Balo concentric sclerosis

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Balo concentric sclerosis
Balo sclerosis.JPG
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.
SpecialtyNeurology Edit this on Wikidata

Balo concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact.[1] It was described by Joszef Balo who initially named it "leuko-encephalitis periaxialis concentrica" from the previous definition,[2] and it is currently considered 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.[3]

It is also common that the clinical course is primary progressive, but a relapsing-remitting course has been reported.[4] It seems that the course gets better with prednisone therapy,[5] 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.


The lesions of the Balo sclerosis belong to the MS lesion pattern III (distal oligodendrogliopathy).[6] Balo concentric sclerosis is now believed to be a variant of pattern III multiple sclerosis[7] and probably due to metabolic problems.[8]

Theoretical models[edit]

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.[9]

Some other researchers maintain that, as in pattern III MS, the mitochondrial respiratory chain complex IV activity is reduced and this could be the culprit of glutamate-mediated axonal injury.[10]

Other two proposed mechanisms are the presence of granulation tissue in old lesions, which is rich in capillaries and could act as an energy reservoir, and the small adherent bands of myelinated tissue. These two mechanisms are proposed based in the assumption of a hypoxic causation principle[11]

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.

Nevertheless, this model is questioned by recent reports that found astrocyte damage, similar to the one found in aquaporin-seropositive neuromyelitis optica. Though no anti-NMO antibodies have been found, the damage is similar, pointing to problems in the water channel of the astrocytes[12][13]

It presents three clinical subtypes: Monophasic, relapsing-remitting and primary rapidly progressive. Cerebrospinal fluid (CSF) is either normal or shows mild mononuclear inflammatory reaction. CSF-restricted oligoclonal bands are present only in a minority of cases[14][15]

Other models[edit]

A mathematical model for concentric sclerosis has been proposed.[16] 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 MRI are distinctive due to their natural concentric shape.

Under a lumbar puncture CSF test, with Baló's concentric sclerosis, as well as patients with pattern III lesions, were recently shown to be mostly negative for CSF-restricted oligoclonal bands.[14][17] Also pattern III patients tend to be negative under the MRZ-reaction (measles, rubeola and zoster viruses)[17]

Paediatric cases[edit]

Balo concentric sclerosis in children has been reported to behave different from adults[18]

Lesions in autopsy and biopsy[edit]

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.[19]

Lesions under MRI[edit]

The features of the MRI and the characteristics of the lesion can be correlated when a biopsy has been taken, providing a way to standardize the future MRI diagnosis[20]

Balo concentric sclerosis lesions can be distinguished from normal lesions on MRI showing alternative hypotensive and hypertensive layers[21]

Balo concentric lesions can be viewed using the myelin water imaging techniques. This is a special MRI sequence that shows the myelin's percentage of water content.[22]

Pattern III lesions, including Baló lesions, have a specific initiation pattern under MRI (MRILIP) consisting in showing Gadolinium enhancement before FLAIR MRI appearance.[23]

Under 7-Tesla MRI balo lesions show a center vein, like in MS.[24]


The disease is more common in Chinese and Filipino populations (both Asiatic) than in caucasoids.[25]


Balo lesions have been reported alone, but also associated to standard multiple sclerosis, neuromyelitis optica, CADASIL and progressive multifocal leukoencephalopathy[26]


Treatment with corticosteroids is usual to relieve inflammation. Recently it has been notice that pattern III lesions are responsive to Mitoxantrone.[27] On the other hand, this pattern is the less responsive to plasmapheresis[28]


Though the disease carries the name of Joszef Balo, it was first described by Otto Marburg in 1906[29] Later, in 1928, Joszef Balo studied the encephalitis periaxialis concentrica in a Hungarian patient, showing also demyelination of the peripheral nervous system.

See also[edit]


