Paulo Selber

An index for quantifying deviations from normal gait

A method is derived to calculate the amount by which a subjects gait deviates from an average normal profile, and to represent this deviation as a single number. The method uses principal component analysis to derive a set of 16 independent variables from 16 selected gait variables. The sum of the square of these 16 independent variables is interpreted as the deviation of the subjects gait from normal. Statistical tests of the methods validity and an initial demonstration of its clinical utility are included. It is found that using this index, increasing clinical involvement corresponds to increasing index score.

MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY

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

Accuracy of intramuscular injection of botulinum toxin A in juvenile cerebral palsy: a comparison between manual needle placement and placement guided by electrical stimulation.

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

Single-event multilevel surgery in children with spastic diplegia: a pilot randomized controlled trial.

Most clinicians who perform botulinum toxin A injections for children with cerebral palsy do so using the “free-hand” or manual technique without using radiologic or electrophysiologic guidance to aid needle placement. The objective of this study was to investigate the accuracy of manual needle placement compared with needle placement guided by electrical stimulation. A total of 1,372 separate injections for upper and lower limb spasticity were evaluated in 226 children with cerebral palsy. The accuracy of manual needle placement compared with electrical stimulation was acceptable only for gastroc-soleus (>75%); it was unacceptable for the hip adductors (67%), medial hamstrings (46%), tibialis posterior (11%), biceps brachii (62%), and forearm and hand muscles (13% to 35%). The authors recommend using electrical stimulation or other guidance techniques to aid accurate needle placement in all muscles except the gastroc-soleus. Further study is needed to determine whether more accurate injecting will lead to better functional outcomes and more efficient use of botulinum toxin A.

Single Event Multilevel Surgery in children with bilateral spastic cerebral palsy: A 5 year prospective cohort study

BACKGROUND Single-event multilevel surgery is considered the standard of care to improve gait and functioning of children with spastic diplegic cerebral palsy. However, the evidence base is limited. This pilot study is the first randomized controlled trial of single-event multilevel surgery, to our knowledge. METHODS Nineteen children (twelve boys and seven girls with a mean age of nine years and eight months) with spastic diplegia were enrolled. Eleven children were randomized to the surgical group and eight, to the control group. The control group underwent a program of progressive resistance strength training. The randomized phase of the trial concluded at twelve months. The control group then exited the study and progressed to surgery, whereas the surgical group continued to be followed in a prospective cohort study. The primary outcome measures were the Gait Profile Score (GPS) and the Gillette Gait Index (GGI). Secondary outcome measures were gross motor function (Gross Motor Function Measure-66 [GMFM-66]), functional mobility (Functional Mobility Scale [FMS]), time spent in the upright position, and health-related quality of life (Child Health Questionnaire [CHQ]). RESULTS A total of eighty-five surgical procedures were performed, with a mean of eight procedures per child (standard deviation, four). The surgical group had a 34% improvement in the GPS and a 57% improvement in the GGI at twelve months. The control group had a small nonsignificant deterioration in both indices. The between-group differences for the change in the GPS (-5.5; 95% confidence interval, -7.6 to -3.4) and the GGI (-218; 95% confidence interval, -299 to -136) were highly significant. The differences between the groups with regard to the secondary outcome measures were not significant at twelve months. At twenty-four months after surgery, there was a 4.9% increase in the GMFM-66 score and improvements in the FMS score, time spent in the upright position, and the physical functioning domain of the CHQ in the surgical group. CONCLUSIONS This study provides Level-II evidence that single-event multilevel surgery improves the gait of children with spastic diplegic cerebral palsy twelve months after surgery. Improvements in other domains, including gross motor function and quality of life, were not observed until twenty-four months after surgery.

A classification system for hip disease in cerebral palsy

BACKGROUND Single Event Multilevel Surgery (SEMLS) is considered the standard of care to improve gait and function in children with bilateral spastic cerebral palsy (BSCP). We have demonstrated in a randomized controlled trial (RCT) of SEMLS, that gait was improved at 12 months after surgery and gross motor function at 24 months after surgery. The question addressed in this study, was to determine if improvements in gait and function, would be maintained at 5 year follow-up. METHODS Nineteen children with BSCP, GMFCS levels II (14 children) and III (5 children), mean age 9.7 years (range 7.7-12.2 years) participated in a prospective cohort study following participation in a RCT, with follow-up to 5 years. Outcome measures were Gait Profile Score (GPS), Gillette Gait Index (GGI), Gait Deviation Index (GDI), Gross Motor Function Measure (GMFM66) and Functional Mobility Scale (FMS). RESULTS Eighteen children have completed follow-up, with interval analysis at 1, 2 and 5 years post SEMLS. One child was excluded because of neurological deterioration and his diagnosis was revised to Hereditary Spastic Paraparesis (HSP). GPS improved by 5.29° and GMFM66 by 3.3% at 5 years post SEMLS. Differences between outcome measures at 1 versus 5 years and 2 versus 5 years (except GMFM66) were not significant, indicating that improvements in gait and gross motor function were stable over time. CONCLUSIONS SEMLS results in clinically and statistically significant improvements in gait and function, in children with BSCP, which were maintained at 5 years after surgery.

