The level of agreement between clinicians' ratings of gait patterns in children with spastic hemiplegia was investigated using the Winters, Gage, and Hicks (WGH) classification scale. Sixteen clinicians (nine physiotherapists, seven orthopaedic surgeons) from six gait laboratories in five different countries rated gait patterns in 34 children with spastic hemiplegia (22 males, 12 females; mean age 10y [SD 3y], range 6–18y). Archived three-dimensional gait reports (kinematic and video data) and videos (video clips only) were rated for each child. Agreement between clinicians was substantial for reports (weighted kappa [wκ] 0.77, range 0.62–0.89). Agreement was lower for videos (wκ 0.63, range 0.39–0.97) and for clinicians' agreement between reports and corresponding videos (wκ 0.62, range 0.47–0.76). Exact agreement was unacceptable for some gait patterns using reports (mean 65%, range 32–74%) and videos (mean 53%, range 35–94%). Not all gait patterns could be rated using the original WGH categories. It was concluded that: (1) agreement is acceptable using the WGH tool; (2) kinematic data from 3D instrumented gait analysis and video should be used together when using the WGH scale; and (3) further refinement of this classification is required.

The purpose of this study was to develop an instrument to measure manual praxis by assessing areas of function considered important in learning and planning movement; use of tools, imitation of gesture, and motor sequencing. Participants included 362 healthy children aged between 3 and 12 years. Their parents completed a questionnaire on hand skills. Performance on all tasks improved with age. However, transitive gestures reached an early ceiling effect at around 5 years of age. Assessment of manual praxis from the parental questionnaire correlated with the child's directly measured competency during a test of object use (P<0.01) but not with the ability to imitate transitive gestures or enact a motor sequence with a ‘novel’ task. Children from schools where a higher number were eligible for free school meals because of low family income had more difficulty with imitation of gesture and motor sequencing to verbal and picture commands (P<0.001, P<0.05, P<0.05). They were neither disadvantaged in tool use nor in appreciation of extracorporeal space. The pattern of errors made in tool use and motor sequencing differed from those described in adults with acquired dyspraxia. These results suggest that assessment of manual praxis in clinical populations should include tool use, imitation of gesture, and motor sequencing and that they should not necessarily be regarded as measuring a unitary function.

The introduction of advanced assisted reproduction technologies (ART) has created opportunities for the treatment of infertility among patients with myelomeningocele (MMC). The aim of this study was to assess the possibility of semen retrieval and to analyse the semen quality in men with MMC. Nine men, aged 22 to 39 with MMC participated in the study. Two participants were able to achieve unassisted ejaculation. Vibratory stimulation was unsuccessfully attempted in the remaining seven participants who then underwent electroejaculation under general anaesthesia. In total, enough spermatozoa for intracytoplasmic spermatozoa injection were retrieved from five participants. In four cases, no spermatozoa were observed in the ejaculates. Testicular biopsies, however, revealed spermatogenesis, and thus a reproductive potential, in one of these men. Therefore, in six of the nine men with MMC, fatherhood seemed possible with modern ART, despite the semen quality generally being very poor.

Hurler syndrome is the most severe form of mucopolysaccharidosis type I. There is a paucity of literature reporting the gross motor abilities of children with untreated Hurler syndrome. The purpose of this case series is to describe the gross motor abilities of one male and three female children (mean age 11.4mo [SD 3.1]; range 9.5–16mo) diagnosed with Hurler syndrome. The children were assessed using the Peabody Developmental Motor Scales, 2nd edition. Gross motor delays were present in all four children at the time of assessment, and were most evident in locomotor abilities for three of the children. All four children had range of motion limitations at multiple joints. This case series provides evidence for early gross motor delays in this population, as well as evidence for specific gross motor abilities of children with untreated Hurler syndrome. It is recommended that children diagnosed with Hurler syndrome be referred to physical therapy services upon diagnosis and that physical therapists be part of the interdisciplinary team involved in the care of children with Hurler syndrome.

The purpose of this study was to compare the cumulative efficacy (three treatment sessions) of botulinum toxin A (BTX-A) alone, casting alone, and the combination of BTX-A and casting in the management of dynamic equinus in ambulatory children with spastic cerebral palsy (CP). Thirty-nine children with spastic CP (mean age 5y 10mo, range 3 to 9y) were enrolled in the study. A multicenter, randomized, double blind, placebo-controlled prospective study was used. Children were randomly assigned to one of three treatment groups: BTX-A only (B), placebo injection plus casting (C), or BTX-A plus casting (B+C). The dosage for the BTX-A injections was 4U/kg per extremity. Assessments were performed at baseline, 3, 6, 7.5, and 12 months with a total of three treatments administered after the evaluations at baseline, 3, and 6 months. Primary outcome measures were ankle kinematics, velocity, and stride length. Secondary outcome measures were ankle spasticity, strength, range of motion, and ankle kinetics. Group B made no significant change in any variable at any time. Groups C and B+C demonstrated significant improvements in ankle kinematics, spasticity, passive range of motion, and dorsiflexor strength. Results of this 1-year study indicate that BTX-A alone provided no improvement in the parameters measured in this study, while casting and BTX-A/casting were effective in the short- and long-term management of dynamic equinus in children with spastic CP.

