Status:

ACTIVE_NOT_RECRUITING

Quantification of Motor Function in Infants With Spinal Muscular Atrophy Treated With Innovative Therapies

Lead Sponsor:

Assistance Publique - Hôpitaux de Paris

Collaborating Sponsors:

URC-CIC Paris Descartes Necker Cochin

Conditions:

Spinal Muscular Atrophy

Eligibility:

All Genders

Up to 2 years

Phase:

NA

Brief Summary

The aim of the study is to propose a method for quantifying motor function in infants with spinal muscular atrophy treated with innovative therapies using inertial sensors.

Detailed Description

Infantile spinal muscular atrophy is a common disease (the second most common fatal autosomal recessive disease after cystic fibrosis), neurodegenerative disorders of childhood causing severe motor im...

Eligibility Criteria

Inclusion

  • Infants of both sexes
  • Suffering from spinal muscular atrophy (diagnosis by genetic study "homozygous deletion of SMN1")
  • Followed up by the Necker Neuromuscular Reference Center (GNMH)
  • Eligible for innovative therapy (gene therapy or pharmacogenetics)
  • age of onset of the disease \<1 year
  • no severe respiratory impairment (dependence on ventilatory support for more than 16 hours per day) or bulbar involvement
  • decision of treatment by a Multidisciplinary Consultation Meeting national of experts
  • Benefiting from social security scheme
  • Informed consent signed by holders of parental authority and the investigator

Exclusion

  • Non-consent of one of the holders of parental authority
  • Respiratory instability (dependence on ventilatory support for more than 16 hours per day) or hemodynamics
  • Contraindication to innovative therapy
  • History of another disease impacting motor skills (neonatal suffering, etc.)

Key Trial Info

Start Date :

March 20 2021

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

March 1 2027

Estimated Enrollment :

35 Patients enrolled

Trial Details

Trial ID

NCT04833348

Start Date

March 20 2021

End Date

March 1 2027

Last Update

September 15 2025

Active Locations (1)

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1

Hôpital Necker-Enfants Malades

Paris, France, 75015