Purpose or Case Report: Metachromatic leukodystrophy is an autosomal recessive lysosomal storage disorder that primarily affects the white matter tracts of the central nervous system. This occurs due to the build up of sulfatides, which leads to demyelinating disease in the brain. On T2 weighted MR imaging, this disorder often exhibits hyperintense periventricular white matter sparing of the subcortical U-fibers. One may also see hypointense stripes and dots within the affected areas. This is commonly referred to as the tigroid or leopard pattern and is a result of peri-vascular sparing. Other features include low signal on T1 weighted images and typically no enhancement on contrast-enhanced T1 weighted images. Metachromatic leukodystrophy can also lead to sulfatide buildup in organs outside the central nervous system, such as the gallbladder, and lead to gallbladder polyposis. This educational exhibit will provide a comprehensive review of the imaging features of metachromatic leukodystrophy, discuss the clinical categorization, and will highlight some of the most common differential diagnoses.
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