Concentric Sclerosis Research Articles

Baló concentric sclerosis: Literature review and report of two cases

BackgroundBaló's concentric sclerosis (BCS) is a rare variant of multiple sclerosis characterized by unique pathological features of alternating demyelination and preserved myelin. ObjectivesTo describe two cases of BCS, radiological and pathological findings and its clinical course. ResultsWe report two distinct cases of BCS that presented with unique MRI findings suggestive of BCS, but with different clinical courses and responses to treatment. The first case demonstrated substantial recovery following corticosteroid therapy, while the second case, initially suspected to be a malignant tumour, showed improvement after surgical intervention and immunoglobulin therapy. ConclusionThese cases highlight the variability in presentation and course of BCS, underscoring the challenges in diagnosis and the importance of considering BCS in the differential diagnosis of demyelinating and tumefactive lesions. The cases also emphasize the potential for favourable outcomes with appropriate management, challenging the traditional view of BCS as uniformly severe.

A rare case of the late onset of Balo concentric sclerosis

A rare case of the late onset of Balo concentric sclerosis

Modern ideas about concentric sclerosis Balo: a clinical case

Balo's concentric sclerosis (BCS) is a rare demyelinating disease of the central nervous system, characterized by concentric areas of demyelination alternating with areas of preserved myelin in the form of a pathognomonic pathomorphological substrate of varying degrees of severity in the form of «annual rings on a tree cut». The pathogenesis of BCS is diverse and complex, today there are several theories of its occurrence, among which hypoxic, vascular and closely related genetic, as well as a number of other theories are distinguished. The BCS clinic is variable, represented by both focal neurological manifestations and general cerebral symptoms. The diagnosis is based on the data of magnetic resonance imaging of the brain. The drugs of choice for BCS therapy are glucocorticosteroids (prednisolone or methylprednisolone).

Intensive receptor blockade and plasma exchange to treat refractory scleroderma renal crisis in patients with agonistic autoantibodies targeting the angiotensin II type 1 and endothelin-1 type A receptors

Scleroderma renal crisis is a rare complication of systemic sclerosis characterized by a rapid decline in kidney function due to acute renal vascular injury. Recently, activating autoantibodies targeting the angiotensin II type 1 receptor and the endothelin-1 type A receptor have been implicated in the pathophysiology of scleroderma renal crisis by sensitizing the angiotensin II type 1 receptor and endothelin-1 type A receptor in renal resistance arteries to their natural ligands. Here, we describe a cohort of 10 patients with scleroderma renal crisis refractory to standard treatment, including blockade of the renin-angiotensin system. Multimodal therapy was initiated, targeting at the removal of anti-angiotensin II type 1 receptor and anti-endothelin-1 type A receptor autoantibodies by plasma exchange and the reduction of vasoconstrictive activity. Further treatment options included angiotensin II type 1 receptor and endothelin-1 type A receptor blockade, iloprost, intravenous immunoglobulins, and immunosuppression. Six patients were hypertensive. On kidney biopsy, concentric intimal sclerosis was present in all patients, whereas acute vascular injury was evident in eight. Levels of anti-angiotensin II type 1 receptor and anti-endothelin-1 type A receptor autoantibodies were significantly reduced by multimodal treatment. Kidney function improved in three patients with histological signs of severe acute renal vascular damage. This report demonstrates that intensive multimodal therapy taking account of potentially pathogenic anti-angiotensin II type 1 receptor and anti-endothelin-1 type A receptor autoantibodies in concert with other vasodilatory interventions provides a salvage option for patients with refractory scleroderma renal crisis.

Balo’s Concentric Sclerosis presenting as Sensorineural Hearing Loss (P14-3.007)

<h3>Objective:</h3> Multiple sclerosis (MS) can affect other cranial nerves apart from optic nerve. We report an interesting case of Balo’s concentric sclerosis (BCS) that presented with sensorineural hearing loss (SNHL). <h3>Background:</h3> BCS is a rare variant of MS. It derives its name from the concentric pattern of demyelination alternating with preserved myelin seen on magnetic resonance imaging (MRI). It typically has a fulminant monophasic clinical course with a fatal outcome although there has been an increasing number of cases reported with a benign clinical course. <h3>Design/Methods:</h3> NA <h3>Results:</h3> 48-year-old presented to our neuro-immunology clinic for evaluation of abnormal MRI which revealed non-enhancing T2 hyperintensities in juxta-cortical, peri-ventricular and infratentorial cerebellar lesions concerning for demyelination. He had tinnitus and SNHL of right ear ongoing for 3–4 months prior to presentation. Extensive workup was pursued to rule out MS mimics including nutritional, autoimmune, and infectious etiologies. LP revealed no evidence of oligoclonal bands. He was originally diagnosed with radiologically isolated syndrome with plans to monitor with MRI brain annually for surveillance. Repeat MRI brain a year later revealed new non-enhancing concentric juxta-cortical lesion along the right frontal operculum exhibiting a laminated appearance with surrounding perilesional edema consistent with BCS. He had no new neurological symptoms in the interim. On re-evaluation, his auditory symptoms were deemed as a clinical event, and he was diagnosed with MS with subsequent initiation of disease modifying therapy. <h3>Conclusions:</h3> BCS may occur simultaneously with MS like lesions or may herald the onset of MS. SNHL is a rare manifestation of MS with demyelinating lesion along the course of vestibulocochlear nerve or nucleus in brain stem. MRI brain can fail to reveal signs of demyelination either due to timing of MRI brain from symptom onset or sub-optimal diagnostic accuracy as vestibular evoked myogenic potentials have detected demyelinating lesions which are invisible on MRI brain. <b>Disclosure:</b> Dr. Potluri has nothing to disclose. Dr. Samaniego has nothing to disclose. Dr. Lannen has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Genentech. Dr. Lannen has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for TG Therapeutics.