  1. ^ Balo J (1928). "Encephalitis periaxialis concentrica". Archives of Neurology and Psychiatry. 19: 242–244. doi:10.1001/archneurpsyc.1928.02210080044002.
  2. ^ Purohit; et al. (2015). "Balo's Concentric Sclerosis with Acute Presentation and Co-Existing Multiple Sclerosis-Typical Lesions on MRI". Case Reports in Neurology. 7 (1): 44–50. doi:10.1159/000380813.
  3. ^ Karaarslan E, Altintas A, Senol U, et al. (August 2001). "Baló's concentric sclerosis: clinical and radiologic features of five cases". American Journal of Neuroradiology. 22 (7): 1362–1367. PMID 11498428.
  4. ^ Moore GR, Berry K, Oger JJ, Prout AJ, Graeb DA, Nugent RA (December 2001). "Baló's concentric sclerosis: surviving normal myelin in a patient with a relapsing-remitting dinical course". Multiple Sclerosis. 7 (6): 375–382. doi:10.1177/135245850100700606. PMID 11795459.
  5. ^ Garbern J, Spence AM, Alvord EC (December 1986). "Balo's concentric demyelination diagnosed premortem". Neurology. 36 (12): 1610–1614. doi:10.1212/WNL.36.12.1610. PMID 3785678.
  6. ^ (Article in Spanish) Archived 2007-04-30 at the Wayback Machine.
  7. ^ Bogdan F. Popescu, MD, Istvan Pirko, and Claudia F. Lucchinetti, Pathology of Multiple Sclerosis: Where Do We Stand? Continuum (Minneap Minn). 2013 Aug; 19(4 Multiple Sclerosis): 901–921, PMC 3915566, doi:
  8. ^ Qiao Ling Cui, Malena Rone, Damla Khan, Melissa Bedard, Guillermina Almazan, Samuel Ludwin, Timophy Kennedy and Jack Antel, Oligodendrogliopathy in Multiple Sclerosis: Relation to Low Glycolytic Metabolic Rate of Oligodendrocytes (I10.004), Neurology April 5, 2016 vol. 86 no. 16 Supplement I10.004
  9. ^ Genetic susceptibility in MS – Steve Hauser. Rare Neuroimmunologic Disorders Symposium [1]
  10. ^ Don J. Mahad, Iryna Ziabreva, Graham Campbell, Nichola Lax, Katherine White, Peter S. Hanson, Hans Lassmann, Douglass M. Turnbull, Mitochondrial changes within axons in multiple sclerosis, DOI:, 17 March 2009
  11. ^ Helmut Barza, Ulrich Barzc, Almut Schreiberd, Morphogenesis of the demyelinating lesions in Baló’s concentric sclerosis, Medical Hypotheses, Volume 91, June 2016, pages 56–61,
  12. ^ Matsuoka Takeshi; et al. (Nov 2010). "Aquaporin-4 astrocytopathy in Baló's disease". Acta Neuropathologica. 120 (5): 651–660. doi:10.1007/S00401-010-0733-7.
  13. ^ Kira J (Jul 2011). "Astrocytopathy in Balo's disease". Multiple Sclerosis. 17 (7): 771–779. doi:10.1177/1352458511400475. PMID 21459811.
  14. ^ a b Jarius S, Würthwein C, Behrens JR, Wanner J, Haas J, Paul F, Wildemann B. Baló's concentric sclerosis is immunologically distinct from multiple sclerosis: results from retrospective analysis of almost 150 lumbar punctures. Journal of Neuroinflammation 2018;15:22. doi:10.1186/s12974-017-1043-y PMID 29347989
  15. ^ Saeed Arif et al, Onion Peel Appearance in Balos Concentric Sclerosis, A Variant of Multiple Sclerosis, Journal of Ayub Medical College, Abbottabad, 2015;27(1)
  16. ^ Khonsari RH, Calvez V (2007). Monk N, ed. "The Origins of Concentric Demyelination: Self-Organization in the Human Brain". PLoS One. 2 (1): e150. doi:10.1371/journal.pone.0000150. PMC 1764710. PMID 17225855.
  17. ^ a b Jarius S, König FB, Metz I, Ruprecht K, Paul F, Brück W, Wildemann B. Pattern II and pattern III MS are entities distinct from pattern I MS: evidence from cerebrospinal fluid analysis. Journal of Neuroinflammation 2017;14:171. doi:10.1186/s12974-017-0929-z PMID 28851393
  18. ^ Linnoila J, Chitnis T (2014). "Balo Concentric Sclerosis in Children: A Case Series". Journal of Child Neurology. 29 (5): 603–607. doi:10.1177/0883073813517294. PMID 24423690.
  19. ^ Lindquist S (2007). "Histopathology and serial, multimodal magnetic resonance imaging in a multiple sclerosis variant". Multiple Sclerosis. 13 (4): 471–482.
  20. ^ Darke, Bahador, Miller, Litofsky, Ahsan. Baló's concentric sclerosis: imaging findings and pathological correlation, Journal of Radiology Case Reports. 2013;7:1-8. doi:10.3941/jrcr.v7i6.1251. PMID 24421937
  21. ^ IANNUCCI et al. Vanishing Balò-like lesions in multiple sclerosis, Journal of Neurology Neurosurgery and Psychiatry 2000;69:399–400
  22. ^ Cornelia Laule et al. Myelin water imaging of multiple sclerosis at 7 T: Correlations with histopathology, NeuroImage volume 40, issue 4, 1 May 2008, pages 1575–1580, doi:10.1016/j.neuroimage.2007.12.008
  23. ^ Charles RG Guttmann et al. Multiple sclerosis lesion formation and early evolution revisited: A weekly high-resolution magnetic resonance imaging study, Multiple Sclerosis Journal, 1–9, doi:10.1177/1352458515600247, 13 July 2015
  24. ^ Behrens JR et al. 7 Tesla MRI of Balo's concentric sclerosis versus multiple sclerosis lesions. Annals of Clinical and Translational Neurology 2018;5:900-912. doi: 10.1002/acn3.572. eCollection 2018 Aug.
  25. ^ Article at
  26. ^ Commentary on Pique et al.’s paper entitled: Peripheral late reactivation of a previously typical monofocal Balo’s concentric sclerosis lesion [2]
  27. ^ Thomas Grüter et al. Mitoxantrone treatment in a patient with multiple sclerosis and pattern III lesions. 03 June 2018,
  28. ^ Lidia Stork et al., Differences in the Reponses to Apheresis Therapy of Patients With 3 Histopathologically Classified Immunopathological Patterns of Multiple Sclerosis, JAMA Neurology 2018;75:428-435. doi:10.1001/jamaneurol.2017.4842
  29. ^ Commentary on Pique et al.’s paper entitled: Peripheral late reactivation of a previously typical monofocal Balo’s concentric sclerosis lesion. Multiple Sclerosis, 2015;21:1084-1086.

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

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