The purpose of this study was to describe problems and resources of adults with cerebral palsy (CP) with special emphasis on locomotion. A questionnaire concerning demographic facts, locomotion, musculoskeletal problems, and present physical activity was mailed to 363 adults with CP. Two hundred and twenty-one adults, (125 male and 96 female; mean age 36 years, range 20 to 58 years) answered the questionnaire. Seventy-seven per cent reported problems with spasticity. Eighty-four per cent lived in their own apartments, with or without home services. Twenty-four per cent worked full-time and 18% had full disability pension. Twenty-seven per cent had never been able to walk, 64% could walk with or without walking aids, 35% reported decreased walking ability, and 9% had stopped walking. Eighty per cent reported contractures and 18% had pain every day. Approximately 60% were regularly physically active, and despite their disability, 54% considered that they were not limited in their ability to move about in the community.

Children with cerebral palsy (CP) have often been described as passive communicators. Their familiar conversation partners tend to direct and control interaction. Such conversation patterns may have various precursors: children's motor impairment, their intelligibility difficulties, and/ or their level of cognitive development. To test the comparative influence of these factors, measures of motor function, speech, communication, cognitive and language skills were applied in 40 children (18 males, 22 females) with CP who were aged from 2 years 8 months to 10 years. These variables were correlated with measures relating to interaction patterns to investigate whether individual features predicted communication style. In this group, poor speech intelligibility was the main predictor of restrictive communication patterns, such as fewer child-initiated conversation exchanges, more simple child communicative acts such as yes/no answers and acknowledgements of the other partner's messages. Results support the provision of therapy to increase children's intelligibility, whether spoken or augmented, such as the introduction of communication aids and training programmes for parents.

Neurological integrity in sick children is difficult to assess clinically. The aim of this study was to determine the predictive value of EEG activity recorded with a bedside EEG analysing monitor in an intensive care unit. EEG activity was monitored in 108 children (age range 2 weeks to 16 years, median 1.7 years) considered at risk for cerebral abnormalities with a cerebral function analysing monitor (CFAM). Recordings were evaluated for features of background EEG activity including mean amplitude, frequencies, and symmetry. Electrical seizure activity was quantified if present. Predictive value of the EEG features was evaluated relative to the clinical neurological outcome after one year. Asymmetrical recordings were not seen in any child with a normal outcome. Suppression of background activity was seen in 75% of the children who died. Seizures were present in 68% of children with a poor outcome. Seventeen of the 32 children (65%) who died had prolonged seizures. Absence of seizures and the presence of superimposed fast EEG activity in response to benzodiazepine infusions correlated with good outcome. A combination of two or more predictive EEG features demonstrated >90% specificity and positive predictive likelihood of poor outcome. EEG features provide information about the functional cerebral integrity of sick children. Changes in cerebral activity detected by the CFAM aid decision making by providing such information readily at the bedside in intensive care.

Although assessment of the quality of movement in children with cerebral palsy (CP) is difficult, the development of the Gross Motor Performance Measure (GMPM) has facilitated this process. In order to determine the interobserver reliability of the GMPM, 36 children with spastic neuromuscular disorders (mean age 7 years, range 4 to 15 years) were evaluated using four of the five dimensions of the GMPM. Percent Agreement, Intraclass Correlations, and Kappas were calculated by both dimension and attribute to determine reliability. In addition, reliability measures were evaluated over time to determine whether reliability improved with continual use of the GMPM. Overall, interobserver reliability was in the ‘fair to good’ category regardless of the reliability measure used in the analysis. Reliability scores improved over time with a greater number of individual item scores moving from the ‘fair to good’ category to the ‘excellent’ category. Results from this study indicate that it is possible to assess reliably the quality of movement in children with CP.

The study aimed to investigate the development of bladder control in children with cerebral palsy (CP) and to determinate subgroups with deviant development of bladder control and a higher risk of not achieving urinary continence. Children and adolescents between the ages of 4 and 18 years with a diagnosis of CP, from six Dutch rehabilitation centres were included in the study (n=601). Parents of these participants were sent a questionnaire, of whom 76% responded. Prevalence of primary urinary incontinence was 23.5%. Survival curves were made to determine the development of achieving bladder control in CP. For participants who achieved urinary continence, the development was delayed in comparison with normally developing children and adolescents. The most important factors influencing the occurrence of urinary incontinence in CP were tetraplegia and low intellectual capacity. At age six, 54% of participants with spastic tetraplegia and 80% with spastic hemiplegia or diplegia gained urinary continence spontaneously. Of those who had low intellectual capacity, 38% were dry at this age.

