Neuroanatomical Models of Muscle Strength and Relationship to Ambulatory Function in Spinal Muscular Atrophy.

ABSTRACT:

Background

Individuals with spinal muscular atrophy (SMA) III walk independently, but experience muscle weakness, gait impairments, and fatigue. Although SMA affects proximal more than distal muscles, the characteristic pattern of selective muscle weakness has not been explained. Two theories have been proposed: 1) location of spinal motor neurons; and 2) differences in segmental innervation.

Objective

To identify neuroanatomical models that explain the selective muscle weakness in individuals with SMA and assess the relationship of these models to ambulatory function.

Methods

Data from 23 ambulatory SMA participants (78.2% male), ages 10-56 years, enrolled in two clinical studies (NCT01166022, NCT02895789) were included. Strength was assessed using the Medical Resear

SUBMITTER: Rodriguez-Torres R 

PROVIDER: S-EPMC9344600 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature