RecruitingPhase 1Phase 2NCT06153966

PrProfile: A Study to Assess the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of ION717

A Phase 1/2a Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Intrathecally Administered ION717 in Patients With Prion Disease


Sponsor

Ionis Pharmaceuticals, Inc.

Enrollment

76 participants

Start Date

Jan 4, 2024

Study Type

INTERVENTIONAL

Conditions

Summary

The purpose of this study is to evaluate the safety, tolerability, pharmacokinetics and pharmacodynamics of intrathecal (IT) delivery of ION717.


Eligibility

Min Age: 18 Years

Inclusion Criteria5

  • A confirmed diagnosis of probable or definite prion disease.
  • Early-stage prion disease at the time of Screening.
  • Willing to meet all study requirements, including travel to Study Center, procedures, measurements and visits.
  • Patients must have a caregiver who is ≥ 18 years old and who is able and willing to facilitate the patient's involvement, to the best of their ability, for the duration of the trial; caregivers must also be able and willing to provide information about themselves and the patient for the duration of the trial.
  • Aged ≥ 18 at the time of informed consent.

Exclusion Criteria5

  • Clinically significant abnormalities in medical history, laboratory tests or physical examination that would render a patient unsuitable for inclusion.
  • Any contraindication or unwillingness to undergo an MRI.
  • Obstructive hydrocephalus, presence of a functional ventriculoperitoneal shunt for the drainage of cerebrospinal fluid (CSF) or an implanted central nervous system (CNS) catheter.
  • Known brain or spinal disease that would interfere with the LP process, CSF circulation or safety assessment.
  • Have any other condition, which, in the opinion of the Investigator would make the patient unsuitable for inclusion or could interfere with the patient participating in or completing the study.

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Interventions

DRUGION717

ION717 will be administered by IT injection.

DRUGPlacebo

Placebo-matching ION717 will be administered by IT injection.


Locations(13)

Massachusetts General Hospital

Boston, Massachusetts, United States

NYU Langone Health

New York, New York, United States

University Hospitals Cleveland Medical Center

Cleveland, Ohio, United States

Royal Melbourne Hospital

Parkville, Australia

McGill University Health Centre

Montreal, Quebec, Canada

Hôpital Universitaire Pitié Salpêtrière

Paris, France

University Medical Center Göttingen

Göttingen, Germany

Tel Aviv Sourasky Medical Center

Tel Aviv, Israel

Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta

Milan, Italy

Mihara Memorial Hospital

Gunma, Isesaki-shi, Japan

National Center of Neurology and Psychiatry

Tokyo, Kodaira-shi, Japan

Neuromuscular Center Yoshimizu Hospital

Yamaguchi, Shimonoseki-shi, Japan

Hospital Clinic De Barcelona

Barcelona, Spain

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NCT06153966


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