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BIIB115 in Healthy Volunteers and Pediatric SMA Patients Previously Treated with Zolgensma

Phase 1
Conditions
Therapeutic area: Diseases [C] - Nervous System Diseases [C10]
Spinal muscular atrophy
MedDRA version: 20.1Level: PTClassification code 10041582Term: Spinal muscular atrophySystem Organ Class: 10010331 - Congenital, familial and genetic disorders
Registration Number
EUCTR2022-000956-12-PL
Lead Sponsor
Biogen Idec Research Limited
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
ot Recruiting
Sex
All
Target Recruitment
62
Inclusion Criteria

Part A:
1.Ability to understand the purpose and risks of the study and provide signed and dated informed consent and authorization to use confidential health information in accordance with applicable privacy regulations.
2.Males aged 18 to 55 years inclusive, at the time of informed consent.
3.Have a body mass index between 18 and 30 kg/m2, inclusive.
4.All participants must practice highly effective contraception as described in Section 12.5 of the protocol.
5.Must be in good health as determined by the Investigator, based on medical history and Screening evaluations.
Part B:
- Age 0.5 to 12 years old, inclusive, at the time of informed consent
- Weight =7 kg at the time of informed consent
- Genetic diagnosis of SMA (5q SMA homozygous survival motor neuron 1 (SMN1)gene deletion or mutation or compound heterozygous mutation)- Survival motor neuron 2 (SMN2) copy number =1
- Must have received IV onasemnogene abeparvovec per the approved label or per guidelines including the steroid regimen and monitoring specified therein
- Treatment with onasemnogene abeparvovec =180 days prior to first BIIB115 dose
- Potential for improvement due to suboptimal clinical status secondary to SMA, as determined by the Investigator

Are the trial subjects under 18? yes
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 38
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

Part A:
1.Any reason, anatomical or otherwise (including abnormal hematology/coagulation) that presents increase of risk of complication from multiple LP procedures required for dosing and CSF collection, per the investigator discretion.
2.History of any clinically significant cardiac, endocrine, gastrointestinal, hematologic, hepatic, immunologic, metabolic, urologic, pulmonary, neurologic, dermatologic, psychiatric, or renal disease, or other major disease, as determined by the Investigator.
3.History of severe allergic or anaphylactic reactions, or of any allergic reactions that in the opinion of the Investigator are likely to be exacerbated by any component of the study treatment including LP procedures.
4.History of, or ongoing malignancy, carcinoma in situ, or high grade dysplasia (with the exception of no more than 1 basal cell carcinoma or squamous cell carcinoma that was completely excised and cured at least 12 months prior to randomization).
5.Systolic blood pressure > 150 mmHg or < 90 mmHg after resting in a supine or semi recumbent position for at least 5 minutes at screening or prior to dosing.
6.Clinically significant 12-lead electrocardiogram abnormalities.
7.Confirmed demonstration of corrected QT interval, using Fridericia’s correction method, of > 450 ms.
8.Plans to undergo elective procedures or surgeries at any time after signing the ICF through the follow-up visit.
9.History of a suicide attempt within 5 years prior to screening or suicidal ideation in the past 6 months
10.History of, or positive test result at Screening for, human immunodeficiency virus.
11.History of hepatitis C infection or positive test result at Screening for hepatitis C virus antibody.
12.Current hepatitis B infection (defined as positive for hepatitis B surface antigen [HBsAg] and/or total hepatitis B core antibody [anti-HBc]). Participants with immunity to hepatitis B from previous natural infection (defined as negative HBsAg, positive anti-HBc, and positive hepatitis B surface antibody [anti-HBs]) or vaccination (defined as negative HBsAg, negative anti-HBc, and positive anti-HBs) are eligible to participate in the study.
13.Chronic, recurrent, or serious infection (e.g., pneumonia, septicemia), as determined by the Investigator, within 90 days prior to Screening or between Screening and Day -1.
14.
•Any value for ALT, AST, bilirubin, or serum creatinine that is above the ULN at Screening or Baseline.
•Any value of hemoglobin that is < 7.45 mmol/L (approx. < 12 g/dL) at Screening or Baseline.
•Any value for platelets that is below the lower limit of normal at Screening or Baseline.
•Any value out of normal range for absolute or differential WBC counts at screening or Baseline.
15.Current enrollment or a plan to enroll in any interventional clinical study of a drug, biologic, or device, in which an investigational treatment or approved therapy for investigational use is administered within 3 months (or 5 half lives of the agent, whichever is longer) prior to randomization.
16.Use of any prescription medication, over-the-counter oral medication that is known to alter hepatic or renal clearance (excluding acetaminophen/paracetamol), or nutraceutical (e.g., St. John’s wort, ginseng, ginkgo biloba) within 28 days prior to Day -1; use of other over-the-counter oral medication, vitamins, dietary supplements, or antacids within 14 days prior to Day -1; and an unwillingness or inability to refrain from this use during study pa

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: Part A: To evaluate the safety and tolerability of single ascending doses of BIIB115 administered via IT bolus injection to HVs<br>Part B: To evaluate the safety and tolerability of multiple ascending doses of BIIB115 administered via IT bolus injection participants with SMA who previously received onasemnogene abeparvovec;Secondary Objective: Part A: To evaluate the single dose PK of BIIB115 administered via IT bolus injection to HVs<br>Part B: To evaluate the PK of multiple ascending doses of BIIB115 administered via IT bolus injection to pediatric SMA participants who previously received onasemnogene abeparvovec;Primary end point(s): Part A: Incidence of AEs/ SAEs<br>Part B: Number of Participants with Adverse Events (AEs) and Serious Adverse Events (SAEs);Timepoint(s) of evaluation of this end point: Part A: Day -1 - EOT<br>Part B: Up to Day 720
Secondary Outcome Measures
NameTimeMethod
Secondary end point(s): Part A:<br>•CSF BIIB115 Concentration<br>•CSF PK Parameters:<br>-t½ <br>•Serum BIIB115 Concentration<br>•Serum PK Parameters:<br>-t½ <br>-AUC0-last<br>-AUC8 <br>-Cmax<br>- Tmax<br>Part B:<br>1. Concentration of BIIB115 in Cerebral Spinal Fluid (CSF)<br>2. Concentration of BIIB115 in Serum<br>3. Terminal Elimination Half-Life (t½) of BIIB115 in Serum<br>4. Area Under the Concentration-Time Curve from Time 0 to Last<br>Measurable Concentration (AUC0-last) of BIIB115 in Serum<br>5. Area Under the Concentration-Time Curve from Time 0 to Infinity<br>(AUCinf) of BIIB115 in Serum<br>6. Maximum Observed Concentration (Cmax) of BIIB115 in Serum<br>7. Time to Reach Maximum Observed Concentration (Tmax) of BIIB115 in Serum;Timepoint(s) of evaluation of this end point: Part A: Day -1 - EOT<br>Part B:<br>1. Days 1 and 360<br>2. Day 1 to Day 720<br>3. Day 1 to Day 720<br>4. Day 1 to Day 720<br>5. Day 1 to Day 720<br>6. Day 1 to Day 720<br>7. Day 1 to Day 720
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