This trial employs a multicenter, randomized, open-label, standard-of-care-controlled design and plans to enroll 50 patients with Type 2 SMA aged 2 to 12 years who have previously received nusinersen. The primary objective of the trial is to evaluate the efficacy of GC101 in treating Type 2 SMA. The secondary objectives are to assess the efficacy, safety, and pharmacokinetic (PK) profile of GC101 in treating Type 2 SMA.
The trial is divided into two groups: one group will receive a single intrathecal injection of GC101 at a dose of 1.2E+14 vg per person and discontinue their previous standard-of-care treatment with nusinersen; the other group will continue their previous standard-of-care treatment with nusinersen. Participants will be randomly assigned to the trial group or the control group in a 1:1 ratio.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
50
Self-complementary recombinant adeno-associated viral vector (scAAV) containing a single-stranded transgene encoding a codon-optimized human SMN1 gene
The Seventh Medical Center of Chinese PLA General Hospital
Beijing, China
RECRUITINGChildren's Medical Center of Peking University First Hospital
Beijing, China
RECRUITINGBeijing Children's Hospital, Capital Medical University
Beijing, China
RECRUITINGNational Children's Medical Center,Shanghai Jiaotong University
Shanghai, China
RECRUITINGShenzhen Children's Hospital
Shenzhen, China
RECRUITINGChildren's Hospital of Soochow University
Suzhou, China
RECRUITINGWuhan Children's Hospital, Tongji Medical College, Huazhong University of Science & Technology
Wuhan, China
RECRUITINGHFMSE score change from baseline
HFMSE Motor Function Assessment (Hammersmith Functional Motor Scale Expanded) is a standardized tool specifically designed to evaluate motor function in patients with spinal muscular atrophy (SMA). It includes 33 test items covering actions such as head control, sitting, standing, walking, stair climbing, jumping, as well as balance and coordination, providing a comprehensive assessment of motor function in the trunk and limbs. The maximum total score is 66 points, with higher scores indicating better motor capabilities.
Time frame: 52 weeks
HFMSE score change from baseline
Time frame: 26 weeks
The proportion of participants with an increase in HFMSE score of ≥3 points from baseline.
Minimal clinically important differences (MCID) were defined as an HFMSE score improvement of ≥3 points.
Time frame: 26 and 52 weeks
Changes in WHO-MGRS motor milestones from baseline.
WHO-MGRS (World Health Organization Multicenter Growth Reference Study) motor development milestones include: Sitting without support Standing with support Crawling on hands and knees Walking with support Standing alone Walking alone
Time frame: 26 and 52 weeks
RULM score change from baseline.
RULM Motor Function Assessment (Revised Upper Limb Module) is a standardized tool designed to evaluate upper limb function in patients with spinal muscular atrophy (SMA). A maximum total score is 37 points. Higher scores indicate better upper limb performance.
Time frame: 26 and 52 weeks
SMAIS score change from baseline.
Spinal Muscular Atrophy Independence Scale (SMAIS)is a patient- and caregiver-reported outcome measure designed to assess the level of assistance required by individuals with Type 2 and non-ambulant Type 3 spinal muscular atrophy (SMA) to perform activities of daily living. Higher scores indicate greater independence, with a range from 0 to 44
Time frame: 26 and 52 weeks
The incidence of adverse events (AEs), serious adverse events (SAEs), and adverse events of special interest (AESIs).
Time frame: 52 weeks
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