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MedPix® Medical Image DatabaseDisease Topic 80
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Contributor: James George Smirniotopoulos, M.D. - Uniformed Services University
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More Like This ? Metachromatic leukodystrophy
Factoid 80 - Created: 1988-01-01 00:00:00-05 - Modified: 2002-04-08 22:01:50-04
ACR Codes: 1.5
diffuse abnormal myelination
most common is late infantile presentation gait disorder and strabismus early in the 2nd year, speech impairment, spasticity, and retardation appear gradually, with steady progression

Cause - deficiency in the enzyme arylsulfatase A => myelin deficiency with accumulation of lipid sulfatide in macrophages and Schwann cells

IMAGING:
CT - low density white matter, progressive atrophy, no enhancement
MR - prolonged T1 and T2
Location:
Brain and Neuro 		Sublocation:
White matter		Category:
Congenital, genetic
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Prepared by: James George Smirniotopoulos, M.D.
Affiliation: Uniformed Services University - || - Author Profile
Approved by: James G. Smirniotopoulos, M.D.
Affiliation: Uniformed Services University - || - Editor Profile
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