Phase 1/2 Clinical Trial of LY3884963 in Patients With Frontotemporal Dementia With Progranulin Mutations (FTD-GRN)

NCT ID: NCT04408625

Last Updated: 2025-12-15

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE1/PHASE2

Total Enrollment

35 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-11-09

Study Completion Date

2031-04-30

Brief Summary

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Study J4B-MC-OKAA is a Phase 1/2, multi-center, open-label ascending dose, first-in-human study that will evaluate the safety and effect of intra-cisternal LY3884963 administration on progranulin protein (PGRN) levels in patients with frontotemporal dementia with progranulin mutations (FTD-GRN). Two escalating dose (low dose and medium dose) cohorts are planned, as well as one bridging cohort which will allocate patients to receive either low or medium dose. The duration of the study is 5 years. During the first year, patients will be evaluated for the effect of LY3884963 on safety, tolerability, immunogenicity, biomarkers, and efficacy. Patients will follow up for an additional 4 years to monitor safety and changes on selected biomarkers and clinical outcomes.

Detailed Description

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Conditions

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Frontotemporal Dementia

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

SEQUENTIAL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Initial Cohort - Low dose

Group Type EXPERIMENTAL

LY3884963

Intervention Type BIOLOGICAL

Participants will receive a single dose of LY3884963, administered intra cisterna magna

Methylprednisolone

Intervention Type DRUG

IV pulses every 2 weeks in the first 3 months.

Optional Sirolimus

Intervention Type DRUG

At the investigators discretion following steroid tolerability issues, patients may receive a loading dose, followed by maintenance dose, followed by dose tapering; administered as concomitant medication

Optional Prednisone

Intervention Type DRUG

If needed and at the investigator discretion, Oral Prednisone may be added to the immunosuppression regimen

Initial Cohort - Medium dose

Group Type EXPERIMENTAL

LY3884963

Intervention Type BIOLOGICAL

Participants will receive a single dose of LY3884963, administered intra cisterna magna

Methylprednisolone

Intervention Type DRUG

IV pulses every 2 weeks in the first 3 months.

Optional Sirolimus

Intervention Type DRUG

At the investigators discretion following steroid tolerability issues, patients may receive a loading dose, followed by maintenance dose, followed by dose tapering; administered as concomitant medication

Optional Prednisone

Intervention Type DRUG

If needed and at the investigator discretion, Oral Prednisone may be added to the immunosuppression regimen

Bridging Cohort - Low dose

Participants enrolled in the Bridging Cohort will be assigned to either low or medium dose in an alternating manner

Group Type EXPERIMENTAL

LY3884963

Intervention Type BIOLOGICAL

Participants will receive a single dose of LY3884963, administered intra cisterna magna

Methylprednisolone

Intervention Type DRUG

IV pulses every 2 weeks in the first 3 months.

Optional Sirolimus

Intervention Type DRUG

At the investigators discretion following steroid tolerability issues, patients may receive a loading dose, followed by maintenance dose, followed by dose tapering; administered as concomitant medication

Optional Prednisone

Intervention Type DRUG

If needed and at the investigator discretion, Oral Prednisone may be added to the immunosuppression regimen

Bridging Cohort - Medium dose

Participants enrolled in the Bridging Cohort will be assigned to either low or medium dose in an alternating manner

Group Type EXPERIMENTAL

LY3884963

Intervention Type BIOLOGICAL

Participants will receive a single dose of LY3884963, administered intra cisterna magna

Methylprednisolone

Intervention Type DRUG

IV pulses every 2 weeks in the first 3 months.

Optional Sirolimus

Intervention Type DRUG

At the investigators discretion following steroid tolerability issues, patients may receive a loading dose, followed by maintenance dose, followed by dose tapering; administered as concomitant medication

Optional Prednisone

Intervention Type DRUG

If needed and at the investigator discretion, Oral Prednisone may be added to the immunosuppression regimen

Cohort 5-Medium Dose

Will enroll up to 10 participants with early phase of disease

Group Type EXPERIMENTAL

LY3884963

Intervention Type BIOLOGICAL

Participants will receive a single dose of LY3884963, administered intra cisterna magna

Methylprednisolone

Intervention Type DRUG

IV pulses every 2 weeks in the first 3 months.

