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Baló's concentric sclerosis (BCS) is a rare, inflammatory demyelinating disease of the central nervous system (CNS). Historically, BCS was thought to be uniformly fatal and diagnosis was based on postmortem findings. With advances in modern neuroimaging, BCS is currently defined by the presence of concentric layered patterns composed of alternating rings of varying intensity. They are best appreciated on gadolinium-enhanced T1-weighted sequences and predominantly occur in the supratentorial cerebral white matter with sparing of cortical U-fibers. The lamellar pattern of the lesions likely reflects bands of demyelination and relative myelin preservation with minimal axonal loss. While BCS falls within the spectrum of atypical demyelinating diseases, there is ongoing debate over whether BCS is a phenotypical variant of multiple sclerosis (MS) or a separate entity. Corticosteroids comprise first-line therapy but there is ongoing controversy regarding appropriate maintenance therapy. First-line MS disease-modifying therapies such as interferon beta-1a are appropriate for patients who fulfill diagnostic criteria for relapsing-remitting MS. Fingolimod should likely be avoided as Baló-like lesions have been reported during its administration or after withdrawal. Monoclonal antibodies such as natalizumab and rituximab are potentially effective at reducing BCS relapses, but alemtuzumab may be relatively ineffective because humoral immunity does not play a central role in BCS pathogenesis., (Copyright © 2021 Elsevier B.V. All rights reserved.)

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.

Background: Baló's concentric sclerosis (BCS) is a rare subtype of tumefactive demyelinating disease with characteristic radiological and pathological features. In the medical literature, less than 10 BCS cases have been reported in the pediatric population., Case: We report the case of a 5-year-old boy who presented to the emergency department with 2 days of left-sided weakness. Magnetic resonance imaging (MRI) revealed 3 tumefactive lesions; further diagnostic studies included MRI spectroscopy, lumbar puncture and biopsy. A final diagnosis of Baló concentric sclerosis was made. He received intravenous methylprednisolone at 30 mg/kg for 5 days, plasma exchange treatment and immunoglobulin G course (2 g/kg/day). The patient was discharged in good condition and asymptomatic; after 8 months of follow-up, he has not presented with new symptoms., Conclusion: Baló's concentric sclerosis (BCS) is a rare variant of tumefactive demyelinating disease with only a handful of cases reported in the pediatric population. It poses a diagnostic challenge and therapeutic enigma, since it is difficult to distinguish from a central nervous system (CNS) neoplasm, infection or other CNS lesions on magnetic resonance imaging (MRI). Our case along with those reported in the literature, highlights the importance of considering BCS as a potential differential diagnosis when assessing tumefactive lesions. Distinguishing tumefactive demyelinating lesions from malignancy or infection is critical for proper patient management and to avoid unnecessary medical or surgical interventions., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Baló's concentric sclerosis (BCS) is considered a variant of inflammatory demyelinating disease closely related to multiple sclerosis characterized by a discrete concentrically layered lesion in the cerebal white matter. Khonsari and Calvez (Plos ONE. 2(2007)) proposed a parabolic-elliptic-ODE chemotaxis model for BCS which describes the evolution of the densities of activated macrophages, cytokine and apoptotic oligodendrocytes. Because "classically activated" M1 microglia can produce cytotoxicity, we introduce a linear production term from the activated microglia in the ODE for pro-inflammatory cytotoxic. For the new BCS chemotaxis model, we first investigate the uniform boundedness and global existence of classical solutions, and then get a range of the chemosensitive rate χ where the unique positive equilibrium point is exponentially asymptotically stable.

Introduction: Baló's concentric sclerosis (BCS) is a rare CNS disorder characterized by alternating bands of demyelination on MRI. One of the main issues is its relationship with multiple sclerosis (MS)., Objectives: To describe 6 BCS patients. To review the risk of developing MS in BCS patients., Methods: We retrospectively recorded clinical and radiological findings of 6 BCS patients and performed a review of the literature., Results: Six patients (5 women) with a mean age of 25 years old were included. Main symptoms were hemiparesis/hemihypoesthesia. On MRI, two patients had a single BCS lesion and four had additional MS-like lesions. Alternating bands were usually more visible on DWI. A patient had reduced central perfusion and SWI hypointensity suggestive of a central vein. Oligoclonal bands were identified in 5/6 patients. After 7 years of follow-up, all patients achieved MS criteria with mild disability (mean EDSS 1.75; 0-4). Our literature review included 65 BCS patients from 30 studies: although CSF oligoclonal bands and the presence of additional MS lesions were associated with subsequent relapses, this was not significant., Discussion/conclusion: Our series allows a detailed MRI description in BCS and gives a new insight into BCS evolution and its strong relationship with MS., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Acute disseminated encephalomyelitis (ADEM) has been linked to immunizations, viral infections, and occasionally to arthropods stings. Its association with scorpion stings has not been described. A 29-year-old man, who after being stung by a scorpion presented right hemiparesis and language impairment. Brain MRI showed subcortical bihemispheric lesion characteristics of ADEM and a bigger one compatible with Baló concentric sclerosis (BCS). Patient was managed with steroids, showing complete clinical improvement. Magnetic resonance imaging (MRI) after 6 months showed cavitation of the bigger lesion with no new findings. In 2 years of follow-up, there is no clinical relapse or new lesions in MRI. Although cases of ADEM have been reported due to venom of chilopoda and hymenoptera, the scorpion sting can be considered as another new cause; this case being even more relevant due to present a Baló-like lesion in MRI., Competing Interests: None