Neurological lesions that cause dyskinetic cerebral palsy (CP) commonly involve ocular movements. This report describes a group of 14 children (nine males, five females) whose CP is associated with severe dyskinetic eye movements. Ages ranged from 4 months to 13 years (mean 6.9 years). Clinical features of this eye movement disorder are discussed and defined. The visual function of these children is slow, variable, and highly inefficient. They are often misdiagnosed as blind, due to cortical visual impairment. Early recognition of dyskinetic eye moment disorder and appropriate developmental and educational management are important.

There were two purposes for this study: to determine the within-subject changes of visual evoked potentials (VEPs) over time, and to evaluate the usefulness of baseline VEP testing in the early diagnosis of increased intracranial pressure (ICP). Twenty children with hydrocephalus between the ages of 4 and 18 years (18 males, 2 females) were evaluated monthly for up to four months. Flash-evoked VEP tests were done in a standardized manner using a Cadwell Quantum 84 evoked potential stimulation and recording instrument. One participant had abnormal VEPs found during a previous episode of shunt malfunction. For all of the other VEP tests, the latency of the N2 response was in the range of adult normal values. For 15 participants with multiple evaluations there were non-significant differences in N2 latency from one session to the next. This study's results provide evidence that within-subject VEP measurements in children with hydrocephalus appear to be relatively stable over limited periods. However, considerable differences were found when comparing VEPs between participants. Further research may provide additional evidence that periodic, routine VEP testing is an effective way to monitor individuals who have shunted hydrocephalus in order to detect increased ICP before the appearance of obvious symptoms.

This study examined the effects of physical therapists' behaviors on the levels of distress and coping of 32 children (19 males, 11 females; age range 2.6 to 9.1 years) during a painful medical procedure. This is the second study to assess children throughout rehabilitation following selective posterior rhizotomy and the first to assess the impact of physical therapists' behaviors. Results of the Child–Adult Medical Procedure Interaction Scale–Revised confirmed the hypotheses that: (1) children exhibit more coping behaviors and fewer distress behaviors over time, and (2) positive relationships exist between coping-promoting behaviors in physical therapists and coping in children, and distress-promoting behaviors in physical therapists and distress in children. Prompts for children to use a coping strategy, reassuring comments, and non-procedural talk by therapists explained 67% of the variance in children's coping. Criticism, reassurance, checking child's status, praise, and empathic statements by therapists explained 65% of the variance in children's distress. Older children and children with higher IQ scores exhibited more coping behaviors.

Gliomatosis cerebri is the unifying term used when diffuse glial infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiological and pathological findings. Adult cases may simulate an acute diffuse encephalomyelitis and show postmortem evidence of a marked swelling of the spinal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10-year-old girl who presented with a history and physical signs suggestive of benign intracranial hypertension. A diffuse encephalomyelopathy occurred, which was complicated by spinal cord swelling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination.

We report a female infant with status epilepticus amauroticus and intractable focal motor seizures associated with congenital cortical dysplasia. EEG demonstrated persistent epileptiform discharges over the right parieto-temporal regions extending to occipital areas. She required cortical resection of the epileptic zone at age 8 months given failure of very high dose antiepileptic combinations. Histological analysis of a sample of cortex resected from the right central parieto-temporal region, identified by electrocorticography as the focus of epileptic activity, showed cortical dysplasia. The seizures ceased and the infant gained full vision after 48 hours. During an 8-year follow-up period she has had a few short-lived seizures, currently controlled with carbamazepine and vigabatrin. Her cognition and speech are intact. This case demonstrates that: (1) resection of a central temporo-parietal focus, which may have spread to the occipital regions, may result in complete visual recovery and cessation of seizures; and (2) EEG should be considered in every infant with alleged delayed visual maturation, to rule out concealed epileptic activity.

A male child with multiple congenital anomalies and developmental delay is described. Cytogenetic evaluation showed that the patient was partially monosomic for the short arm of chromosome 18 and partially trisomic for the short arm of chromosome 16: a combination of chromosomal syndromes not previously described.

Vitamin E (a-tocopherol) has antioxidant properties which prevent free radical damage to the nervous system, and there is a well-recognized association between vitamin-E deficiency and ataxia. This may result from such causes as malabsorption, the result of intestinal and liver disease, or severe malnutrition. There are also a number of hereditary conditions which can cause this deficiency. The classical abnormalities in fat-soluble vitamin-E deficiency progress from hyporeflexia, ataxia, limitation in upward gaze, and strabismus to long-tract defects, muscle weakness, visual field constriction, and centrocecal scotomata.