We describe a 9-year-old female with thoracic epidural haematoma. The clinical course simulated Guillain-Barré syndrome (GBS) so intravenous immunoglobulin therapy was started at the paediatric clinic. Magnetic resonance imaging (MRI) 3 days after admission showed thoracic epidural haematoma between T2 and T8. An emergency laminectomy was performed and the patient's neurological symptoms began to improve immediately after surgery and she made a full recovery during the 2 weeks of follow-up. Time is a very important factor in achieving reversibility of symptoms of compressive cord lesions, such as spinal epidural haematoma, and MRI is mandatory for patients with progressive paraplegia, even though the signs and symptoms might suggest GBS.

There is now substantial evidence to suggest that the presence of inflammatory molecules within the fetal circulation and brain is associated with damage to vulnerable oligodendrocyte precursor cells before 32 weeks' gestation and long-term white matter damage (WMD); however, the nature of the inflammatory stimulus remains unclear. The common association between ascending bacterial infection from the vagina, haematogenous spread of periodontal organisms, chorioamnionitis, preterm delivery, and neonatal brain injury provides indirect clinical evidence supporting an important role for bacteria. Animal experiments in which live bacteria, or endotoxins, lead to the classical focal necrotic lesions of periventricular leukomalacia add further support to this concept. The isolation of bacteria-specific DNA from only a small proportion of cases where there is evidence of histological chorioamnionitis, suggests that there may be non-bacterial causes of fetal inflammation. Viruses make attractive alternative candidates; they are a common cause of maternal pyrexia, can cross the placenta (e.g. cytomegalovirus and rubella), are known to cause WMD and microcephaly, and are difficult to isolate. A recent study of viral DNA in stored neonatal blood spot specimens suggested a possible link between viral exposure and later cerebral palsy, particularly in preterm babies. Dammann and Leviton have been even more specific, identifying maternal pestivirus (PV) infection as a hypothetical cause of WMD in preterm neonates.

Eliasson et al. are to be warmly congratulated on the publication of their excellent paper ‘The Manual Ability Classification System (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability’ in this issue of Developmental Medicine and Child Neurology.

Systematic studies of cognitive development in individuals with congenital giant interhemispheric cysts (GIC) without neurosurgical intervention are rare. However, data from such studies are of great importance in deciding whether or not to perform cerebral shunting in otherwise asymptomatic patients. In this study, psychomotor function was examined in 13 children (three females, 10 males; mean age at follow-up: females 1 year 6 months, males 3 years 2 months; age range: females 13 months to 4.2 years, males 10 months to 10 years) all diagnosed with GIC during the neonatal period after indicative foetal ultrasound. Eight patients received neurosurgical treatment due to increased intracerebral pressure during the first year of life. Psychomotor development was evaluated using Griffith's Psychomotor Developmental Scales, the Sniders–Oomen Non-verbal Intelligence Scale for Young Children and the Kaufmann Assessment Battery for Children. The majority of patients showed normal intelligence, irrespective of the presence or absence of neurosurgical intervention. Evidence was found that the cognitive outcome might be related to an association of GIC with agenesis of the corpus callosum or additional congenital cerebral malformations, such as gyration anomalies. Main postoperative complications were chronic subdural effusions with spastic cerebral (hemi)-paresis; were present in three patients. In view of the risk of severe postoperative neurological complications and a comparable psychomotor development in patients without neurosurgical treatment, recommendations for early neurosurgical intervention in otherwise asymptomatic patients with a foetal or neonatal diagnosis of GIC should be viewed with caution.

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’.

In paediatric outpatient departments it is not uncommon for a child to complain of muscle pain and stiffness. These may be labelled as ‘growing pains’, a diagnosis of exclusion when no obvious cause is found. Pain and stiffness occur most frequently at night and are located usually in the leg muscles, and not in the joints. They are intermittent, and tend to occur after the child has had an especially active day, so that a detailed history which notes pain after almost any amount of exercise (as opposed to stress at the site of the pain) will reveal the differences from McArdle's disease. If examination results are entirely normal investigations are to be avoided unless the symptoms persist. If they do, a number of causes may be found, from orthopaedic abnormalities to metabolic disorders. This paper is concerned with one of the latter: glycogenosis type V or McArdle's disease, a rare autosomal recessive condition resulting from muscle phosphorylase deficiency, an enzyme which initiates the breakdown of glycogen in skeletal muscle. Cardiac muscle is not significantly affected. The condition usually presents in the first two decades of life, but can occur in infancy and adulthood.

