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.
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http://dx.doi.org/10.1007/s00455-015-9627-0 | DOI Listing |
BMC Public Health
December 2024
Department of Pathology, University of Cambridge, Tennis Court Road, Cambridge, CB2 1QP, UK.
Background: Our primary focus was Schistosoma mansoni infection and schoolchildren. Within communities the social environment may promote individual risk of infection for the school-aged children. There will also be demographic groups who are not targeted or reached by preventive chemotherapy campaigns.
View Article and Find Full Text PDFMov Disord Clin Pract
December 2024
Second Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Mov Disord Clin Pract
December 2024
Department of Otorhinolaryngology, TU Dresden, Smell and Taste Clinic, Dresden, Germany.
Am J Chin Med
December 2024
Department of Neurology, The First Affiliated, Hospital of Anhui University of Traditional Chinese Medicine, Hefei, P. R. China.
Wilson's disease (WD) is a hereditary condition marked by abnormalities in copper metabolism, which precipitate a spectrum of neurological symptoms and cognitive impairments. Emerging research has highlighted ferroptosis (FPT) as a distinct type of programmed cell death, potentially linked to various cognitive dysfunctions. Nevertheless, the connection between FPT and cognitive impairment in Wilson's disease (WDCI) remains largely enigmatic.
View Article and Find Full Text PDFEur J Hum Genet
December 2024
Estonian Genome Center, Institute of Genomics, University of Tartu, Tartu, Estonia.
Population-based biobanks enable genomic screening to support initiatives that prevent disease onset or slow its progression and to estimate the prevalence of genetic diseases in the population. Wilson's disease (WD) is a rare genetic copper-accumulation disorder for which timely intervention is crucial, as treatment is readily available. We studied WD in the Estonian Biobank population to advance patient screening, swift diagnosis, and subsequent treatment.
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