H. Kerr Graham

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

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.

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

Botulinum toxin type A (BTX-A) is increasingly being used for the treatment of childhood spasticity, particularly cerebral palsy. However, until very recently, all such use in this indication has been unapproved with no generally accepted treatment protocols, resulting in considerable uncertainty and variation in its use as a therapeutic agent. In view of the increasing awareness of, and interest in, this approach to the treatment of spasticity, and also the recent licensing in a number of countries of a BTX-A preparation for treating equinus deformity in children, it would seem timely to establish a framework of guidelines for the safe and efficacious use of BTX-A for treating spasticity in children. This paper represents an attempt, by a group of 15 experienced clinicians and scientists from a variety of disciplines, to arrive at a consensus and produce detailed recommendations as to appropriate patient selection and assessment, dosage, injection technique and outcome measurement. The importance of adjunctive physiotherapy, orthoses and casting is also stressed.

The Gait Profile Score and Movement Analysis Profile

The Gait Deviation Index (GDI) has been proposed as an index of overall gait pathology. This study proposes an interpretation of the difference measure upon which the GDI is based, which naturally leads to the definition of a similar index, the Gait Profile Score (GPS). The GPS can be calculated independently of the feature analysis upon which the GDI is based. Understanding what the underlying difference measure represents also suggests that reporting a raw score, as the GPS does, may have advantages over the logarithmic transformation and z-scaling incorporated in the GDI. It also leads to the concept of a Movement Analysis Profile (MAP) to summarise much of the information contained within kinematic data. A validation study on all children attending a paediatric gait analysis service over 3 years (407 children) provides evidence to support the use of the GPS through analysis of its frequency distribution across different Gross Motor Function Classification System (GMFCS) and Gillette Functional Assessment Questionnaire (FAQ) categories, investigation of intra-session variability, and correlation with the square root of GGI. Correlation with GDI confirms the strong relationship between the two measures. The study concludes that GDI and GPS are alternative and closely related measures. The GDI has prior art and is particularly useful in applications arising out of feature analysis such as cluster analysis or subject matching. The GPS will be easier to calculate for new models where a large reference dataset is not available and in association with applications using the MAP.

The Functional Mobility Scale (FMS).

We devised a new Functional Mobility Scale (FMS) to describe functional mobility in children with cerebral palsy, as an aid to communication between orthopaedic surgeons and health professionals. The unique feature of the FMS is the freedom to score functional mobility over three distinct distances, chosen to represent mobility in the home, at school and in the wider community. We examined the construct, content, and concurrent validity of the FMS in a cohort of 310 children with cerebral palsy by comparing the FMS to existing scales and to instrumented measures of physical function. We demonstrated the scale to be both valid and reliable in a consecutive population sample of 310 children with cerebral palsy seen in our tertiary referral center. The FMS was useful for discriminating between large groups of children with varying levels of disabilities and functional mobility and sensitive to detect change after operative intervention.

MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY

©2003 British Editorial Society of Bone and Joint Surgery doi.10.1302/0301-620X.85B2.14066

A randomized clinical trial of strength training in young people with cerebral palsy

2.00 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 2.0 to 2.5 per 1000 live births.1 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 encephalopathy.2 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 unchanging.3 To this we would add a third feature, that it results in progressive musculoskeletal pathology in most affected children.4 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 malformations.1 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 common.1,6 The most common topographical syndromes are spastic hemiplegia, spastic diplegia and spastic quadriplegia which is also known as ‘whole-body involvement’.1,3,6

Hip displacement in cerebral palsy

This randomized clinical trial evaluated the effects of a home-based, six-week strength-training programme on lower limb strength and physical activity of 21 young people (11 females, 10 males; mean age 13 years 1 month, SD 3 years 1 month; range 8 to 18 years) with spastic diplegic cerebral palsy (CP) with independent ambulation, with or without gait aids; (Gross Motor Function Classification System levels I to III). Compared with the 10 controls, the 11 participants in the strength-training programme increased their lower limb strength (combined ankle plantarflexor and knee extensor strength as measured by a hand-held dynamometer) at 6 weeks (F(1,19)=4.58, p=0.046) and at a follow-up 12 weeks later (F(1,18)=6.25, p=0.041). At 6 weeks, trends were also evident for improved scores in Gross Motor Function Measure dimensions D and E for standing, running and jumping, and faster stair climbing. A relatively short clinically feasible home-based training programme can lead to lasting changes in the strength of key lower-limb muscles that may impact on the daily function of young people with CP.

