|LETTER TO THE EDITOR
|Year : 2022 | Volume
| Issue : 2 | Page : 122-123
Balo's concentric sclerosis and borderline forms of multiple sclerosis
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
Department of Medicine, Federal University of Santa Maria, Santa Maria, RS, Brazil
|Date of Submission||23-Dec-2021|
|Date of Acceptance||24-Jan-2022|
|Date of Web Publication||08-Apr-2022|
Jamir Pitton Rissardo
Av. Roraima, 1000 - Camobi, Santa Maria, RS 97105-900
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rissardo JP, Fornari Caprara AL. Balo's concentric sclerosis and borderline forms of multiple sclerosis. Apollo Med 2022;19:122-3
We read the article entitled “Balo's Concentric Sclerosis with Bilateral Cerebral Hemisphere Involvement” on the esteemed “Apollo Medicine” with great interest. Sharma et al. reported a case of a middle-aged adult who was diagnosed with Balo's concentric sclerosis (BCS), in which the lesions were found in both cerebral hemispheres. Steroids and mycophenolate mofetil were started. After 3 months, the patient had a significant improvement in his neurological symptoms.
BCS is a rare multiple sclerosis (MS) variant defined by concentric rings of alternating demyelinated and partially myelinated fibers. The clinical course of BCS was traditionally depicted as acute monophasic, but many other clinical forms were discovered in the past decades. Furthermore, it is noteworthy that favorable outcomes with treatment were reported even though the natural history of the disease varies and may result in death or severe disability.
Herein, we would like to discuss more this uncommon pathology. [Table 1] describes the main characteristics of BCS, Schilder's disease, and Marburg MS.,,,,
|Table 1: Borderline forms, pseudotumefactive variants, and atypical variants of multiple sclerosis|
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Ayrignac et al. reviewed the risk of developing MS in more than 60 BCS participants. They did not find any clinical or laboratorial risk factor directly related to the development of MS. However, cerebrospinal fluid oligoclonal bands and the presence of additional MS-like lesions on the initial neuroimaging were significantly associated with subsequent relapses. Furthermore, it is suggested that larger imaging studies with 3-Tesla or more powerful magnetic fields should be done.
In a recent Greek case series, it was proposed a new possible BCS classification and different therapeutic concepts based on each BCS-subgroup characteristic. The three subgroups suggested by the authors were BCS with or without coexisting nonspecific white matter lesions, BCS with typical MS lesions and good response to methylprednisolone, and BCS with typical MS lesions and poor response to methylprednisolone. It is worthy of mentioning that this study provides a new algorithm for BCS management based on radiological features at disease onset and the response to different immunotherapies.
Arenas Vargas and colleagues reported a case of Balo's disease in a 5-year-old boy. In this context, BCS in pediatric individuals was only described in about 10 individuals. The authors suggest that BCS should be considered in the differential diagnosis when evaluating tumefactive demyelinating lesions in the pediatric population. Furthermore, the majority of the pediatric individuals affected by BCS are under 10 years of age without gender predominance. Interesting, the lesions found were bilateral and/or multiple with at least one showing a concentric pattern.
A retrospective review assessed the clinical, radiologic, and pathology records of 40 BCS cases from the United States and Australia tertiary care centers. Jolliffe et al. showed that radiological BCS and BCS-like lesions are significantly correlated with pattern III immunopathology, which is characterized by oligodendrocyte dystrophy and composed mainly of T-lymphocytes with macrophages and activated microglia. Understanding BCS's pathophysiology is essential for early diagnosis and the development of targeted therapies, which could prevent severe forms of the disease and its complications.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Sharma M, Menon B, Manam G. Balo's concentric sclerosis with bilateral cerebral hemisphere involvement. Apollo Med 2021;18:37-8. [Full text]
Ayrignac X, Letourneau-Guillon L, Carra-Dallière C, Duquette P, Girard M, Poirier J, et al.
From Baló's concentric sclerosis to multiple sclerosis: A series of 6 patients. Mult Scler Relat Disord 2020;42:102078.
Tzanetakos D, Vakrakou AG, Tzartos JS, Velonakis G, Evangelopoulos ME, Anagnostouli M, et al.
Heterogeneity of Baló's concentric sclerosis: A study of eight cases with different therapeutic concepts. BMC Neurol 2020;20:400.
Arenas Vargas LE, Bedoya Morales AM, Rincón Carreño C, Espitia Segura OM, Penagos N. Balo's concentric sclerosis: An atypical demyelinating disease in pediatrics. Mult Scler Relat Disord 2020;44:102198.
Jolliffe EA, Guo Y, Hardy TA, Morris PP, Flanagan EP, Lucchinetti CF, et al
. Clinical and radiologic features, pathology, and treatment of Baló concentric sclerosis. Neurology 2021;97:414-22.