Pelizaeus Merzbacher disease

  • X-linked disease leading to hypomyelination. 
  • Typically presents in young males and has a protracted course.
MRI
  • Diffuse high T2 signal in the white matter with involvement of the sub-cortical U-fibres in all cases.
  • Relative sparing of the perivascular white matter, which produces a ‘tigroid’ appearance like metachromatic leukodystrophy.



 

Leave a Comment

Your email address will not be published.

Scroll to Top