The good news in the paper from Dolk and colleagues is that the severity of disabilities and overall rates of cerebral palsy (CP) in very low birthweight infants born in Northern Ireland decreased during the 1990s. This followed an increase in the 1980s, observed in many places following the introduction of assisted ventilation to neonatal intensive care. However, according to their paper the CP rates for births <1000g were still higher between 1994 and1997 than those observed in the early 1980s.

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 heterogenity of disorders covered by the term cerebral palsy (CP) may severely hinder communication between families on the one hand and health professionals, such as physiotherapists, paediatricians, and occupational therapists on the other. Common ground for communication about the child with CP is crucial for aspects such as diagnosis, treatment, care, management, and research. In his later work, Ludwig Wittgenstein introduced the concept of ‘language games’ (Sprachspiele) to underline that our use of language is permanently governed by rules, but not always by the same rules. On a daily basis we are involved in a large number of ‘language games’, where confusion and misunderstanding usually arise when a statement in one ‘language game’ is interpreted according to the rules of another. Speaking the same language, i.e. being engaged in the same ‘language game’ was one of the primary goals in the development of the Gross Motor Function Classification System, the recent redefinition of CP, as well as the recent development of the Manual Ability Classification System (MACS; also see Morris et al. p 950). This commentary focusses on the communication aspect of the MACS.

Literacy is a powerful tool for communication, with particular potential for users of augmentative and alternative communication (AAC). This is dramatically illustrated by John Carey's comment in his preface to Christopher Nolan's book Under the Eye of the Clock. He describes the author's experience of release when he surmounted his severe motor and speech impairments to express the written words, ‘He played rapturously with them, making them riot and lark about, echoing, alliterating, and falling over one another.’ However, unfortunately even in the presence of normal intellect, this acquisition of literacy for childhood AAC users proves elusive. The paper by Sandberg in this month's issue is, therefore, a welcome contribution to the small body of literature that addresses this important area.

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’.

Between June 1993 and December 1996, 276 term newborn infants with encephalopathy and 564 randomly selected term controls were enrolled in a population-based study of moderate and severe term newborn encephalopathy (NE) in Western Australia. During comprehensive neurobehavioural and cognitive follow-up of all patients and controls at 3 years and again at 5 years of age we found an unexpected but strong association between NE and autism spectrum disorders (ASDs). A diagnosis of ASD by age 5 years was reached using criteria according of the Diagnostic Statistical Manual, 4th edition. Linking records to the Western Australian Disability Services Commission Register ensured that no child in the study with ASD was missed. By age 5 years, 37 (13.4%) infants with NE and one (0.2%) control had died. Among the 239 survivors of NE, 12 (5%) were diagnosed with an ASD. Of these, 10 (4.2%) met the full criteria for autism, one had pervasive developmental disorder–not otherwise specified, and one had Asperger syndrome. Among the 563 surviving controls, five (0.8%) were diagnosed with an ASD: three with autism, one with autism/possible Asperger syndrome, and one with Asperger syndrome. Compared with the controls, the children who had experienced NE were 5.9 times (95% confidence interval 2.0–16.9) more likely to have been diagnosed with an ASD.

Over recent decades, cardiology has evolved into a highly evidence-based specialty. Guided by the results of large, multicentre, randomized trials of patients with common conditions, cardiologists are usually in the happy position of knowing how best to treat patients, once they have been diagnosed with various conditions or are deemed at risk of developing them. This has led to the implementation of standardized approaches to treatment. The progressive uniformity of care has improved outcomes – preserving quality of life and prolonging survival. Arguably, however, cardiologists are now less comfortable in situations of clinical uncertainty, when evidence is inadequate or does not exist to guide decision making. This is well illustrated by the difficulty experienced by many patients in obtaining appropriate care for the cardiac aspects of a neuromuscular disorder. From a patient's perspective, cardiologists seem unfamiliar with their condition and, usually, to favour therapeutic nihilism.

In recent years, bisphosphonates, primarily intravenous (iv) pamidronate, have become very widely used in children with severe osteogenesis imperfecta (OI). This has occurred without the well-controlled clinical trials usually required before a medication becomes ‘standard care’. Without placebo-controlled trials it is difficult to define precisely the risks and the benefits, but in children with very severe OI and numerous fractures the risk/benefit ratio is so clearly favorable that widespread use of pamidronate in this situation appears appropriate.

Philippi and colleagues' excellent randomized clinical trial (RCT: p 5) led me to reflect on two issues that I believe are worth considering. I hope these remarks may provoke further discussion among both the authors and readers.

The Manual Ability Classification System (MACS) has been developed to classify how children with cerebral palsy (CP) use their hands when handling objects in daily activities. The classification is designed to reflect the child's typical manual performance, not the child's maximal capacity. It classifies the collaborative use of both hands together. Validation was based on the experience within an expert group, a review of the literature, and thorough analysis of children across a spectrum of function. Discussions continued until consensus was reached, first about the constructs, then about the content of the five levels. Parents and therapists were interviewed about the content and the description of levels. Reliability was tested between pairs of therapists for 168 children (70 females, 98 males; with hemiplegia [n=52], diplegia [n=70], tetraplegia [n=19], ataxia [n=6], dyskinesia [n=19], and unspecified CP [n=2]) between 4 and 18 years and between 25 parents and their children's therapists. The results demonstrated that MACS has good validity and reliability. The intraclass correlation coefficient between therapists was 0.97 (95% confidence interval 0.96–0.98), and between parents and therapist was 0.96 (0.89–0.98), indicating excellent agreement.

Following obstetrical brachial plexus injury, infants are unable to learn specific patterns of movement due to the disruption of neural pathways. Even with successful reinnervation (spontaneously or post surgical reconstruction), function can be suboptimal due to overactivity in antagonist muscles preventing movement of reinnervated muscles. Botulinum toxin type A (BTX-A) was used to temporarily weaken antagonistic muscles early in the reinnervation process following brachial plexus injury, with the aim of facilitating functional improvement. A case series of eight children (five females, three males; mean age 12.5mo [SD 6.43]; range 5–22mo) with significant muscle imbalances but evidence of reinnervation were given BTX-A injections into the triceps, pectoralis major, and/or latissimus dorsi muscles. After a single injection, all parents reported improvement in function. Active Movement Scale total score changed significantly between pre BTX-A and 1 month (p=0.014), and 4 months (p=0.022) post BTX-A injection. It is proposed that BTX-A facilitated motor learning through improved voluntary relaxation of antagonist muscles while allowing increased activity in reinnervated muscles.

The ketogenic diet has been in use since the early 1920s as a treatment for epilepsy. It is a high fat, low protein, and low carbohydrate diet which was developed to mimic the effects of fasting, which has been found to decrease seizure activity. The diet produces ketosis, as does fasting, but the role of ketosis in seizure control is not known. These days, the diet is generally used to treat seizures which do not respond to drug therapy.

We describe trends in the prevalence of cerebral palsy (CP) by birthweight group, and in the severity of motor impairments and presence of associated intellectual impairment, in Northern Ireland from 1981 to 1997 (n=909; 510 males, 399 females; total population 415936 live births) using data from a population-based register of CP. Children with suspected CP or who died before 1 year of age and those with CP of postneonatal origin were excluded. Prevalence of CP was 2.2 per 1000 live births without significant change over time. Among very-low-birthweight (<1500g) live births, prevalence was 44.5 per 1000 (95% confidence interval 32.3–59.8) from 1994 to 1997, with evidence of a statistically significant decline in prevalence since the mid- to late 1980s accompanied by a decrease in the severity of motor impairment and likelihood of intellectual impairment. Among moderately-low-birthweight (1500–2499g) children there was weaker evidence of a peak prevalence in the late 1980s. Prevalence among normal-birthweight infants did not change significantly, but outcome in terms of severity of motor impairment and intellectual impairment improved in the 1990s. Occurrence of bilateral spasticity from 1994 to 1997 was associated with greater severity and likelihood of intellectual impairment for normal-birthweight individuals than for low- or very-low-birthweight individuals.

The study by Sullivan et al. (p 877) aimed to measure energy balance and body composition in children with severe cerebral palsy (CP) who were fed either orally or by gastrostomy tube. The authors show that feeding by gastrostomy tube, using a commercial formula, may lead to disproportionate (excess) fat deposition in these children. They note that while caloric supplementation of children with eating impairments is associated with weight gain, the nutritional composition of a diet for this group of children has yet to be determined.

This study compared the efficacy of Adeli suit treatment (AST) with neurodevelopmental treatment (NDT) in children with cerebral palsy (CP). Twenty-four children with CP, Levels II to IV according to the Gross Motor Function Classification System (GMFCS), were matched by age and functional status and randomly assigned to the AST or NDT treatment groups. In the AST group (n=12; eight males, four females; mean age 8.3y [SD 2.0]), six children had spastic/ataxic diplegia, one triplegia and five spastic/mixed quadriplegia. In the NDT group (n=12; nine males, three females; mean age 8.1y [SD 2.2]), five children had spastic diplegia and seven had spastic/mixed quadriplegia. Both groups were treated for 4 weeks (2 hours daily, 5 days per week, 20 sessions). To compare treatments, the Gross Motor Function Measure (GMFM-66) and the mechanical efficiency index (EIHB) during stair-climbing were measured at baseline, immediately after 1 month of treatment, and 10 months after baseline. The small but significant time effects for GMFM-66 and EIHB that were noted after 1 month of both intensive physiotherapy courses were greater than expected from natural maturation of children with CP at this age. Improvements in motor skills and their retention 9 months after treatment were not significantly different between the two treatment modes. Post hoc analysis indicated a greater increase in EIHB after 1 month (p=0.16) and 10 months (p=0.004) in AST than that in NDT, predominantly in the children with higher motor function (GMFCS Levels II and III). The results suggest that AST might improve mechanical efficiency without a corresponding gain in gross motor skills, especially in children with higher levels of motor function.

