Disclosing a neurodevelopmental disorder to parents is always difficult, in spite of current training in this task. Regardless of how well trained we are, we will always find the task difficult and often worry we have got it ‘wrong’. However, I have found myself almost thanked when telling parents that their child still has a problem. Often these parents have sought a second or third opinion after being told their child was healthy.

It is 110 years since Sir William Osler published his little book on the cerebral palsies of childhood. Less than 10 years later Freud published his observations. And if we are looking for landmarks, perhaps the next is Crothers and Paine's book, published 40 years ago. Ingram's ‘Paediatric Aspects of Cerebral Palsy’ follows this, published in 1974.

No area of developmental neurology is more complex than the specific learning disorders (SLD): the terminology is a morass; the epidemiology, in consequence, is extremely difficult; diagnosis is a problem due to changing symptomatology with age (3-year-olds can't be dyslexic or can they?); the extent to which investigations are initiated is financially and biologically controversial; and management may range from pharmacotherapy to educational strategies through to alternative therapies. Because these children very clearly attract the attention of educationalists and health professionals, they often fall into a divide where neither professional is fully involved. Many clinicians report that they frequently see children in late childhood/early adolescence where the biological background to a SLD has not been identified, resulting in behaviour disturbances which may be criminal.

From studies within the USA, Israel, Norway and France, it has been estimated that the incidence of brain injury in children up to 14 years of age is 180 out of 100 000 per annum. Between 40 and 50% of children dying from acute traumatic injuries in the USA during 1991 had brain injury as part of their diagnosis and this proportion has been born out by other reports. However, death rates of children admitted for brain injury from whatever cause, which range from 2–3% to 14%, are not very helpful as the aetiology and admission criteria can vary widely.

Aicardi's presentation on epilepsy at the 1998 International Child Neurology Association conference in Ljubljana, Slovenia, started dramatically by using the iceberg, with larger amounts of ice below the surface, as an analogy for epilepsy. He then talked about the phenomena associated with epilepsy - the learning and behaviour problems. Beckung and Uvebrant titled their 1997 paper on epilepsy ‘Hidden dysfunction in childhood epilepsy’. ‘Hidden’ and ‘under the surface’ maybe, but hidden from whom? I suspect young people with epilepsy and their parents have been well aware for many years of some of these associated problems. But health professionals presented with children with epilepsy have concentrated too much on the convulsive episode itself and ignored the broader spectrum.

Five papers in this issue report on outcome studies, all of which follow up children who presented with illnesses in the neonatal period. The studies by Ruggieri et al. and Watanabe et al. are principally concerned with the outcome of a specific condition. The former examines spinal-cord injury and the latter, convulsions. Both are chiefly concerned with how the seizures progress and how the specific signs relate to neurological findings. Both mention general functional outcomes – Watanabe discusses psychomotor delay, while Ruggieri concentrates on learning and behaviour disorders – but neither explains how they were measured.

Humankind has always been fascinated by the concept of depicting and recording human motion. During the Renaissance, Giovanni Alfonso Borelli, a student of Galileo, was among the first scientists to analyse motion while developing his theory of muscle action based upon mechanical principles. Early static interpretations of morbid anatomy by Da Vinci and Vesalius were ‘brought to life’ by the discovery that electricity caused muscle activity, as described by Luigi Galvani in 1791. The first scientific, systematic evaluation of muscle function was conducted by Duchenne who described the function of individual muscles of the human body in a monumental work, Physiologie des Mouvements, published in 1867.

As a graduate psychology student in the early 1960s, I came to the University of Iowa Hospital School with much energy, considerable naivety, and many questions. Do children with cerebral palsy (CP) have difficulties with psychological adjustment, what do their families need, can we really have an effect on their quality of life, do we know anything that really makes a difference in the long term? What do we mean by ‘they have to accept their disability’, what standard is the baseline from which to best measure improvement, and what is ‘normal’ adjustment if a child has a physical disability? Over the last three decades, has practice changed for the better for the child with CP? Have our perspectives changed on any of these questions?

