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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. [ABSTRACT FROM AUTHOR]

Background/objectives This study investigated the craniofacial morphology of young individuals with congenital or childhood onset myotonic dystrophy type 1 (DM1) compared to healthy subjects. The study also followed growth changes in their facial morphology over a 5-year period. Materials/methods Lateral cephalograms of the 26 subjects (young patients with DM1 from west and south Sweden) were taken at baseline and after a 5-year period. These radiographs were compared with normal standards based on healthy individuals from the Michigan Growth Study, according to their age and sex, using paired t -tests (P < 0.05). Results On examination of initial radiographic measurements, patients with DM1 showed, in the sagittal plane, larger ANB and smaller SNPg angles. Analysis of the vertical plane showed the mandibular plane angle (ML-NSL) and the intermaxillary angle (ML-NL) to be larger. During the 5-year follow-up period, the intermaxillary angle (ML-NL) remained the same in the group with DM1 whereas this angle decreased in healthy individuals. Limitations For ethical reasons, historical cephalometric norms were used to compare the growth and the craniofacial morphology of patients with DM1. Conclusions/implications Young patients with DM1 had already from the beginning a more retrognathic profile and hyperdivergent skeletal aberration with a steep mandibular plane and large intermaxillary angle when compared with healthy individuals. The intermaxillary angle did not decrease during the observation period, contrary to what was observed in healthy individuals. [ABSTRACT FROM AUTHOR]

Abstract: Background: Myotonic dystrophy type 1 (DM1) is a slowly progressive multi‐systemic disease with an autosomal‐dominant inheritance caused by a mutation on chromosome 19 (19q13.3). Aims: To explore speech characteristics in a group of individuals with the congenital and childhood‐onset forms of DM1 in terms of intelligibility, speech‐sound production, nasality and compensatory articulation. A further aim was to analyse whether speech characteristics were correlated to subforms of DM1 and if speech outcome could be related to muscle strength. Methods & Procedures: Fifty native Swedish speakers (7–29 years old) with the congenital and childhood‐onset forms of DM1 and 13 healthy controls participated in the study. The intelligibility of spontaneous speech, speech‐sound production — single‐word and sentence repetition — including percentage consonants correct (PCC) and compensatory articulation, were evaluated by speech–language pathologists from video recordings. A nasometer and lip‐force meter were used for objective evaluations of nasality and orofacial strength. Outcomes & Results: In severe (n = 9) and mild congenital DM1 (n = 13), all participants had impaired intelligibility to some degree, while this applied to 79% of those with childhood DM1 (n = 28). PCC for bilabials were 53.9% in severe congenital DM1, 57.4% in mild congenital DM1 and 85.3% in childhood DM1; the corresponding results for dentals were 69.3%, 59.2% and 87.3%. Bilabials were most often compensated for with interdental or labiodental articulation. Dentals were substituted with interdental articulation. Velars were seldom affected. The mean nasalance score was high in the study group compared with controls and with normative data and the majority had weak lips. Maximum lip force, as well as the mean nasalance score, correlated significantly with the intelligibility score. Conclusions & Implications: 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. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Myotonic dystrophy type 1 (DM1) is a slowly progressive neuromuscular disease. Most children and adolescents with DM1 have weak lips and impaired lip function. The primary aim of the present study was to investigate if regular training with an oral screen could strengthen the lip muscles in children and adolescents with DM1. If lip strength improved, a secondary aim would be to see if this could have an immediate effect on lip functions such as lip mobility, eating and drinking ability, saliva control, and lip articulation. Methods: Eight school aged children and adolescents (7–19 years) with DM1 were enrolled in an intervention study with a single group counterbalanced design. After three baseline measurements four children (Subgroup A) were randomly chosen to start 16 weeks therapy while the others (Subgroup B) acted as controls without therapy. After 16 weeks the subgroups changed roles. During treatment the participants exercised lip strength with an oral screen for 16min, 5 days/week. Lip force, grip force (control variable), and lip articulation were followed-up every fourth week. At baseline, after treatment, and after maintenance, the assessment protocol was completed with measurements of lip mobility using 3D motion analysis and parental reports concerning eating ability and saliva control. Results: Seven of eight participants improved maximal lip strength and endurance but only four showed significant change. Increased lip strength did not automatically lead to improved function. There was a wide intra-individual variation concerning speech and eating ability within and between assessments. The treatment programme could be carried out without major problems but the frequency and the effect of training were affected by recurrent infections in some. Conclusions: Maximal lip force and lip force endurance can improve in school aged children and adolescents with DM1. Improved lip strength alone cannot be expected to have an effect on lip articulation, saliva control, or eating and drinking ability in this population. Lip strengthening exercises can be a complement but not a replacement for speech therapy and dysphagia treatment. A prefabricated oral screen is an easy to use tool suitable for strengthening lip exercises. [Copyright &y& Elsevier]

