Foreword 2

Oral Presentations 3

Posters 23

Index 53

Children with congenital oro-motor disorders (COMD) are not numerous. However, as a group, they merit special consideration as management of diseases leading to COMD is complex, owing to their protracted and varying course, the diverse areas of health care involved, and the significant amount of resources their treatment requires.

The traditional model of motor weakness derived from the works of Hughlings Jackson is that of a negative phenomenon following a cortical injury such as stroke, in contrast to the positive phenomena of brisk reflexes and the release of primitive, complex reflexes. The physiology of orderly motor neuron recruitment and de-recruitment elaborated by Henneman, in which the smallest, weakest, and slowest motor neurons are recruited first followed by increasingly large, powerful, and fast motor neurons, provided a physiological explanation for weakness following a cortical insult. This was especially so with the later work of Evarts who demonstrated that the frequency of firing of Betz cells (upper motor neurons) in the cerebral cortex specified the strength of isometric contractions. Maintaining a contraction is underpinned by pools of upper and lower motor neurons being recruited in rotation by relay as it were. Reduced populations and sizes of such upper motor neurons would result in poorly sustained firing of neurons and hence weakness. However, in cerebral palsy (CP), the injury is seldom a focal lesion in the motor cortex but more usually bilateral lesions affecting subcortical white matter. Moreover, the brains of children with CP are immature and still developing. The work of Sutherland demonstrating persistence of the wrap-around electromyogram discharges throughout the gait cycle, and subsequently of Leonard, support a model of ‘developmental co-contraction’ as the default muscle activation pattern out of which, in the uninjured state, refined, selective motor strategies emerge to provide fluency and elegance of movement. As Leonard and colleagues demonstrated, children with CP never shake off this co-contracting pattern which results in clumsy, poorly executed, and energy-intensive movements. According to the landmark study by Tedroff et al. (p 789) children who have a hemiplegia or diplegia frequently select the wrong muscles when attempting to perform a maximum voluntary contraction. This miss-selection often involves an antagonist or remote muscle from the intended joint of action. A consequence of these findings necessitates a revision of the essence of the motor disorders traditionally referred to as ‘spastic’.

We examined muscle recruitment patterns in children with cerebral palsy (CP) and comparison children without CP under conditions of maximum voluntary contractions. Three groups of children participated in the study: (1) 12 children with diplegic CP (eight males, four females; age range 4–10y, mean age 7y [SD 2y 4mo]); (2) six children with hemiplegic CP (four males, two females; age range 5–10y, mean age 7y 4mo [SD 2y]); and (3) 13 comparison children with normal motor function (seven males, six females; age range 4–11y, mean age 7y 2mo, [SD 2y]). The children with CP were classified according to the Gross Motor Function Classification System: eight were Level I, five were Level II, four were Level III, and one was Level IV. Surface electromyography was recorded from four proximal and distal lower extremity (LE) muscles. Children with CP more frequently activated a muscle other than the intended prime mover first, compared with the comparison children, especially when the prime mover was a distal muscle. For example, during ankle plantar flexion, when the lateral gastrocnemius muscle was the prime mover, children with hemiplegia showed preactivation of the tibialis anterior muscle and children with diplegia showed medial hamstring coactivation. In conclusion, children with CP showed considerable differences to the comparison children in how LE muscles were voluntarily activated. Greater understanding of muscle recruitment patterns under a variety of tasks may provide new directions for motor control retraining or other forms of intervention.

The goal was to compare children with hemiplegia with those with diplegia within Gross Motor Functional Classification System (GMFCS) levels using multiple validated outcome tools. Specifically, we proposed that children with hemiplegia would have better gait and gross motor function within levels while upper extremity function would be poorer. Data were collected on 422 ambulatory children with cerebral palsy: 261 with diplegia and 161 with hemiplegia, across seven centers. Those with hemiplegia in each level performed significantly and consistently better on gait or lower extremity function and poorer on upper extremity and school function than those with diplegia. In GMFCS Level II, the group with hemiplegia walked faster (p=0.017), scored 6.6 points higher on Dimension E of the Gross Motor Function Measure (p=0.017), 6.7 points lower on Upper Extremity subscale of the Pediatric Outcomes Data Collection Instrument, and 9.1 points lower on WeeFIM self-care (p=0.002). Basing motor prognosis on GMFCS level alone may underestimate lower extremity skills of children with hemiplegia, and overestimate those of children with diplegia.