The Battery for Rapid Evaluation of Cognitive Functions (Batterie Rapide d'Evaluation des Fonctions Cognitives: BREV) is a quick test to screen children with higher-functioning disorders and to define the patterns of their disorders. After standardization tests in 500 normally developing children aged 4 to 8 years, validation consisted of comparative evaluation of the specificity and sensitivity of the BREV with a wide reference battery in 202 children with epilepsy (108 males, 94 females; mean age 6 years 6 months, SD 1 year 8 months). Children were divided into 10 age groups from 4 to 8 years of age and represented eight epileptic syndromes. The reference battery included verbal and non-verbal intelligence assessment using the Wechsler scale, oral language assessment with a French battery for oral language study, drawing with the Rey figure, verbal and visuo-spatial memory with the McCarthy scale subtest and the Rey figure recall, and educational achievement with the Kaufman subtests. Every function evaluated with the BREV was significantly correlated with the reference battery testing a similar function (p=0.01 to 0.001). Specificity and sensitivity of the BREV verbal and non-verbal scores were correlated with those of the Wechsler scale in more than 75% of children. The BREV, therefore, appears to be a reliable test which has been carefully standardized and validated and is valuable in screening for cognitive impairment in children.

Cortical visual impairment (CVI) refers to bilateral impairment of vision that is usually due to damage occurring perinatally in the visual cortex and/or optic radiations. The most common cause of this damage is hypoxia, and other causes include encephalitis, meningitis, and trauma. Relatively little research has been done to quantify visual abilities in children with CVI. In the present study, we used an electrophysiological technique (visual evoked potentials) to measure two aspects of spatial vision in 35 infants and children with CVI (15 females, 20 males; mean age 3 years 6 months, SD 3 years 5 months; age range 4 months to 16 years). We measured each child's grating acuity (resolution for detecting high-contrast stripe patterns) and vernier acuity (resolution for localizing pattern elements). Performance on grating acuity and vernier acuity in individuals with CVI was compared with that of age-matched individuals with normal vision, and it was found that vernier acuity was relatively lower than grating acuity in children with CVI. Results support the theory that vernier acuity is cortically mediated, and suggest that vernier acuity is a more sensitive measure than grating acuity for quantifying vision deficits in patients with CVI.

Giant axonal neuropathy (GAN) is a chronic progressive disease of the peripheral nervous system, but lesions can also occur in the central nervous system (CNS), especially in the brainstem and cerebellum. Its mode of inheritance is autosomal recessive, with parents of affected children often being consanguineous, but sporadic cases have been reported.

A male child, born at 37+5 gestational weeks (GWs) (birthweight 2000g) after intrauterine growth retardation (IUG; –3 SD), to a mother treated during pregnancy for asthma, developed periventricular leukomalacia and retinopathy with total retinal detachment in the left eye and partial detachment in the right eye. Apart from basic asthma treatment with terbutalin, budesonid, and fenoterolhydrobromid throughout the pregnancy, she was treated with intravenous or oral cortisone for 6.5 weeks from 28+5 GWs. In addition she developed deep venous thrombosis at 29 GWs and was treated with heparin until delivery. Psychotic symptoms during the 31st GW were treated with diazepam, haloperidol, and levomepromazin. Functional sequelae for the child were visual impairment (visual acuity 5/60), uneven intellectual profile (Wechsler Pre-school and Primary Scale of Intelligence, Verbal IQ 94 and Performance IQ 32 at 8y of age), and autistic-like behaviour. The possibility that pre- and perinatal risk factors (e.g. severe maternal illness, IUGR, and cortisone treatment) in a term infant may create conditions for developing eye and brain pathologies commonly closely related to preterm birth should be considered.

Botulinum toxin has rapidly gained wide acceptance as a treatment for focal spasticity, and is becoming more readily available. Early studies established its effectiveness, but many issues are still open for discussion. After a brief review of the historical and scientific development of botulinum toxin as a clinical tool and an outline of previous work in the field, some important questions are discussed in the light of more recent trials.

A neuropsychological assessment was conducted to study cognition, with emphasis on memory, information processing/learning ability, and executive functions in boys with Duchenne muscular dystrophy (DMD). A group of 20 boys with DMD, aged 7 to 14 years (mean age 9 years 5 months, SD 2 years 2 months), was contrasted with 17 normally developing age-matched comparison individuals, using specific neuropsychological tests (Block Span, Digit Span, Story Recall, Rey Auditory Verbal Learning Test, Rey Complex Figure Test, Spatial Learning Test, Verbal Fluency, Trail Making Test, Tower of London, Memory for Faces, and Raven's Coloured Progressive Matrices). The DMD group performed significantly worse on all aspects of memory, learning, and executive functions. There was no significant difference in general intellectual ability between the two groups. Analyses of group differences indicate that problems in short-term memory are the most apparent, suggesting specific cognitive deficits. The differences between the groups were similar for both verbal–auditory and visuospatial tests, thus contradicting the idea that cognitive deficits are related to type of stimulus presented. It is concluded from this study that short-term memory deficits might play a critical role in the cognitive impairment and intellectual development seen in those with DMD.