Walking and weakness in children: a narrative review of gait and functional ambulation in paediatric neuromuscular disease

Abstract

Background: Weakness is the primary impairment in paediatric neuromuscular diseases, impacting gait and gaitrelated functional activities in ambulant children affected by these rare and often degenerative diseases. Gait speed is an indicator of health and disability, yet gait is a complex, multi-faceted activity. Using the International Classification of Function, Health and Disability (ICF) model, assessment of gait and functional ambulation should consider the impairments, activity limitations and participation restrictions due to disease, and factors related to the environment and the individual person. Methods: This narrative review involved a literature search of databases including Medline, Embase and Pubmed from 1946 to October 2019. Inclusion criteria included assessments of gait, endurance and ambulatory function in paediatric (0-18 years) neuromuscular diseases. Results: Fifty-two papers were identified reporting assessments of gait speed, timed function, endurance and ambulatory capacity, gait-related balance and qualitative descriptive assessments of gait function and effect of disease on gait and gait-related activities. Gait speed is an indicator of disability and children with neuromuscular disease walk slower than typically developing peers. Increasing disease severity and age were associated with slower walking in children with Duchenne muscular dystrophy and Charcot-Marie-Tooth disease. The six-minute walk test is used widely as a test of endurance and ambulatory capacity; six-minute walk distance was substantially reduced across all paediatric neuromuscular diseases. Endurance and ambulatory capacity was more limited in children with spinal muscular atrophy type 3, congenital muscular dystrophy and older boys with Duchenne muscular dystrophy. Only a few papers considered normalisation of gait parameters accounting for the effect on gait of height in heterogeneous groups of children and linear growth in longitudinal studies. Balance related to gait was considered in five papers, mainly in children with Charcot-Marie-Tooth disease. There was limited investigation of factors including distance requirements and terrain in children's typical environments and personal factors related to self-perception of disease effect on gait and gait-related function.

In vivo operating lengths of the gastrocnemius muscle during gait in children who idiopathically toe‐walk

“…During gait, children who ITW operated at more plantarflexed angles, but at longer fascicle lengths than TD children. This coincided with observed differences in the peak of the moment-angle and moment-length relationships, measured from MVCs, which showed that children who ITW had the greatest strength at more plantarflexed angles and an Equinus gait has previously been linked with ankle, knee and hip extensor weakness in other clinical populations (Hampton et al, 2003;Kennedy et al, 2020;Morozova et al, 2017;Wiley & Damiano, 1998).…”

“…to contribute to equinus gait (Hampton et al, 2003;Kennedy et al, 2020;Morozova et al, 2017;Wiley & Damiano, 1998). For children who ITW, there has been an indication that there may be some small effects of ankle joint weakness (De Oliveira et al, 2021); however, to our knowledge, muscle strength around the hip or knee joint has never been objectively studied in children who ITW.…”

Gait as a Quantitative Translational Outcome Measure in Angelman Syndrome

“…Our study showcases translational and longitudinal use of quantitative motor metrics for a genetic NDD. Clinically, quantitative gait research has gained traction across many NDDs and the idea to tailor attention on gait in preclinical studies is now also gaining momentum (IDDRC working group, AGENDA working group, personal communications) for Rett syndrome, neurofibromatosis, Down syndrome, and other NDDs (Hampton et al, 2004;Jequier Gygax et al, 2020;Kennedy et al, 2020;Layne et al 2018;Rahn et al, 2020;Summers et al, 2015;Wilson et al, 2020). Data from our work showed analogous measures of gait phenotypes in rodents that are being quantified currently in AS clinics (Jessica Duis MD, personal communication).…”

Gait Parameters in Healthy Preschool and School Children Assessed Using Wireless Inertial Sensor

“…There are many articles describing gait being connected with different disabilities, such as cerebral palsy, amputation, or Charcot–Marie–Tooth disease, as well as a neuromotor delay [ 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 ]. Since typically developed children move in a different way, the normative data for this group of age are also desirable [ 5 , 14 , 15 ].…”