Development of literacy skills was studied in six children (one male, five females) with severe speech impairments and cerebral palsy (CP). These skills were related to intellectual development, phonological abilities, and short-term memory. Three of the children were diagnosed with dystonia, and three with diplegia. They had no, or severely restricted, independent mobility (Gross Motor Function Classification System Level IV for four children and Level V for two), and severe fine motor problems, including difficulty with pointing. As they had no intelligible speech, the Bliss system was the primary communication mode. Assessments were made at approximately 6, 9, and 12 years of age. The results revealed that the children had difficulties acquiring literacy skills, although intellectual level and phonological ability predicted otherwise. Positive development during the first 3 years was followed by an arrest. A conspicuous decrease in IQ points was also found. Thus, phonological ability does not seem to have the same predictive power for literacy development in children with severe speech impairments and CP as in typically developing children. Further studies are needed to clarify the role of phonological abilities, working memory, and strategies used in literacy acquisition in these children. Such studies might also clarify the importance of articulatory abilities in early literacy acquisition.

A randomized placebo-controlled trial was carried out to investigate the efficacy of neuromuscular electrical stimulation (NMES) and threshold electrical stimulation (TES) in strengthening the quadriceps muscles of both legs in children with cerebral palsy (CP). Sixty children (38 males, 22 females; mean age 11y [SD 3y 6mo]; age range 5–16y) were randomized to one of the following groups: NMES (n=18), TES (n=20), or placebo (n=22). Clinical presentations were diplegia (n=55), quadriplegia (n=1), dystonia (n=1), ataxia (n=1), and non-classifiable CP (n=2). Thirty-four children walked unaided, 17 used posterior walkers, six used crutches, and the remaining three used sticks for mobility. Peak torque of the left and right quadriceps muscles, gross motor function, and impact of disability were assessed at baseline and end of treatment (16wks), and at a 6-week follow-up visit. No statistically significant difference was demonstrated between NMES or TES versus placebo for strength or function. Statistically significant differences were observed between NMES and TES versus placebo for impact of disability at the end of treatment, but only between TES and placebo at the 6-week follow-up. In conclusion, further evidence is required to show whether NMES and/or TES may be useful as an adjunct to therapy in ambulatory children with diplegia who find resistive strengthening programmes difficult.

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 aim of this study was to test the efficacy of low doses of pamidronate in increasing bone mineral density (BMD) in non-ambulatory children and adolescents with cerebral palsy (CP). Twenty-three non-ambulatory children and adolescents (12 females, 11 males; mean age 10y [SD 5y], range 4y 1mo–17y 11mo) with severe spastic quadriplegic CP and low BMD were recruited from a multidisciplinary clinic. Severity of CP was graded at Level IV (n=10) and Level V (n=13) using the Gross Motor Function Classification System. Patients received intravenous pamidronate (4.12mg/kg/y, maximum 45mg/d) every 4 months. Lumbar spine and femoral neck BMD were measured at baseline and after 4 and 12 months. Twelve months after the first dose of pamidronate there was a significant increase in lumbar spine and femoral neck BMD (p<0.01 for both sites) and z scores compared with baseline values (p<0.01 for both sites). Mean BMD z scores increased 1.6 points for femoral neck and 1.9 points for lumbar spine after 12 months of pamidronate treatment. Serum intact parathyroid hormone increased significantly and cross-linked N-teleopeptide of type I collagen decreased significantly at 12 months. No significant side effect was noted. Low doses of pamidronate are well tolerated and significantly increase BMD in non-ambulatory children and adolescents with CP.

The aim of this study was to assess the rate of hip dislocation at different ages in children with bilateral spastic cerebral palsy attending special schools in southern Derbyshire, UK, between 1985 and 2000. The medical notes of 110 individuals (68 males, 42 females) were obtained. They were divided into four groups according to the Gross Motor Function Classification System (GMFCS). We determined whether or not their hips were dislocated at the ages of 5, 10, and 15 years, and the kind of surgery performed in each case. The percentage of individuals with one or both hips dislocated increased with age and with severity of disease. Of those in GMFCS Level II (n=18), none had dislocations; Level III (n=16), none had dislocations at ages 5 and 10, but 11% had by the age of 15; Level IV (n=35), 8% had dislocations by age 5, 19% by age 10, and 30% by age 15; Level V (n=41), 22% had dislocations by age 5, 48% by age 10, and 50% by age 15. Forty-two per cent of individuals with hip dislocation had not had previous preventive surgery. Twenty-one per cent of hips operated on still proceeded to dislocation. We conclude that there was a high rate of hip dislocation, especially in GMFCS groups Levels IV and V, and that this often occurred very early. Preventive surgery avoided dislocation in many children. However, orthopaedic referral was often not made before dislocation was discovered, or the referral was made too late for surgery on soft tissue to be successful. These results may be compared with those from current programmes of hip management, involving radiological surveillance and early use of conservative and surgical interventions.