Clinicians use a range of clinical and objective measures to quantify the positive and negative features (impairments) of the upper motor neurone syndrome. These measures play an important role in the assessment and selection of suitable candidates for intervention and monitoring of outcome. Intervention strategies often focus on the positive features; however, outcome may be more contingent upon the severity of the negative features. The clinical protocol for patient selection and treatment used by our multidisciplinary team is presented, together with details of the assessment procedure. Measurement tools in routine use are described, including: the Modified Ashworth Scale, the Modified Tardieu Scale (‘R1’), muscle length by joint range of motion ‘R2’, three-dimensional gait analysis, assessments of strength by the Medical Research Council Scale, Selective Motor Control, the Gross Motor Function Measure and the Observational Gait Scale. Three case studies of children with cerebral palsy who underwent botulinum toxin type A treatment as part of their management of gait disorder are presented, a 2-year-old girl with mild hemiplegia (‘true equinus’), a 3-year-old boy with moderate hemiplegia (‘apparent equinus’) and a 6-year-old girl with diplegia, where a targeted approach was used to treat a distal problem and resulted in correction Of a proximal problem.

Objective measurement of clinical findings in the use of botulinum toxin type A for the management of children with cerebral palsy

In the subacute and chronic stages of spastic paresis, stretch-sensitive (spastic) muscle overactivity emerges as a third fundamental mechanism of motor impairment, along with paresis and soft tissue contracture. Part II of this review primarily addresses the pathophysiology of the various forms of spastic overactivity. It is argued that muscle contracture is one of the factors that cause excessive responsiveness to stretch, which in turn aggravates contracture. Excessive responsiveness to stretch also impedes voluntary motor neuron recruitment, a concept termed stretch-sensitive paresis. None of the three mechanisms of impairment (paresis, contracture, and spastic overactivity) is symmetrically distributed between agonists and antagonists, which generates torque imbalance around joints and limb deformities. Thus, each may be best treated focally on an individual muscle-by-muscle basis. Intensive motor training of the less overactive muscles should disrupt the cycle of paresis-disuse-paresis, and concomitant use of aggressive stretch and focal weakening agents in their more overactive and shortened antagonists should break the cycle of overactivity-contracture-overactivity.

Pathophysiology of spastic paresis. II: Emergence of muscle overactivity.

Recommendations for the use of botulinum toxin type A in the management of cerebral palsy

©2003 British Editorial Society of Bone and Joint Surgery doi101302/0301-620X85B214066 $200 J Bone Joint Surg [Br] 2003;85-B:157-66 Cerebral palsy is the most common cause of physical disability affecting children in developed countries, with an incidence of 20 to 25 per 1000 live births1 It is not a single entity but a heterogeneous collection of clinical syndromes, characterised by abnormal motor patterns and postures Although in most parts of the world the orthopaedic burden secondary to poliomyelitis and myelomeningocele is declining rapidly, the prevalence of cerebral palsy is static or increasing It is the most common diagnosis after trauma in most paediatric orthopaedic units and is therefore of enormous strategic importance in terms of allocation of resources, planning and service delivery The definitions of cerebral palsy have undergone a number of refinements by developmental paediatricians and neurologists They stress two features First, cerebral palsy is the result of a lesion in the immature brain, which is nonprogressive; it is a static encephalopathy2 It is clearly important to differentiate cerebral palsy from progressive neurological conditions from the standpoint of both taxonomy and clinical management Secondly, cerebral palsy results in a disorder of posture and movement which is permanent but not unchanging3 To this we would add a third feature, that it results in progressive musculoskeletal pathology in most affected children4 It is inappropriate to emphasise that the cerebral lesion is static without clearly stating that the musculoskeletal pathology will be progressive In Little’s original description5 of spastic diplegia, prominence was given to the description of the musculoskeletal deformities The newborn child with cerebral palsy usually has no deformities or musculoskeletal abnormalities at birth Scoliosis, dislocation of the hip and fixed contractures develop during the rapid growth of childhood Cerebral palsy is a useful term which describes a large group of children with motor impairment from many causes and expressed as a wide variety of clinical syndromes The preferred term is therefore ‘the cerebral palsies’6 It was formerly considered that most cases of cerebral palsy were the result of obstetric misadventure Careful epidemiological and brain-imaging studies suggest that it frequently has antenatal antecedents and is often multifactorial Recent studies also point to an increasing number of specific aetiological factors including intrauterine infections and inherited malformations1 These investigations will in time lead to both primary prevention and secondary minimisation of cerebral injury The increase in the incidence of cerebral palsy in preterm infants is because of neonatal intensive care and a rise in multiple births The rates of cerebral palsy in babies born at term are steady, despite strategies to reduce birth asphyxia The most important issue in cerebral palsy is the elucidation of the causal pathways from population-based epidemiological studies This will lead to primary prevention, which in a chronic, incurable condition is clearly the most humane and cost-effective strategy Cerebral palsy is subdivided according to the movement disorder and its topographical distribution Spastic and mixed motor disorders account for more than 85% of children on current registers; dyskinetic cerebral palsy is much less common1,6 The most common topographical syndromes are spastic hemiplegia, spastic diplegia and spastic quadriplegia which is also known as ‘whole-body involvement’1,3,6

