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metachromatic leukodystrophy, Scholz type leukodystrophy or Greenfield's disease

Three forms described in childhood depending on age of onset & clinical symptoms. Pathology: -> yellowish-brown staining of nerves with cresyl violet (metachromasia) due to break down of myelin into metachromatic material containing sulfatides Clinical manifestations: -> mental retardation

Related

arylsulfatase-A (cerebroside sulfatase, [contains: arylsulfatase A component B; arylsulfatase A component C], ARSA) sulfatide; galactocerebroside sulfate

General

metachromatic leukodystrophy

Properties

ACCUMULATION: sulfatide CEREBROSIDE-DIHEXOSE-SULPHATE DEFICIENCY: aryl sulfatase-A

Database Correlations

OMIM 250100

References

  1. Textbook of Biochemistry with Clinical Correlations, 3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 459
  2. Greenfield's Neuropathology 1992 pg 744