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PRION-1: Quinacrine for Human Prion Disease

This study has been completed.
Information provided by:
Medical Research Council Identifier:
First received: March 3, 2005
Last updated: April 6, 2015
Last verified: April 2015
PRION-1 aims to assess the activity and safety of Quinacrine (Mepacrine hydrochloride) in human prion disease. It also aims to establish an appropriate framework for the clinical assessment of therapeutic options for human prion disease that can be refined or expanded in the future, as new agents become available.

Condition Intervention
Prion Disease Drug: Quinacrine

Study Type: Interventional
Study Design: Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Treatment
Official Title: PRION-1: Quinacrine for Human Prion Disease. A Partially Randomized Patient Preference Trial to Evaluate the Activity and Safety of Quinacrine in Human Prion Disease

Resource links provided by NLM:

Further study details as provided by Medical Research Council:

Primary Outcome Measures:
  • Time to death
  • proportion of responders, with "responders" defined as patients showing either clinical improvement or lack of deterioration in 3 key neurological and neuropsychiatric measures

Secondary Outcome Measures:
  • Mini Mental State Examination (MMSE)
  • Clinician's Dementia rating (CDR)
  • Rankin score
  • Alzheimer's Disease Assessment Scale - Cognitive (ADAS-Cog)
  • Glasgow coma score
  • Barthel Activities of Daily Living (ADL)
  • magnetic resonance imaging scan (MRI)
  • electro-encephalogram (EEG)
  • cerebrospinal fluid (CSF)

Estimated Enrollment: 160
Study Start Date: June 2004
Estimated Study Completion Date: March 2007
Detailed Description:

The human prion diseases have been traditionally classified into Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) disease and kuru. They can alternatively be classified into three causal categories: sporadic, acquired and inherited. The appearance of a new human prion disease, variant CJD (vCJD), in the United Kingdom from 1995 onwards, and the experimental evidence that this is caused by the same prion strain as that causing bovine spongiform encephalopathy (BSE) in cattle, has raised the possibility that a major epidemic of vCJD will occur in the United Kingdom and other countries as a result of dietary or other exposure to BSE prions. These concerns have led to intensified efforts to develop therapeutic interventions.

Quinacrine has been previously used to treat other diseases such as malaria; however, it was found to have serious side effects and is no longer licensed in the United Kingdom. There is only very limited evidence from laboratory tests for the potential use of quinacrine in human prion disease, and the evidence to date for any possible clinical benefit is very scarce. The PRION-1 trial is being undertaken since there are no other drugs currently available which are considered suitable for human evaluation.


Ages Eligible for Study:   12 Years and older   (Child, Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No

Inclusion Criteria:

  • Aged 12 years or more, diagnosed with any type of human prion disease.

Exclusion Criteria:

  • In a coma, or in a pre-terminal phase of disease such that prolongation of the current quality of life would not be supported
  • Known sensitivity to quinacrine
  • Been taking any other putative anti-prion therapy for less than 8 weeks
  Contacts and Locations
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Please refer to this study by its identifier: NCT00104663

United Kingdom
National Prion Clinic
London, United Kingdom, WC1N 3BG
Sponsors and Collaborators
Medical Research Council
Principal Investigator: John Collinge, MD, FRCP MRC Prion Unit
Study Director: Janet Darbyshire, MBChB, FRCP MRC Clinical Trials Unit
  More Information Identifier: NCT00104663     History of Changes
Other Study ID Numbers: Version 1.1
Grant ID:71361
Study First Received: March 3, 2005
Last Updated: April 6, 2015

Keywords provided by Medical Research Council:
Creutzfeldt-Jakob disease
new variant CJD

Additional relevant MeSH terms:
Prion Diseases
Central Nervous System Infections
Central Nervous System Diseases
Nervous System Diseases
Neurodegenerative Diseases
Anticestodal Agents
Antiplatyhelmintic Agents
Antiparasitic Agents
Anti-Infective Agents
Antiprotozoal Agents
Antinematodal Agents
Antineoplastic Agents
Enzyme Inhibitors
Molecular Mechanisms of Pharmacological Action processed this record on August 18, 2017