Immediate Postural Change in a Child with Choreic Movement Disorder Using Elasto-Compressive Bodysuit

1. Introduction

Cerebral palsy (CP) is one of the most common physical disorders of childhood, defined as a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or child brain. [1].

The scientific literature of the last ten years has increasingly focused on the importance of early diagnosis of children with cerebral palsy to provide early rehabilitation care that can promote and optimize child neuroplasticity, prevent complications, and enhance parent and caregiver well-being. [2]

Depending the type of movement disorder, cerebral palsy is classified in spastic, dyskinetic including dystonia and athetosis, ataxic and hypotonic. About 10% of cerebral palsy is represented by the dyskinetic forms, characterized by abnormal movements, abrupt changes in tone and the persistence of primitive reflexes that limit the ability to organize and execute voluntary movements and the ability to maintain posture. The dyskinetic form of CP represents one of the most frequent forms of movement disorder in childhood [3] and are frequently associated with disorder of cerebral cortex, basal ganglia, cerebellum and other motor pathways which are the cause of a brain injury.[4]

According to the recent literature, the most evident therapeutic approaches for the rehabilitation of dyskinetic forms of child cerebral palsy are taping, mobility, strength trainings and environmental enrichment to promote task performance. [5,6,7]

A pharmacological approach using baclofen, trihexyphenidyl, and benzodiazepines is often necessary to accompany rehabilitation treatment. Tetrabenazine also acts as a weak postsynaptic dopamine-2 receptor antagonist. The efficacy of this treatment is limited by the lack of clinical trials on long-term effects in this patient population and potential dose-related side-effects including drowsiness, nausea, parkinsonism, depression, and insomnia. [6]

Dynamic movement orthoses (DMO) are elasto-compressive tools used in the rehabilitation of children with motor disabilities and in children with cerebral palsy. [7] The literature review on preventive and rehabilitative interventions in child with cerebral palsy identifies the use of DMO as an intervention that is likely to benefit these children. [5] The use of bodysuit is part of the interventions that can benefit the motor function [5]. The aim of these orthoses is to improve postural stability, posture symmetry, and movement, without restricting functional movement. The elastic fabric consists of individual panels that are sewn together so that pressure is applied to the trunk and limbs, combined with zips to allow for ease of application. The use of DMOs, to support the rehabilitation of children with motor disabilities, has been extensively used in clinical practice and in various scientific works to promote normalization of muscle tone, increased perception of body position in space, symmetry, posture and movement, stability and proximal control, reduction of pain [8] [9].

Many types of DMO are used in clinical practice associated with standard therapy or specific treatment programs. These include Theratogs suit, Adeli suit treatment, Lycra, FLEXA®, SPIO, Spinecor, SelLi suit. [7] These suits are dynamic orthoses available in different designs from full body suits to smaller garments such as sleeves/gloves and leggings. Although there is no consensus regarding the advantages and disadvantages of wearing these suits, they are used in clinical practice with specific rehabilitation programme and monitoring by therapists. [7]

FLEXA®

Among the DMO, the FLEXA® represents one of the most frequently used in our clinical practice due to its adaptability and flexibility; it can be applied as a bodysuit, glove, or tight-fitting legging that completely covers the trunk, arms, and legs. FLEXA® is less invasive than traditional orthoses, is breathable and has a wide weave. It has seven lines of force, and it can be reinforced to exert specific directional forces without restricting functional movement. The pressure applied by body improved stability and a decreasing effect of external force vectors. It can contain and reduce involuntary movements, favouring realignment and giving at the muscles a biomechanical advantage. The FLEXA® is a specific DMO that has been widely used in clinical practice in different type of children with motor and orthopaedic disorders. This type of DMO is distinguished from others using innovative materials and attention to packaging, elements that contribute to comfort and resistance. The base of the bodysuit is made of a natural-synthetic fibre. Cotton is the component in direct contact with the skin. A significant aspect of the FLEXA® is the use of elasticised velcro, which allows prolonged use of the brace, adapting to the patient's growing and changing needs. The materials used for the FLEXA® are supplied by European manufacturers (Germany and Italy).

