The aim of the study was to evaluate the health of children with cerebral palsy (CP) using a global assessment of quality of life, condition-specific measures, and assessments of health care use. A multicenter population-based cross-sectional survey of 235 children, aged 2 to 18 years, with moderate to severe impairment, was carried out using Gross Motor Function Classification System (GMFCS) levels III (n=56), IV (n=55), and V (n=122). This study group scored significantly below the mean on the Child Health Questionnaire (CHQ) for Pain, General Health, Physical Functioning, and Impact on Parents. These children used more medications than children without CP from a national sample. Fifty-nine children used feeding tubes. Children in GMFCS level V who used a feeding tube had the lowest estimate of mental age, required the most health care resources, used the most medications, had the most respiratory problems, and had the lowest Global Health scores. Children with the most severe motor disability who have feeding tubes are an especially frail group who require numerous health-related resources and treatments. Also, there is a relationship among measures of health status such as the CHQ, functional abilities, use of resources, and mental age, but each appears to measure different aspects of health and well-being and should be used in combination to reflect children's overall health status.

The purpose of this study was to examine the effectiveness of the hinged ankle–foot orthosis (HAFO), posterior leaf spring (PLS), and solid ankle–foot orthosis (SAFO), in preventing contracture, improving efficiency of gait, and enhancing performance of functional motor skills in 30 children (21 male, 9 female; mean age 9 years 4 months; age range 4 to 18 years,) with spastic hemiplegia. Following a 3-month baseline period of no ankle–foot orthosis (AFO) use, each AFO was worn for 3 months after which ankle range of motion, gait analysis, energy consumption, and functional motor skills were assessed. The HAFO and PLS increased passive ankle dorsiflexion and normalization of ankle rocker function during gait. Normalization of knee motion in stance was dependent upon the knee abnormality present and AFO configuration. The HAFO was the most effective in controlling knee hyperextension in stance, while PLS was the most effective in promoting knee extension in children with >10° knee flexion in stance. Energy efficiency was improved in 21 of the children, with 13 of these children demonstrating the greatest improvement in HAFO and PLS. Improvements in functional mobility were greatest in the HAFO and PLS.

From 1975 to 1 January 1999, 1103 neurologically involved patients (mean age 13.2 years; 686 males, 417 females) referred with problematic drooling, or sialorrhea, were assessed at a pediatric rehabilitation center by a team consisting of an otolaryngologist, speech pathologist, and a dentist. The initial standard treatment for persistent sialorrhea (in the compliant or aware patient) is oral-motor training. A group of 522 patients with persistent significant drooling after a minimum of 6 months of oral-motor training, or profuse drooling in the presence of a low cognitive level, underwent surgery, usually when over 6 years of age. From 1978 to 1 January 1998, the operation of first choice was submandibular duct relocation (SDR), and was completed in a total of 226 patients. Midway through 1988, sublingual gland excision was also completed at the time of submandibular duct relocation (SDRSGE); 249 of these procedures have been completed to January 1st 1999. Those patients who had SDRSGE had significantly fewer complications that required additional surgery than those that had SDR only. However, the impact of surgery on the drooling as evaluated in subsets of both groups (SDR n=115, SDRSGE n=106) was statistically similar. The study of 11 children with salivary gland radionuclitide scans to determine the effect of submandibular duct surgery on gland function was inconclusive. The pattern of oral-motor function in 26 children studied after SDRSGE surgery suggested that those children with severe impairment of volitional motor function and profuse drooling tended to have a poorer outcome following surgery compared to those with milder impairments.

It has recently been suggested that lycra garments are helpful for children with cerebral palsy (CP). Twelve children, with athetosis, ataxia, and spasticity, were fitted with lycra garments (Kendall-Camp UK Ltd). Scores on the Paediatric Evaluation of Disability Inventory (PEDI) scales were determined before and after wearing the garment for at least 6 hours a day for 6 weeks. Five children with motor problems representative of the whole group were investigated during a reach-and-grasp task by kinematic motion analysis; reflective markers were used with and without the garment. Carers were given a questionnaire concerning the practicalities of using the garments. All 12 children made improvements in at least one of the functional scales of the PEDI, and scores for the whole group showed significant gains (Wilcoxon χ2 test, self-help p<0.01; mobility p<0.5; social p<0.1). These changes were usually slight, although noticed by carers. Six children made gains of at least one scale of the caregiver assistance scores, two of the children showed losses (due to difficulties removing the garment for toileting), and four showed no change. Motion analysis indicated that (1) two children with athetosis had improved proximal stability in sitting and in smoothness of arm movements, (2) one child with ataxia had improved in proximal and distal stability, and (3) two children with spasticity had more jerky movements, although one improved in proximal stability. All children had problems in wearing the garments, including problems with toileting and incontinence of urine; the parents of only one child wanted to continue using it. Results suggest that the functional benefit of lycra garments for children with CP is mainly due to improvements in proximal stability but this should be weighed against the inconvenience and loss of independence.

