 Metachromatic leukodystrophy MLD, an autosomal recessively inherited lysosomal storage disorder, causes a deficiency of arylsulfatase A. This results in accumulation of sulfated glycolipids sulphatide within lysosomes of myelin forming cells in the central and peripheral nervous system and to a lesser extent in lysosomes of cells comprising the liver, kidneys, and gallbladder. The disease is characterized by progressive demyelination with wide variability in clinical onset and severity. Depending upon the age at onset and disease progression, MLD may be classified as late infantile 6 months to 4 years, early juvenile 4 to 6 years, late juvenile 6 to 16 years, and adult 16 years. In the late infantile and early juvenile forms, blindness, gait disturbances, loss of speech, loss of hearing, and quadriparesis are common signs. In older children and adults the disease may present with gait disturbances, mental regression, and behavioral abnormalities. Disease progression, also variable, results in death within a few years to several decades; however, disease progression among affected siblings seems to follow a similar course, unlike many other leukodystrophies. 