title: Huntington chorea presenting with motor neuron disease.
abstract: BACKGROUND
  There have been a few case reports of motor neuron disease in association with Huntington disease (HD).
  
  
  OBJECTIVE
  To describe a patient presenting with prominent fasciculations, chorea, and possible amyotrophic lateral sclerosis (ALS) in whom genetic testing revealed HD mutation.
  
  
  METHODS
  Case report.
  
  
  METHODS
  University of Texas Southwestern Medical Center, Dallas. Patient  A 69-year-old man with chorea and fasciculations.
  
  
  METHODS
  Genetic and electrophysiologic testing.
  
  
  METHODS
  Genetic test result, electrophysiologic test result, and physical examination.
  
  
  RESULTS
  A 69-year-old man with long-standing depression and failing memory presented with muscle twitches of 8 months' duration. He was found to have choreoathetoid movements and distal weakness on neurological examination. Electrophysiologic studies revealed evidence of motor neuron disease. Genetic test showed CAG repeat of 40 on chromosome 4, confirming the diagnosis of HD.
  
  
  CONCLUSIONS
  Motor neuron disease can rarely occur in patients with HD and could be one of its presenting features.
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX 75390-8897, USA.
authors.forename: Hamid
authors.initials: H
authors.lastname: Sadeghian
authors.forename: Padraig E
authors.initials: PE
authors.lastname: O'Suilleabhain
authors.forename: James
authors.initials: J
authors.lastname: Battiste
authors.forename: Jeffrey L
authors.initials: JL
authors.lastname: Elliott
authors.forename: Jaya R
authors.initials: JR
authors.lastname: Trivedi
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics; D002574:Cervical Vertebrae; D002894:Chromosomes, Human, Pair 4 / Q000235:genetics*; D003937:Diagnosis, Differential; D004594:Electrophysiology; D005207:Fasciculation / Q000235:genetics; D005820:Genetic Testing; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000175:diagnosis* / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000235:genetics*; D009460:Neurologic Examination; D055009:Spondylosis / Q000150:complications / Q000601:surgery; D019680:Trinucleotide Repeat Expansion