title: Diagnosing variant Creutzfeldt-Jakob disease: a retrospective analysis of the first 150 cases in the UK.
abstract: BACKGROUND
  Establishing an early clinical diagnosis in variant Creutzfeldt-Jakob disease (vCJD) can be difficult, resulting in extended periods of uncertainty for many families and sometimes a view that patients have been subjected to unnecessary investigations. This issue is accentuated by the progressive nature of vCJD and by the difficulty in achieving a confident clinical diagnosis before an advanced stage of illness. Although diagnostic delay may be a result of the non-specific early clinical features, a systematic analysis of the process of diagnosis was undertaken, with the aim of trying to achieve earlier diagnosis of vCJD.
  
  
  METHODS
  Retrospective case file analysis was undertaken of the first 150 definite and clinically probable cases of vCJD identified by the UK surveillance system.
  
  
  RESULTS
  There is a significant interval between illness onset and presentation to a primary care physician, which is influenced by the nature of the initial clinical features. Neurological review is invariably sought following the development of clinical signs and a diagnosis is then established relatively quickly. Despite the progressive clinical course, a confident clinical diagnosis is not usually achieved until a relatively advanced stage of illness (mean time to diagnosis 10.5 months) with a more rapid clinical progression accounting for those cases diagnosed earlier after symptom onset.
  
  
  CONCLUSIONS
  Early clinical diagnosis in vCJD is not possible in the great majority of cases because of non-specific initial symptoms. Once neurological signs develop, a diagnosis is usually made promptly but this is often at a relatively advanced stage of illness. The inherent delays in the diagnosis of vCJD have implications for those involved in both public health and therapeutics.
authors.affiliation: Western General Hospital, Edinburgh, UK.
authors.forename: C A
authors.initials: CA
authors.lastname: Heath
authors.forename: S A
authors.initials: SA
authors.lastname: Cooper
authors.forename: K
authors.initials: K
authors.lastname: Murray
authors.forename: A
authors.initials: A
authors.lastname: Lowman
authors.forename: C
authors.initials: C
authors.lastname: Henry
authors.forename: M A
authors.initials: MA
authors.lastname: MacLeod
authors.forename: G
authors.initials: G
authors.lastname: Stewart
authors.forename: M
authors.initials: M
authors.lastname: Zeidler
authors.forename: J M
authors.initials: JM
authors.lastname: McKenzie
authors.forename: R S G
authors.initials: RS
authors.lastname: Knight
authors.forename: R G
authors.initials: RG
authors.lastname: Will
mesh_terms: D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D003937:Diagnosis, Differential; D003943:Diagnostic Techniques, Neurological / Q000706:statistics & numerical data; D042241:Early Diagnosis; D006801:Humans; D012017:Referral and Consultation / Q000706:statistics & numerical data; D012189:Retrospective Studies; D006113:United Kingdom