Changes of gait pattern in children with Charcot-Marie-Tooth disease type 1A: a 18 months follow-up study

Abstract

BackgroundIn a previous study we identified 3 different gait patterns in a group of children with CMT1A disease: Normal-like (NL), Foot-drop (FD), Foot-drop and Push-off Deficit (FD&POD). Goal of the present study was to perform a follow-up evaluation of the same group of patients to analyze possible changes of gait features in relation to disease progression or specific therapy.MethodsNineteen children with CMT1A were evaluated clinically (CMT-Examination Score and Overall Neuropathy Limitation Scale) and through gait analysis 18.2±1.5 months after a baseline evaluation. Meanwhile, 3 of them had foot surgery.ResultsFifteen out of the 16 non-operated patients significantly changed at least one of the two parameters associated to primary signs (FD and/or POD). Eleven participants worsened at least one parameter and 9 improved one parameter. CMTES significantly worsened for the group of non-operated patients. However, there was no change in CMTES score in 4 patients and in ONLS score in 11. At subgroup level, participants originally belonging to NL group showed a trend towards a foot-drop deficit (−15%, ns); FD and FD&POD subgroups did not change their primary signs, although significant changes were identified individually. All 3 patients operated have improved push-off and proximal joint patterns during walking. Clinical scores did not change within any sub-group.ConclusionsSubtle changes occurring in 1.5 year in gait features of CMT1A children can be instrumentally identified. Such changes show a large inter-subject variability, with some patients even improving their walking pattern. There is anecdotal evidence that foot surgery may improve the push-off phase of gait.

Gait in children and adolescents with Charcot‐Marie‐Tooth disease: a systematic review

“…The search yielded an initial 454 articles (Fig. ), with a final yield of 10 eligible articles (Chan et al, ; Newman et al, ; Burns et al, ; ; ; Rose et al, ; Blyton et al, ; Ferrarin et al, ; ; Ounpuu et al, ) .…”

“…Preliminary review of the 10 articles identified studies and samples that were described in more than one article, with data from seven independent studies identified. Notably, two articles appeared to describe gait of one sample (Ferrarin et al, ; ) and another three described a sample from a clinical trial of ascorbic acid (Burns et al, ; ; Blyton et al, ) (Correspondence with the lead author confirmed that two articles, Blyton et al ( ) and Burns et al ( ) , were post hoc analyses of baseline data from the Ascorbic acid study, Burns et al ( ) .). Table lists study characteristics of included articles.…”

“…Both RCTs were intervention studies with CMT placebo groups and recorded gait characteristics as secondary outcome measures (Burns et al, ; Rose et al, ) . Four studies had a primary aim of describing gait characteristics; all compared findings to their respective gait laboratory reference group of typically developing (TD) controls with statistical analysis (Chan et al, ; Newman et al, ; Ferrarin et al, ; ; Ounpuu et al, ) . Two of these studies subgrouped data according to gait patterns (Ferrarin et al, ; ; Ounpuu et al, ) .…”

“…Four studies had a primary aim of describing gait characteristics; all compared findings to their respective gait laboratory reference group of typically developing (TD) controls with statistical analysis (Chan et al, ; Newman et al, ; Ferrarin et al, ; ; Ounpuu et al, ) . Two of these studies subgrouped data according to gait patterns (Ferrarin et al, ; ; Ounpuu et al, ) . One further article compared gait data from the ascorbic acid trial (Burns et al, ) to published normative values and grouped data into age bands across childhood and adolescence but did not provide statistical analysis (Burns et al, ) .…”

Gait analysis of fixed bearing and mobile bearing total knee prostheses during walking: Do mobile bearings offer functional advantages?

“…The majority of studies comparing FB and MB prostheses have used questionnaire based outcome measures that have been shown to be less sensitive than gait analyses when detecting changes in gait [11]. Gait analysis has been previously used to measure functional outcome following TKR surgery [12], with current systems able to calculate the biomechanics about the knee to a high degree of accuracy, establishing gait analysis as an important tool in the clinical management of knee problems [13].…”