title: Diagnosis and Management of Drooling in Children With Progressive Dystonia: A Case Series of Patients With MEGDEL Syndrome.
abstract: Drooling is a common problem in children with progressive dystonia. The authors noted a 58% incidence of drooling in 22/38 children with MEGDEL, a rare neurodegenerative cause of dystonia and report on the clinical course of four patients. Drooling of varying severity and subsequent respiratory problems were treated at the authors' multidisciplinary saliva-control outpatient clinic. One patient improved on antireflux medication, the second after medication with drooling as side effect was changed. Two other patients underwent salivary gland surgery, one of whom significantly improved; the other died shortly after surgery. The heterogeneity of the cases presented shows the need for stepwise and personalized treatment. The authors recommend the following: (1) optimize the treatment of the underlying neurological condition and replace medication that stimulates saliva secretion; (2) treat constipation, scoliosis, and gastroesophageal reflux if there is still a risk of chronic aspiration of saliva; (3) perform more intense/invasive treatment (botulinum toxin, salivary gland surgery).
authors.affiliation: Department of Paediatrics, Amalia Children's Hospital, Radboud University Medical Centre, Nijmegen, Netherlands.
authors.forename: Dorian
authors.initials: D
authors.lastname: Blommaert
authors.affiliation: Department of Rehabilitation, Donders Centre for Neuroscience, Radboud University Medical Centre, Nijmegen, Netherlands Karen.vanHulst@radboudumc.nl.
authors.forename: Karen
authors.initials: K
authors.lastname: van Hulst
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Centre, Nijmegen, Netherlands.
authors.forename: Frank J A van den
authors.initials: FJ
authors.lastname: Hoogen
authors.affiliation: Department of Paediatric Neurology, Donders Centre for Neuroscience, Radboud University Medical Centre, Nijmegen, Netherlands.
authors.forename: Corrie E
authors.initials: CE
authors.lastname: Erasmus
authors.affiliation: Department of Paediatrics, Amalia Children's Hospital, Radboud University Medical Centre, Nijmegen, Netherlands Department of Paediatrics, Salzburger Landeskliniken, Paracelsus Medical University, Salzburg, Austria.
authors.forename: Saskia B
authors.initials: SB
authors.lastname: Wortmann
mesh_terms: D000293:Adolescent; D002675:Child, Preschool; D019468:Disease Management; D018450:Disease Progression; D020821:Dystonic Disorders / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D012798:Sialorrhea / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*
keywords: 3-methylglutaconic aciduria; CLPB; SERAC1; botulinum toxin A; saliva control; salivary gland surgery; stepwise approach