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Saturday, July 12th 2008

First Session - CJD 2008: Treatment

Chairman: Neil Cashman, M.D.

Presentations:

  • Introduction
  • Prions
  • Prion Vaccination
  • A review of human prion disease treatment
  • Doxycycline treatment in Creutzfeldt-Jakob disease
  • Doxycycline treatment as a therapeutic approach in Creutzfeldt-Jakob-Disease
  • An Update on the First U.S. Treatment Trial for Sporadic Jakob-Creutzfeldt Disease (sCJD)


Introduction

Length: 4 min (chaptered)
Presenter: Neil Cashman

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Prions

Length: 28 min (Chaptered)
Presenter: Valerie Sim

  • PRION DISEASE - A review of experimental drug treatments
  • Normal prion protein
  • TSE-associated prion protein
  • Therapeutic targets - Where to begin?
  • ...

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Prion Vaccination

Length: 25 min (Chaptered)
Presenter: Thomas Wisniewski

  • CONFORMATIONAL DISORDERS
  • Current Vaccination Trials Targeting Amyloid b in Alzheimer’s Disease
  • Vaccination with Ab Peptides as Treatment for Alzheimer’s Disease
  • Balance of Benefit versus Toxicity
  • Initial Results of Human Active Vaccination for AD

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A review of human prion disease treatment

Length: 24 min (Chaptered)
Presenter: Richard Knight

  • Introduction
  • A mixed audience - different backgrounds - different perspectives
  • Human prion diseases
  • Treatments: How do you find them?
  • What do treatments do?
  • ...

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Doxycycline treatment in Creutzfeldt-Jakob disease

Length: 21 min (Chaptered)
Presenter: Fabrizio Tagliavini

  • New Drug Therapy...
  • Effectiveness of Anthracycline...
  • Tetracycline
  • Tetracyclines affect prion infectivity
  • The efficacy of Tetracyclines...
  • Molecular basis of anti-prion activity in vitro experiments
  • ...

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Doxycycline treatment as a therapeutic approach in Creutzfeldt-Jakob-Disease

Length: 13 min (Chaptered)
Presenter: Inga Zerr

  • Doxycycline in Patients with CJD
  • Outline of the clinical trial
  • Follow up
  • Observational trial
  • Preliminary results
  • ...

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An Update on the First U.S. Treatment Trial for Sporadic Jakob-Creutzfeldt Disease (sCJD)

Length: 19 min (Chaptered)
Presenter: Michael D. Geschwind

  • Actual Diagnoses of UCSF Potential CJD Referrals 8/2001-7/2008
  • Quinacrine & Chlorpromazine effective in vitro
  • Study Design: Randomized, double-blinded, placebo controlled (delayed start)
  • Status of potential CJD cases referred to the trial (2/22/05 to 7/8/08)
  • Geographic Distribution – Referrals vs. Enrollments
  • ...

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Second Session - Surveillance Centers Reports

Chairman: Robert Will, M.D., FRCP(E)

Presentations:

  • National Prion Disease Pathology Surveillance Center
  • Surveillance Center Reports: the UK
  • CJD in Germany
  • A decade of prospective CJD surveillance in Canada
  • CJD Surveillance in Mexico


National Prion Disease Pathology Surveillance Center

Length: 23 min (Chaptered)
Presenter: Pierluigi Gambetti

  • The Goals of Surveillance
  • Diagnosing a Case
  • National Prion Disease Pathology Surveillance Center - Cases Examined - (June 2008)
  • Cases Examined as Percent of Cases Expected
  • Increasing the Number of Cases
  • ...

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Surveillance Center Reports: the UK

Length: 12 min (Chaptered)
Presenter: RG Will

  • CLINICAL FEATURES OF SPORADIC AND VARIANT CJD
  • Time to suspect sCJD for Cases and Non Cases in 1993/4 and 2003/4
  • vCJD: Time to Suspect CJD by year of onset (n=147)
  • REFERRALS TO NCJDSU 1 MAY 1990 – 31 DECEMBER 2007
  • DIAGNOSTIC CLASSIFICATION OF REFERRALS
  • ...

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CJD in Germany

Length: 15 min (Chaptered)
Presenter: Inga Zerr

  • Surveillance data
  • NRZ
  • Differential diagnosis (neuropathology)
  • Confirmed sCJD classified as ‘other‘ (n=39)
  • Increase of atypical subtypes?
  • ...

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A decade of prospective CJD surveillance in Canada

Length: 13 min (Chaptered)
Presenter: Gerard H. Jansen

  • Canadian National Blood Safety Surveillance (1998)
  • Yearly Referrals to CJD-SS April 1, 1998-February 29, 2008
  • Inventory of Notifications & Cases
  • Prion disease cases
  • CJD Cases (n=359)
  • ...

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CJD Surveillance in Mexico

Length: 17 min (Chaptered)
Presenter: Victor Sanchez

  • Population Health coverage
  • Obtain preliminary prevalence
  • Figures
  • Photos
  • CJD Surveillance in Mexico
  • ...

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