Background: Myelinoclastic diffuse sclerosis (MDS; also termed Schilder's disease) is a rare inflammatory demyelinating disorder of the central nervous system characterised by demyelination of vast areas of the white matter. It is unclear whether MDS is a variant of multiple sclerosis (MS) or a disease entity in its own right., Objective: To compare the cerebrospinal fluid (CSF) features of MDS with those of MS., Methods: Retrospective analysis of the CSF profile of all patients with MDS reported in the medical literature between 1960 and 2018., Results: The most striking finding was a substantial lack of oligoclonal bands (OCBs) in MDS, which were absent in at least 77% (30/39) of all lumbar punctures (LP) in the total cohort and in 86% in the subgroup of patients with normal very long-chain fatty acid serum ratios (VLCFA). Almost all cases published in the past 15 years were negative for OCBs. These findings are in contrast to MS, in which OCBs are present in up to 98% of cases (p < 0.00001 when compared with reference works in MS; both in adult and in pediatric patients). CSF pleocytosis was absent in at least 79% (46/58) of all LP (p < 0.0001 vs. MS) and in 92% (24/26) of LPs in the VLCFA-tested subgroup. CSF total protein levels were elevated in 56% of all LPs (p < 0.0001 vs. MS) and in 63% of LPs in the VLCFA-tested subgroup and were often higher than in typical MS (> 100 mg/dL in 13/22; up to 220 mg/dL). EBV serum antibodies, which are present in virtually all patients with MS, and the so-called MRZ (measles/rubella/zoster) reaction, a highly specific marker of MS, were absent in all of the few patients tested. In addition, we discuss further differences between MS and MDS, taking into account also Schilder's original comprehensive case description from 1912., Conclusion: In the majority of patients diagnosed with MDS, CSF features differ significantly from those typically found in MS and are more similar to those previously reported in patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-positive encephalomyelitis, aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders or Baló's concentric sclerosis. Our data suggest that MDS and MS are immunopathologically distinct entities in the majority of cases.

Aim: To study the clinical and biochemical features of atypical variants of multiple sclerosis (MS) (tumefactive demyelination (TD), Balo's concentric sclerosis (BCS)) and acute disseminated encephalomyelitis (ADEM))., Material and Methods: Forty-two patients were studied, including 32 patients with atypical variants of MS (6 patients with BCS and 26 patients with TD) and 10 patients with ADEM. The control group included 20 healthy volunteers. Clinical characteristics and EDSS scores were evaluated. Antibodies to aquaporin 1 (AQP1-IgG), aquaporin 4 (AQP4-IgG), antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) and aquaporin 1 (AQP1) in serum and cerebrospinal fluid (CSF) were detected using ELISA., Results and Conclusion: BCS and TD occurred both in isolation and comorbid with MS (in 50% of cases with BCS, 50% of cases with TD). Atypical symptoms of MS were detected in 50% of cases of CFS, 15.4% of cases of PD. The levels of CSF cytosis and CSF protein were not significantly different between the groups. The levels of AQP1-IgG, AQP4-IgG, AQP1, MOG-IgG in serum with BCS, TD and ADEM were significantly higher than in the control group. No significant differences were found between atypical variants of MS. A correlation between a high level of MOG-IgG and the EDSS score in BCS was shown. MOG-IgG may have a pathogenetic significance in BCS. Further studies of AQP1-IgG, AQP4-IgG and MOG-IgG in patients with atypical variants of MS are needed.

Balo's concentric sclerosis (BCS) is a rare demyelinating disease, first described by Hungarian neurologist Josef Balo in 1928. BCS occurs predominantly in young adults, the average age of onset of the disease - 34 years (range from 3 to 62 years). Our case report describes a 27-year woman with acute onset progressive right-side pyramidal weakness, MRI results showed a variant of demyelination as Balo's concentric sclerosis.