Neurology has always been considered to be based on a sound knowledge of basic neuroscience with a ‘Sherlock Holmes’ approach to localization in the brain. At a recent meeting of the editorial board of this journal, we were told by our distributors that some of the Clinics in Developmental Medicine had too much emphasis on basic neuroscience. I have been described as belligerent because of my constant nagging at these meetings about the lack of good basic research papers in the field of paediatric neurology. However, looking at other paediatric journals and performing a mental geographical tour of known paediatric research units, there does seem to be a genuine paucity of fundamental research in this area.

How, as medical professionals, do we improve our skills so that we can enhance the life of our patients with developmental concerns? What is the best use of our learning time? Is our knowledge going to be as helpful to our patients as that which they acquire themselves? Do we learn more from observation and reading, or attending meetings? These are questions you may ask yourself when you pick up each issue of this journal.

Terminology means ‘a set of words used in a specially understood or defined sense’. From the standpoint of an international journal welcoming English language material from all over the world, it is apparent that widespread differences in meaning and uses of terminology exist in the field of developmental medicine. Differences in terminology matter, as Bax and Mac Keith commented, ‘First, foremost, surely, is how careful we must be in our language. If words are used... (that) confuse us they may in fact harm the patient’.

Of 55200 Maltese children born in the Mediterranean islands of Malta and Gozo in the birth years 1981 to 1990 inclusive, 134 fitted the case definition of cerebral palsy (CP). Children were allocated to prenatal, perinatal, and postnatal groups according to strict criteria and to various CP syndromes using the Swedish classification. The study was undertaken between 1994 and 1996 and the period prevalence rate was calculated. The control group comprised 134 children who were matched for sex and born immediately before or after the index subjects in the same hospital. Data related to risk factors were extracted from medical records and collected by interview with parents/carers. Unadjusted odds ratios (OR) with 95% confidence intervals for a range of risk factors were calculated. For risk factors with a relatively high prevalence in the population, such as prematurity and low birthweight, ORs were calculated from case-control data. For low-prevalence risk factors, such as breech presentation and multiple pregnancy, ORs were calculated against the whole population data for the 10-year birth cohort. The prevalence rate of CP in the geographically defined, stable population of the Maltese islands was similar to that in the UK and higher than that for Sweden, but the rates for different types of CP were markedly different from most developed countries, with a higher rate of spastic tetraplegias. Risk factors were as expected and similar to those reported from other developed countries. The study shows a strong social gradient with a higher than expected proportion of CP in children of unskilled manual workers.

Neuropsychological and behavioral status were examined in 57 children aged 7 to 16 years with complex partial seizures (CPS) and compared with 27 sibling control children of the same age. Epilepsy had a significant effect on both cognitive and behavioral adjustment measures. Children with CPS had significant impairment across all seven cognitive domains assessed, reflective of a profile of relatively diffuse and generalized cognitive dysfunction. Age at onset of recurrent seizures was the strongest and most consistent predictor of adequacy of cognitive functioning; earlier age at onset was associated with poorer cognitive status. Children with CPS also had more problems compared with sibling control children on measures of social and school competence and internalizing behavior problems, but not externalizing behaviors. Further, frequency of seizure activity in the past year, rather than age at seizure onset, emerged as the strongest predictor of these behavioral difficulties. These findings are discussed in the context of understanding the impact of CPS on cognition and behavioral adjustment, and identifying the contribution of various aspects of the neurodevelopmental course of CPS to these issues.

This study examines the executive function (EF) skills of extremely-low-birthweight (ELBW) children at school compared with their peers. Thirty children with ELBW and 50 control children (both with a mean age of 62±4 months) were administered tests of EF including the Tower of Hanoi task, Finger Sequencing task, and Tapping Test. Children with ELBW, including those who scored more than 1 SD below the mean on the Peabody Picture Vocabulary Test – Revised, scored significantly lower than their peers on all executive tasks. There was limited correlation between EF and previous general quotient index scores obtained at routine assessment using the McCarthy Scales of General Ability at 4 years of age for the children with ELBW. Results suggest that children with ELBW are at risk for deficits in ‘executive’ behaviours including planning, sequencing, and inhibition which may have implications for later learning.

