title: Hypersialorrhea in Wilson's Disease.
abstract: Hypersialorrhea, corresponding to excessive salivation is a symptom frequently reported in Wilson's disease, especially in its neurological form. The prevalence of this frequent complaint has not been often evaluated. During a 7-month period, 87 consecutive Wilson's disease patients answered to the simple question "do you have the sensation of excess saliva in your mouth?" to evaluate the frequency of this symptom. A sub-sample of 10 consecutive Wilson's disease patients with drooling was recruited to undergo quantitative and qualitative measures to evaluate the mechanism of hypersialorrhea. Excessive drooling or excess saliva was found in 46 % of patients followed at the French Reference Centre. Ninety-eight percent of them presented neurological symptoms and drooling was found in only one patient without neurological symptoms. Our study showed that patients with a complaint of excessive saliva produced significantly higher quantities of saliva at rest than controls. Endoscopic examination was abnormal in six patients. A significant decrease of swallowing frequency, longer swallow latencies, and poor swallowing capacities may partly explain the salivary stasis. Oropharyngeal sensitivity disorders were present in 50 % of our patients. The decrease of the swallowing frequency observed in all patients could be related to cognitive and behavioral abnormalities with initiation difficulties objectified by longer latencies triggered by all the ingested volumes. This study confirmed the hypothesis of a multifactorial origin of hypersialorrhea in patients who have been diagnosed in Wilson's disease. It was essential to evaluate drooling with a multidisciplinary consultation to better identify the underlying mechanisms and to implement strategies for speech therapy and therapeutic adaptation.
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France. cnr.wilson@aphp.fr.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Trocello
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Karima
authors.initials: K
authors.lastname: Osmani
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Michaela
authors.initials: M
authors.lastname: Pernon
authors.affiliation: Service ORL, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Gérard
authors.initials: G
authors.lastname: Chevaillier
authors.affiliation: Service ORL, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: de Brugière
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Remy
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Emilie
authors.initials: E
authors.lastname: Wenisch
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Cousin
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France.
authors.forename: Nadège
authors.initials: N
authors.lastname: Girardot-Tinant
authors.affiliation: French Reference Centre for Wilson's Disease, AP-HP, Hôpital Lariboisière, 2 Rue Ambroise Paré, 75010, Paris, France. france.woimant@aphp.fr.
authors.forename: France
authors.initials: F
authors.lastname: Woimant
mesh_terms: D000328:Adult; D003679:Deglutition; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D007828:Laryngoscopy; D008297:Male; D008875:Middle Aged; D012798:Sialorrhea / Q000209:etiology*
keywords: Deglutition; Deglutition disorders; Drooling; Dysphagia; Hypersialorrhea; Wilson’s disease