This paper reports the development and validation of a disease-specific measure of health status and well-being of children with severe cerebral palsy (CP). The Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) was constructed from recommendations from caregivers, healthcare providers, and review of other measures. Items spanning six domains are rated on an ordinal scale. Standardized scores (0–100) are reported for each domain and in total. Primary caregivers (n=77) of 45 males and 32 females between 5 and 18 years of age (mean age 13y 5mo [SD 3y 4mo]) with CP, categorized by the Gross Motor Function Classification System (GMFCS) level, completed the CPCHILD. Caregivers of children with severe CP (GMFCS Levels IV and V) also completed a second administration of the CPCHILD 2 weeks after the first. The mean CPCHILD score for children with severe CP was 56.2 (SD 15.7; range 24–93). The mean CPCHILD scores for children in GMFCS Levels I to V were 22.0, 38.2, 23.0, 44.5, and 59.3 respectively (p<0.001). Reliability was tested in 41/52 caregivers who reported no change in health status between the two administrations of the CPCHILD. The intraclass correlation coefficient was 0.94 (95% confidence interval 0.90–0.97). The CPCHILD seems to be a reliable and valid measure of caregivers' perspectives on the health status, functional limitations, and well-being of these children.

We assessed the influence of equinus gait treatments on the vertical displacement of the body's centre of mass (COM) in 21 patients with cerebral palsy (14 males, 7 females; mean age 8y 9mo [SD 2y]; range 3y 7mo–17y) presenting different topographical types (quadriplegia, n=1; diplegia, n=6; right hemiplegia, n=6; and left hemiplegia, n=8). Vertical COM displacement was computed from ground reaction forces, and lower limb kinematics was recorded simultaneously. Equinus gait was treated with non-operative treatments (i.e. botulinum toxin injections and stretching casts) in 14 patients, and with operative treatments in seven patients. After non-operative treatments, the entire ankle displacement shifted towards dorsiflexion throughout the gait cycle, but the amplitude of the third foot rocker (TR) and vertical COM displacement remained unchanged. However, after operative treatments, the amplitude of TR increased and vertical COM displacement decreased. A negative linear correlation was found between the former variables in all the patients where 53% of the changes in their vertical COM displacement, after equinus gait treatments, were explained by the changes in TR amplitude. In fact, TR remains a main gait determinant, reducing the vertical COM displacement after equinus gait treatment and influencing the general gait pattern.

We investigated gait in newly diagnosed children with autism. From our previous study with 6- to 14-year-olds, we hypothesized that motor symptoms indicative of basal ganglia and cerebellar dysfunction would appear across the developmental trajectory of autism. Two groups were recruited: children with autism (eight males, three females; mean age 5y 10mo [SD 9mo]; range 4y 4mo–6y 9mo) and a comparison group of typically developing children (eight males, three females; mean age 5y 9mo [SD 1y 1mo]; range 4y 3mo–7y 2mo). The GAITRite Walkway was used to gather data from average gait and intra-walk measurements. Experienced physiotherapists analyzed gait qualitatively. Groups were matched according to age, height, weight, and IQ; although not statistically significant, IQ was lower in the group with autism. Spatiotemporal gait data for children with autism were compatible with findings from patients with cerebellar ataxia: specifically, greater difficulty walking along a straight line, and the coexistence of variable stride length and duration. Children with autism were also less coordinated and rated as more variable and inconsistent (i.e. reduced smoothness) relative to the comparison group. Postural abnormalities in the head and trunk suggest additional involvement of the fronto-striatal basal ganglia region. Abnormal gait features are stable across key developmental periods and are, therefore, promising for use in clinical screening for autism.

This paper examines ethnic differences in the attainment of developmental milestones during infancy and possible explanations for observed differences are investigated. Data from the first survey of the Millennium Cohort Study (n=15 994; 8212 males, 7782 females; mean age 9.2mo [SD 0.5]) were examined. We found that Black Caribbean (odds ratio [OR]=0.23, 95% confidence interval [CI]=0.11–0.48), Black African (OR 0.31, 95% CI 0.18–0.55), and Indian (OR 0.55, 95% CI 0.33–0.93) infants were less likely to show delay in the attainment of gross motor milestones compared with White infants after adjustment for a range of explanatory variables. Pakistani and Bangladeshi infants were more likely to have delays in fine motor development (OR 1.69, 95% CI 1.21–2.35 and OR 2.17, 95% CI 1.17–4.02 respectively) and communicative gestures (OR 4.19, 95% CI 1.47–11.94 and OR 7.64, 95% CI 3.96–14.76 respectively), but these differences were explained by socioeconomic factors and markers of cultural tradition. In conclusion, unexplained ethnic differences were seen in the attainment of gross motor milestones, with Indian, Black Caribbean, and Black African children less likely to be delayed (in adjusted models). Increased likelihood of fine motor and communicative gesture delays among Pakistani and Bangladeshi infants, that disappear when socioeconomic factors are taken into account, point to the need to address deprivation among these groups to reduce the likelihood of developmental delay and possible longer term behavioural and cognitive problems and consequent opportunities throughout life.

