title: Observing Huntington's Disease: the European Huntington's Disease Network's REGISTRY.
abstract: BACKGROUND
  Huntington's disease (HD) is a rare triplet repeat (CAG) disorder. Advanced, multi-centre, multi-national research frameworks are needed to study simultaneously multiple complementary aspects of HD. This includes the natural history of HD, its management and the collection of clinical information and biosamples for research.
  
  
  METHODS
  We report on cross-sectional data of the first 1766 participants in REGISTRY, the European Huntington's Disease Network's (EHDN), multi-lingual, multi-national prospective observational study of HD in Europe. Data collection (demographics, phenotype, genotype, medication, co-morbidities, biosamples) followed a standard protocol.
  
  
  RESULTS
  Phenotype, and the HD genotype, of manifest HD participants across different European regions was similar. Motor onset was most common (48%) with a non-motor onset in more than a third of participants. Motor signs increased, and cognitive abilities and functional capacity declined as the disease burden (CAGn-35.5) X age) increased. A life-time history of behavioural symptoms was common, but the behavioural score was not related to disease burden. One fifth of participants had severe psychiatric problems, e.g. suicidal ideation and attempts, and/or irritability/aggression, with psychosis being less common. Participants on anti-dyskinetic medication had a higher motor and lower cognitive score, were older, and more prone to physical trauma. A higher motor and a lower cognitive score predicted more advanced disease.
  
  
  CONCLUSIONS
  The unparalleled collection of clinical data and biomaterials within the EHDN's REGISTRY can expedite the search for disease modifiers (genetic and environmental) of age at onset and disease progression that could be harnessed for the development of novel treatments.
authors.affiliation: Department of Neurology, University of Ulm, Ulm Germany
authors.forename: Michael
authors.initials: M
authors.lastname: Orth
authors.forename: Olivia J
authors.initials: OJ
authors.lastname: Handley
authors.forename: Carsten
authors.initials: C
authors.lastname: Schwenke
authors.forename: Stephen B
authors.initials: SB
authors.lastname: Dunnett
authors.forename: David
authors.initials: D
authors.lastname: Craufurd
authors.forename: Aileen K
authors.initials: AK
authors.lastname: Ho
authors.forename: Edward
authors.initials: E
authors.lastname: Wild
authors.forename: Sarah J
authors.initials: SJ
authors.lastname: Tabrizi
authors.forename: G Bernhard
authors.initials: GB
authors.lastname: Landwehrmeyer