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Phase 3 N=11 Randomized Triple-blind Treatment

Short and Long Term Treatment With 4-AP in Ambulatory SMA Patients

Spinal Muscular Atrophy

Enrolled (actual)
11
Serious AEs
0.0%
Results posted
Sep 2024
Primary outcome: Primary: Six Minute Walk Test (6MWT) With Kinematic Evaluation of Gait (Short Term) — 293.05; 299.50 meters

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
4-aminopyridine (Drug); Placebo (Drug)
Age
Adult · 18+ yrs
Sex
All
Sponsor
Columbia University
Primary completion
Sep 2015

Outcome Measures

OutcomeResultp-value
PRIMARY
Six Minute Walk Test (6MWT) With Kinematic Evaluation of Gait (Short Term)
293.05; 299.50
PRIMARY
Six Minute Walk Test (6MWT) With Kinematic Evaluation of Gait (Long Term)
299.10; 295.20
SECONDARY
Hammersmith Functional Motor Scale, Expanded (HFMSE) (Short Term)
50.84; 50.43
SECONDARY
Hammersmith Functional Motor Scale, Expanded (HFMSE) (Long Term)
50.90; 50.30
SECONDARY
Manual Muscle Testing (MMT) Total Score (Short Term)
181.86; 182.23
SECONDARY
Manual Muscle Testing (MMT) Total Score (Long Term)
177.80; 181.10
SECONDARY
Motor Unit Number Estimation (MUNE)
190.86; 194.26

Summary

The purpose of this study is to assess whether 4-AP (Dalfampridine-ER, Ampyra) improves walking ability and endurance in adult patients with Spinal muscular atrophy (SMA) Type 3 compared to placebo and whether the duration of treatment affects outcome.

Eligibility Criteria

Inclusion Criteria

  • Aged 18 to 50 years at the time of enrollment
  • Have genetically confirmed SMA 3 (homozygous absence of SMN1 exon 7)
  • Ability to walk at least 25 meters without assistance
  • Be free of major orthopedic deformities (i.e. scoliosis, contractures)
  • Normal Cystatin C clearance (> 80 ml/min)

Exclusion Criteria

  • Patients with a history of seizures
  • Patients with any renal impairment
  • Inability to comply with the study procedures
  • Unstable medical illness
  • Any ventilatory assistance
  • Taking experimental medication for SMA other than under this protocol
  • Pregnancy or lactation
  • Menstruating women, not sterilized or not using effective birth control
  • Planning to undergo scoliosis surgery within the next 10 months
  • Inability to give informed consent
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01645787). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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