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Evolution of Balo concentric sclerosis over time. Axial diffusion weighted imaging, apparent diffusion coefficient (ADC) map, and postcontrast T1-weighted images of the lesion acquired at presentation (Day 0), Day 67, and Day 252 show evolution of diffusion changes and contrast enhancement with time. Graph of relative ADC over time shows relatively reduced diffusion in the periphery compared to the central part of the lesion. This was most pronounced at presentation, decreased but still present at Day 67, and nearly resolved at Day 252.
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Balo concentric sclerosis (BCS) is a rare, atypical demyelinating disease, which may rapidly progress to become severe and fatal. Advanced neuroimaging has proven helpful for early diagnosis, classification, prognostication, and monitoring of progression in multiple sclerosis, but has not been fully explored in BCS. We present the case of a 27-year...
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Multiple sclerosis (MS) is a heterogenous condition with differences between patients regarding disease presentation, imaging features, disease activity, prognosis and treatment responses. Following the discovery of new biomarkers, the concept of MS has evolved, with syndromes previously considered to be its variants now recognised as separate enti...
Citations
... This result is similar to our case report in which T1-gadolinium lesion volume reduced from 8.23 mL (at baseline) to 0 mL in the subsequent follow-up visits. In accordance with a previously described case of BCS of increased choline from cellular membrane turnover and decreased NAA from neuronal injury in the MRS, our patient had a low baseline NAA + NAAG/Cr + PCr that has gradually and consistently improved at M6 and M12 [21] . ...
Balo's concentric sclerosis (BCS) is a rare subtype of multiple sclerosis. Advanced MRI metrics, such as magnetization transfer ratio (MTR), fractional anisotropy (FA), mean diffusivity (MD), and the ratio of total N-acetylaspartate concentration/total creatine concentration (tNAA/tCr) using proton magnetic resonance spectroscopy (¹H-MRS), are commonly used in research studies to investigate the effect of a disease modifying therapy (DMT). We report a patient diagnosed with BCS, receiving ocrelizumab, and provide a comparison of the lesion volume, T1-gadolinium lesion volume, MTR, FA, MD, and MRS metrics at baseline, 6- and 12-month follow-up. There was a reduction in Balo's lesion volume on fluid-attenuated inversion recovery (FLAIR) imaging observed in our patient from baseline (23.925 mL) to 12-month follow-up (2.391 mL), with the largest decrease from baseline to 6-month follow-up (3.650 mL). There was no T1-gadolinium enhancement seen at month 6 and 12. The MTR of the lesion did not change significantly (baseline = 50.9%, 6-month = 49.9%, 12-month =50.1%) but the FA increased from 0.188 (at baseline) to 0.304 (at 6 months), while the 12-month follow-up FA was 0.297. We also noted a reduction in MD from baseline (1.333 × 10⁻³ mm²/s) to 6-month follow-up (1.037 × 10⁻³ mm²/s), while the 12-month follow-up MD was 1.086 × 10⁻³ mm²/s. There was a 10.3% increase in tNAA/tCr from 1.583 (at month 0) to 1.747 (at month 12). Our results demonstrate for the first time a direct effect of ocrelizumab on BCS lesions. To validate our findings, more observations are needed in a larger group of BCS patients.
... To fit the gamma function to concentration-time curves, the range of the signal peak was initially specified in each curve [27]; then, using the Minimum Square Error method, Equation (3) was fitted to the extracted peak. Figure 2 shows one patient sample for the signal intensity curve in an arbitrary pixel, its conversion to concentration-time curve, and the gamma-fitted function. ...
This retrospective and exploratory study investigated the efficiency of the 3T perfusion magnetic resonance imaging (MRI) at the classification of MS lesion subtypes. For the MS lesion subtype classification, firstly, it was necessary to segment all MS lesions. Therefore, a Bayesian classifier based on the adaptive mixture method was used to segment all lesions, and an artificial neural network (ANN) employed a multi‐layer Perceptron as a subtype classifier. The Bayesian classifier accomplished the segmentation of lesions using Fluid Attenuated Inversion Recovery automatically, and the ANN part was used as a subtype classifier that worked based on extracted information from perfusion MRI (i.e. Mean Transit Time and Cerebral Blood Volume maps) along with the intensity information of the conventional multi‐channel MRI in segmented lesions. Adding 3‐Tesla perfusion MRI to the proposed model for the subtype classification led to an increment of about 7% and 13% in the sensitivity of acute and chronic lesion classifications, respectively. The sensitivity of T2 lesions did not meaningfully change. The overall accuracy of the classification for acute, chronic, and T2 lesion classifications was 96.1%, 90.5%, and 92.9%, respectively. The proposed architectures reached high sensitivity in discrimination between MS lesion subtypes when 3T perfusion MRIs were used.
... Acute lesions of BCS appear to be a result of type II hypersensitivity reactions carried out by T cells, B cells, macrophages, and other inflammatory factors [53,54]. The hypersensitivity reaction may be triggered by factors related to autoimmunity, infection, genetics, and the environment [3,13,[55][56][57]. Common pathologic features of demyelinated bands in Baló lesions include oligodendrocyte loss, hypertrophic astrocytes closely associated with oligodendrocytes, well-preserved axons, lipid-filled foamy macrophages, and scattered perivascular inflammatory infiltrates [13,17,30,58,59]. ...