Optional Sirolimus

Intervention Type DRUG

At the investigators discretion following steroid tolerability issues, patients may receive a loading dose, followed by maintenance dose, followed by dose tapering; administered as concomitant medication

Optional Prednisone

Intervention Type DRUG

If needed and at the investigator discretion, Oral Prednisone may be added to the immunosuppression regimen

Interventions

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LY3884963

Participants will receive a single dose of LY3884963, administered intra cisterna magna

Intervention Type BIOLOGICAL

Methylprednisolone

IV pulses every 2 weeks in the first 3 months.

Intervention Type DRUG

Optional Sirolimus

At the investigators discretion following steroid tolerability issues, patients may receive a loading dose, followed by maintenance dose, followed by dose tapering; administered as concomitant medication

Intervention Type DRUG

Optional Prednisone

If needed and at the investigator discretion, Oral Prednisone may be added to the immunosuppression regimen

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Men or women aged 30 to 85 years (inclusive), at the time of informed consent.
* Body weight range of ≥40 kg (88 lbs) to ≤110 kg (242 lb) and a BMI of 18 to 34 kg/m2.
* Has symptomatic frontotemporal dementia (FTD), including mild behavioral, cognitive, motor or language impairment per Investigator's assessment (behavioral-variant FTD, primary progressive aphasia-FTD, FTD with corticobasal syndrome, or a combination of syndromes are allowed for enrollment).
* Score ≥0.5 and ≤15 on CDR plus NACC FTLD sum of boxes (Cohorts 1-4 only). Note: In Cohort 5 only patients with CDR plus NACC FTLD with sum of boxes ≥0.5 and ≤9 AND global score of 0.5 or 1 will be enrolled.
* Stable use of background medications at least 8 weeks prior to LY3884963 dosing.
* Carrier of a pathogenic progranulin gene (GRN) mutation.
* Negative screening test for Mycobacterium tuberculosis (MTB) or documented negative MTB test within 1year prior to screening.
* Age- and gender-appropriate cancer screenings are up-to-date and completed.
* Patient and/or patient's legally authorized representative has the ability to understand the purpose and risks of the study, and provide written informed consent and authorization to use protected health information.
* Women of nonchildbearing potential must be either surgically sterile (hysterectomy, bilateral tubal ligation, salpingectomy, and/or bilateral oophorectomy at least 26 weeks before Screening) or postmenopausal, defined as spontaneous amenorrhea for at least 2 years, with follicle stimulating hormone level in the postmenopausal range at Screening based on the central laboratory's range.
* Men and women of childbearing potential (i.e., ovulating, premenopausal, and not surgically sterile) must use a highly effective method of contraception consistently and correctly for the duration of the study, including the long term follow up. Highly effective methods of contraception are those that, alone or in combination, result in a failure rate of less than 1% per year when used consistently and correctly (i.e., perfect use) and include the following for female patients of childbearing potential:

* Combined (estrogen and progestogen containing) oral, intravaginal, or transdermal hormonal contraception associated with inhibition of ovulation.
* Oral, injectable, or implantable progestogen-only hormonal contraception associated with inhibition of ovulation.
* Intrauterine device.
* Intrauterine hormone-releasing system.
* Bilateral tubal ligation or bilateral tubal occlusion (performed at least 3 months prior to Screening).
* Vasectomized partner (performed at least 3 months prior to Screening).
* Sexual abstinence (no sexual intercourse), if in line with the patient's usual and preferred lifestyle.
* Acceptable forms of contraception for male patients include:

* Sexual abstinence (no sexual intercourse), if in line with the patient's usual and preferred lifestyle.
* History of vasectomy (performed at least 3 months prior to Screening, with documented absence of sperm in the ejaculate) in combination with condom.
* Condom with spermicide used together with highly effective female contraceptive methods if the female partner(s) is of childbearing potential (see above for list of acceptable female contraceptive methods).