Aim. The aim of this longitudinal study was to evaluate changes in oral health, orofacial function, and dental care in children with myotonic dystrophy type 1 (DM1) in comparison with a control group. Methods. Thirty-six DM1 patients and 33 control patients out of originally 37 in each group were examined on two occasions about 4 years apart. Caries, plaque, and gingivitis were registered, mouth opening capacity assessed and the ability to cooperate in dental treatment estimated. Questionnaires concerning different aspects of oral health and care, symptoms of temporomandibular dysfunction (TMD), and dental trauma were also used. Results. The DM1-patients, in particular the boys, had significantly more caries, plaque, and gingivitis than the control patients on both occasions and the increase in decayed missing or filled permanent teeth (DMFT) and surfaces (DMFS) was significantly larger. They received more dental care and had lower cooperation ability. Mouth opening capacity and increase of it was significantly lower and symptoms of TMD were significantly more frequent. Conclusions. DM1 patients, as they grow older, have increasing amounts of plaque and risk of caries and gingivitis. They have more TMD problems. Behaviour management problems do not seem to decrease with age. Increased prophylactic care is essential for DM1 patients. [ABSTRACT FROM AUTHOR]

Myotonic dystrophy or dystrophia myotonica (DM) is a hereditary neuromuscular multisystem disease with a varying clinical expressivity and severity. The objective of this study was to assess the oral health in children with myotonic dystrophy and to compare it with a control group. Fifty-six DM patients, aged 2.7–18.0 yr, were compared with age- and gender-matched control patients with respect to caries, plaque, and gingivitis. Oral function and signs of temporomandibular dysfunction (TMD) were assessed, and the ability to co-operate in dental treatment was estimated. Questionnaires concerning eating habits, dental care, traumatic injuries to teeth, and orofacial function were also used. The DM patients had significantly more caries, plaque, and gingivitis than did control patients. They had more TMD problems and lower co-operation ability than the healthy control persons. General sedation was frequently needed to carry through dental treatment. DM patients are at risk of caries, gingivitis and TMD problems, and need intensified prophylactic care. Behavior management problems are common. [ABSTRACT FROM AUTHOR]

Myotonic dystrophy (DM) is a neuromuscular disorder caused by an expansion of a CTG repeat sequence on chromosome 19q13. The aim of the present study was to describe the characteristics and prevalence of oral motor dysfunction in a cohort of children and adolescents with DM and to correlate different aspects of oral motor function with the type of DM and sex. Fifty-six individuals with DM (30 males, 26 females; median age 13y 2mo; range 2y 6mo-21y 5mo) were compared with healthy controls. They were divided into four subgroups: severe congenital DM ( n=18); mild congenital DM ( n=18); childhood DM ( n=18); and classical DM ( n=2). A speech-language pathologist assessed different variables of oral motor function, intelligibility, and lip force. The families used a questionnaire to report on eating difficulties and drooling. All individuals with DM had impaired facial expression. Intelligibility was moderately or severely reduced in 30 patients (60%), excluding six patients without speech. Most had a moderate or severe impairment of lip motility (76.0%), tongue motility (52.2%), and lip force (69.2%), causing deviant production of bilabial and dental consonants. The families reported problems with eating (51.9%) and drooling (37.0%). Oral motor dysfunction was most prominent in congenital DM, and males were more affected than females. [ABSTRACT FROM AUTHOR]