The aim of this study was to evaluate cognitive development at the onset of West syndrome (WS) with regard to electroencephalogram (EEG) patterns and visual function. Twenty-five patients (14 males, 11 females) at the onset of spasms (T0) in WS and 2 months later (T1) underwent a full clinical evaluation, including neuroimaging, cognitive assessment, video-EEG, and visual function. Mean age of the patients at spasm onset was 5.9 months (SD 2.5; range 2 to 13mo). Cognitive development, assessed with Griffiths Mental Development Scales (GMDS), was generally impaired at T0 and was significantly related to visual function (p<0.001) at both T0 and T1. In general, there was a specific major impairment in the eye–hand coordination scale of the GMDS which tended to disappear after 2 months in less severe cases. At the onset of spasms, sleep EEG organization seemed to be better related to cognitive abilities than awake hypsarrhythmia. These results support a close link between visual function and cognitive competence in WS and provide additional information to improve the understanding of possible mechanisms underlying cognitive impairment.

We describe four children with dysmorphic syndrome with severe learning disability (SLD). Their chromosomes had been normal on conventional cytogenetic examination. However, screening using a multiprobe fluorescence in situ hybridisation (FISH) technique for subtelomeric abnormalities revealed a deletion of the p arm of chromosome 1. The physical features include body asymmetry, microcephaly, distinctive facies with deep-set eyes, sharply defined eye sockets, and mid-face hypoplasia; the neurodevelopmental profile was characterised by SLD, motor delay with hypotonia, markedly delayed visual maturation, and postural asymmetry together with epilepsy. This phenotype is consistent with that described for partial monosomy for 1p36.3.

The assumption that children born preterm have difficulties in maintaining active attention was tested in passive and active tasks. Twenty 5-year-old children born preterm at 26 to 32 weeks gestational age were compared with 20 children born at term, matched for age and IQ, using an auditory paradigm. In the passive task participants had to watch a videotape of a cartoon and ignore auditory stimuli. In the active task they had to detect a rare tone (the ‘target’ tone; 10% of the tones presented) among frequent tones (the ‘standard’ tone; 90%). Accuracy and reaction time were analysed, and event-related potentials (ERPs) were recorded at the scalp sites Fz, Cz, T3, T4, Pz, Oz, and two electrodes for the left mastoid (passive task); and Fz, F7, F8, Cz, T3, T4, Pz, and Oz (for the active task). Behavioural and electrophysiological data were analysed with repeated-measure ANOVAs. The results showed a significant group effect only on the active task. The preterm group scored fewer correct hits (correct detection of target tone) and were less efficient in their attentional strategy as assessed by ERP components.

The aim of this cross-sectional study was to determine the prevalence of cerebral palsy (CP) among children in Turkey between the ages of 2 and 16 years. Samples were selected from cities, towns, districts, and villages using the cluster sampling method; 41861 children were selected. Data was collected by parental interview and physical examination. One hundred and eighty-six children were identified with CP. The prevalence of CP was determined as 4.4 per 1000 live births and included postnatally acquired CP. Origin of CP was classified as prenatal in 49 (26.6%), perinatal/neonatal in 34 (18.5%), postnatal in 11 (5.9%), and unclassifiable in 90 participants (48.9%; data was unobtainable for two individuals). Type of CP was diplegia in 39.8% of children, hemiplegia in 28%, tetraplegia in 19.9%, ataxia in 5.9%, and dyskinetic in 6.4%. Prenatal factors were seen more frequently in the groups with a high socioeconomic status while perinatal factors were encountered more often in those with a low socioeconomic status (p<0.05). Place of residence and sex had no significant effect on the prevalence of CP (p>0.05). This cross-sectional study shows that the prevalence of CP in Turkey is higher than that in developed countries but the aetiology is probably similar. Although the high prevalence of CP in Turkey could originate from an increased level of obstetric and neonatal problems, the lack of a possible aetiological factor in approximately half the children suggests that the high level might also be due to other factors, such as genetic disorders.

