Mechanisms and Treatment of Exercise Intolerance and Persistent Fatigue in Spinal Muscular Atrophy

Description

This study will focus on the pathophysiological underpinnings of reduced exercise capacity and fatigue in ambulatory patients with spinal muscular atrophy (SMA). There has been laboratory evidence to suggest that the molecular mechanisms underlying mitochondrial biogenesis may be vulnerable to survival motor neuron (SMN) protein deficiency. This is an observational, single visit study including 34 ambulatory SMA patients treated with SMN repletion therapies (risdiplam or nusinersen) for at least 6 months at enrollment.

Conditions

Spinal Muscular Atrophy

Study Overview

Study Details

Study overview

This study will focus on the pathophysiological underpinnings of reduced exercise capacity and fatigue in ambulatory patients with spinal muscular atrophy (SMA). There has been laboratory evidence to suggest that the molecular mechanisms underlying mitochondrial biogenesis may be vulnerable to survival motor neuron (SMN) protein deficiency. This is an observational, single visit study including 34 ambulatory SMA patients treated with SMN repletion therapies (risdiplam or nusinersen) for at least 6 months at enrollment.

Mechanisms and Treatment of Exercise Intolerance and Persistent Fatigue in Spinal Muscular Atrophy

Mechanisms and Treatment of Exercise Intolerance and Persistent Fatigue in Spinal Muscular Atrophy

Condition
Spinal Muscular Atrophy
Intervention / Treatment

-

Contacts and Locations

New York

Columbia University Irving Medical Center, New York, New York, United States, 10032

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

For general information about clinical research, read Learn About Studies.

Eligibility Criteria

  • * Genetic confirmation of SMA with laboratory documentation of homozygous deletion of survival motor neuron (SMN1) exon 7;
  • * At least 8 years of age at time of signing Informed Consent Form (or assent)
  • * Children or adults currently receiving treatment, for at least 6 months, with SMN repletion therapy, either with
  • * Able to walk independently at least 25 meters
  • * Able to tread a stationary cycle ergometer.
  • * Unable to walk 25 meters independently.
  • * Use of investigational medications intended for the treatment of SMA within 30 days prior to study entry.
  • * The presence of any contraindication to exercise according the American College of Sports Medicine (ACSM) criteria.

Ages Eligible for Study

8 Years to 55 Years

Sexes Eligible for Study

ALL

Accepts Healthy Volunteers

No

Collaborators and Investigators

Columbia University,

Jacqueline Montes, PT, EdD, PRINCIPAL_INVESTIGATOR, Columbia University

Study Record Dates

2025-09-01