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metachromatic leukodystrophy n.
An inherited metabolic disorder characterized by myelin loss, accumulation of metachromatic lipids in the white matter of the central and peripheral nervous systems, a marked excess of sulfatidates in white matter and in urine, progressive paralysis, and dementia.
rare inherited metabolic disease in which the lack of a key enzyme causes loss of the protective myelin sheath from the white matter of the brain, resulting in psychological disturbances, mental deterioration, and sensory and motor defects.