Concentric Sclerosis Research Articles

Comment: Ischemia or demyelination—or both?

Vascular pathology and, more specifically, compromised capillaries have long been implicated in the pathogenesis of Balo concentric sclerosis (BCS).1 More recently, hypoxia-like tissue injury and corresponding counter-regulatory mechanisms, such as upregulation of heat shock proteins and HIF1α in oligodendrocytes, have been proposed as underlying the conspicuous concentric lesion formation.2 And although classic BCS is clearly …

Baló's Concentric Sclerosis in Multiple Sclerosis

Baló's Concentric Sclerosis in Multiple Sclerosis

A rare case of Balo concentric sclerosis showing unusual clinical improvement and response with oral prednisolone

The present report is a rare case of Balo Concentric Sclerosis. Most cases have either been diagnosed post mortem or have succumbed to the disease after being diagnosed ante mortem. In our case, the patient showed a dramatic response to treatment, and after a one-year follow-up, he was asymptomatic, with no relapses or residual effect of the illness.

Spotlight on the November 8 Issue

#### Notable in Neurology This issue features an article that identifies a KCNA2 mutation that caused dominantly inherited episodic ataxia in association with mild infantile-onset seizures, as well as generalized and focal epilepsies, and another article that clarifies the pathogenic factors and mechanisms underlying the development of concentric demyelinating lesions in Balo disease. Another featured article examines the utility and reliability of volumetric MRI in measuring disease progression in the 4-repeat tauopathies progressive supranuclear palsy and corticobasal syndrome. ### Alemtuzumab improves preexisting disability in active relapsing-remitting MS patients![Graphic][1] ![Graphic][2] The authors characterized the effects of alemtuzumab treatment on … [1]: /embed/inline-graphic-1.gif [2]: /embed/inline-graphic-2.gif

Pitfalls in the diagnosis of a tumefactive demyelinating lesion: A case report

IntroductionIn rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. We report a patient with a space-occupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as Balo concentric sclerosis and a glioma. This case report highlights important diagnostic clues in the differential diagnosis of Balo concentric sclerosis.Case presentationA 20-year-old Caucasian woman with acute onset of left-sided weakness and numbness was admitted to hospital with neurologic signs of left-sided hemiparesis and hypoesthesia. Brain magnetic resonance imaging showed a mass lesion of abnormal signal intensity with concentric enhancing rings in the right parietal lobe, without perifocal edema. The characteristic concentric pattern detected on the magnetic resonance images was highly suggestive of Balo disease, and corticosteroids were administered. Evoked potentials, cerebrospinal fluid analysis, and magnetic spectroscopy findings were not specific, and glioma was also included in the differential diagnosis. A stereotactic biopsy was not diagnostic.After one month the patient showed moderate clinical improvement, and during 12 months follow-up, no further relapses occurred. In the follow-up magnetic resonance imaging, the concentric pattern had completely disappeared, and only a low-signal, gliotic lesion remained.ConclusionWe hope this case presentation will advance our understanding of clinical and radiologic appearance of Balo concentric sclerosis, which is a rare demyelinating disease. Although this is a specific entity, it has a broader clinical impact across medicine, because it must be differentiated from other space-occupying lesions in the central nervous system.

High-dose steroid treatment in a patient with Balò disease diagnosed by means of magnetic resonance imaging.

Balò disease, also called concentric sclerosis, is a rare variant of multiple sclerosis that has been historically characterized as a monophasic, rapidly progressive, often fatal demyelinating disease. The diagnosis of Balò disease is based on the pathognomonic concentric ring pattern seen on magnetic resonance images. The authors describe the case of a 30-year-old woman with Balò disease who was initially treated with an unconventionally high dose of steroids and had a positive long-term clinical outcome with near-complete resolution of her neurologic symptoms. The authors also report MRI findings of concentric lesions before steroid treatment and resolution of the lesions shortly after and several months after steroid treatment. Given the outcome in this patient, we propose using high doses of steroids in the early treatment of patients with Balò disease. However, further studies are needed.

Balo′s concentric sclerosis involving bilateral thalami

Balo's concentric sclerosis (BCS) is a rare inflammatory demyelinating disease of central nervous system, pathologically characterized by alternate bands of demyelination and preserved myelin tissue. Before the era of magnetic resonance imaging (MRI), most cases of BCS were diagnosed on postmortem examination. MRI allows for noninvasive diagnosis by demonstrating characteristic changes which closely parallels the histopathological features of BCS. We report a case of 26-year-old female with BCS involving bilateral thalami, with typical MRI appearance.

