Balo's concentric sclerosis: An atypical demyelinating disease in pediatrics

Balo’s concentric sclerosis (BCS) is a rare demyelinating disease known as Multiple Sclerosis (MS) lesion type III. It is a disease of the white matter of the brain characterized by a round lesion with variable concentric myelinated and demyelinated layers, appearing as “onion bulb.” We present a case of BCS and discuss the imaging findings and management strategies of this disease. A 26-y-old male developed headache, weakness, and numbness of limbs. Magnetic resonance imaging (MRI) showed concentric lamellar like demyelinating lesions at the subcortical regions. The patient’s neurological symptoms were consistent with the MRI findings.

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Balo's concentric sclerosis: a clinical case

Neurology neuropsychiatry Psychosomatics ◽

10.14412/2074-2711-2018-3-97-102 ◽

2018 ◽

Vol 10 (3) ◽

pp. 97-102

Author(s):

I. V. Litvinenko ◽

G. N. Bisaga ◽

V. S. Chirsky ◽

R. F. Gimadutdinov ◽

P. S. Dynin ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Biopsy Specimen ◽

Clinical Case ◽

Demyelinating Disease ◽

Clinical Manifestations ◽

Brain Biopsy ◽

Diagnostic Value ◽

Pathological Process ◽

Baló’S Concentric Sclerosis ◽

Immunohistochemical Techniques

This paper describes a case of Balo's concentric sclerosis, a rare demyelinating disease of the central nervous system (CNS), which is currently classified as multiple sclerosis. In recent years, there has been a more favorable clinical course of Balo's sclerosis. The significant polymorphism of clinical manifestations of the disease, its neuroimaging pattern, and laboratory tests cause difficulties diagnosing this pathology. Its differentiation with CNS tumors presents a particular challenge. So it also happens in the described clinical case, when computed tomography revealed the signs of space-occupying lesion, the histological pattern of concentric focus biopsy specimen indicated the presence of protoplasmic astrocytoma. However, immunohistochemical analyses of the biopsy specimen, immunological examination of cerebrospinal fluid, as well as the typical magnetic resonance imaging changes of Balo's sclerosis could suggest the demyelinating nature of the pathological process. The article shows that immunohistochemical techniques for examining a brain biopsy specimen and immunological assays of blood and cerebrospinal fluid are of great diagnostic value.

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Heterogeneity of Baló’s concentric sclerosis: a study of eight cases with different therapeutic concepts

BMC Neurology ◽

10.1186/s12883-020-01971-2 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

D. Tzanetakos ◽

A. G. Vakrakou ◽

J. S. Tzartos ◽

G. Velonakis ◽

M. E. Evangelopoulos ◽

...

Keyword(s):

Demyelinating Disease ◽

Brain Mri ◽

Immunosuppressive Agents ◽

Disease Onset ◽

White Matter Lesions ◽

Poor Response ◽

Therapeutic Concepts ◽

The Mean ◽

Disease Modifying ◽

Baló’S Concentric Sclerosis

Abstract 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.

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7 Tesla MRI demonstrates vascular pathology in Baló’s concentric sclerosis

Multiple Sclerosis Journal ◽

10.1177/1352458512445302 ◽

2012 ◽

Vol 19 (1) ◽

pp. 120-122 ◽

Cited By ~ 16

Author(s):

M Berghoff ◽

MU Schlamann ◽

S Maderwald ◽

AE Grams ◽

M Kaps ◽

...

Keyword(s):

Demyelinating Disease ◽

Vascular Pathology ◽

7 Tesla ◽

Motor Deficits ◽

Off Label Use ◽

Lipid Turnover ◽

Severe Motor ◽

7 Tesla Mri ◽

Baló’S Concentric Sclerosis ◽

Underlying Pathology

Baló’s concentric sclerosis (BCS) is an inflammatory demyelinating disease related to multiple sclerosis; its underlying pathology remains unclear. At 7 T MRI in a 19-year-old female BCS patient, microhaemorrhages and ectatic veins were found in T2 hyperintense regions, features which have not been previously reported in conjunction with BCS, and these findings may support the view that vascular pathology plays a role in BCS. MRS data suggest that neuron loss and lipid turnover still took place months after a remission. Plasma exchange was effective in treating a relapse with severe motor deficits, and the off-label use of natalizumab was successful in maintaining remission in this patient.

