Sudanophilic Leukodystrophy

Diagnosis:

A type of leukodystorphy. A type of dysmyelination

Findings on investigations:

Imaging:

  • White matter (hemispheres, cerebellum and brainstem) and grey matter
  • CT: hypodense
  • T2 MRI: hyperintense white matter. Hypointense lentiform nucleus, thalamus, substantia nigra, dentate nucleus

Pathology:

Sudanophilic material in macrophages.
Reduced oligodendrocytes.
Tigroid appearance: abnormal white matter background, normal neurons.

Subtype:

  • Pelizaeus-Merzbacher disease

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