title: Dysphagia in Huntington's Disease: Correlation with Clinical Features.
abstract: Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by motor impairment, cognitive decline and psychiatric disorders. Dysphagia is a pathologic condition that increases morbidity and mortality of the affected people. Our aim was to evaluate dysphagia in a group of HD patients in view of motor, cognitive and functional decline. Thirty-seven genetically confirmed HD patients were submitted to clinical evaluations of swallowing. Bedside Swallowing Assessment Scale (BSAS) was used. Dysphagia Outcome and Severity Scale (DOSS) was applied for a preliminary classification of swallowing difficulties. All patients were also evaluated by the Unified Huntington's Disease Rating Scale (UHDRS). A group of 39 controls comparable for sex and age were recruited for BSAS scores normalisation. The BSAS scores indicated that in our HD cohort, 32.4% presented relevant or severe dysphagia. The DOSS levels were significantly correlated with main clinical features, such as age, disease duration and motor impairment, with special regard to lingual protrusion ability, dysarthria and bradykinesia. The total functional capacity (TFC) and cognitive scales did not show significant correlation with DOSS levels. The results of clinical examination of swallowing indicated that dysphagia is a prevalent motor symptom of HD.
authors.affiliation: Department of Basic Medical Sciences, Neurosciences and Sensory Organs, School of Medicine, University of Bari, Bari, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: de Tommaso
authors.forename: Angela
authors.initials: A
authors.lastname: Nuzzi
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Dellomonaco
authors.forename: Vittorio
authors.initials: V
authors.lastname: Sciruicchio
authors.forename: Claudia
authors.initials: C
authors.lastname: Serpino
authors.forename: Claudia
authors.initials: C
authors.lastname: Cormio
authors.forename: Giovanni
authors.initials: G
authors.lastname: Franco
authors.forename: Marisa
authors.initials: M
authors.lastname: Megna
mesh_terms: D000328:Adult; D003072:Cognition Disorders / Q000453:epidemiology / Q000209:etiology; D003680:Deglutition Disorders / Q000453:epidemiology* / Q000209:etiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications*; D008297:Male; D008875:Middle Aged; D015995:Prevalence