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Therapeutic Plasma Exchange Application in Children Requires Individual Decision

Journal of Pediatric Intensive Care ◽

10.1055/s-0040-1714098 ◽

2020 ◽

Author(s):

Gürkan Atay ◽

Demet Demirkol

Keyword(s):

Plasma Exchange ◽

Vascular Access ◽

Pediatric Patients ◽

Demyelinating Disease ◽

Pediatric Intensive Care ◽

Therapeutic Plasma Exchange ◽

Transplantation Rejection ◽

Access Problems ◽

Uremic Syndrome

AbstractTherapeutic plasma exchange (TPE) is a treatment administered with the aim of removing a pathogenic material or compound causing morbidity in a variety of neurologic, hematologic, renal, and autoimmune diseases. In this study, we aimed to assess the indications, efficacy, reliability, complications, and treatment response of pediatric patients for TPE. This retrospective study analyzed data from 39 patients aged from 0 to 18 years who underwent a total of 172 TPE sessions from January 2015 to April 2018 in a tertiary pediatric intensive care unit. Indications for TPE were, in order of frequency, macrophage activation syndrome (28.2%, n = 11), renal transplantation rejection (15.4%, n = 6), liver failure (15.4%, n = 6), Guillain–Barre's syndrome (15%, n = 6), hemolytic uremic syndrome (7.7%, n = 3), acute demyelinating disease (7.7%, n = 3), septic shock (5.1%, n = 2), and intoxication (5.1%, n = 2). No patient had any adverse event related to the TPE during the procedure. The TPE session was ended prematurely in one patient due to insufficient vascular access and lack of blood flow (2.6%). In the long term, thrombosis due to the indwelling central catheter occurred (5.1%, n = 2). TPE appears to be an effective first-stage or supplementary treatment in a variety of diseases, may be safely used in pediatric patients, and there are significant findings that its area of use will increase. In experienced hands and when assessed carefully, it appears that the rate of adverse reactions and vascular access problems may be low enough to be negligible.

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Сauses of Dizziness in Patients with Suspected Stroke

Russian Sklifosovsky Journal Emergency Medical Care ◽

10.23934/2223-9022-2018-7-3-217-221 ◽

2018 ◽

Vol 7 (3) ◽

pp. 217-221

Author(s):

E. V. Shevchenko ◽

G. R. Ramazanov ◽

S. S. Petrikov

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Demyelinating Disease ◽

Heart Rhythm ◽

Diagnostic Methods ◽

Deficiency Anemia ◽

Resonance Imaging ◽

Peripheral Vestibulopathy ◽

Suspected Stroke ◽

The Brain

Background Acute dizziness may be the only symptom of stroke. Prevalence of this disease among patients with isolated dizziness differs significantly and depends on study design, inclusion criteria and diagnostic methods. In available investigations, we did not find any prospective studies where magnetic resonance imaging, positional maneuvers, and Halmagyi-Curthoys test had been used to clarify a pattern of diseases with isolated acute dizziness and suspected stroke.Aim of study To clarify the pattern of the causes of dizziness in patients with suspected acute stroke.Material and methods We examined 160 patients admitted to N.V. Sklifosovsky Research Institute for Emergency Medicine with suspected stroke and single or underlying complaint of dizziness. All patients were examined with assessment of neurological status, Dix-Hollpike and Pagnini-McClure maneuvers, HalmagyiCurthoys test, triplex scans of brachiocephalic arteries, transthoracic echocardiography, computed tomography (CT) and magnetic resonance imaging (MRI) of the brain with magnetic field strength 1.5 T. MRI of the brain was performed in patients without evidence of stroke by CT and in patients with stroke of undetermined etiology according to the TOAST classification.Results In 16 patients (10%), the cause of dizziness was a disease of the brain: ischemic stroke (n=14 (88%)), hemorrhage (n=1 (6%)), transient ischemic attack (TIA) of posterior circulation (n=1 (6%)). In 70.6% patients (n=113), the dizziness was associated with peripheral vestibulopathy: benign paroxysmal positional vertigo (n=85 (75%)), vestibular neuritis (n=19 (17%)), Meniere’s disease (n=7 (6%)), labyrinthitis (n=2 (1,3%)). In 6.9% patients (n=11), the cause of dizziness was hypertensive encephalopathy, 1.9% of patients (n=3) had heart rhythm disturbance, 9.4% of patients (n=15) had psychogenic dizziness, 0.6% of patients (n=1) had demyelinating disease, and 0.6% of patients (n=1) had hemic hypoxia associated with iron deficiency anemia.Conclusion In 70.6% patients with acute dizziness, admitted to hospital with a suspected stroke, peripheral vestibulopathy was revealed. Only 10% of patients had a stroke as a cause of dizziness.

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