| Literature DB >> 29563764 |
Hanneke E Borst1,2, Gillian S Townend1, Mirjam van Eck3,4, Eric Smeets1, Mariëlle van den Berg5,6, Aleid Laan7,8, Leopold M G Curfs1.
Abstract
This study aimed to determine whether there is a relationship between abnormal foot position and standing and walking ability in individuals with Rett syndrome (RTT), a rare neurological condition primarily affecting females, often accompanied by impaired gross motor function and musculoskeletal deformities. Through means of an online survey, physiotherapists were asked to share information about their work and experience with individuals with RTT. They were asked about their clients' scores on the Rett Syndrome Gross Motor Scale and measures of their foot deformity, passive range of motion of dorsiflexion of the foot, use of supportive footwear, pressure load on the foot, and symmetry in weight bearing. 45 physiotherapists gave answers relating to 67 individuals with RTT who ranged in age from 2 to over 50 years. Almost 80% had an abnormal foot position which required support of special shoes or orthoses. Approximately 55% experienced abnormal pressure load on the foot and 65% demonstrated asymmetrical weight-bearing; 22% could sit independently and 17% were able to stand and walk independently. Of all the variables investigated, only abnormal distribution of pressure on the foot and asymmetry in weight bearing through the legs were found to be (negatively) correlated with standing and walking ability. Physiotherapists can use this information to give advice on othopedic support for the feet of individuals with RTT.Entities:
Keywords: Foot deformities; Orthosis; Rett Syndrome Gross Motor Scale; Rett syndrome; Walking; Weight bearing
Year: 2018 PMID: 29563764 PMCID: PMC5842496 DOI: 10.1007/s10882-017-9585-6
Source DB: PubMed Journal: J Dev Phys Disabil ISSN: 1056-263X
Description of study cohort (N = 67)
| Categories | Number (%) | |
|---|---|---|
| Age range (years) | 2–4 | 2 (3%) |
| 4–12 | 19 (28%) | |
| 12–19 | 22 (33%) | |
| 19–40 | 18 (27%) | |
| 40–50 | 3 (4.5%) | |
| >50 | 3 (4.5%) | |
| Type of abnormal foot position | Equinus | 18 (27%) |
| Flatfoot (flexible) | 15 (22%) | |
| Flatfoot (rigid) | 12 (18%) | |
| Other | 7 (10.5%) | |
| No problems | 10 (15%) | |
| Nil response | 5 (7.5%) | |
| Type of foot support | Semi-orthopedic footwear | 14 (21%) |
| Orthopedic footwear | 17 (25.5%) | |
| Ankle-foot orthosis (AFO) | 15 (22%) | |
| Other | 4 (6%) | |
| Not required | 10 (15%) | |
| Nil response | 7 (10.5%) | |
| Full foot on the floor (when standing with bare feet) | Yes | 24 (36%) |
| Partial | 4 (6%) | |
| No | 30 (45%) | |
| Nil response | 9 (13%) | |
| Symmetrical weight bearing through both legs (when standing) | Yes | 12 (18%) |
| No | 46 (69%) | |
| Nil response | 9 (13%) |
Levels of support for motor skills
| Maximum support | Medium support | Minimal support | Independent | Total | |
|---|---|---|---|---|---|
| Sitting on the floor |
| 9 (14%) | 4 (6%) | 21 (32%) | 65 |
| Sitting on a chair | 18 (27%) | 20 (30%) | 6 (9%) |
| 67 |
| Sitting on a stool |
| 11 (17%) | 9 (14%) | 14 (22%) | 64 |
| Transfer from sitting to standing |
| 19 (31%) | 2 (3%) | 9 (15%) | 61 |
| Standing for 3 s |
| 14 (25%) | 7 (12%) | 10 (18%) | 56 |
| Standing for 10 s |
| 13 (23%) | 4 (7%) | 10 (18%) | 56 |
| Standing for 20 s |
| 10 (18%) | 4 (7%) | 10 (18%) | 55 |
| Walking 10 steps |
| 12 (23%) | 1 (2%) | 9 (17%) | 53 |
| Transfer from floor to standing |
| 6 (11%) | 1 (2%) | 3 (5%) | 56 |
| Sidestepping |
| 10 (18%) | 3 (5%) | 2 (4%) | 55 |
| Turning 180˚ degrees |
| 9 (17%) | 4 (7%) | 4 (7%) | 54 |
| Bending to pick up an object from the floor and return to standing |
| 6 (12%) | 2 (4%) | 1 (2%) | 51 |
| Stepping over an obstacle |
| 6 (11%) | 5 (10%) | 2 (4%) | 52 |
| Walk up or down a slope |
| 8 (15%) | 6 (11%) | 2 (4%) | 53 |
| Running |
| 1 (2%) | 0 (0%) | 1 (2%) | 52 |
For each item, the level of support with the highest score is shown in bold
Motor skills scores
| Average score | SD | Range | N | |
|---|---|---|---|---|
| PROM dorsiflexion (right) | 0,35 | 16,8 |
| 66 |
| PROM dorsiflexion (left) | 1,88 | 14,6 |
| 66 |
| Subscale Sitting | 3,76 | 3,4 | 0–9 | 63 |
| Subscale Standing and Walking | 5,04 | 7,68 | 0–27 | 48 |
| Subscale Challenge | 0,54 | 1,56 | 0–9 | 50 |
| Total RSGMS score | 8,68 | 11,3 | 0–45 | 47 |
Normal PROM dorsiflexion in adults is 20 degrees. Responses ranged from -70 degrees dorsiflexion (indicating a great impairment in ankle motion) to 30 degrees dorsiflexion (normal range of motion)
Spearman’s rho for independent and dependent variables
| Item walking | Subscale Standing and Walking | Total score | |
|---|---|---|---|
| Abnormal foot position | −0.078 (p 0.576) | −0.031 (p 0.833) | −0.024 (p 0.868) |
| Foot support | 0.066 (p 0.646) | 0.065 (p 0.663) | 0.162 (p 0.282) |
| PROM left | −0.159 (p 0.250) | 0.193 (p 0.179) | 0.144 (p 0.4323) |
| PROM right | 0.027 (p 0.846) | 0.187 (p 0.194) | 0.163 (p 0.264) |
| Pressure load on the bare foot when standing | −0.422 (p 0.002)* | −0.470 (p 0.001)* | −0.507 (p 0.000)* |
| Symmetrical weight bearing through both legs (when standing) | −0.225 (p 0.117) | −0.198 (p 0.188) | −0.323 (p 0.031)* |
*p-value <0.05
Fig. 1Average scores on the standing and walking subscale according to abnormal foot position
Fig. 2Average scores on the standing and walking subscale according to type of foot support
Fig. 3Frequencies of abnormal pressure load on the foot by age group
Fig. 4Relationship between age and PROM dorsiflexion of right ankle
Fig. 5Relationship between age and PROM dorsiflexion of left ankle