抄録
Spinocerebellar ataxias （SCAs） are progressive neurodegenerative disorders marked by cerebellar degeneration. Symptoms of these diseases are motor uncoordination, particularly of gait. Additionally, SCAs show clinical signs relating to oral functions including speech and swallowing. The incidence of these diseases is extremely rare, occurring in approximately 18.5/100,000 in Japan. This report describes a case of surgical orthodontic treatment for mandibular prognathism with SCAs.
The male patient developed walking disturbance at 12 years of age; occasionally, speech disturbance has occurred. At the age of 19, he was referred to our clinic for surgical orthodontic treatment with a chief complaint of mandibular prognathism. The Department of Neurology of our hospital revealed SCAs and genetic testing showed autosomal recessive spastic ataxia of Charlevoix-Saguenay （ARSACS）.
Preoperative orthodontic treatment started at the age of 30. SCAs are progressive ataxia diseases, which can affect orthognathic surgery under general anesthesia, so it is necessary to shorten the preoperative period. For this reason, we selected non-extraction of both jaws. Bimaxillary orthognathic surgery at the age of 31 corrected the mandibular prognathism, and a good profile and occlusion were obtained. Five years have passed since the orthodontic treatment, and his occlusal condition is stable.
There is only one report of surgical orthodontic treatment for these diseases. SCAs can cause impairment of motor coordination including the stomatognathic system, which can be a factor of relapse. In this report, we discuss the treatment results and stability after treatment from the viewpoint of these disorders progressively affecting motor function including the orofacial region.
