Balo concentric sclerosis

バロー同心円硬化症

WordNet

1. having a common center; "concentric rings" (同)concentrical, homocentric
2. any pathological hardening or thickening of tissue (同)induration

PrepTutorEJDIC

1. 中心を同じくする,同心の
2. (組織・血管などの)硬化,硬化症
3. bariumの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/12/04 14:32:25」(JST)

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Balo concentric sclerosis
Classification and external resources
Typical aspects of Baló's concentric sclerosis. (a) Original case of Baló; several anastomoses are located in the lower half of the lesion. (b) Lesion centered by a veinule showing ring fragmentation in a constrained area. (c) Lesion. (d) Progress of the pathologic process from a center located in a constrained area, showing formation of bands. Loyez staining (myelin in black, destroyed areas in white); scale bars: 1 cm.
ICD-10	G37.5
ICD-9	341.1
DiseasesDB	11849

Balo concentric sclerosis is one of the borderline forms of multiple sclerosis.

Balo concentric sclerosis is a demyelinating disease similar to standard multiple sclerosis, but with the particularity that the demyelinated tissues form concentric layers. Scientists used to believe that the prognosis was similar to Marburg multiple sclerosis, but now they know that patients can survive, or even have spontaneous remission and asymptomatic cases.

It is also common that the clinical course is primary progressive, but a relapsing-remitting course has been reported. It seems that the course gets better with prednisone therapy, although evidence of this is anecdotal and such conclusions are difficult to accept given that there are cases where patients spontaneously recover whether the patient was on steroid therapy or not.

Pathophysiology

The lesions of the Balo sclerosis belong to the MS lesion pattern III (distal oligodendrogliopathy).

According with Dr. Lucchinetti investigations, in Balo's concentric sclerosis, the rings may be caused by a physiological hypoxia (similar to that caused by some toxins or viruses) in the lesion, which is in turn countered by expression of stress proteins at the border. This expression and counter-expression forms rings of preserved tissue within the lesion and rings of demyelinated tissue just beyond where the previous attack had induced the protective stress proteins. Hence, subsequent attacks form concentric rings.

Ultimately, this expanding lesion causes the progressive picture typically seen. However, in some patients, the pathology underlying the disease appears to burn out and hence the disease may halt, hence the patients who spontaneously recover. The mechanisms triggering attacks and recovery remain uncertain.

Recently, a mathematical model for concentric sclerosis has been proposed. Authors review the previous pathogenic theories, discuss the link between concentric sclerosis and Liesegang's periodic precipitation phenomenon and propose a new mechanism based on self-organization.

Lesions under autopsy and biopsy

A report comparing 1H-magnetic resonance spectroscopy, magnetization transfer and diffusion tensor imaging with histopathology in a patient with Balo's concentric sclerosis, found that inflammation was traced by fractional anisotropy and increased lactate. In contrast, magnetization transfer ratio and the diffusion coefficient show a loss of tissue in the rings of the lesion.

Epidemiology

The disease is more common in Chinese and Filipino populations (both Asiatic) than in caucasoids.

See also

• Multiple sclerosis
• The Lesion Project

References

Khonsari RH, Calvez V (September 2007). "Concentric demyelination by self-organization: a new hypothesis for Baló's sclerosis". Nat Clin Pract Neurol 3 (9): E1. doi:10.1038/ncpneuro0619. PMID 17805242.

External links

• Balo sclerosis at mult-sclerosis.org

UpToDate Contents

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English Journal

• Eccentric development of Balo's concentric sclerosis: detected by magnetic resonance diffusion-weighted imaging and magnetic resonance spectroscopy.

• Chen F1, Liu T, Li J, Xing Z, Huang S, Wen G, Lu G.
• The International journal of neuroscience.Int J Neurosci.2014 Sep 3. [Epub ahead of print]
• Background: The concentricity of BCS has captured wide attention; the findings of the current study may provide useful information on the centrifugal pathogenesis of BCS. Objective: This study aims to evaluate the performance of MRI, DWI and MRS in elucidating the pathogenesis of Balo's lesions expa
• PMID 25051427

• Baló's concentric sclerosis.

• Hardy TA1, Miller DH2.
• The Lancet. Neurology.Lancet Neurol.2014 Jul;13(7):740-6. doi: 10.1016/S1474-4422(14)70052-3.
• Baló's concentric sclerosis is often regarded as a rare variant of multiple sclerosis. Patients with this disorder present with acute or subacute neurological deterioration, with MRI showing one or more concentrically multilayered ring-like lesions usually in the cerebral white matter. Historically
• PMID 24943346

• Balo concentric sclerosis in children: a case series.

• Linnoila J1, Chitnis T.
• Journal of child neurology.J Child Neurol.2014 May;29(5):603-7. doi: 10.1177/0883073813517294. Epub 2014 Jan 14.
• Baló concentric sclerosis is a unique and rare phenomenon in demyelinating disease. Typically thought of as a subtype of multiple sclerosis, Baló concentric sclerosis is characterized pathologically by striking rings of demyelination alternating with areas of preserved myelination. Its exact preva
• PMID 24423690

Related Links

• Baló's Concentric Sclerosis: Clinical and Radiologic Features ...

Baló's concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis. The initial terminology for this entity was leuko-encephalitis periaxialis concentrica, which is based on its ...

• Pathognomonic MR Imaging Findings in Balo Concentric ...

Balo concentric sclerosis (BCS) is widely believed to be a rare variant of multiple sclerosis. It may present as clinically similar to multiple sclerosis, affecting young adults with mild cognitive impairment without frank ...

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★リンクテーブル★

リンク元	「バロー同心円硬化症」
関連記事	「concentric」「concentric sclerosis」「Ba」

「バロー同心円硬化症」

　　[★]

英

Balo concentric sclerosis, Balo's concentric sclerosis

同

同心円性硬化症 同心円硬化症 concentric sclerosis

• 原因不明の中枢性脱髄疾患
• 大脳白質において脱髄領域と正常領域が交互に同心円状に配列する病態を呈する症候
• 多発性硬化症などで見られる。

「concentric」

　　[★]

• adj.

• 同一中心の、同心の、同心円状の。同軸を有する。集中的な

関

(adj.)eccentric 偏心の

関

afferent、centripetal、concentrically

　 　

「concentric sclerosis」

　　[★] バロー同心円硬化症、同心円性硬化症

「Ba」

　　[★] バリウム barium