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Updated: Jan 14, 2026

A Versatile Murine Model of Subcortical White Matter Stroke for the Study of Axonal Degeneration and White Matter Neurobiology

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Endogenous Repair in Vanishing White Matter.

Bonnie C Plug¹, Jodie H K Man¹, Marjolein Breur¹

¹Department of Child Neurology, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam Leukodystrophy Center, Amsterdam, the Netherlands.

Annals of Neurology

|October 27, 2025

Summary

Vanishing white matter disease shows regional differences in repair potential. Preserved glial cell function in the pons allows spontaneous repair, unlike the frontal white matter where glial dysfunction leads to degeneration.

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Area of Science:

Neuroscience
Cellular Pathology
Neurodegenerative Diseases

Background:

Vanishing white matter is a leukodystrophy with variable disease severity.
Cerebral and cerebellar white matter degenerate, while deep gray matter and brainstem lesions show temporary improvement, suggesting endogenous repair.
Repair is absent in cerebral and cerebellar white matter, prompting investigation into cellular pathology differences.

Purpose of the Study:

To investigate if cellular pathology differences explain the regional variation in repair potential in vanishing white matter.
To compare astrocyte, microglia, and oligodendrocyte pathology in severely affected frontal white matter versus less affected pons white matter.

Main Methods:

Postmortem human brain tissue analysis from vanishing white matter patients and controls.
Assessment of astrocytes, microglia, oligodendrocytes, myelin, and axons.
Comparison of frontal white matter (severe) and pons white matter (mild).

Main Results:

In frontal white matter, glial cells (astrocytes, microglia, oligodendrocytes) showed abnormal morphology and failed mature functions.
In pons white matter, glial cells were relatively preserved with evidence of better-preserved functions.
These neuropathological differences suggest a basis for divergent regional repair potential.

Conclusions:

Loss of glial cell function in cerebral white matter correlates with irreversible degeneration.
Preserved glial function and inflammatory response in the pons coincide with spontaneous repair in vanishing white matter.
Understanding pons repair mechanisms could lead to new treatment strategies for this incurable disease.