Children with Angelman syndrome may be more prone to developing cavities in their teeth, due to the growth of thin enamel with an abnormal protein content, according to a new study.
The study, “The effect of Angelman syndrome on enamel and dentin mineralization,” was published in the journal Special Care in Dentistry.
The loss of a gene called ubiquitin-protein ligase E3A (UBE3A) in the brain causes Angelman syndrome and leads to a wide array of symptoms, including intellectual disability, a lack of speech, and abnormal motor skill development. Those living with Angelman also typically have a number of oral motor challenges, including drooling and excessive chewing.
The dental aspects of Angelman are not well understood. Three studies covering 12 patients have dealt with such issues, and described some dental anomalies such as defects in enamel formation and a molar with a single root, compared with the more common two to three roots. Of note, enamel is one of the four major tissues — the one that is usually visible — that make up teeth.
Now, two researchers from the Barzilai Medical University Center, in Askelon, Israel, analyzed the mineral composition in baby and permanent teeth of two children with Angelman, ages 12 and 15, compared with two age-matched healthy individuals (controls).
The children with Angelman displayed a variety of symptoms, including developmental delay, frequent laughter, and seizures.
Their dental development showed abnormalities related to oral function, including widely spaced teeth and tongue protrusion, in which the tongue presses forward too far in the mouth.
Similar amounts of nitrogen were found in the dentin — tissue normally found in the interior of teeth — and enamel of the children with Angelman. However, the enamel of the children without Angelman showed no nitrogen. According to the investigators, this indicated that, in the context of Angelman, the protein content normally found within dentin was also present in enamel.
The enamel of the second baby molar showed higher concentrations of calcium and phosphate, while that of the neighboring third molar had less. The mesiodens, an extra tooth between and slightly behind the incisors, showed very little mineralization but had high amounts of organic ions (carbon and oxygen).
The mineral composition of the mesiodens and the third adult molar of the children with Angelman differed from those of the healthy children and had variable structural features, the researchers found.
The dental implications of the altered mineral compositions of the teeth of these children implied that they are more prone to cavities, a feature which the investigators say is seen in treated cases.
“[Angelman syndrome] affects morphology and mineralization of enamel,” the researchers concluded. “These findings show that [Angelman syndrome] also affects odontogenesis [the formation and development of teeth] in addition to the known oral motor challenges.”