title: Pharyngo-esophageal motility disturbances in patients with myotonic dystrophy.
abstract: BACKGROUND
  Esophageal motility is often disturbed in patients with myotonic dystrophy. The esophageal motor derangement pattern and its correlation with esophageal and peripheral motor symptoms is not well defined. Our aims were to evaluate: 1) pharyngo-esophageal motor abnormalities in these patients; 2) the relationship between motor involvement and clinical manifestations; and 3) the correlation between pharyngo-esophageal motility abnormalities and peripheral neuromuscular involvement.
  
  
  METHODS
  We compared data from 18 patients and 18 healthy controls. Neuromuscular affectation was quantified with a five-point muscular disability rating scale. Pharyngo-esophageal symptoms were assessed with a directed questionnaire, whereas motility was evaluated by means of manometry.
  
  
  RESULTS
  Myotonic dystrophy patients had diminished pharyngeal contraction amplitude, upper esophageal sphincter basal pressure, and esophageal body contraction amplitude compared with the control group (P < 0.001). No signs of esophageal myotony were evident. Simultaneous esophageal waves after more than 40% of liquid swallows were found in 80% of patients. No relationship between esophageal manometric alteration and esophageal or peripheral motility symptoms was elicited.
  
  
  CONCLUSIONS
  In patients with myotonic dystrophy pharyngo-esophageal motility is severely deranged in both amplitude and coordination. These abnormalities may be present even if symptoms referred by the patient or the severity of the disease is not remarkable.
authors.affiliation: Neurology Dept, Vall d'Hebron General University Hospital, Barcelona, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Modolell
authors.forename: F
authors.initials: F
authors.lastname: Mearin
authors.forename: J S
authors.initials: JS
authors.lastname: Baudet
authors.forename: J
authors.initials: J
authors.lastname: Gámez
authors.forename: C
authors.initials: C
authors.lastname: Cervera
authors.forename: J R
authors.initials: JR
authors.lastname: Malagelada
mesh_terms: D000293:Adolescent; D000328:Adult; D015154:Esophageal Motility Disorders / Q000503:physiopathology*; D004943:Esophagogastric Junction / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008365:Manometry / Q000379:methods; D008875:Middle Aged; D009043:Motor Activity; D009223:Myotonic Dystrophy / Q000503:physiopathology*; D009468:Neuromuscular Diseases / Q000503:physiopathology; D017933:Peripheral Nervous System / Q000503:physiopathology; D010614:Pharynx / Q000503:physiopathology*; D018709:Statistics, Nonparametric