Authors: Lever, Teresa¹; Gorsek, Ambre²; Cox, Kathleen²; O'Brien, Kevin³; Capra, Norman⁴; Hough, Monica²; Murashov, Alexander⁵

Source: Dysphagia, Volume 24, Number 2, June 2009 , pp. 180-195(16)

Publisher: Springer

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 (~110 days), and disease end-stage (~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.

Keywords: Amyotrophic lateral sclerosis; ALS; Dysphagia; Superoxide dismutase; SOD1; SOD1-G93A; Mouse; Deglutition; Deglutition disorders

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s00455-008-9190-z

Affiliations: 1: Department of Communication Sciences and Disorders, College of Allied Health Sciences, East Carolina University, 600 Moye Blvd., Suite 3310, Greenville, NC, 27858, USA, Email: levert@ecu.edu 2: Department of Communication Sciences and Disorders, College of Allied Health Sciences, East Carolina University, 600 Moye Blvd., Suite 3310, Greenville, NC, 27858, USA 3: Department of Biostatistics, College of Allied Health Sciences, East Carolina University, Greenville, NC, 27858, USA 4: Department of Biomedical Sciences, Baltimore College of Dental Surgery, University of Maryland Dental School, Baltimore, MD, 21201, USA 5: Department of Physiology, Brody School of Medicine, East Carolina University, Greenville, NC, 27858, USA

Publication date: 2009-06-01

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• In this Subject: Ear, Nose & Throat ,  Gastroenterology ,  Radiology & Imaging
• By this author: Lever, Teresa ;  Gorsek, Ambre ;  Cox, Kathleen ;  O'Brien, Kevin ;  Capra, Norman ;  Hough, Monica ;  Murashov, Alexander

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