Dental Problem in Angelman Syndrome

Dental Problem in Angelman Syndrome
5
(1)

People with Angelman syndrome can have dental problems in addition to the mental and physical disabilities that mark this disease. Many of these problems are cosmetic. However, some can lead to an increased risk of cavities, difficulty eating or drinking, and drooling.

Here is more information about how a child’s mouth and teeth may be affected by Angelman syndrome, and possible treatment options.

What dental problems are common in Angelman syndrome?

Angelman syndrome patients have several characteristic abnormalities related to their mouth and teeth. Many have diastemas (gaps between their teeth), prognathism (an “underbite” or “overbite,” where the upper and lower jaw are misaligned), forward thrusting of the tongue, which can lead to an opening between the top and bottom teeth, and drooling.

Patients may also frequently like to suck or chew on objects or their hands. They are also known to grind their teeth during the day.

How might Angelman syndrome affect the teeth?

It is not clear how Angelman syndrome causes such oral characteristics as spaces between teeth and prognathism.

A recent study found that the enamel (the hard outside covering of teeth) of children with Angelman syndrome was unusually thin and contained nitrogen, while that of unaffected children did not. Both sets of children’s dentin (the layer just below the enamel) contained nitrogen, but the enamel of patients’ teeth alone had nitrogen in quantities similar to the dentin. This led the investigators to propose that patients’ tooth enamel may have some of the same proteins as the dentin. They also found that several of the patients’ teeth had variations in different minerals, which may lead to an increased risk of cavities.

Dental treatments of Angelman syndrome patients

The primary form of dental care for Angelman patients is good oral hygiene, and limits on consumption of sweets and sugary beverages. Since most patients have developmental delays and motor challenges, parents will need to ensure proper brushing. Regular dental checkups can also help to prevent cavities. Since children with Angelman syndrome may not be cooperative during dental procedures, restraint or general anesthesia may be necessary.

Treatment is usually not necessary for spaces between teeth and prognathism unless these impair the patient’s ability to eat and acquire sufficient nutrition. In such cases, jaw surgery or orthodontic devices like a chincup or face mask — or a combination of the two — may help.

An occupational or speech therapist would be able to suggest exercises aiming to improve oral motor skills. These could also help to lessen tongue protrusion and drooling.

 

Last updated: Jan. 4, 2020

***

Angelman Syndrome News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. 

Brian holds a Ph.D. in Biomedical Engineering from Case Western Reserve University and a Bachelors of Science in Biomedical Engineering from Georgia Institute of Technology. He has co-authored numerous scientific articles based on his previous research in the field of brain-computer interfaces and functional electrical stimulation. He is also passionate about making scientific advances easily accessible to the public.
Total Posts: 0
Özge has a MSc. in Molecular Genetics from the University of Leicester and a PhD in Developmental Biology from Queen Mary University of London. She worked as a Post-doctoral Research Associate at the University of Leicester for six years in the field of Behavioural Neurology before moving into science communication. She worked as the Research Communication Officer at a London based charity for almost two years.
×
Brian holds a Ph.D. in Biomedical Engineering from Case Western Reserve University and a Bachelors of Science in Biomedical Engineering from Georgia Institute of Technology. He has co-authored numerous scientific articles based on his previous research in the field of brain-computer interfaces and functional electrical stimulation. He is also passionate about making scientific advances easily accessible to the public.
Latest Posts
  • disrespectful
  • obesity
  • gene therapies
  • drooling

How useful was this post?

Click on a star to rate it!

Average rating 5 / 5. Vote count: 1

No votes so far! Be the first to rate this post.

As you found this post useful...

Follow us on social media!

We are sorry that this post was not useful for you!

Let us improve this post!

Tell us how we can improve this post?