A neurodevelopmental model for understanding the spectrum of developmental disabilities postulates that developmental delay, dissociation, and deviance reflect underlying central nervous system dysfunction. In this study, we determined the occurrence of the dissociated and developmentally deviant behavioral profile of attention-deficit–hyperactivity disorder (ADHD) among children with and without developmental cognitive delays (borderline-to-mild intellectual disability) in a population-based birth cohort (n=5718). Among the 70 children identified to have borderline-to-mild intellectual disability, 30% (n=21; 7 females, 14 males) met research criteria for ADHD, compared to only 6.4% of children without intellectual disability (n=358; 88 females, 270 males). The odds ratio for comorbid ADHD for children with versus without borderline-to-mild intellectual disability was 6.3 (95% confidence interval: 3.7–10.6; p<0.001). These data illustrate that developmental dissociation and/or deviance, as represented by ADHD, are more likely to occur in the context of developmental delay, represented by borderline-to-mild intellectual disability.

Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7y 4mo–12y 10mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p<0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p<0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p<0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability.

Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) has a cerebral cortical localization and its phenotype was thought to be purely central. This study of seven children with CBPS (five males, two females; mean age 5y [SD 3y 6mo]; range 1mo–11y 10mo) documents electrophysiological evidence of lower motor neuron involvement in association with congenital contractures (limb or jaw) in six of the seven children studied. This is not an expected association and does not conform to the traditional lesional classification system of the cerebral palsies. Possible pathogenic mechanisms are discussed but this association of upper and lower motor neuron involvement is likely to be a previously unsuspected part of a genetic or other pathogenic sequence.

Magnetic resonance spectroscopy (MRS) of a 12-year-old female patient with glutaric aciduria type II was compared with data obtained from four healthy age- and sex-matched volunteers. In the clinically active phase, conventional magnetic resonance imaging showed mild ventricular dilatation. Frontal lobe choline/creatine (Cho/Cr) ratio (1.98) was higher than the ratios reported for the comparison participants (1.64 [SD 0.21]). The N-acetylaspartate/creatine (NAA/Cr) ratio (1.95) was lower than normal limits (2.66 [SD 0.23]). After successful riboflavin treatment and dietary restriction for proteins, the NAA/Cr ratio was within the normal range (2.44) and Cho/Cr ratio was below the normal range (1.15), suggesting riboflavin-responsive multiple acyl-coA dehydrogenase deficiency. An elevated Cho/Cr ratio and decreased NAA/Cr ratio is consistent with a demyelinating process in the active phase of glutaric aciduria type II. MRS helps to monitor the progress of the disease and the efficacy of treatment by revealing changes in NAA/Cr and Cho/Cr ratios.

A female aged 12 months with developmental delay and left-sided reverse ocular bobbing (rapid deviation of the eye upward and a slow return to the horizontal position) was observed. At birth, an upper left gingival mass, pathologically diagnosed as a benign granular cell tumour, was removed. On computed tomography (CT), left middle cerebral pedincular and midpontine lesions were seen. Magnetic resonance imaging revealed additional white matter hamartomas, corticosubcortical tubers, and subependymal nodules. The patient was re-examined at 36 months. Her general developmental quotient was equivalent to 23 months of age. Third cranial nerve functions and auditory brainstem response were normal. Her abnormal eye movements were still present. Reverse bobbing is usually observed in patients who are unconscious and who have significant pontine pathology and disruption of the reticular formation. This case is the first tuberous sclerosis-related ocular bobbing case to our knowledge and is interesting as the causative lesion was relatively mild, and ocular horizontal movements were preserved.

Passive stretching is widely used for individuals with spasticity in a belief that tightness or contracture of soft tissues can be corrected and lengthened. Evidence for the efficacy of passive stretching on individuals with spasticity is limited. The aim of this review was to evaluate the evidence on the effectiveness of passive stretching in children with spastic cerebral palsy. Seven studies were selected according to the selection criteria and scored against the Physiotherapy Evidence Database scale. Effect size and 95% confidence intervals were calculated for comparison. There was limited evidence that manual stretching can increase range of movements, reduce spasticity, or improve walking efficiency in children with spasticity. It appeared that sustained stretching of longer duration was preferable to improve range of movements and to reduce spasticity of muscles around the targeted joints. Methods of passive stretching were varied. Further research is required given the present lack of knowledge about treatment outcomes and the wide use of this treatment modality.

‘Social integration of adults with cerebral palsy’ Michelsen et al. DMCN Vol 48: 643–649

We would like to correct an error that was printed in the above mentioned article:

p 649: In Appendix 1 the values in the n columns and the percentage columns have been transposed: the values on the left are the percentages and those on the right are the n values.

We sincerely apologize for this error.