title: An animal model of oral dysphagia in amyotrophic lateral sclerosis.
abstract: Relatively little is known about the underlying neuropathology of dysphagia in amyotrophic lateral sclerosis (ALS); thus, effective treatments remain elusive. Tremendous progress toward understanding and treating dysphagia in ALS may be possible through the use of an animal model of dysphagia in ALS research; however, no such animal model currently exists. The most logical candidate to consider is the SOD1-G93A transgenic mouse, the most widely investigated animal model of ALS. To investigate whether this animal model develops dysphagia, oral behaviors (lick and mastication rates) of SOD1-G93A transgenic mice (n = 30) were evaluated at three time points based on hind limb motor function: asymptomatic (60 days), disease onset (approximately 110 days), and disease end-stage (approximately 140 days). Age-matched nontransgenic littermates (n = 30) served as controls. At each time point, lick and mastication rates were significantly lower (p < 0.05) for transgenic mice compared with controls. Histologic analysis of the brainstem showed marked neurodegeneration (vacuolation) of the trigeminal and hypoglossal nuclei, two key motor components involved in mastication and licking behaviors. These results demonstrate a clinicopathologic correlation of oral dysfunction in SOD1-G93A transgenic mice, thereby establishing the SOD1-G93A transgenic mouse as a bona fide animal model of oral dysphagia in ALS.
authors.affiliation: Department of Communication Sciences and Disorders, College of Allied Health Sciences, East Carolina University, 600 Moye Blvd., Suite 3310, Greenville, NC 27858, USA. levert@ecu.edu
authors.forename: Teresa E
authors.initials: TE
authors.lastname: Lever
authors.forename: Ambre
authors.initials: A
authors.lastname: Gorsek
authors.forename: Kathleen T
authors.initials: KT
authors.lastname: Cox
authors.forename: Kevin F
authors.initials: KF
authors.lastname: O'Brien
authors.forename: Norman F
authors.initials: NF
authors.lastname: Capra
authors.forename: Monica S
authors.initials: MS
authors.lastname: Hough
authors.forename: Alexander K
authors.initials: AK
authors.lastname: Murashov
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D000818:Animals; D015992:Body Mass Index; D003680:Deglutition Disorders / Q000209:etiology*; D018450:Disease Progression; D005247:Feeding Behavior; D008409:Mastication; D051379:Mice; D023421:Models, Animal; D013482:Superoxide Dismutase
chemical_list: C109415:SOD1 G93A protein; D013482:Superoxide Dismutase