- This is an autosomal recessive disease presenting at less than 2 years of age.
- It is the most common leukodystrophy and is associated with gait disturbance and learning disability.
- Death occurs within 5 years.
- Progressive, symmetrical white matter lesions.
- It involves the periventricular white matter and cerebellum, but spares the subcortical white matter.
- Perivascular white matter is relatively spared, which gives the white matter a stripy ‘tigroid’ appearance.
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