Diagnostic Role of Target Lesion on Diffusion-Weighted Imaging

Target lesions on diffusion-weighted imaging are uncommon and their significance not well appreciated. To assess the diagnostic value of this neuroimaging finding, a case of cerebral aspergillosis is presented and the literature reviewed. The diffusion-weighted magnetic resonance imaging features of target lesions in a case of cerebral aspergillosis with neuropathologic correlate is presented and 8 other cases manifesting this neuroimaging finding are reviewed for etiology, patient immune status, lesion number, enhancement, and location. The etiologies included cerebral aspergillosis, Balo concentric sclerosis, and acute necrotizing encephalopathy. The cerebral aspergillosis cases were immunocompromised with multiple lesions in 4 of 5 patients. The acute necrotizing encephalopathy and Balo concentric sclerosis patients were immunocompetent with bilateral thalamic lesions in the former and multiple random or solitary lesions in the later. Enhancement was seen in 5 patients. Target lesions on diffusion-weighted imaging are compelling for a diagnosis of cerebral aspergillosis in immunocompromised patients and for acute necrotizing encephalopathy in immunocompetent patients when lesions are bilateral thalamic and Balo concentric sclerosis when white matter is involved.

Demyelinating lesions with features of Balo's concentric sclerosis in a patient with active hepatitis C and human herpesvirus 6 infection

Demyelinating lesions with features of Balo's concentric sclerosis in a patient with active hepatitis C and human herpesvirus 6 infection

Aquaporin-4 astrocytopathy in Baló’s disease

Baló's concentric sclerosis (BCS) is considered to be a rare variant of multiple sclerosis and characterized by alternating rings of demyelinated and preserved myelin layers. The mechanism underlying BCS remains to be elucidated. Recently, occurrence of concentric rings of Baló was described in the brainstem of a patient with neuromyelitis optica (NMO). Because selective loss of aquaporin-4 (AQP4) and vasculocentric deposition of complement and immunoglobulins are characteristic in NMO, we aimed to assess AQP4 expression in the concentric demyelinating lesions of BCS patients. We evaluated AQP4 expression relative to expression of another astrocytic marker (glial fibrillary acidic protein), the extent of demyelination, lesion staging and perivascular deposition of complement and immunoglobulin in four cases with BCS, and 30 individuals with other neurological diseases. All cases with BCS demonstrated extensive AQP4 loss in both demyelinated and myelinated layers of all actively demyelinating lesions, with perivascular lymphocytic cuffing of T cells, but no deposition of immunoglobulins or complement around vessels. These findings suggest that AQP4 loss occurs in heterogeneous demyelinating conditions, namely NMO and BCS. Furthermore, acute BCS lesions are characterized by extensive AQP4 loss without vasculocentric deposition of complement or immunoglobulin.

The pathological spectrum of CNS inflammatory demyelinating diseases

Inflammatory demyelinating diseases of the central nervous system (CNS) occur throughout the world and are the leading cause of nontraumatic neurological disability in young adults. They represent a broad spectrum of disorders that vary in their clinical course, regional distribution, and pathology. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. Multiple sclerosis (MS), the most common inflammatory demyelinating CNS disease affecting approximately one million adults, shares the basic pathological hallmark of CNS inflammatory demyelination. Advances based on recent systematic clinicopathologic-serologic correlative approaches have led to novel insights with respect to the classification of this disorder, the pathologic substrate of disability, a better understanding of the underlying pathogenic mechanisms involved in lesion formation, as well as the clinical relevance of cortical demyelination and normal appearing white matter pathology. In addition to prototypic MS, these diseases include Marburg variant of acute MS, Balo's concentric sclerosis, neuromyelitis optica, acute disseminated encephalomyelitis, and tumefactive MS. The last decade has seen a resurgence of interest in examining the lesions of these inflammatory demyelinating CNS disorders with newer and more sophisticated immunological and molecular tools. Herein, we review the clinicopathologic features of these CNS inflammatory demyelinating disorders and discuss recent advances in understanding their immunopathogenesis.

Spinal cord involvement in Balo's concentric sclerosis

We present a patient with a history of myelitis, who had a steroid refractory attack of CNS inflammatory demyelinating disease that developed into cerebral concentric sclerosis of Balo after plasma exchange. The acute inflammatory disease involved the spinal cord, a phenomenon rarely demonstrated. This patient fulfilled the McDonald criteria for multiple sclerosis. Plasmapheresis did not have a beneficial effect, but the patient stabilised at high EDSS after treatment with mitoxantron.

Balò′s concentric sclerosis presenting with benign clinical course and multiple sclerosis-like lesions on magnetic resonance images

Balò's concentric sclerosis (BCS) is a rare primary demyelinating disease of central nervous system (CNS) and is considered to be a variant of multiple sclerosis (MS). It is characterized by a severe, rapidly evolving course with CNS lesions consisting of concentric rings of demyelination alternating with myelination in the white matter. We report a patient with BCS from mainland China diagnosed on magnetic resonance imaging (MRI) findings. In addition to two contrast enhanced open ring lesions. MRI also showed multiple small white matter lesions in both the hemispheres. The patient had a good response to corticosteroids and had a benign prognosis during a follow-up period of two years.

