Balo concentric sclerosis
| Balo concentric sclerosis | |
| ICD-10 | G37.5 |
|---|---|
| ICD-9 | 341.1 |
| DiseasesDB | 11849 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [8]
Synonyms and keywords: Sudanophilic cerebral sclerosis; diffuse sclerosis of Schilder; encephalitis periaxalis diffusa; myelinoclastic diffuse sclerosis
Overview
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.[1]
It is also common that the clinic course is primary progressive, but a relapsing-remitting course has been reported. [2]It seems that the course gets better with prednisone therapy[3], 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).[4]
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. [5].
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[6]. 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.
Epidemiology
The disease is more common in Chinese and Filipino populations (both Asiatic) than in caucasoids[7].
Related Chapters
References
- ↑ Baló's Concentric Sclerosis: Clinical and Radiologic Features of Five Cases [1]
- ↑ Balo's concentric sclerosis: surviving normal myelin in a patient with a relapsing-remitting clinical course[2]
- ↑ Balo's concentric demyelination diagnosed premortem [3]
- ↑ (Article in Spanish) [4]
- ↑ Genetic susceptibility in MS – Steve Hauser. Rare Neuroimmunologic Disorders Symposium [5]
- ↑ The Origins of Concentric Demyelination: Self-Organization in the Human Brain, Khonsari & Calvez, PLoS ONE 2(1):e150)[6]
- ↑ Article at mult-sclerosis.org [7]
Concentric demyelination by self-organization: a new hypothesis for Baló's sclerosis, Khonsari RH, Calvez V, Nat Clin Pract Neurol 2007 3:E1
External link
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Balo concentric sclerosis On the Web |
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American Roentgen Ray Society Images of Balo concentric sclerosis |
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Risk calculators and risk factors for Balo concentric sclerosis |