The aim of the study was to test the hypotheses that elevated cerebral lactate, detected by proton spectroscopy performed within 18 hours of suspected birth asphyxia, is associated with adverse outcome, and that increased lactate can be used to predict adverse outcome. Thirty-one term infants suspected of having had birth asphyxia and seven control infants underwent proton magnetic resonance spectroscopy, using three-dimensional chemical shift imaging, within 18 hours of birth. Adverse outcome was defined as death or neurodevelopmental impairment at 1 year of age or more. Nine infants had an adverse outcome. The other 22 and all of the control infants remained normal. Median (range) lactate/creatine plus phosphocreatine (lactate/creatine) ratios in the abnormal, the normal, and the control group were 1.14 (0.17 to 3.81), 0.33 (0 to 1.51), and 0.05 (0 to 0.6) respectively (P=0.003). Lactate/creatine >1.0 predicted neurodevelopmental impairment at 1 year of age with sensitivity of 66% and specificity of 95%, positive and negative predictive values of 86% and 88%, and a likelihood ratio of 13.2. Elevated cerebral lactate/creatine within 18 hours of birth asphyxia predicts adverse outcome.

Autism and Rett syndrome (RS) are both developmental disorders of unknown origin. Autism is a behaviourally defined syndrome. RS, which affects girls only, is characterized by a profound learning disability following early normal development, with a consistent cluster of clinical features. Differentiation of RS from infantile autism in the very early stages of the disorders is not always easy. Both syndromes still lack discriminative laboratory markers for accurate diagnosis and differentiation. We decided to compare the CSF nerve-growth factor (NGF) levels of children with infantile autism and children with RS using enzyme-linked immunosorbent assay (ELISA). Our findings of mainly normal CSF NGF in autism and low to negligible values in RS are in agreement with the different morphological and neurochemical findings (brain growth, affected brain areas, neurotransmitter metabolism) in the two syndromes. CSF NGF could be used as a biochemical marker for differentiation of patients with autism from those with RS.

In 73 patients with myelomeningocele (mean 17.2 years, range 5 to 40 years) the classification of level of lesion was studied according to six commonly used classification systems and to the ambulation groups of Hoffer. The distribution of the patients into classes of thoracic-level lesions was the same for four classification systems in 10 patients and for two classification systems into categories of level L3 in 14 patients. For the other patients discrepancies occurred between systems for lesions of level L3 and downward. None of the patients was consistently categorized in the functional ambulation groups of Hoffer using all classification systems. The results show that it is not possible to compare neurological lesion levels classified according to the different systems described in this study and consequently that the distribution into the functional ambulation groups of Hoffer varies. To enhance communication and facilitate comparing the results of treatment we suggest using some basic criteria for patient documentation.

The aim of this retrospective study was to ascertain whether physiological derangement and potential secondary brain insult from raised intracranial pressure (ICP) or reduced cerebral perfusion pressure (CPP) in non-accidental head injury (NAHI) influences outcome. Any child who had a diagnosis of NAHI and had ICP monitoring or measurements during the acute illness was entered in the study. Seventeen children with an average age 5.1 months (range 1 to 20 months) were identified. Details of the acute encephalopathy, lowest mean arterial blood pressure (MAP), mean of maximum ICP measured, lowest CPP, and neurodevelopmental outcome at follow up were obtained from the hospital case notes. Seshia's (1994) outcome classification scale was used. The lowest CPP was very significantly related to outcome (P=0.0047, τ= −0.544). Mean of maximum ICP did not correlate with outcome. The lowest MAP was significantly related to outcome (P=0.039). It was concluded that the degree of secondary brain insult from reduced CPP influences outcome.

This study investigated the accuracy of prediction of neurodevelopmental outcome at 1 year using cerebral proton magnetic resonance spectroscopy (MRS) and structured neonatal neurological assessment in term infants after presumed hypoxic–ischaemic brain injury. Eighteen control infants and 28 infants with presumed hypoxic–ischaemic brain injury underwent proton MRS investigation. Studies were carried out as soon as possible after the cerebral insult, most within 48 hours. Infants had an early structured neurological assessment at a median of 19 hours (range 0 hours to 9 days) from the presumed hypoxic–ischaemic insult and a late assessment at a median of 7 days (range 3 to 25 days) during recovery. The maximum cerebral peak–area ratio lactate:N-acetylaspartate measured by proton MRS accurately predicted adverse outcome at 1 year with a specificity of 93% and positive predictive value of 92%. Neurological assessment had a tendency for false-positive predictions. However, both early and late neurological examination can be used as a reliable indicator for a favourable outcome at 1 year having negative predictive values of 100% and 91% respectively.