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| Författare |
Lotta Sjögreen Monica Engvall Anne-Berit Ekström Anette Lohmander Stavros Kiliaridis Mar Tulinius |
|---|---|
| Publicerad i | Developmental medicine and child neurology |
| Volym | 49 |
| Nummer/häfte | 1 |
| Sidor | 18-22 |
| ISSN | 0012-1622 |
| Publiceringsår | 2007 |
| Publicerad vid |
Institutionen för odontologi Institutionen för neurovetenskap och fysiologi, sektionen för klinisk neurovetenskap och rehabilitering Institutionen för kliniska vetenskaper |
| Sidor | 18-22 |
| Språk | en |
| Länkar |
dx.doi.org/10.1111/j.1469-8749.2007... |
| Ämnesord | Adolescent, Articulation Disorders, diagnosis, epidemiology, physiopathology, Child, Child, Preschool, Cohort Studies, Cross-Sectional Studies, Dyskinesia, Drug-Induced, diagnosis, epidemiology, physiopathology, Female, Humans, Lip, physiopathology, Male, Myotonic Dystrophy, epidemiology, genetics, Observer Variation, Parents, Phonetics, Prevalence, Severity of Illness Index, Sex Distribution |
| Ämneskategorier | Medicin och Hälsovetenskap |
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.
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