and Arterial Spin Labeling can be applied in order to differentiate BCS from CNS neoplasms [9,10,11,12]
Summary
Baló’s Concentric Sclerosis (BCS) is a rare heterogeneous demyelinating disease with a variety of phenotypes on Magnetic Resonance Imaging (MRI). Other typical radiological features include enhancement of the active layers of demyelination on contrast-enhanced T1-weighted images and diffusion restriction usually in the outermost rings on DWI [7]. These imaging findings are useful in the differential diagnosis of BCS from other tumefactive or atypical CNS lesions e.g. neoplasms, vasculitis [8]; advanced MRI techniques such as Magnetic Resonance Spectroscopy, Diffusion Tensor Imaging and Arterial Spin Labeling can be applied in order to differentiate BCS from CNS neoplasms [9,10,11,12]. Our analysis suggests distinct BCS subgroups with different responses to treatment
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