‘Good food’, I remarked argumentatively ‘should be the occasion and not the topic of good conversation’. I was rounded on by my friends who berated me for such a naïve remark. From antiquity to the present day the experience of good food, the mealtime, has been a major feature of civilization and the cultural importance unimpeachable. Just so. But perhaps my view was biased by having spent time with families with children who have been hard to feed. Preparing the right food for them (good food) and helping the child consume that food over many hours can cause endless distress. When we started our first Feeding Clinic at our Child Development Centre I naïvely imagined that most of the children who came to see us would be there with social and behavioural reasons for the feeding difficulty (and there are such of course). But we soon saw children with all sorts of disabilities and it was distressing to find, as a clinician, that children referred for ‘feeding difficulties’ proved to have a neurological problem and we were telling parents that was the case at the same time as trying to help them with the problems that had brought them to the clinic. It is in these children that alternative feeding methods have to be considered. Already for many of them nasogastric feeding had been a feature of their early lives. Sampson-Fang and her colleagues have reviewed more than a decade of not very robust evidence of the value of gastrostomy feeding in children with cerebral palsy (CP; DMCN 2003, 45: 415–426). But it is important to remember that CP is not the only diagnostic category where we see feeding problems (e.g. cardiac, genetic conditions).

The aim of this prospective cohort study was to evaluate the impact of gastrostomy tube feeding on the quality of life of carers of children with cerebral palsy (CP). Short-Form 36 version II was used to measure quality of life in carers of 57 Caucasian children with CP (28 females, 29 males; median age 4y 4mo, range 5mo to 17y 3mo) six and 12 months after insertion of a gastrostomy tube. Responses were calibrated against a normative dataset (Oxford Healthy Life Survey III). Six months after gastrostomy feeding was started, a substantial rise in mean domain scores for mental health, role limitations due to emotional problems, physical functioning, social functioning, and energy/vitality were observed. At 12 months after gastrostomy placement, carers reported significant improvements in social functioning, mental health, energy/vitality (mean increase >9.8 points; p<0.03), and in general health perception (mean increase 6.35 points; p=0.045) compared with results at baseline. Moreover, the values obtained for these domains at 12 months were not significantly different from the normal reference standard. Carers reported a significant reduction in feeding times, increased ease of drug administration, and reduced concern about their child's nutritional status. This study has demonstrated a significant, measurable improvement in the quality of life of carers after insertion of a gastrostomy feeding tube.

Fourteen participants (six females, eight males) ranging in age from 7 years 11 months to 18 years 2 months (mean 11y 7mo) with a confirmed diagnosis of spastic cerebral palsy (CP) were included in the study. Participants included those who drooled (CP+, n=14); age- and sex-matched children with spastic CP who were dry to mild and never to infrequent droolers (CP–, n=14) as well as typically developing peers (CTRL, n=14) served as controls. Frequency of swallowing was measured by using simultaneous cervical ausculation and videotaping of the head and neck. Saliva production was measured with the Saxon test, a simple gauze-chewing procedure. In addition, Pediatric Evaluation of Disability Inventory (PEDI), Test of Nonverbal Intelligence-3 (TONI-3), dysarthria severity scale, and Gross Motor Function Classification System (GMFCS) scores were obtained for each participant. Both groups of participants with CP tended to swallow less frequently than typically developing participants and tended to produce less saliva than typically developing controls; however, these differences were not statistically significant. No correlation was found between amount of saliva produced and amount drooled (r=0.245). An analysis of variance (ANOVA) conducted on the PEDI functional skills mean scores indicated significant differences between the three groups (F(2,39)=23.522, p<0.0001). Likewise, an ANOVA conducted on the TONI-3 scores revealed statistically significant differences between the three groups (F(2,39)=31.761, p<0.0001). A Spearman's rho correlation indicated that GMFCS scores were not significantly correlated with drooling severity (Spearman's rho correlation=0.3951, p=0.037). Drooling severity was found to be positively correlated with dysarthria severity (Spearman's rho correlation=0.82, p<0.0001). These findings suggest that drooling in patients with CP is related to swallowing difficulties rather than hypersalivation.

Botulinum toxin type A (BTX-A), serial casting, and combined treatment for spastic equinus were compared by chart review. Fifty-five patients with diplegia (n=21), hemiplegia (n=17), quadriplegia (n=6), and other diagnoses (n=11) were reviewed. Thirty-one patients had Gross Motor Function Classification System (GMFCS) scores of I or II, 10 had a GMFCS score of III, and eight had GMFCS scores of IV or V (for six there were no available data). Mean age was 7 years 1 month (SD 4 y 8mo). The combined group showed a significantly greater increase in passive range of motion (ROM) of the ankle joint in comparison with BTX-A alone (p=0.0002), with a mean of 17° (SD 11.2) versus 5° (SD 6.7), and casting alone showed a significantly greater increase in comparison with BTX-A alone (p=0.0001), with a mean of 15° (SD 4.5) versus 5° (SD 6.7). With cast number controlled, change in ROM after casting with and without BTX-A was not significantly different (p=0.37). There was no significant difference between casting with or without BTX-A, and both improved ROM to a greater degree than BTX-A alone. Casting demonstrated a significantly more robust impact on range of motion than BTX-A alone.