Note: Individuals who are in exclusively same sex relationships (as their preferred and usual lifestyle) are not required to use contraception.

* Men must agree to use a condom during any sexual intercourse (including male patients who have had a vasectomy) and abstain from sperm donation for the duration of the study, including long-term follow-up.
* Women must agree to abstain from egg donation for the duration of the study, including long-term follow-up.
* Women of childbearing potential cannot be pregnant or lactating/breastfeeding and must have a negative result for the serum pregnancy test (β-human chorionic gonadotropin) at Screening.
* Patient must agree to abstain from blood donation for the first year following gene transfer.
* Patient has a reliable study partner/informant (e.g. family member, friend) willing and able to participate in the study as a source of information on the patient's health status and cognitive and functional abilities.
* Patient is not dependent on a walker or wheelchair.
* Patient is living in the community (i.e. not in nursing home); some levels of assisted living may be permitted at the discretion of the investigator.
* Pneumococcal pneumonia and shingles vaccines are required within 10 years of Screening allowed to be performed during Screening but must be given at least 4 weeks prior to initiation of immunosuppressant regimen.

Exclusion Criteria

* Diagnosis of a significant CNS (central nervous system) disease other than frontotemporal dementia (FTD) that may cause FTD symptoms or confound study objectives.
* Brain or cervical spine magnetic resonance image (MRI)/MRA imaging showing clinically significant abnormality considered to prevent intracisternal magna (ICM) injection.
* Hypersensitivity or contraindications to corticosteroid, and/or sirolimus use.
* Clinical evidence of peripheral symmetric sensory polyneuropathy (stable sensory mononeuropathies and radiculopathies are not exclusionary).
* Concomitant disease or condition within 6 months of screening that could interfere with, or treatment of which might interfere with, the conduct of the study or that would, in the opinion of the investigator, pose an unacceptable safety risk to the patient or interfere with the patient's ability to comply with study procedures
* Clinically significant laboratory test result abnormalities assessed at screening.
* Participation within 3 months prior to screening in another therapeutic investigational drug or device study with purported disease-modifying effects on FTD, unless it can be documented that the patient received placebo only.
* Any type of prior gene or cell therapy.
* Live vaccines in the 4 weeks prior to Screening. NOTE: Pneumococcal vaccine and/or shingles vaccine administration is allowed at least 4 weeks prior to initiation of immunosuppressant regimen.
* Use of blood thinners in the 2 weeks prior to screening, or anticipated use of blood thinners during the study. Antiplatelet therapies are acceptable if the patient is medically able to temporarily stop 48 hours to 7 days (depending on the antiplatelet medication used) prior to and at least 48 hours after ICM injection and LP. Note: the use of blood thinners as part of prophylaxis or treatment of an emergent VTE or another AE during the study does not exclude the patient, unless there is a baseline high risk of thromboembolic events, and use of blood thinners is highly anticipated in the opinion of the Investigator.
* Contraindications or intolerance to imaging methods (MRA, MRI, and/or computed tomography \[CT\]), including claustrophobia and intolerance to contrast agents used for MRI, MRA, or CT (including, but not limited to, gadolinium contrast agents and iohexol).
* Contraindications to general anesthesia or deep sedation.
* Positive urine test for drugs of abuse (including opiates, amphetamines, cocaine, barbiturates, and phencyclidine) without prescription at Screening and on Day 1.
Minimum Eligible Age

30 Years

Maximum Eligible Age

85 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Eli Lilly and Company

INDUSTRY

Sponsor Role collaborator

Prevail Therapeutics

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Travis Lewis, MD, PhD

Role: STUDY_DIRECTOR

Prevail Therapeutics

Locations

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k2 Medical Research-Maitland

