• 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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