title: Proposed diagnostic criteria for cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS).
abstract: OBJECTIVE
  Diagnosis of ataxic disorders is an important clinical challenge upon which prognostication, management, patient solace, and, above all, the hope of future treatment all rely. Heritable diseases and the possibility of affected offspring carry the added burden of portending adverse health, social and financial ramifications.
  
  
  RESULTS
  Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is an inherited multisystem ataxia compromising cerebellar, vestibular, and sensory function. It is not uncommon, but despite early attempts the genetic defect is yet to be identified. As the search for the causative gene continues, we have found it useful to further define this syndrome in terms of its likely phenotype.
  
  
  CONCLUSIONS
  We propose staged diagnostic criteria based on the identified pathology in CANVAS. We envisage that these criteria will aid the clinician in diagnosing CANVAS and the researcher in further elucidating this complex disorder.
authors.affiliation: University of Melbourne (DJS), Royal Victorian Eye & Ear Hospital, Melbourne, Australia; Department of Neuroscience (LR), St Vincent's Hospital, Melbourne, Australia; Department of Anatomical Pathology (CAL), Alfred Hospital, Melbourne, Australia; Vestibular Research Laboratory (HGM), School of Psychology, University of Sydney, Australia; Department of Neuroscience (GMH), Monash University, Melbourne, Australia; and Department of Neurology (ES), Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: David J
authors.initials: DJ
authors.lastname: Szmulewicz
authors.affiliation: University of Melbourne (DJS), Royal Victorian Eye & Ear Hospital, Melbourne, Australia; Department of Neuroscience (LR), St Vincent's Hospital, Melbourne, Australia; Department of Anatomical Pathology (CAL), Alfred Hospital, Melbourne, Australia; Vestibular Research Laboratory (HGM), School of Psychology, University of Sydney, Australia; Department of Neuroscience (GMH), Monash University, Melbourne, Australia; and Department of Neurology (ES), Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Leslie
authors.initials: L
authors.lastname: Roberts
authors.affiliation: University of Melbourne (DJS), Royal Victorian Eye & Ear Hospital, Melbourne, Australia; Department of Neuroscience (LR), St Vincent's Hospital, Melbourne, Australia; Department of Anatomical Pathology (CAL), Alfred Hospital, Melbourne, Australia; Vestibular Research Laboratory (HGM), School of Psychology, University of Sydney, Australia; Department of Neuroscience (GMH), Monash University, Melbourne, Australia; and Department of Neurology (ES), Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Catriona A
authors.initials: CA
authors.lastname: McLean
authors.affiliation: University of Melbourne (DJS), Royal Victorian Eye & Ear Hospital, Melbourne, Australia; Department of Neuroscience (LR), St Vincent's Hospital, Melbourne, Australia; Department of Anatomical Pathology (CAL), Alfred Hospital, Melbourne, Australia; Vestibular Research Laboratory (HGM), School of Psychology, University of Sydney, Australia; Department of Neuroscience (GMH), Monash University, Melbourne, Australia; and Department of Neurology (ES), Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Hamish G
authors.initials: HG
authors.lastname: MacDougall
authors.affiliation: University of Melbourne (DJS), Royal Victorian Eye & Ear Hospital, Melbourne, Australia; Department of Neuroscience (LR), St Vincent's Hospital, Melbourne, Australia; Department of Anatomical Pathology (CAL), Alfred Hospital, Melbourne, Australia; Vestibular Research Laboratory (HGM), School of Psychology, University of Sydney, Australia; Department of Neuroscience (GMH), Monash University, Melbourne, Australia; and Department of Neurology (ES), Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: G Michael
authors.initials: GM
authors.lastname: Halmagyi
authors.affiliation: University of Melbourne (DJS), Royal Victorian Eye & Ear Hospital, Melbourne, Australia; Department of Neuroscience (LR), St Vincent's Hospital, Melbourne, Australia; Department of Anatomical Pathology (CAL), Alfred Hospital, Melbourne, Australia; Vestibular Research Laboratory (HGM), School of Psychology, University of Sydney, Australia; Department of Neuroscience (GMH), Monash University, Melbourne, Australia; and Department of Neurology (ES), Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Elsdon
authors.initials: E
authors.lastname: Storey