To investigate whether the decrease in mean birthweight of the smallest infants surviving with a very low birthweight (VLBW) in the past two decades is associated with an increase in intellectual deficit rates, we compared the results obtained in two different follow-up studies: one included a cohort of children of VLBW (<1500g; n=102, 52% male) born in the early 1980s; the other included a cohort of children of extremely low birthweight (ELBW; <1000g; n=183, 46% male) born in the mid-1990s. Intellectual development was evaluated in both cohorts. McCarthy Scales of Children's Abilities were used in the cohort from the early 1980s; Wechsler's Preschool and Primary Scale of Intelligence – Revised was used in the cohort from the mid-1990s. The children were assessed at age 4–5 years, and a control group was included for each of the two studies. Mean scores in the measures of intelligence found at the assessment were recalculated to standard deviation scores from the two control groups. We found no difference in the standard deviation scores between the two cohorts despite the mean birthweight being 353g (p<0.001) lower in the cohort from the mid-1990s, and the overall survival rate of infants of VLBW having increased. Our results suggest that the improved survival of the smallest babies in the 1980s and 1990s has been achieved without an increase in the intellectual deficit in the surviving children.

Preterm infants are at increased risk for cognitive disorders, including impairments in recognition memory. This study evaluated the effects of extreme prematurity on the neural pathway for auditory recognition memory using event-related potentials (ERPs), a neurophysiological technique widely used in cognitive neuroscience. ERPs were recorded at term postmenstrual age in 35 preterm infants born at less than 32 weeks' gestation (22 males, 13 females; mean birthweight ([BW] 1154g, SD 374g) with normal brain ultrasounds, compared with 40 healthy, term newborns (1 to 3 days of age; 20 males, 20 females; BW 3672g, SD 420g). Because infants must be able to detect and discriminate sounds before recognizing them, two paradigms were used to assess these functions. The first evaluated the detection and discrimination of speech sounds. The second tested recognition of the mother's voice compared with a stranger's. Results showed significantly different patterns of speech sound discrimination in preterm infants compared with term infants. No evidence of maternal voice recognition was elicited from the preterm infants. No specific patterns of auditory detection or discrimination were associated with patterns of recognition memory, suggesting that the function of multiple neural pathways may have been altered in this group of preterm infants. These results provide a functional corroboration of magnetic resonance imaging studies showing effects of prematurity on early brain development, even among preterm infants with normal cranial ultrasonography.

This study examined the relationship between lead exposure and attention regulation in children from low-income families. Children with lead exposure were expected to have lower attention regulation abilities than children who were not exposed. The 57 participants (39% male, 61% female) were children aged 4 and 5 years (mean age 4y 7mo [SD5mo]); 24 children were lead-exposed (10–29µg/dl) and 33 were non-exposed comparisons (1–6µg/dl). Both groups qualified to be enrolled in programs for low-income families. A puzzle-matching task was completed in parent–child and child-alone conditions. Attention allocation and puzzle-matching performance were assessed in the two conditions. Children who were lead-exposed demonstrated less self-regulated attention than comparisons and had poorer puzzle performance in the child-alone condition. Attention patterns did not differ in the parent–child condition. This study extends previous knowledge concerning effects of lead exposure on preschool children from disadvantaged environments and suggests that lead exposure affects specific attention regulation abilities.

Salla disease, a free sialic acid storage disorder, is one of the 36 currently known disorders in Finland that form the Finnish disease heritage. Salla disease leads to learning disability with a wide clinical variation. Two main categories of the disease have been classified: a conventional subtype and a severe subtype with more severe defects. We present detailed neurocognitive profiles of 41 Finnish patients with Salla disease (19 females, 22 males; age range 11mo to 63y, median 19y). The neurocognitive development of patients with Salla disease was assessed by psychological and neuropsychological testing. All patients were also examined by a paediatric neurologist and a speech therapist. The characteristic cognitive profile consisted of a lower non-verbal performance (mean developmental age 13mo) compared with linguistic skills (mean developmental age 17mo). In particular, spatial and visual-constructive impairments were typical of these patients. Tactile and visual discrimination of forms was poor. Tasks demanding hand–eye coordination, maintenance of visual attention, and those requiring short-term visual memory and executive skills were performed better. Receptive language skills were notably better compared with expressive speech. The patients' interactive and non-verbal communication skills were quite strong. Another typical pattern with Salla disease was severe motor disability. After the second decade of life, the decline in these skills was more pronounced than patients' cognitive deterioration. Our results indicate that even though there is a considerable variation in the clinical findings of patients with Salla disease, the characteristic neurocognitive profile of the disease can be outlined.

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.

Cerebral folate deficiency (CFD) can be defined as any neurological syndrome associated with low cerebrospinal fluid (CSF) 5-methyltetrahydrofolate (5MTHF), the active folate metabolite, in the presence of normal folate metabolism outside the nervous system. CFD could result from either disturbed folate transport or from increased folate turnover within the central nervous system (CNS).