Developmental enamel defects in primary teeth have been found at least twice as frequently in children with cerebral palsy or mental retardation as in control children, and frequently also in children with sensori-neural hearing deficits. The developing tooth germ is sensitive to a range of systemic disturbances, some of which may also affect neurologic development. Because the enamel cannot recover once it is damaged, it may provide a repository of information on the timing and nature of insults potentially affecting other ectodermally derived structures, including the brain. This paper reviews the literature on developmental defects of enamel in primary teeth, asking whether these might be useful as biological markers of the timing and in some cases the nature of insults. Among systemic factors related to development of enamel that might also have implications for neurologic development are certain genetic disorders including tuberous sclerosis, premature birth, neonatal nutritional disturbances (especially hypocalcemia), viral infections (such as rubella and cytomegalovirus during gestation), thyroid disorders, and maternal diabetes. It is concluded that further research is warranted concerning whether developmental defects of dental enamel can be useful markers for the timing of intra-uterine or perinatal events associated with certain neurologic and sensory disorders of children.
Vitamin B12-responsive neonatal megaloblastic anemia and homocystinuria with associated reduced methionine synthase activity