Serial proton MR spectroscopy and diffusion tensor imaging in infantile Balo’s concentric sclerosis

IntroductionProton magnetic resonance spectroscopy (MRS) and diffusion tensor imaging (DTI) yield different parameters for characterizing the evolution of a demyelinating white matter disease. The purpose was to elucidate biochemical and microstructural changes in Balo’s concentric sclerosis lesions and to correlate the findings with the clinical course.MethodsLocalized short-echo time MRS and DTI were performed over 6 years in a left occipital lesion of a female patient (age at onset 13.8 years) with Balo’s concentric sclerosis. A right homonym hemianopsia persisted.ResultsMetabolite patterns were in line with initial active demyelination followed by gliosis and partial recovery of neuroaxonal metabolites. Fractional anisotropy and mean diffusivity of tissue water remained severely altered. Fiber tracking confirmed a disruption in the geniculo-calcarine tract as well as involvement of the corpus callosum.ConclusionMRS and DTI depict complementary parameters, but DTI seems to correlate better with clinical symptoms.

Mitochondrial defects in acute multiple sclerosis lesions

Multiple sclerosis is a chronic inflammatory disease, which leads to focal plaques of demyelination and tissue injury in the CNS. The structural and immunopathological patterns of demyelination suggest that different immune mechanisms may be involved in tissue damage. In a subtype of lesions, which are mainly found in patients with acute fulminant multiple sclerosis with Balo's type concentric sclerosis and in a subset of early relapsing remitting multiple sclerosis, the initial myelin changes closely resemble those seen in white matter stroke (WMS), suggesting a hypoxia-like tissue injury. Since mitochondrial injury may be involved in the pathogenesis of such lesions, we analysed a number of mitochondrial respiratory chain proteins in active lesions from acute multiple sclerosis and from WMS using immunohistochemistry. Functionally important defects of mitochondrial respiratory chain complex IV [cytochrome c oxidase (COX)] including its catalytic component (COX-I) are present in Pattern III but not in Pattern II multiple sclerosis lesions. The lack of immunohistochemically detected COX-I is apparent in oligodendrocytes, hypertrophied astrocytes and axons, but not in microglia. The profile of immunohistochemically detected mitochondrial respiratory chain complex subunits differs between multiple sclerosis and WMS. The findings suggest that hypoxia-like tissue injury in Pattern III multiple sclerosis lesions may be due to mitochondrial impairment.

Magnetic Resonance Imaging in Balo's Concentric Sclerosis

Concentric high signal intensity zones on T2-weighted magnetic resonance images of brain strongly suggest Balo's concentric sclerosis (BCS), a rare but recognized variant of multiple sclerosis. Differentiating BCS from multiple sclerosis or neoplasm can be difficult clinically. The pathognomonic MRI appearance can dramatically influence the course of the disease, allowing earlier diagnosis and therapy of the disease which was once considered to have an invariably fulminant and fatal course.

A note on X linked adrenoleukodystrophy (Addison–Schilder syndrome)

In one of the unsurpassed medical works of the nineteenth century,1 2 Thomas Addison (1793–1860) when investigating a “peculiar form of anaemia” described the classic symptoms of adrenal cortical failure...

Multiple sclerosis: Classification revisited reveals homogeneity and recapitulation

Multiple sclerosis: Classification revisited reveals homogeneity and recapitulation

Esclerose concêntrica de Baló – relato de caso

Objetivos: O caso descrito a seguir busca incentivar a suspeição do médico frente a um provável diagnóstico de Esclerose Concêntrica de Baló (ECB). Métodos:Relato de caso associado à revisão da literatura científica a partir artigos publicados sobre o tema. Resultados: Há grande dificuldade na determinação da etiopatologia da ECB, já que há amplas áreas de desmielinização do sistema nervoso central (SNC) que podem ser observadas em várias doenças neurológicas. A RNM faz grande suspeição diagnóstica, mas a confirmação só é realmente obtida através de biópsia cerebral. O tratamento consiste no uso de esteróides em doses altas com neuroimagem seriada de controle. Discussão: Uma vez determinada a natureza inflamatória da lesão, a dificuldade de determinar sua etiopatologia reside no fato de que amplas áreas de desmielinizaçãodo sistema nervoso central (SNC) podem ser observadas em várias doenças neurológicas.Clinicamente os pacientes com esclerose concêntrica de Balo se apresentam com sintomas agudos ou subagudos, sugerindo lesão expansiva encefálica. A doença tem um curso imprevisível, que varia de uma rápida evolução em semanas ou meses sem remissão até uma condição autolimitada que incluem vários sintomas cognitivos como cefaléia, afasia, distúrbios de comportamento, disfunção visual e ataxia.

Mathematical description of concentric demyelination in the human brain: Self-organization models, from Liesegang rings to chemotaxis

Baló’s concentric sclerosis (BCS) is an atypical variant of multiple sclerosis (MS) characterized by remarkable concentric demyelination patterns in the white matter of the brain and in the spinal chord. In Khonsari and Calvez (2006), we introduced a very simple model based on cellular self-organization, which reproduced quite accurately the quantitative and qualitative aspects of this disease. The present paper provides more details on the mathematical framework underlying our precedent work. We first review the basics of the chemotaxis aggregation principle and the modeling of the Liesegang rings (LR) phenomenon. We then present our local macrophages recruitment model, which derives from the reconsideration of the classical analogy between LR and BCS. This new model provides several non-obvious insights, such as the link between the level of aggressivity of the disease and the emergence of patterns.