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. &#x0D; 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.

Teaching Neuro Images : Acute neurologic deficits due to Baló concentric sclerosis

A previously healthy 40-year-old man developed acute left-sided weakness and numbness that progressed over 2 weeks. Physical examination revealed decreased sensation to pinprick in the left arm and leg and left leg weakness. MRI supported a diagnosis of Baló concentric sclerosis (BCS), a rare demyelinating disease (figure). The concentric bands of differing intensities on MRI are due to alternating layers of normal and demyelinated tissue that is classically observed with BCS.1,2 Patients with BCS commonly present with acute neurologic symptoms, which can be due to a solitary lesion. The clinical course can be fulminant and progressive or benign. In the case presented, the patient's symptoms improved within 1 week of starting high-dose glucocorticoids.

Marburg Variant of Multiple Sclerosis: A Case Report

Multiple sclerosis (MS) is a demyelinating disorder with central nervous system (CNS) involvement, leading to varied presentations. It is an autoimmune disease characterised by chronic inflammation, demyelination, gliosis (plaques or scarring) and neuronal loss with relapsing or progressive course. MS plaques typically develop at different times and in different CNS locations (i.e., MS is said to be disseminated in time and space). The clinical course is extremely variable, ranging from a relatively benign condition to a rapidly evolving and incapacitating disease requiring profound lifestyle adjustments. MacDonald’s criteria are frequently used to diagnose MS. Rapidly, evolving severe forms of MS such as Marburg’s variant and Balo’s concentric sclerosis account for &lt;4% of total incidence of MS. The clinical course is rapid and often fatal. The exact aetiology behind this malignant nature of Marburg’s variant MS is unknown. In this case report, we will be discussing one such rare presentation of Marburg variant of MS.

Tumefactive Balo-Like Concentric Sclerosis in Treatment with Fingolimod

Tumefactive Balo-Like Concentric Sclerosis in Treatment with Fingolimod

Knowledge and awareness on Balo's disease among dental students: A survey.

Balo's disease is an uncommon central nervous system disorder causing demyelination and is a form of multiple sclerosis. Concentric sclerosis, leukoencephalitis periaxialis concentrica, is also known as Balo's disease. Concentric sclerosis signalizes the bands of intact myelin and alternating rings of myelin loss present in several parts of the brainstem and brain. The present study aimed to assess and create awareness about Balo's disease among dental undergraduates. The study included an online survey about the knowledge and awareness of Balo's disease among dental students. A questionnaire was prepared, uploaded, and distributed in an online portal. The latest software version of SPSS was used to analyze the collected data. Descriptive statistical analysis and Chi-square test were done, and the P value was calculated. The survey showed that 54.55% of males and 45.45% of females participated in this survey. Most of the respondents (87.88%) knew about Balo's disease, whereas only 12.12% of participants were not aware of the disease. It was found that most of dental students are aware of Balo's disease. The study also showed that male respondents are more aware than the females.

Balo's concentric sclerosis or multiple sclerosis? A demyelinating disease in a 36‐year‐old woman

Balo's concentric sclerosis or multiple sclerosis? A demyelinating disease in a 36‐year‐old woman

Fulminant Balo Disease.

Fulminant Balo Disease.

Increasing role of imaging in differentiating MS from non-MS and defining indeterminate borderline cases.

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 entities, including aquaporin-4 (AQP4)-antibody (Ab) neuromyelits optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG)-Ab disease (MOGAD). In line with their distinct pathology, the newly emerging conditions have imaging characteristics which are dissimilar to typical MS. Progress in reclassifying such demyelinating CNS conditions has highlighted the challenge in meaningful categorisation of atypical presentations at the borders of MS, such as antibody-negative neuromyelitis optica-like syndromes, tumefactive demyelinating lesions, or Balo's concentric sclerosis. In this review, we discuss the increasing role of imaging in distinguishing MS from non-MS CNS inflammatory/demyelinating conditions and defining undetermined borderline cases. This progress relies both on better characterisation of imaging features of these conditions on conventional imaging in terms of their appearance and location, as well as on the implementation of novel image acquisition and/or post-processing techniques allowing for more in-depth lesion assessment, including the presence of a central vein sign or paramagnetic rim.

