bluuebunny/crossref_metadata_2025 huggingface dataset:
Outcome at 1 year of neonatal encephalopathy in Kathmandu, Nepal
Authors: Ellis Matthew , Manandhar Nilu , Shrestha Prakash S , Shrestha Laxman , Manandhar Dharma S , De L Costello Anthony M
Published: July 2010

To determine the outcome at 1 year of neonatal encephalopathy (NE) and to estimate the possible contribution of birth asphyxia to childhood disability in a low‐income South Asian country, a prospective cohort study was undertaken in the principal maternity hospital of Kathmandu, where over 50% of local women give birth. From a total population cohort of 21 609 live births, 131 term infants with NE (after exclusion of cases associated with neonatal sepsis, congenital malformations, or primary hypoglycaemia) and 208 term control infants were recruited. Of these, 102 (78%) infants with NE and 106 (51%) control infants were followed‐up to 1 year of age. Outcome measures were death or neurodevelopmental impairment, graded as major, minor or none. Of the 131 term infants with NE, 83 were graded with moderate or severe NE according to conventional definition. By 1 year of age, 45 (44%) of the infants with NE had died, 18 (18%) had severe impairments, and two (2%) had minor impairments; four (4%) of the control subjects had died and two (2%) had minor impairments. Most deaths in subjects with NE occurred in the early neonatal period; NE carried no excess risk of death beyond the neonatal period. Of the 18 children with major impairment, 14 (78%) had spastic tetraplegic cerebral palsy and eight (44%) had multiple impairments. Compared with the control group the relative risk of death by 1 year was 5 (95% CI 1.4 to 15) for mild NE, 8 (95% CI 3 to 23) for moderate, and 26 (95% CI 10 to 67) for severe. Twenty‐seven of 38 (71%) infants with moderate NE either died or survived with major impairment. An upper estimate for the prevalence of major neuroimpairment at 1 year attributable to birth asphyxia is 1 per 1000 live births in this population.
Bone age and linear skeletal growth of children with cerebral palsy
Authors: Kong C K , Tse P W T , Lee W Y
Published: July 2010

The aim of this study was to compare the linear growth of children with cerebral palsy (CP) with that of children without CP. The segmental lengths (humerus, ulna, femur, tibia, and spine), recumbent length, body weight, and bone age of 62 children with CP (age range 2.25 to 14 years, mean 7.13 years) were measured and compared with 68 children without CP (age range 1.50 to 12.67 years, mean 6.73 years). The results show that bone‐age delay is common in children with CP (68% with a delay of more than 1 year). The linear growth of children with CP is similar to that of children without CP when bone age is used instead of chronological age. There is a strong correlation between segmental lengths and body height. Hence, this study favours using segmental lengths for estimating height in children with CP and using the comparison group as a reference for this purpose. Nutritional and non‐nutritional factors on bone‐age delay are discussed.
Sequential neuromotor examination in children with intrauterine cocaine/ polydrug exposure
Authors: Belcher Harolyn M E , Shapiro Bruce K , Leppert Mary , Butz Arlene M , Sellers Sherry , Arch Ellen , Kolodner Ken , Pulsifer Margaret , Lears Mary Kathleen , Kaufmann Walter E
Published: July 2010