Paediatric quality of life instruments: a review of the impact of the conceptual framework on outcomes.

BACKGROUND Hip displacement is considered to be common in children with cerebral palsy but the reported incidence and the proposed risk factors vary widely. Knowledge regarding its overall incidence and associated risk factors can facilitate treatment of these children. METHODS An inception cohort was generated from the Victorian Cerebral Palsy Register for the birth years 1990 through 1992, inclusive, and multiple data sources pertaining to the cohort were reviewed during 2004. Gross motor function was assessed for each child and was graded according to the Gross Motor Function Classification System (GMFCS), which is a valid, reliable, five-level ordinal grading system. Hip displacement, defined as a migration percentage of >30%, was measured on an anteroposterior radiograph of the pelvis with use of a reliable technique. RESULTS A full data set was obtained for 323 (86%) of 374 children in the Register for the birth years 1990 through 1992. The mean duration of follow-up was eleven years and eight months. The incidence of hip displacement for the entire birth cohort was 35%, and it showed a linear relationship with the level of gross motor function. The incidence of hip displacement was 0% for children with GMFCS level I and 90% for those with GMFCS level V. Compared with children with GMFCS level II, those with levels III, IV, and V had significantly higher relative risks of hip displacement (2.7, 4.6, and 5.9, respectively). CONCLUSIONS Hip displacement is common in children with cerebral palsy, with an overall incidence of 35% found in this study. The risk of hip displacement is directly related to gross motor function as graded with the Gross Motor Function Classification System. This information may be important when assessing the risk of hip displacement for an individual child who has cerebral palsy, for counseling parents, and in the design of screening programs and resource allocation.

Analgesic effects of botulinum toxin A: a randomized, placebo-controlled clinical trial.

With an increasing number of paediatric quality of life (QOL) instruments being developed, it is becoming difficult for researchers and clinicians to select the most appropriate instrument. Reviews of QOL instruments tend to report only basic properties of the instruments such as domains and psychometric properties. This paper seeks to appraise critically the conceptual underpinnings of paediatric QOL instruments. A systematic review was conducted to identify QOL instruments for children aged 0 to 12 years, and to examine and compare their conceptual frameworks, definitions employed, and structure. Both generic and condition‐specific measures were reviewed. Fourteen generic and 25 condition‐specific QOL instruments were identified. Eleven types of definition of QOL and health‐related QOL and three theories of QOL were identified. QOL was measured by a variety of domains including emotional, social and physical health, and well‐being. Items commonly assessed difficulties, or intensity/frequency of feelings/symptoms, in contrast to positive aspects of life and happiness. The findings highlight the diversity that is apparent in the conceptualization of paediatric QOL and draw attention to the lack of empirical evidence for many of the fundamental assumptions. The impact of the conceptual underpinnings of the instruments on the resulting QOL scores is discussed.

Cerebral palsy in Victoria : Motor types, topography and gross motor function

Postoperative pain in children with spastic cerebral palsy (CP) is often attributed to muscle spasm and is difficult to manage using opiates and benzodiazepines. Adductor‐release surgery to treat or prevent hip dislocation in children with spastic CP is frequently performed and is often accompanied by severe postoperative pain and spasm. A double‐blinded, randomized, placebo‐controlled clinical trial of 16 patients (mean age 4.7 years) with a mainly spastic type of CP (either diplegic or quadriplegic in distribution) was used to test the hypothesis that a significant proportion of postoperative pain is secondary to muscle spasm and, therefore, might be reduced by a preoperative chemodenervation of the target surgical muscle by intramuscular injection of botulinum toxin A (BTX/A). Compared with the placebo, BTX/A was found to be associated with a reduction in mean pain scores of 74% (P<0.003), a reduction in mean analgesic requirements of approximately 50% (P<0.005), and a reduction in mean length of hospital admission of 33% (P<0.003). It was concluded that an important component of postoperative pain in the patient population is due to muscle spasm and this can be managed effectively by preoperative injection with BTX/A. These findings may have implications for the management of pain secondary to muscle spasm in other clinical settings.