SUMMARY These guidelines present an overall strategy for oral healthcare based on the principles of achieving a disease-free, pain-free and safe mouth. The standards of care referred to in the document seek to provide guidelines for the care and treatment that is no less a standard provided for an individual that does not have this condition. Such care and treatment will take into account the health and safety of each individual within the context of their condition. Particular emphasis is placed on dental professionals working within a multidisciplinary team, focusing on prevention of oral disease and providing treatment appropriate to the various stages of the progression of this condition. It is intended that by providing and subsequently promoting these guidelines, it will focus the dental professions work on this condition and the implementation of good care for people with Huntington’s disease.

The deviant production of bilabial consonants, interdental articulation and hypernasal speech are characteristic features of dysarthria in congenital and childhood DM1. Dysarthria is more frequent and more severe in congenital DM1 compared with childhood DM1. Most individuals with congenital DM1 and childhood-onset DM1 will need speech therapy from a young age. For some children with incomprehensible speech or severe neurodevelopmental disorders, alternative and augmentative ways of communication will be part of the treatment.

Summary Background/objectives This research aimed to study the malocclusions of children and adolescents with myotonic dystrophy type 1 (DM1), in respect to healthy individuals, and trace the occlusal changes that occurred in these individuals during growth. Materials/methods Thirty-six dental casts, from children and adolescents with DM1 living in western and southern Sweden, were compared with a control group of 50 healthy individuals. To identify potential changes in occlusal traits, 26 casts were assessed and followed-up over a median time of 9 years. Independent samples t-tests were used to compare the two groups and their changes over time. Paired samples t-tests tested changes over time within each group (P < 0.05). Results DM1 patients had a higher prevalence of anterior open bite, posterior crossbite, and Class III malocclusions. When compared to controls, patients presented smaller upper and lower intermolar as well as intercanine widths. In both groups, the individuals revealed longitudinal changes with a decrease in both upper and lower arch lengths and an increase on the palatal vault height. During the follow-up period, the prevalence of malocclusions remained almost the same, only significantly differing regarding the changes that occurred between groups referred to the upper intermolar width, which decreased among DM1 patients. Conclusions/implications In comparison to healthy controls, children and adolescents with DM1 have shown already at an early age a higher prevalence of both anterior open bite and posterior crossbite. These occlusal traits did not change with time apart from the upper narrow intermolar width, which further decreased with time.

The primary aim was to study the interaction between oral hygiene, oral care, saliva production, and oral motor function in individuals with myotonic dystrophy type 1 (DM1). A secondary aim was to study how oral hygiene, oral care, and saliva flow rate are affected by gender, age, and subgroup of DM1 in this study population. The study comprised 52 individuals, seven to 29 years of age, divided into two subgroups of DM1, the congenital (N = 24) and childhood‐onset forms (N = 28). A combined dental and oral motor examination was performed and the participants or caregivers answered a questionnaire with questions about general health and disabilities, medication, dental care, and oral health. Sixteen individuals with a plaque‐, gingivitis‐, or calculus‐index score of 5–6 were considered to have poor oral hygiene. There were no significant differences between subgroups (age, gender, or form of DM1) in terms of the occurrence of calculus, gingivitis, plaque, or saliva flow rate. The mean value of the unstimulated whole saliva flow rate was 0.7(±0.44) mL/min. An open mouth at rest and oral motor dysfunction were frequent findings. The majority of Swedish children and young adults with the congenital or childhood form of DM1 have fair or poor oral hygiene, with a high occurrence of plaque and gingivitis. As a group, individuals with DM1 and poor oral hygiene have a higher frequency of caries and they report less satisfaction with their oral care at home and the quality of dental care received compared with those with good oral hygiene.

Background A small group of dental professionals interested in the Oral Health Care of people with Huntington's disease were invited by the European Huntington's Disease Network to provide guidelines for Oral Health. Aim To provide guidelines which would be an initial part of an evolving process in the improvement of Oral Health. Methods Guidelines was produced during two meetings based on: A literature review, information from other oral health organizations, the professional clinical opinion of the group. Results A review of the literature found little evidence in this field. A structured approach described the provision of Oral Health Care under prevention and treatment proposing recommendations related to early, middle and late stages of the condition. The importance of interdisciplinary work was stressed. Outcome A strategy for oral health was defined. The overall strategy for oral health care will be based on the principles of achieving a disease free, pain free, and safe mouth The standards of care referred to in the guidelines will seek to provide the care and treatment that is no less a standard than an individual that does not have this condition. Such care and treatment will take into account the health and safety of each individual within the context of their condition. Conclusions The group recognized the need for further quality research. Interdisciplinary work was considered to be a vital part of the guidelines, and it was intended that the group should establish such links.