A further advantage offered by the FLEXA® is the possibility of customization, responding to the requests of physician and therapists and providing a personalised solution for each patient.

Although its large use in clinical practice, no specific study has been published in literature about the use in both adult and paediatric patients. The specific use of FLEXA is reported exclusively in a case report that explains the use of a FLEXA glove in a boy with cerebral palsy as an alternative to the use of neuromuscular tape to increase autonomy and independence. [10].

The purpose of the present case study is to describe the application of FLEXA® in a female child of 18 months of correct age with a choreic form of cerebral palsy.

2. Materials and Methods

This case report details about the application of FLEXA® in a female child, corrected age (CA) 18 months, with cerebral palsy, specifically with a choreic movement disorder. The child was born prematurely at 32 weeks and 6 days due to premature rupture of membranes and was urgently delivered via caesarean section. At term age, a MRI indicated extensive gliotic-malacic changes, cortical necrosis, and hemosiderin deposits primarily in the posterior periventricular white matter and basal nuclei. By 4 months CA, a neurological structured evaluation [2] identifying motor delays with a predominant extensor tone at the axial level and a significant visual deficit. The child used Valproic acid from the 9 months CA due to the presence of electroclinical episodes characterized by focal onset epileptic spasms. During follow-up, a psychomotor development using the Bayley-III scale at 12 months CA revealed delays in cognitive and language development, as well as in socio-emotional behaviour and adaptive functioning. Genetic analyses for isolated congenital heart defects and through array- Comparative Genomic Hybridization (array-CGH) were conducted, with normal results. At 12 months CA, significant axial hypotonia, lack of trunk control, and poor, chaotic motility were observed. At the 15-month CA, the child shows a level V at the Gross Motor function classification system E & R (12% at Gross Motor Function system-66) [1,11,12] and the motility evolved into a choreiform movement disorder affecting the trunk, limbs, and peribuccal area. Due to the presence of movement disorders that impacted the motor performance, the family was introduced to the use of a DMO (FLEXA®) from 18 months CA, to improve axial control, alignment, and stabilization. An occupational therapist performed the measures and fitted the FLEXA® before ordering it and educated the family for use. At the time of introduction of the DMO the child was not taking medication for chorea.

Outcomes

The subsequent follow-up noted good compliance with the bodysuit and improved postural stability and reduced abrupt limb movements. The (MD-CRS) 0-3 was used to evaluate the effect of the DMO (FLEXA®). This scale is age-specific and identify the presence of the movement disorder by pointing out its prevalent pattern; it also evaluates how much the movement disorder affects motor functions and activities of daily living, and rate the intensity of the disorder in different body regions. The MD-CRS allow monitoring of the disorder over time and the effectiveness of pharmacological, surgical or rehabilitative treatment. [13] The scale is composed of two sections: General assessment (part I) and Movement Disorder (MD) assessment (part II). Part I is to allow a global assessment of the child from observing how much the movement disorder affects motor function, oral/verbal function, level of responsiveness to the environment, and in older children, personal autonomies. Part II assesses the severity of movement disorder in different parts of the body. Scoring the scale to generate an Index I related to Part I (General assessment), an Index II related to Part II (Movement-disorder severity), and a Global Index. Each Index can range from 0 to 1 and it is divided into five classes, according to the severity of movement disorder. Class 1 included a 0–0.2 Index (healthy), class 2 a 0.2–0.4 Index (mildly affected), class 3 a 0.4–0.6 Index (moderately affected), class 4 a 0.6–0.8 Index (severely affected), and class 5 a 0.8–1 Index (profoundly affected). The MD-CRS scale is validated and extensively used in clinical practice for the evaluation of rehabilitative and pharmacological treatments due to its easy reproducibility and it’s inter and intra-rater reliability. [13,14,15,16]