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Astrocytopathy in Balo's disease
1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. kira@neuro.med.kyushu-u.ac.jp
Abstract:
Baló's disease is characterized by alternating rings of demyelination and preserved myelin. As additional multiple sclerosis (MS)-like lesions often coexist in Baló's cases, Baló's disease is regarded as a variant of MS. In demyelinated areas, many hypertrophic astrocytes are present in close contact with oligodendrocytes, which often show apoptotic features. In the outermost layer of preserved myelin, stress proteins involved in tissue preconditioning are abundant in oligodendrocytes. The peri-plaque perimeter is thus assumed resistant to subsequent attack, thereby leaving a layer of preserved myelin. In some cases, Baló's concentric rings develop step by step in a centrifugal direction, whereas many other cases show simultaneous enhancement of multiple rings. Therefore tissue preconditioning and successive ring formation does not fully describe the mechanism of the disease. We recently reported that in four Filipino Baló's patients, aquaporin-4 (AQP4) was extensively lost in glial fibrillary acidic protein-positive hypertrophic astrocytes, both in demyelinated and myelinated layers of all actively demyelinating lesions. None of six further patients with MRI-confirmed Baló's disease were seropositive for anti-AQP4 antibody. I propose that AQP4 astrocytopathy, in the absence of anti-AQP4 antibody, is characteristic of Baló's disease. This hypothesis should be tested in future experimental studies.
Insights
Baló's disease, a variant of multiple sclerosis (MS), shows characteristic concentric rings. Loss of aquaporin-4 (AQP4) in astrocytes, without antibodies, may define this condition.
Area of Science:
- Neurology
- Neuroimmunology
- Pathology
Background:
- Baló's disease presents as concentric demyelination rings, often co-occurring with multiple sclerosis (MS)-like lesions.
- Hypertrophic astrocytes and oligodendrocyte apoptosis are observed in demyelinated areas.
- The mechanism of Baló's concentric ring formation remains incompletely understood.
Purpose of the Study:
- To investigate the role of aquaporin-4 (AQP4) in Baló's disease pathology.
- To determine if AQP4 alterations are characteristic of Baló's disease, independent of anti-AQP4 antibodies.
Main Methods:
- Immunohistochemical analysis of brain tissue from Baló's disease patients.
- Assessment of AQP4 and glial fibrillary acidic protein (GFAP) expression in demyelinated and myelinated lesions.
- Serological testing for anti-AQP4 antibodies.
Main Results:
- Extensive loss of AQP4 was observed in hypertrophic astrocytes within active demyelinating lesions in Filipino Baló's patients.
- This AQP4 loss occurred in both demyelinated and myelinated areas.
- No anti-AQP4 antibodies were detected in any of the studied patients with MRI-confirmed Baló's disease.
Conclusions:
- Aquaporin-4 astrocytopathy, characterized by AQP4 loss in astrocytes without circulating antibodies, is proposed as a hallmark of Baló's disease.
- This finding suggests a novel pathogenic mechanism for Baló's disease, distinct from antibody-mediated conditions.
- Further experimental studies are warranted to validate this hypothesis.
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