Motor Unit Number Estimation (MUNE) in Adults With Spinal Muscular Atrophy (SMA)

Completed

Conditions studied: Spinal Muscular Atrophy

In brief

The primary objective of this research protocol is to study and follow the course of motor neuron loss in individuals with spinal muscular atrophy (SMA) using the electrophysiological technique of motor unit number estimation (MUNE). This study is based on the hypothesis that the electrophysiological technique of motor unit number estimation (MUNE) and compound muscle action potential (CMAP) provide sensitive indicators to assess the severity and progression of disease in adults with SMA.

Key facts

Study ID

NCT04139343

Run by

Ohio State University

People needed

67

Starts

2018-08-10

Expected to finish

2025-12-31

Last updated by the study team

2026-02-23

Who can join

Age: 17 and older, up to 70. Sex: any. Healthy volunteers: accepted.

You may qualify if…

• 1. Adults age 17 to 70 with SMA type 2 or 3. The diagnosis of SMA must be documented by the homozygous deletion of both SMN1 genes on standard genetic tests for the disorder, and the determination of type 2 SMA by history of ability to maintain a sitting position when placed, or type 3 by the ability to stand and walk independently. Wheelchair mobile patients will not be excluded from the study.
• Interest in participating and the ability to travel to the study site on an agreed upon date on four occasions over a total interval of twenty-four months.
• Be in good health with the exception of SMA. Assessments will be rescheduled for a later date in the event of any intercurrent illness that might affect performance on the assessment.
• Inclusion criteria for control subjects:
• Healthy adults age 17 to 70
• Interest in participating and the ability to travel to the study site

You may not qualify if…

• Patients unable to tolerate the assessment by virtue of associated medical conditions, respiratory failure with ventilator dependence, or an obligatory need for orthotics that cannot be removed during the evaluation.
• Patients who are, in the investigator's opinion, mentally or legally incapacitated, preventing informed consent, or is unable to read and understand written material including the consent 3. Patients with a pacemaker or cardiac device
• Exclusion criteria for control subjects:
• Subjects with any neurological disorder
• Subjects with chronic medical illness that will interfere with their ability to tolerate the study
• Subjects who are, in the investigator's opinion, mentally or legally incapacitated, preventing informed consent, or is unable to read and understand written material including the consent.

Where it is running

• The Ohio State University Medical Center — Columbus, Ohio, United States

Full record on ClinicalTrials.gov

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