Botulinum neurotoxin A: An unusual systemic effect

In population‐based studies, hip displacement affects approximately one‐third of children with cerebral palsy (CP). Given the extreme range of clinical phenotypes in the CP spectrum, it is unsurprising that hip development varies from normality, to dislocation and degenerative arthritis. Numerous radiological indices are available to measure hip displacement in children with CP; however, there is no grading system for assessing hip status in broad categorical terms. This makes it difficult to audit the incidence of hip displacement, determine the relationship between hip displacement and CP subtypes, assess the outcome of intervention studies, and to communicate hip status between health care professionals. We developed a categorical, radiographic classification of hip morphology based on qualitative indices and measurement of the key continuous variable, the migration percentage of Reimers. One hundred and thirty‐four radiographs were reviewed of 52 female and 82 male adolescents with CP who were at, or close to, skeletal maturity (mean age 16y 1mo [SD 1y 4mo] range 14y to 19y 1mo). Twenty‐nine were classified at Gross Motor Function Classification System level I, 25 at level II, 27 at level III, 24 at level IV, and 29 at level V. A classification system was developed to encapsulate the full spectrum of hip morphology in CP, with and without intervention.

Multilevel Surgery for Equinus Gait in Children with Spastic Diplegic Cerebral Palsy: Medium-term Follow-up with Gait Analysis

Abstract:  Systemic effects from Botulinum neurotoxin A are uncommon but can have serious consequences. We report the case of a boy with severe cerebral palsy who developed deterioration in respiratory and oromotor function following repeated injections of Botulinum neurotoxin A. Caution is needed in using this treatment in children with severe cerebral palsy and pseudobulbar palsy.

Sacral-level myelomeningocele : Long-term outcome in adults

BACKGROUND In children with spastic diplegia, surgery for ankle equinus contracture is associated with a high prevalence of both overcorrection, which may result in a calcaneal deformity and crouch gait, and recurrent equinus contracture, which may require revision surgery. We sought to determine if conservative surgery for equinus gait, in the context of multilevel surgery, could result in the avoidance of overcorrection and crouch gait as well as an acceptable rate of recurrent equinus contracture at the time of medium-term follow-up. METHODS This was a retrospective, consecutive cohort study of children with spastic diplegia who had had surgery for equinus gait between 1996 and 2006. All children had distal gastrocnemius recession or differential gastrocnemius-soleus complex lengthening, on one or both sides, as part of single-event multilevel surgery. The primary outcome measures were the Gait Variable Scores (GVS) and Gait Profile Score (GPS) at two time points after surgery. RESULTS Forty children with spastic diplegia, Gross Motor Function Classification System (GMFCS) level II or III, were included in this study. There were twenty-five boys and fifteen girls. The mean age was ten years at the time of surgery and seventeen years at the time of final follow-up. The mean postoperative follow-up period was 7.5 years. The mean ankle GVS improved from 18.5° before surgery to 8.7° at the time of short-term follow-up (p < 0.005) and 7.8° at the time of medium-term follow-up. The equinus gait was successfully corrected in the majority of children, with a low rate of overcorrection (2.5%) and a high rate of recurrent equinus (35%), as determined by sagittal ankle kinematics. Mild recurrent equinus was usually well tolerated and conferred some advantages, including contributing to strong coupling at the knee and independence from using an ankle-foot orthosis. CONCLUSIONS Surgical treatment for equinus gait in children with spastic diplegia was successful, at a mean of seven years, in the majority of cases when combined with multilevel surgery, orthoses, and rehabilitation. No patient developed crouch gait, and the rate of revision surgery for recurrent equinus was 12.5%.

Supramalleolar derotation osteotomy of the tibia, with T plate fixation. Technique and results in patients with neuromuscular disease.

We reviewed long-term outcome of 46 adult patients with sacral-level myelomeningocele with an average age of 23 years (range, 18-38). Data were collected from the medical records, computerized database, and a questionnaire. Thirty patients had high sacral lesions (weak gluteus medius, gluteus maximums, and gastroc/soleus), and 16 had low sacral lesions (fair to normal-strength gluteus medius, gluteus maximus, and gastroc/soleus). Thirteen patients had signs of tethered-cord syndrome, and 12 underwent neurosurgical release. Thirty-nine patients underwent a total of 217 orthopaedic surgeries. At the final follow-up, 41 (89.13%) patients were community ambulators. Thirty-two required no external support for walking. Twenty-nine patients used some kind of orthosis. Aggressive management of tethered-cord syndrome, surgical correction of musculoskeletal deformities, and avoidance of arthrodesis at the foot level may be the main factors in accounting for these results.