Maitland, Florida, United States

Site Status RECRUITING

PPD Phase 1 Clinic, 100 West Gore Street, Suite 202

Orlando, Florida, United States

Site Status COMPLETED

Lahey Hospital & Medical Center, 41 Burlington Mall Road

Burlington, Massachusetts, United States

Site Status RECRUITING

Hospital of the University of Pennsylvania, 3 West Gates Building, 3400 Spruce Street

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

Royal Prince Alfred Hospital, Brain & Mind Research Institute, 94 Mallet Street

Camperdown, New South Wales, Australia

Site Status RECRUITING

UZ Leuven, Neurologie Herestraat 49

Leuven, , Belgium

Site Status RECRUITING

AP-HM Hôpital de La Timone

Saint-Pierre, Marseille, France

Site Status COMPLETED

Centre Mémoire de Ressources

Lille, , France

Site Status RECRUITING

Le Ber, Institut du Cerveau et de la Moelle Epinière

Paris, , France

Site Status RECRUITING

Hospital Clinic de Barcelona, Villaroel 170 Servicio de Neurología

Barcelona, , Spain

Site Status COMPLETED

Hospital Universitario de Donostia, Servicio De Neurologia, Consultas Externas Neurologia, San Sebastian, Guipúzcoa

Donostia / San Sebastian, , Spain

Site Status COMPLETED

University College London,Queen Square, Dementia Research Building, London,

London, , United Kingdom

Site Status RECRUITING

Countries

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United States Australia Belgium France Spain United Kingdom

Central Contacts

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Prevail Therapeutics

Role: CONTACT

(917) 336-9310

Facility Contacts

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Gina Lagares

Role: primary

321-204-9552

Catia Melo

Role: primary

781-744-1930

Dahlia Kamel

Role: primary

215-662-6134

Dewi Pane

Role: primary

61 95154540

Carine Schildermans

Role: primary

3126345500

Laëtitia Breuilh

Role: primary

+33 320446728

Sylvie Coudoin

Role: primary

01.42.16.57.62

Miguel Alvarez

Role: primary

References

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Sevigny J, Uspenskaya O, Heckman LD, Wong LC, Hatch DA, Tewari A, Vandenberghe R, Irwin DJ, Saracino D, Le Ber I, Ahmed R, Rohrer JD, Boxer AL, Boland S, Sheehan P, Brandes A, Burstein SR, Shykind BM, Kamalakaran S, Daniels CW, David Litwack E, Mahoney E, Velaga J, McNamara I, Sondergaard P, Sajjad SA, Kobayashi YM, Abeliovich A, Hefti F. Progranulin AAV gene therapy for frontotemporal dementia: translational studies and phase 1/2 trial interim results. Nat Med. 2024 May;30(5):1406-1415. doi: 10.1038/s41591-024-02973-0. Epub 2024 May 14.

Reference Type DERIVED
PMID: 38745011 (View on PubMed)

De BP, Rosenberg JB, Selvan N, Wilson I, Yusufzai N, Greco A, Kaminsky SM, Heier LA, Ricart Arbona RJ, Miranda IC, Monette S, Nair A, Khanna R, Crystal RG, Sondhi D. Assessment of Safety and Biodistribution of AAVrh.10hCLN2 Following Intracisternal Administration in Nonhuman Primates for the Treatment of CLN2 Batten Disease. Hum Gene Ther. 2023 Sep;34(17-18):905-916. doi: 10.1089/hum.2023.067.

Reference Type DERIVED
PMID: 37624739 (View on PubMed)

Related Links

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https://www.nature.com/articles/s41591-024-02973-0

Progranulin AAV gene therapy for frontotemporal dementia

Other Identifiers

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J4B-MC-OKAA (PRV-FTD101)

Identifier Type: -

Identifier Source: org_study_id

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