The electromyographic (EMG) responses to tendon taps at the ankle and ensuing muscular twitch forces and temporal parameters were studied at varying angles across the joint range in 18 children, aged 3 to 14.9 years, with congenital hemiparetic cerebral palsy and 22 healthy (control) children, aged 3 to 13.6 years. Those subjects with hemiparesis were community ambulators without assistance. In all subjects, passive muscle stretch caused a waxing of the reflex EMG and twitch force near neutral (with the sole of the foot at right angles to the tibia) and a diminution of these with further dorsiflexion. Twitch times increased with each dorsiflexing increment, being slowest at maximum dorsiflexion and fastest at the resting plantarflexion angle. Heterogeneity of the hemiparetic-limb data is evident when compared with data of non-paretic and unaffected limbs, with clear differences in the clonic (fast twitch) as opposed to non-clonic (slow twitch) muscles. In four cases with clinical clonus, clonus frequency was reduced by passive dorsiflexion. Plaster immobilization for 1 month produced clonus which was previously absent in one subject, and caused a fast-twitch phenotype to emerge in two subjects. Follow-up after heel-cord lengthening in one subject showed that clonus frequency diminished from 9 to 3 Hz with slowing and strengthening of muscle-twitch phenotype. Short- and long-term peripheral manipulations appear to regulate neuromuscular excitability according to whether muscles are loaded or unloaded. Although damage to the nervous system provides the setting for reflex excitability, the data suggest that the muscle length (which specifies the joint angle) and the muscle-twitch phenotype of any given limb for any given case appear to dictate the actual speed and strength of reflex muscle-twitch and clonus profiles. This study illustrates how peripheral manipulations of muscles and tendons may alter the expression of what have hitherto been considered as exclusively central phenomena.

This study explored the relation between a motor-free visual perceptual deficit, different visual–motor integration deficits, and different motor skills in children with developmental coordination disorder (DCD). Thirty-six children (22 males), aged 9 or 10 years, with DCD and a control group (n=36), matched for age and sex, were assessed with the Movement Assessment Battery for Children (MABC), a ball-catching test, a jumping test, a timed response task to a visual moving stimulus, and the Beery-Buktenica Developmental Test of Visual–Motor Integration, incorporating copying, visual discrimination, and tracing tasks. Children with DCD performed significantly worse than the control group on all measures. The visual discrimination task did not correlate significantly with any of the motor tasks. The visual timing task correlated significantly with the ball-catching test in the DCD group. The copying test was significantly correlated with the MABC in the DCD group. The association between visual–perceptual deficits and motor tasks was shown to be task specific.

Nothing in clinical medicine is one hundred per cent certain. Part of a doctor's education involves learning how to cope with the anxiety that uncertainty in decisions affecting life and death inevitably produces. This paper examines: (1) the role of anxiety – both rational and irrational – in the provision of health care; (2) the effects of uncertainty upon the doctor–patient relationship; (3) the threat uncertainty poses to medical authority (and the assumption of infallibility that props it up); (4) the contribution of clinical uncertainty to the rising popularity of alternative therapies; and (5) the clash between the medical and the legal understanding of how certainty should be defined, particularly as it affects the paediatric community. It concludes by suggesting some strategies that might facilitate successful navigation between the opposing and ever-present forces of certainty and uncertainty.

An historical cohort study was conducted to investigate the rate and extent of neurological recovery in obstetric brachial plexus injury (OBPI) and to identify possible prognostic factors in a cohort of children with OBPI from birth to 7 years. All children (n=56; 31 females, 25 males) with OBPI were evaluated at fixed time intervals by one examiner. They underwent a final neurological examination at a mean age of 3 years 10 months (range 1 to 7 years). Neurological outcome was not as favourable as is often reported: complete neurological recovery occurred in 37 out of 56 children (66%). In half of these there was delayed recovery, in which case complete neurological recovery was assessed from 1.5 to 16 months of age (median age 6.5 months, SD 4.2 months). External rotation and supination were the last to recover and recovered the least. Although biceps function at three months was considered to be the best indicator for operative treatment, external rotation and supination were found to be better in predicting eventual complete recovery. Initial symptoms directly post partum were not found to be prognostic. Functional outcome was mainly reported to be good.

The effects of adapted spoons on food spilling and movement kinematics (movement duration, dysfluency) were examined in people with tetraparetic cerebral palsy (CP). We varied the thickness of the spoon's handle (diameters of 1, 3, and 5cm), the substance with which the spoon was filled (sugar or water), and the size of the bowl into which the spoon had to be emptied (diameter 5 or 11cm). Ten adolescents with tetraparesis (five males, five females; mean age 17y 2mo [SD 1y 3mo]; range 14y 7mo–18y 6mo) and 12 healthy comparison participants (four males, eight females; mean age 25y 6mo [SD 3y 0mo]; range 20y 0mo–30y 0mo) participated. Four participants with tetraparesis could walk independently, while the other six sat unstrapped in a wheelchair. The task consisted of picking up and transporting the filled spoon and emptying it into a bowl. Movements of the hand were recorded by a three-dimensional motion tracking device. When the spoon's handle was thickened participants with CP were faster in picking up the spoon and transporting it to the bowl, in particular when the spoon was filled with water. Importantly, this faster performance did not lead to increased spilling. These results are the first systematic test of the supposed beneficial effect of adapted spoons on movement performance in people with CP.

