Poorer Physical Activity in CMT Children Linked with Higher Disability

Children with Charcot-Marie-Tooth disease have reduced physical activity and ambulation, factors linked with greater disability, a study suggests. 

The study, “Physical activity of children and adolescents with Charcot-Marie-Tooth neuropathies: A cross-sectional case-controlled study” was published in the journal PLOS One.

Charcot-Marie-Tooth (CMT) leads to progressive muscle weakness, which limits patients’ ability to perform physical activities involving walking, running, and jumping. Reduced physical activity is known to have a negative impact on health, and children with limited physical abilities are at risk of poorer health outcomes, including obesity, even without underlying disease.

In this study, the researchers evaluated the impact of CMT-related disability in ambulatory function and physical activity of children and adolescents with CMT compared to age- and sex-matched healthy controls.

The study included 100 children, 50 with CMT and 50 age- and gender-matched controls, with a mean age of 12.5 years. Participants were recruited from The Royal Children’s Hospital (RCH), Melbourne and the Children’s Hospital, Westmead (CHW), Sydney in Australia. Almost 75% of the CMT children had been diagnosed with Charcot-Marie-Tooth disease type 1A (CMT1A) .

The participants answered the physical activity questionnaire — child (PAQ-C) — to assess physical activity, a self-report questionnaire where the children were asked to report the type and intensity of their physical activity using a scale ranging from “none” to “seven or more” in the seven days prior to their study visit.

The children’s mobility was assessed via the 6-minute walk test (6MWT). The children with CMT also answered the Walk-12 questionnaire.

The Walk-12 questionnaire is a self-reported survey that assesses children’s experience and perception of the limitations of their disease as it relates to gait and gait-related activities. The 6MWT measures the maximum distance an individual can walk for six minutes on a hard, flat surface. The person walks at his or her own pace and can stop and rest as many times as needed.

The results showed that children with CMT were less active than the controls: the estimated weekly physical activity in the CMT group was 283.6 minutes compared to 315.8 minutes a week in the control group.

The children with CMT walked significantly shorter distances in 6MWT versus those walked by the control group, a mean of 507.7 versus 643.3 meters, a difference of 135.6 meters.

Moreover, researchers observed a correlation between lower physical activity in CMT children with increased disability and reduced ambulation.

“Physical activity and functional ambulation are adversely and significantly affected, and are associated with greater disability, in children with CMT,” researchers stated.

“Healthcare clinicians, researchers, and funding agencies ought to engage with and promote opportunities for children with CMT to be more physically active, be it through participation in structured, evidence-based exercise and training programs, or community-based recreational sporting programs,” the study said.