This study aimed to describe clinical findings, pedigrees, and possible environmental risk factors in children with clinical anophthalmos and remnant microphthalmos in either eye in southern India. Twenty-four children (14 male, 10 female; mean age 10.3 years, age range 1.3 to 18 years,) were recruited from schools for the blind, hospitals, and community-based rehabilitation programmes in Andhra Pradesh, India, over 1 year. Family members were examined, and mothers interviewed. Fifteen children had anophthalmos and nine had remnant microphthalmos in one or both eyes. Twelve children had associated systemic findings, of which six were major and six were minor abnormalities. Information on consanguinity was available in 19 children, 12 of whom had consanguineous parents. Five children had a positive family history. Two mothers had a history of night blindness, and one had a history of pesticide exposure during pregnancy. High rates of consanguinity suggest a genetic recessive aetiology.

This study investigates the presence of sensory modulation dysfunction (SMD) among children with attention-deficit-hyperactivity disorder (ADHD). Twenty-six children with ADHD (mean age 8.3 years, 18 males, 8 females), and 30 typically developing children (mean age 8.2 years, 21 males, 9 females) were tested using a laboratory procedure that gauges responses to repeated sensory stimulation by measuring electrodermal reactivity (EDR). Parental report measures of limitations in sensory, emotional, and attentional dimensions were administered using the Short Sensory Profile, the Leiter International Performance Scale–Revised, Parent Rating subscales, and the Child Behavior Checklist (CBCL). Compared to the typical sample, the children with ADHD displayed greater abnormalities in sensory modulation on both physiological and parent-report measures. The children with ADHD also displayed more variability in responses. Within the group with ADHD, levels of SMD were highly correlated with measures of psychopathology on the CBCL. Implications of findings relate to the importance of considering sensory processing abilities in a subgroup of children with ADHD.

Nine children (five males, four females; age range 6 years 1 month to 11 years 1 month) affected by benign epilepsy of childhood with centrotemporal or Rolandic spikes (BECRS) with EEG evidence of marked activation of interictal epileptic discharges (IEDs) during sleep, and nine unaffected control children matched for age, sex, and socioeconomic status, were enrolled in a prospective study. At the time of detection of IED activation during sleep, patients showed a mean Full-Scale IQ score within the normal range, but significantly below that of control participants; neuropsychological assessment revealed disorders in visuospatial short-term memory (Corsi's Block Tapping Test), attention, and cognitive flexibility (Trail Making Test and Stroop Color-Word Test), picture naming, and fluency (Benton's Naming Test and Word Fluency), visuoperceptual skill (Ghent-Poppelreuter and Street Gestalt Completion Tests) and visuomotor coordination (Bender Test). After detection of IED activation during sleep, children were followed up for 2 years. At the time of IED remission (T1), neuropsychological re-evaluation showed a notable increase in IQ score and a significant improvement (t-test: p<0.007) in visuomotor coordination, non-verbal short-term memory, sustained attention and mental flexibility, picture naming, and visual–perceptual performance. At T1, patients' performance did not differ from the controls (Mann–Whitney U test).

We report on a male infant with pyridoxine dependency and seizures from birth, controlled with pharmacological doses of pyridoxine at 4 months of age. Seizures stopped between 30 and 80 days of age when very-low doses of pyridoxine were given in a multivitamin supplement . Daily dose was 0.5mg that corresponded to 0.08 to 0.16 mg/kg/day when weight gain is considered. In previous reports doses have ranged from 0.2 to 30 mg/kg/day. Another distinctive feature was that this infant went into a coma and developed hypotonia and irregular breathing when pyridoxine was given by enteral tube which has usually been reported when the vitamin is given intravenously. Use of low doses of pyridoxine in multivitamin supplements could be a confounding factor for early diagnosis and appropriate treatment of pyridoxine-dependent seizures.

Pyridoxine dependency was one of the first genetically mediated seizure disorders to be recognized. However, the biochemical abnormality and gene locus are still unknown. Knowledge about the condition has come from case reports and small clinical series. However, new features are still being recognized both clinically, as well as more predictably, from the application of new imaging techniques. Early onset seizures responsive to pyridoxine exist which do not appear to be the same disorder. In some parts of the world, high-dose pyridoxine is used in the treatment of other major seizure disorders of young children such as West syndrome.

Congenital facial weakness involves various clinical manifestations, each of which raises specific problems. The incidence of congenital facial palsies ranges from 1.8 to 7.5‰ of births. Comparison of clinical and neurophysiological data enables aetiologies to be differentiated, pathogenesis to be evaluated, and the prognosis to be assessed.