Metachromatic Leukodystrophy

  • Etiology: arylsulfatase-A deficiency
  • Imaging: symmetrical confluent involvement of white matter (butterfly), starts at core of centrum semiovale sparing periventricular white matter and U-fibers
  • Clinical: most common leukodystrophy

Radiology Cases of Metachromatic Leukodystrophy

MRI of metachromatic leukodystrophy
Axial T2 (left) and FLAIR (right) MRI without contrast of the brain show bilateral symmetrical confluent increased signal throughout the white matter in a butterfly pattern.