title: Premotor-motor excitability is altered in dopa-responsive dystonia.
abstract: BACKGROUND
  Dopa-responsive dystonia is clinically dominated by a combination of dystonia and parkinsonism, both known to be associated with abnormal activity in premotor-motor circuits.
  
  
  METHODS
  To probe premotor-motor excitability, we used a transcranial magnetic stimulation dual pulse conditioning paradigm in 15 genetically confirmed dopa-responsive dystonia patients and 20 controls under different medication states. We also determined silent periods, short-latency afferent inhibition, interhemispheric inhibition, and short-interval intracortical inhibition and facilitation.
  
  
  RESULTS
  In contrast to healthy controls, no motor cortex inhibition was seen after premotor conditioning regardless of the dopaminergic state in patients. The duration of the ipsilateral silent period was increased in the OFF state, and short-latency afferent inhibition was reduced in the ON compared with the OFF state.
  
  
  CONCLUSIONS
  Premotor-motor circuits appear hyporesponsive in dopa-responsive dystonia.
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.|Department of Neurology, University of Luebeck, Germany.
authors.forename: Anne
authors.initials: A
authors.lastname: Weissbach
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.
authors.forename: Tobias
authors.initials: T
authors.lastname: Bäumer
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.|Department of Neurology, University of Luebeck, Germany.
authors.forename: Norbert
authors.initials: N
authors.lastname: Brüggemann
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.|Department of Neurology, University of Luebeck, Germany.
authors.forename: Vera
authors.initials: V
authors.lastname: Tadic
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.
authors.forename: Simone
authors.initials: S
authors.lastname: Zittel
authors.affiliation: Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Bastian
authors.initials: B
authors.lastname: Cheng
authors.affiliation: Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Götz
authors.initials: G
authors.lastname: Thomalla
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.
authors.forename: Christine
authors.initials: C
authors.lastname: Klein
authors.affiliation: Institute of Neurogenetics, University of Luebeck, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Münchau
mesh_terms: D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D020821:Dystonic Disorders / Q000235:genetics / Q000473:pathology*; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology*; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000503:physiopathology*; D009433:Neural Inhibition / Q000502:physiology; D011930:Reaction Time; D050781:Transcranial Magnetic Stimulation / Q000379:methods; D055815:Young Adult
keywords: Parkinsonism; dopa-responsive dystonia; dystonia; motor cortex; premotor cortex