Balo's concentric sclerosis (BCS) is considered a variant of multiple sclerosis characterized by concentric lamella of alternating demyelinated and partially myelinated tissues. It is a rare and a relatively acute condition. Attacks may proceed rapidly over weeks or months, typically without remission, like Marburg's variant, resulting in death or severe disability. However, the majority of cases have a more benign, self-limiting course with spontaneous remission. Magnetic resonance imaging is a primary imaging modality in the diagnosis of BCS. Treatment with intense immunosuppression may be indicated in patients with more aggressive form. New reports reveal more evidence regarding the pathophysiology and treatment strategies.

Cocaine abuse may cause stroke, metabolic or multifocal inflammatory leukoencephalopathy. We described a patient with cocaine abuse who presented with Balo's type acute multifocal leukoencephalopathy. Magnetic Resonance Imaging (MRI) of the brain showed onion like patchy concentric ring enhancement on T1-weighted MRI with gadolinium. Balo's Concentric Sclerosis like radiological findings related to cocaine has not been reported. Levamisole is now frequently used as an ingredient in cocaine and may cause leukoencephalopathy. It is recommended to check urine levamisole levels in patients with cocaine-induced leukoencephalopathy with or without mimicking Balo's Concentric Sclerosis. On the other hand, it is also possible that the cocaine use was coincidental and this was a demyelinating case arising de novo in patient who uses cocaine., (Copyright © 2017 Elsevier B.V. All rights reserved.)

This article presents two clinical cases of patients diagnosed with Balo's concentric sclerosis. Distinctive features of the pathogenesis in the aspect of differential diagnosis from other forms of multiple sclerosis and possible treatment are discussed.

Background: The concentricity of BCS has captured wide attention; the findings of the current study may provide useful information on the centrifugal pathogenesis of BCS., Objective: This study aims to evaluate the performance of MRI, DWI and MRS in elucidating the pathogenesis of Balo's lesions expanding., Methods: Six clinically diagnosed BCS cases were reviewed, and the findings obtained by MRI, DWI and MRS were analyzed. DWI data were available for six patients, with the DWI and ADC imaging locations being central and peripheral layers of the index lesion. At TE 144ms, we calculated metabolite ratios of MRS at different depths of the demyelinating lesions and compared with the lesion on the opposite normal side for two patients., Results: The ADC values of 18 typical concentric lesions revealed that the central lesion had the highest ADC value, followed by the internal ring, and the outermost layer had the lowest ADC value. The reduction in NAA/Cr and the increase in Cho/Cr were more evident in the central lesion than in the internal and outermost ring., Conclusion: The findings of DWI and MRS indicate Balo's concentric rings develop gradually and centrifugally. Of course, this hypothesis remains to be proved by further experimental studies.

Balo's concentric sclerosis (BCS) is a rare demyelinating disorder of the central nervous system. The humanised monoclonal antibody alemtuzumab has shown efficacy in another demyelinating disorder, relapsing-remitting multiple sclerosis. We aimed to explore its efficacy in treatment-refractory BCS. A 52-year-old male with radiologically confirmed progressive BCS resistant to steroids, plasmapharesis and cyclophosphamide was administered a standard protocol of alemtuzumab. Treatment failed to slow his decline; he died 6 months after administration. Why alemtuzumab induced no clinical or radiological impact may be multifactorial. We review the evidence directing BCS therapy and propose the next steps for exploring this potentially fatal condition.

Baló's concentric sclerosis is a primary inflammatory central nervous system demyelinating disease that is considered a rare, radiographically and pathologically distinct variant of multiple sclerosis. Baló's concentric sclerosis is characterized by alternating rings of demyelinated and myelinated axons, and it is most frequently diagnosed postmortem by autopsy or, more recently, by magnetic resonance imaging without pathologic verification. This report is of a case of Baló's concentric sclerosis in which the patient presented with left-sided focal sensorimotor deficits. The patient's lesion demonstrated characteristics of Baló's concentric sclerosis by magnetic resonance imaging, but since a neoplastic process was also suspected initially, the patient underwent a surgical biopsy. This pathology sample now provides the opportunity to correlate the tissue diagnosis of demyelination with characteristic magnetic resonance imaging findings; this comparison is infrequently found in the literature.

Abstract: Baló''s concentric sclerosis (BCS) lesions display specific metabolite changes detected by magnetic resonance spectroscopy (MRS). We report on two cases of BCS lesions examined by MRS; the first case was evaluated 36 days after the onset of symptoms, whereas the second case was evaluated 9 days after the onset of symptoms. MRS data were obtained from single voxels located in the lesion and in the contralateral region. Relative to the creatine/phosphocreatine peak, BCS lesions displayed decreases of N-acetyl aspartate and increases of choline, myo-inositol (mI), glutamine/glutamate (Glx), lactate and lipid+macromolecule signals, in agreement with previous reports. In addition, previously unreported decreases of mI (−19% to −29%) and increases of Glx (+55% to +198%) were measured; these could be useful in characterizing BCS lesions. [Copyright &y& Elsevier]