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.

To confirm the presence of apneusis in patients with hypoxic–ischemic encephalopathy and to clarify which factors influence their respiratory patterns, polygraphic studies were performed on two patients. Apneusis was clinically suspected in both patients who had severe brainstem damage. In one subject, inputs of vagal afferents from the gastrointestinal tract and the urinary bladder often resulted in extreme tachypnea instead of apneusis. Lung inflation facilitated expiration during inspiratory arrest. Expiration preceded a periodic inhibition of rigospastic discharge in the right biceps muscle. In the other subject, prolonged inspiratory pauses with cyanosis occurred with or without preceding epileptic seizure. Both phenytoin dose reduction and treatment with tandospirone, a serotonin-1A agonist, were effective in improving the respiratory distress in this subject.

Twenty children (11 females; age range 8y 2mo to 14y 7mo, mean 10y 6mo, SD 1y 8mo) with electroclinically typical rolandic epilepsy (RE), were investigated to demonstrate possible occurrence of abnormal oromotor functions and oral sensibility, linguistic problems, and impaired auditory discrimination. Twelve children were treated with an antiepileptic drug (carbamazepine, valproate, or sulthiame). They were compared with an age- and sex-matched control group of 24 children (14 females; age range 8y 2mo to 14y 5mo, mean 11y, SD 1y 8mo). The test battery included tests for facial expressions, lip and tongue movements, repeated syllables, and articulation using nonsense and tongue-twisting words. Sensibility on tongue and lips was tested with two-point discrimination. As language tests, Rapid Confrontation Naming, orthographic, and phonologic decoding tests were used. A dichotic listening test was performed in 13 children with RE and in 14 controls. Compared to control children, those with RE had significantly greater problems concerning tongue movements (p<0.05) and articulation (nonsense words and tongue-twisting words; p<0.01), and worse performance on dichotic listening (p<0.05). Oro-lingual sensibility and results of language tests did not differ between those with RE and control children. The results indicate that children with RE have distinct but mild problems with oromotor performance and auditory discrimination.

Convulsive status epilepticus (CSE) is the most common medical neurological emergency and continues to be associated with significant morbidity and mortality. The two primary determinants of outcome from CSE are underlying aetiology and seizure length. It is unclear whether a prolonged seizure worsens the prognosis of an underlying aetiology or whether a more severe underlying disorder is more likely to result in CSE. Acceptance of the former hypothesis means that seizures need rapid treatment as their early termination could potentially reduce brain damage and morbidity. Pathophysiology, epidemiology, and outcomes from CSE have recently been reviewed elsewhere.

We report a case of a 7-month-old male with primary intracranial haemorrhage 2 months after infection with varicella zoster virus (VZV). His initial clinical course was complicated by seizures and right hemiparesis; when last seen at 22 months the only positive finding was of left hand preference. Although the literature has recently established the association of arterial ischaemic stroke and VZV infection, primary intracranial haemorrhage has been reported only in one case. The child reported here had anterior interhemispheric haemorrhage due to a focal arteritis of the left anterior cerebral artery. The vascular abnormality was transient and had radiological features compatible with either a focal arteritis or vasospasm as a direct result of blood surrounding the vessels. We postulate that direct invasion of VZV caused extensive inflammation of the vessel wall and aggressive tissue penetration resulting in necrotizing angiitis and intracranial haemorrhage. We suggest that VZV infection should be considered a potential risk factor for intracranial haemorrhage in children.

We have developed a gait nomogram based on dynamic similarity to characterize and compare neuromuscular function. We used temporal–distance data based on 669 normal participants (age range 5 to 98 years), and 78 children and young adults with spastic diplegia (43 males, 35 females; mean age 10y 8mo, standard deviation 3y 11mo, range 5 to 20y), all of whom were independent ambulators. A new statistical algorithm known as fuzzy clustering was implemented and five cluster centres were identified, each representing distinct walking strategies adopted by children with cerebral palsy. Using just three easily obtained parameters – leg length in metres, stride length in metres, and cadence in steps per minute – our program calculates a child's dimensionless step length and step frequency, generates the individual's membership values for each of the five clusters, and plots the gait nomogram. The clinical utility of our approach has been demonstrated for two test participants with spastic diplegia, using pre- and postoperative data (one neurosurgical and one orthopaedic), where changes in membership of the five clusters provide objective measures of improvement in their neuromuscular function.