Концентричний склероз Бало: науковий огляд і особисте спостереження

Концентричний склероз Бало (КСБ) — рідкісне демієлінізуюче захворювання, що характеризується появою як специфічного патоморфологічного субстрату концентричних вогнищ демієлінізації за типом кілець на спилі дерева або зрізі цибулини з шарами ремієлінізації і демієлінізації, що чергуються. Патогенез КСБ вірогідно відповідає варіанту дистальної олігодендроцитопатії. Також висловлюються припущення про гіпоксичний принцип причинності демієлінізуючих уражень при КСБ і розсіяному склерозі. Справжня частота КСБ невідома, проте вважається, що ця патологія належить до рідкісних захворювань нервової системи з дещо більшою поширеністю в країнах Азії. Більшість випадків захворювання припадає на вік 20–50 років. Симптоматика найчастіше розвивається підгостро, протягом декількох днів або тижнів. Клінічні прояви КСБ зазвичай представлені вираженою вогнищевою неврологічною симптоматикою (пірамідні, мозочкові симптоми, ретробульбарний неврит, когнітивні порушення) у поєднанні із загальномозковою (головний біль, епілептичний синдром), часто з когнітивним дефіцитом. У цереброспінальній рідині при КСБ можуть виявлятися невеликий лімфоцитарний плеоцитоз, нормальний рівень білка, облігатні для розсіяного склерозу олігоклональні IgG відзначаються в 10 % хворих. До типових нейровізуалізаційних ознак цієї патології при проведенні магнітно-резонансної томографії відносять концентричні кільця, що нагадують завитки або спил дерева, на Т2- і Т1-зважених зображеннях із чергуванням структур, змінених і незмінених у відповідному режимі MP-сигналу. У лікуванні КСБ зазначаються дані лише IV класу доказовості. Основними методами вибору терапії цієї патології є: пульс-терапія глюкокортикостероїдними препаратами, високооб’ємний плазмаферез або цитостатики (мітоксантрон). У статті подано клінічний розбір діагностики цієї патології в чоловіка 52 років і результати секційного дослідження.

Classical Onion Bulb-Like Appearance in Balo's Concentric Sclerosis.

Classical Onion Bulb-Like Appearance in Balo's Concentric Sclerosis.

Balo’s concentric sclerosis mimicking an intracranial space occupying lesion in a Nigerian adult male: A case report

Balo’s concentric sclerosis (BCS) is a rare demyelinating disease often classified as a variant of multiple sclerosis. Typically, brain MRI shows concentric lamellae of demyelination alternating with normal zones of myelin preservation. Given its presentation with mass effect, it may be mistaken for other intracranial space occupying lesions (ICSOL), especially in our sub-region were neuroimaging facilities are scarce or not easily affordable. To the best of the authors' knowledge, this is the first reported case of BCS in sub-Saharan Africa. Aims: We report the management and early outcome of a young Nigerian man managed for BCS in our hospital.

Baló Concentric Sclerosis: Clinical, Radiologic, and Pathologic Observations

Balo concentric sclerosis (BCS) is a rare demyelinating disease with a variable prognosis. Researchers retrospectively evaluated radiologic, clinical,

Spotlight on the July 27 Issue

#### Notable in Neurology This Week This issue features an article that compares the rate of remission associated with different surgical procedures for thymectomy in patients with myasthenia gravis; another describes the clinical, radiologic, and pathologic features of patients with Baló concentric sclerosis. A Historical Neurology article looks at the role of microbiologist Clarence Joseph Gibbs on the adoption and use of the “Creutzfeldt-Jakob disease” eponym. ### Accuracy of a Deep Learning System for Classification of Papilledema Severity on Ocular Fundus Photographs ![Graphic][1]</img> Patients with severe papilledema from raised … [1]: /embed/inline-graphic-1.gif

Speech Disorders Revealing Balo's Concentric Sclerosis: A Case Report

Introduction: Balo's concentric sclerosis (BCS) is a particular and rare demyelinating disease of the white matter of the brain that was first described by J. Balo in 1928 and considered as a rare variant of multiple sclerosis. The clinical manifestations usually consist of aphasia, behavioral disturbances, and seizures. This term results from a pattern of concentric aspect of the lesions with alternating areas of demyelination and areas of normal myelin in various parts of the brain and spinal cord. BCS was described as monophasic, progressive, and treated as a disease with a fulminant course. Case report: We report a case of 38 years old woman who developed slurring of speech with mild swallowing disorders for solid foods accompanied by vertigo. Her MRI showed “onion peel-like” demyelinating lesions over the subcortical areas of the left parietal lobe. Treatment given to the Patient was IV methylprednisolone followed by oral corticosteroids, with a favorable outcome without clinical recurrence after two years of follow-up. Conclusion: BCS is a rare disease with an unpredictable course, the pathognomonic brain MRI features help to secure the diagnosis. The clinical case described is interesting because of the rarity of this entity.

Balo's Concentric Sclerosis with Bilateral Cerebral Hemisphere Involvement

Balo's concentric sclerosis (BCS) is a variant of multiple sclerosis which differs in terms of clinical presentation, neuroimaging, and neuropathology. It is known for its characteristic “concentric ring” appearance on radioimaging. We present an interesting case of BCS with bilateral cerebral hemisphere involvement.