... The sequential treatment approach of corticosteroids followed by customized second-line therapy has been uniformly reported in recent BCS literature [8,19,43,44,55,[72][73][74][75][76][77][89][90][91][92][93][94][95][96][97][98]. In the absence of highquality evidence, therapeutic modalities should be considered on a case-by-case basis. ...
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.
... Maybe the MRS technique will be of help in the further related study. Combining two or more fMRI tools can help with a comprehensive analysis of neuropathological changes from multiple perspectives (Yeo et al., 2018). That is called multimodal fMRI analysis technique. ...
Tinnitus refers to sound perception in the absence of external sound stimulus. It has become a worldwide problem affecting all age groups especially the elderly. Tinnitus often accompanies hearing loss and some mood disorders like depression and anxiety. The comprehensive adverse effects of tinnitus on people determine the severity of tinnitus. Understanding the mechanisms of tinnitus and related discomfort may be beneficial to the prevention and treatment, and then getting patients out of tinnitus distress. Functional magnetic resonance imaging (fMRI) is a powerful technique for characterizing the intrinsic brain activity and making us better understand the tinnitus neural mechanism. In this article, we review fMRI studies published in recent years on the neuroimaging mechanisms of tinnitus. The results have revealed various neural network alterations in tinnitus patients, including the auditory system, limbic system, default mode network, attention system, and some other areas involved in memory, emotion, attention, and control. Moreover, changes in functional connectivity and neural activity in these networks are related to the perception, persistence, and severity of tinnitus. In summary, the neural mechanism of tinnitus is a complex regulatory mechanism involving multiple networks. Future research is needed to study these neural networks more accurately to refine the tinnitus models.
... 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 also be applied in order to differentiate BCS from CNS neoplasms [9][10][11][12]. However, in addition to the typical BCS mass lesion on MRI, other MS-like plaques can also coexist [13]. ...
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.
With the recent advancement in computer technology, we can extract the picture of the brain as a network. The aim of this study is to constructs large scale individual anatomical brain networks using regional gray matter cortical thickness from individual subject’s magnetic resonance imaging (MRI) data, as well as to investigate changes with normal aging in global network organization. The dataset includes 183 healthy subjects sMRI data with an age range from 50 to 80 plus. For all brain networks, we calculated the global network measures and nodal network measures by using network analysis toolkit GRETNA. From global network measurements we calculated small-world measurements and network efficiency measurements, from nodal measurements we calculated node clustering coefficient (CC) and node efficiency at a wide-range of threshold values. All small world measurements showed more clustering at all the given threshold values than random networks and a alike least path length, indicative of that they were “small world”. To analyze the effect normal ageing on networks organization, the networks of subjects were categorized into three age groups (50s, 60s, and 70 over). The global and nodal network measurements of each group were statistically analyzed to investigate the significant difference in network organization with in age groups. Results shows that the age has no significance effect in global measurements of brain network. However, by analysis the nodal measures of brain network between age group, network nodes from brain frontal lobe and temporal lobe showed age related significant difference. The results obtained from the proposed study suggest that this network method can deliver a concise network-level picture of brain organization and be used from the outlook of composite networks to investigate inter-individual variability in brain morphology.
In the last decades, some atypical demyelinating diseases of the central nervous system have been better described and differentiated from classical phenotypes of multiple sclerosis. These conditions have been more precisely defined over time based on clinico-radiological and laboratory criteria, such as in the case of neuromyelitis optica. However, other entities still remain not fully understood, even though they have been known and studied for a long time. Additionally, the etiology of other recently described rare entities, such as solitary sclerosis or diffuse confluent or cavitary demyelinating lesions, has not yet been adequately clarified. In all these cases, the differential diagnosis remains difficult even with the use of advanced MRI techniques. A broad spectrum of knowledge of such conditions, as well as their main differential diagnoses, is essential to guide the diagnosis.
The aim of our study was to describe two cases of Balò’s Concentric Sclerosis (BCS) and further to review the state‐of‐the‐art literature. This dissertation is the result of a critical analysis matching the literature and our professional experience. Data were synthesized into a narrative review. BCS is often referred to as a variant of Multiple Sclerosis (MS), it is still unclear whether BCS is an acute variant of MS or a distinct entity that happens to coexist with MS. BCS and MS‐like lesions might be present at the same time. BCS lesions are characterized by large concentric “onion‐like” shape on MRI T2‐weighted images composed of alternating hypointense and hyperintense layers. On contrast‐enhanced T1‐weighted images, BCS active lesions usually show enhancing and non‐enhancing pattern. The advancing edge of demyelination could be represented by peripheral restricted diffusion and contrast enhancement. Balo’s lesions is mainly found in the supratentorial white matter, however the cerebellum, the brainstem, and the spinal cord might be affected as well. Our two cases both show onion‐like lesions, case 1 demonstrates typical BCS features while case 2 is atypical and could be classified as probably BCS or as a BCS‐like lesion in the course of MS. It is important for radiologists to be able to recognize this type of lesions and to cooperate with clinicians in order to help them perform earlier diagnosis and treatment, determining a better clinical outcome for patients affected by BCS even without histological confirmation, still considered the gold standard.
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.
