title: Clinical evaluation of oropharyngeal dysphagia in Machado-Joseph disease.
abstract: BACKGROUND
  In Machado-Joseph disease, poor posture, dystonia and peripheral neuropathy are extremely predisposing to oropharyngeal dysphagia, which is more commonly associated with muscular dystrophy.
  
  
  OBJECTIVE
  To evaluate the clinical characteristics of oropharyngeal dysphagia in Machado-Joseph disease patients.
  
  
  METHODS
  Forty individuals participated in this study, including 20 with no clinical complaints and 20 dysphagic patients with Machado-Joseph disease of clinical type 1, who were all similar in terms of gender distribution, average age, and cognitive function. The medical history of each patient was reviewed and each subject underwent a clinical evaluation of deglutition. At the end, the profile of dysphagia in patients with Machado-Joseph disease was classified according to the Severity Scale of Dysphagia, as described by O'Neil and collaborators.
  
  
  RESULTS
  Comparison between dysphagic patients and controls did not reveal many significant differences with respect to the clinical evaluation of the oral phase of deglutition, since afflicted patients only demonstrated deficits related to the protrusion, retraction and tonus of the tongue. However, several significant differences were observed with respect to the pharyngeal phase. Dysphagic patients presented pharyngeal stasis during deglutition of liquids and solids, accompanied by coughing and/or choking as well as penetration and/or aspiration; these signs were absent in the controls.
  
  
  CONCLUSIONS
  Oropharyngeal dysphagia is part of the Machado-Joseph disease since the first neurological manifestations. There is greater involvement of the pharyngeal phase, in relation to oral phase of the deglutition. The dysphagia of these patients is classified between mild and moderate.
authors.affiliation: Hospital das Clínicas, Universidade de São Paulo, SP, Brazil.
authors.forename: Sabrina Mello Alves
authors.initials: SM
authors.lastname: Corrêa
authors.forename: Valter Nilton
authors.initials: VN
authors.lastname: Felix
authors.forename: Jonas Lírio
authors.initials: JL
authors.lastname: Gurgel
authors.forename: Rubens A A
authors.initials: RA
authors.lastname: Sallum
authors.forename: Ivan
authors.initials: I
authors.lastname: Cecconello
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D003680:Deglutition Disorders / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000150:complications* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012720:Severity of Illness Index; D055815:Young Adult