Paper Report for: Blommaert_2016_J.Child.Neurol_31_1220
Reference
Title: Diagnosis and Management of Drooling in Children With Progressive Dystonia: A Case Series of Patients With MEGDEL Syndrome Blommaert D, van Hulst K, Hoogen FJ, Erasmus CE, Wortmann SB Ref: Journal of Child Neurology, 31:1220, 2016 : PubMed
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).
        
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Blommaert D, van Hulst K, Hoogen FJ, Erasmus CE, Wortmann SB (2016) Diagnosis and Management of Drooling in Children With Progressive Dystonia: A Case Series of Patients With MEGDEL Syndrome Journal of Child Neurology31: 1220-6
Blommaert D, van Hulst K, Hoogen FJ, Erasmus CE, Wortmann SB (2016) Journal of Child Neurology31: 1220-6