title: Assessment of swallowing in motor neuron disease and Asidan/SCA36 patients with new methods.
abstract: BACKGROUND
  We report on a unique complication of cerebellar ataxia and motor neuron disease named Asidan/SCA36 with a high frequency of tongue atrophy. We aimed to elucidate dysphagia in amyotrophic lateral sclerosis (ALS) and spinal, bulbar muscular atrophy (SBMA), and Asidan/SCA36 patients with new methods.
  
  
  METHODS
  Patients diagnosed with ALS (n=20), SBMA (n=6), and Asidan (n=12) were included. A videofluoroscopic swallow study (VFS), an assessment of maximal tongue pressure (MTP), and impedance pharyngography (IPG) were applied.
  
  
  RESULTS
  The frequencies of VFS abnormalities were 70%, 50%, and 33% in ALS, SBMA, and Asidan/SCA36, respectively. Compared with control subjects (31.6 ± 6.3 kPa, mean ± SD), MTP was significantly decreased in ALS patients and SBMA patients, but was relatively preserved in Asidan patients. ALS patients performed more swallowing actions (Ns) detected by IPG than did control subjects, but SBMA and Asidan/SCA36 patients performed similar Ns to control subjects.
  
  
  CONCLUSIONS
  VFS showed a higher frequency of swallowing abnormalities in ALS patients. MTP and IPG measurements showed the most severe involvement in ALS patients and a relatively preserved swallowing function in SBMA and Asidan/SCA36 patients.
authors.affiliation: Department of Neurology Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Okayama, Japan.
authors.forename: Nobutoshi
authors.initials: N
authors.lastname: Morimoto
authors.forename: Toru
authors.initials: T
authors.lastname: Yamashita
authors.forename: Kota
authors.initials: K
authors.lastname: Sato
authors.forename: Tomoko
authors.initials: T
authors.lastname: Kurata
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Toshimasa
authors.initials: T
authors.lastname: Kusuhara
authors.forename: Naomichi
authors.initials: N
authors.lastname: Murata
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000503:physiopathology; D003679:Deglutition / Q000502:physiology*; D003680:Deglutition Disorders / Q000209:etiology* / Q000503:physiopathology*; D005260:Female; D005471:Fluoroscopy; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D020966:Muscular Disorders, Atrophic / Q000175:diagnosis / Q000503:physiopathology; D010614:Pharynx / Q000503:physiopathology; D012372:ROC Curve; D020754:Spinocerebellar Ataxias / Q000235:genetics / Q000503:physiopathology*; D014059:Tongue / Q000503:physiopathology