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Technology Insight: can neuroimaging provide insights into the role of ischemia in Baló's concentric sclerosis?

Abstract

Baló's concentric sclerosis (BCS) has long been considered to be a variant of multiple sclerosis. Although BCS was initially described over 100 years ago, relatively few antemortem cases have been identified, and the exact pathogenesis remains unknown. Inflammatory protective ischemic preconditioning has recently been suggested as a mechanism by which the typical concentric rings of the BCS lesion are formed. Advanced neuroimaging can provide important in vivo markers of disease progression that can assist in the diagnosis and management of patients with BCS. In this Review, we discuss evidence from longitudinal neuroimaging studies that supports the role of ischemic preconditioning in BCS.

Key Points

  • The lesion in Baló's concentric sclerosis (BCS) is characterized by a pattern of concentric alternating bands of myelinated and partially demyelinated neurons

  • Ischemic preconditioning proteins might protect against demyelination in an outwardly expanding BCS lesion, causing the formation of a 'spared' ring of myelinated tissue surrounded by another ring of demyelination outside of the region of protection

  • It is difficult to clinically distinguish BCS from other demyelinating diseases such as acute disseminated encephalomyelitis and multiple sclerosis; MRI is needed to confirm the diagnosis

  • Restricted diffusion, indicative of cytotoxic edema typically associated with ischemia, has now been reported in several BCS cases

  • Magnetic resonance spectroscopy shows that acute BCS lesions have decreased N-acetylaspartate peaks (consistent with neuronal loss), increased choline and lipid peaks (suggestive of increased cell membrane turnover and gliosis), and increased lactate peaks (consistent with impaired aerobic metabolism)

  • Ischemia is likely to have a role in the pathogenesis of BCS and pattern type III multiple sclerosis lesions

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Figure 1: Pathology of classic Baló's concentric sclerosis
Figure 2: Proposed pathogenesis of Baló's concentric sclerosis
Figure 3: Fluid-attenuated inversion recovery MRI study demonstrating classic abnormalities consistent with Baló's concentric sclerosis

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Acknowledgements

The authors would like to thank Dr Steven Galetta and Dr Dina Jacobs for their helpful comments and suggestions. This work was supported by a University-wide AIDS Research Program Grant (CF05-SD-301) and an American Foundation for AIDS Research Grant (106729-40-RFRL), both awarded to BM Ances.

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Correspondence to Beau M Ances.

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Supplementary information

Supplementary Table 1

Existing literature of radiological findings associated with Baló's concentric sclerosis. (DOC 161 kb)

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Mowry, E., Woo, J. & Ances, B. Technology Insight: can neuroimaging provide insights into the role of ischemia in Baló's concentric sclerosis?. Nat Rev Neurol 3, 341–348 (2007). https://doi.org/10.1038/ncpneuro0519

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