Musculoskeletal aspects of cerebral palsy

Classifications of gait patterns in spastic diplegia have been either qualitative, based on clinical recognition, or quantitative, based on cluster analysis of kinematic data. Qualitative classifications have been much more widely used but concerns have been raised about the validity of classifications, which are not based on quantitative data. We have carried out a cross-sectional study of 187 children with spastic diplegia who attended our gait laboratory and devised a simple classification of sagittal gait patterns based on a combination of pattern recognition and kinematic data. We then studied the evolution of gait patterns in a longitudinal study of 34 children who were followed for more than one year and demonstrated the reliability of our classification. Children with spastic diplegia usually walk independently but most have an easily recognised disorder of gait which may include deviations in the sagittal plane such as toewalking, flexed-stiff knees, flexed hips and an anteriorly tilted pelvis with lumbar lordosis. 1 When compared with their peers many also walk at a reduced speed, with increased energy expenditure and impaired functional capability. 2-4 Instrumented gait analysis gives detailed information and quantitative measurements. By a process of clinical interpretation this may help the clinician to understand the gait pattern and perhaps to plan appropriate intervention. 5,6 However, experienced clinicians often describe gait patterns using a combination of clinical examination and clinical observation. In 1986, Rang, Silver and De La Garza 7 described a number of gait patterns in spastic diplegia and classified them on a purely observational basis, related to spasticity or contracture of muscles which work in the sagittal plane. They observed associations between contractures of the psoas and lumbar lordosis, of the adductors and scissoring, of the hamstrings and knee flexion, of rectus femoris and stiff knee gait and of gastrocsoleus and tip-toe gait. By linking these observed patterns to specific shortening of the muscles, the association with management was implied. 7

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Sagittal gait patterns in spastic diplegia

The role of intramuscular botulinum toxin A in the treatment of 26 children with cerebral palsy was evaluated. The indication for injection was the presence of a dynamic contracture of lower-limb muscles interfering with positioning or walking. Spastic target muscles were identified by clinical examination and, in ambulant children, by gait analysis. Between 50 and 320 units of botulinum toxin were injected into each muscle group to a total dose of 100 to 400 units per child. The effects of injection were monitored by repeated clinical examination and gait analysis. There were no clinically detectable systemic side-effects, and all but one patient had a reduction in tone, which occurred within three days and persisted for two to four months. There were significant improvements in ambulatory status and in sagittal-plane kinematics. In some cases these gains persisted after the tone-reducing effects of the toxin had worn off.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1469-8749.1994.tb11864.x

I. S. Corry

Papers

Botulinum toxin in the management of the lower limb in cerebral palsy