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Title: Clinical aspects of veriant CJD in the UK, 1995-2005
Author: Heath, Craig A.
Awarding Body: University of Aberdeen
Current Institution: University of Aberdeen
Date of Award: 2008
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Aims: 1. Explore the process of diagnosis and determine the factors responsible for diagnostic delay. 2. Explore the process of surveillance and determine the effectiveness of voluntary referral. 3. Determine the value of the current WHO diagnostic criteria. 4. Provide a comprehensive overview of the clinical and investigative phenotype of vCJD. ISO cases were identified in the UK between 1995 and 2005. The median age at onset was 26. The median duration of illness was 14 months although a single case remains alive after 70 months. There is a significant delay between onset and the first point of medical contact (mean of 3.4 months) and a significant delay between onset and neurological review (mean of 7.4 months). The clinical phenotype has remained relatively stereotyped with almost 70% of patients present with psychiatric features and most neurological features developing after the first six months. Following this development, a relentless deterioration ensues with a median time to become bed-bound of only 13 months. Brain MRI remains the most important diagnostic aid in vCJD with over 90% of patients providing a positive scan. Tonsil biopsy may provide support for the clinical diagnosis but should only be considered if genuine doubts remain following non-invasive investigations. In their current form the WHO diagnostic criteria are both sensitive and specific with a sensitivity of over 80% and a specificity of 100%. A voluntary system of case referral has been an overwhelming success in the UK with all suspected cases referred. The current WHO diagnostic criteria may be of value to both researcher and clinician alike. Despite voluntary referral, clinicians throughout the UK have been exceptionally supportive of the NCJDSU resulting in an effective surveillance programme.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available