Data are presented on 157 newborn infants followed sequentially in a randomized home‐based nursing‐intervention trial for drug‐exposed infants with follow up at 3 (N=118), 6 (N=124), and 12 months (N=77). The objectives of this study were to describe the longitudinal neurodevelopmental status of a cohort of children with intrauterine exposure to illicit drugs during their gestation, characterize the evolution of early tone abnormalities in a polydrug‐exposed cohort, and determine whether neuromotor outcome is associated with drug‐exposure patterns. For analysis, infants were grouped based on maternal drug‐use pattern and the presence of drug metabolites in the neonatal drug screen. The sequential neuromotor examination was used at each age to define the neuromotor status of six domains and define categorical classifications as either normal, suspect, or abnormal. Multiple patterns of neuromotor abnormalities were observed during the neonatal period; most resolved over time. Axial hypotonia was a prominent finding in the neonatal period; however, it was infrequent in abnormal examinations at 12 months. Increased lower‐extremity tone was a less frequent finding during the neonatal period. Infants whose neonatal urine drug screen was positive for both cocaine and opiates, were more likely than infants with negative urine drug screens, cocaine only, or opiate only drug screen results to have abnormal neuromotor examinations; while positive maternal drug screens for concurrent cocaine and opiate use were associated with peripheral hypertonia. Persistence of increased leg‐extensor tone was found in 67% of the abnormal examinations at 12 months. Acquisition of rolling and walking was delayed in the drug‐exposed cohort.
Melatonin treatment of non‐epileptic myoclonus in children
Authors: Jan James E , Connolly Mary B C , Hamilton Don , Freeman Roger D , Laudon Moshe
Published: July 2010

Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non‐epileptic, and one had epileptic and non‐epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.
Cognitive functioning in children with typical cri du chat (5p–) syndrome
Authors: Cornish K M , Bramble D , Munir F , Pigram J
Published: July 2010

This study is the first attempt to assess systematically the cognitive functioning in children diagnosed with typical cri du chat syndrome (CDCS) using neuropsychological test measures. Twenty‐six children aged between 6 years 4 months and 15 years 5 months (mean 8 years 3 months) completed a battery of tasks measuring IQ level, receptive and expressive language skills, and articulation. Twenty‐four children were in the severe learning‐disability range with no specific verbal or performance profile. Using more finely tuned measures of cognition, however, a clear discrepancy in the pattern of language functioning was found with better receptive than expressive language skills. One implication of these findings is that parents and professionals should be more optimistic about the capacities of children with CDCS to understand more complex verbal commands than their expressive language skills would suggest.
Head circumference in ELBW babies is associated with learning difficulties and cognition but not ADHD in the school‐aged child
Authors: Stathis Stephen L , O'Callaghan Michael , Harvey Jacqueline , Rogers Yvonne
Published: July 2010

This study examines whether a small head circumference (HC) and low head‐circumference growth velocity (HGV) during the first year of life predict consequences at school age in learning, cognition, and concentration. A total of 124 extremely‐low‐birthweight (ELBW) infants (birthweight 500 to 999 g) born between 1977 and 1986 were eligible for follow‐up at the corrected ages of 4, 8, and 12 months and 2, 4, and 6 years. Infants were categorized as having a small HC (<3% or 3 to 10%) on the basis of the 1990 British growth data which allowed standardized z ‐scores to be calculated for HC, independent of gestation and corrected age. HGV measurements were calculated using differences in the HC z ‐scores. In 1995, parents of 87 children agreed to participate in a study of learning and attention at school age. Attention‐deficit–hyperactivity disorder (ADHD) was assessed using the Du Paul Rating Scale. Academic performances were based on a teacher questionnaire dealing with aspects of reading, writing, mathematics, and spelling. A child was considered to have a learning difficulty if academic problems were present in at least one of these four areas. Intellectual ability was assessed using the McCarthy Scale at 6 years. HC <3% and 3 to 10% at 8 months’corrected age was strongly associated with school‐aged learning problems (P=0.004), with a moderate specificity (70%), positive predictive value (PPV) (67%), and sensitivity (67%). HGV ?10% from birth to 4 months was also associated with learning problems at school age (P=0.01) with a higher specificity (98%) and PPV (88%) but lower sensitivity (20%). A logistic regression analysis was performed with the risk for
Premature sexual development in individuals with neurodevelopmental disabilities
Authors: Siddiqi Siraj U , Van Dyke Don C , Donohoue Patricia , McBrien Dianne M
Published: July 2010

Studies on precocious puberty have primarily focused on children with typical patterns of growth and cognitive development. This study reviewed diagnostic data from the records of 15 719 patients with neurodevelopmental disabilities for diagnoses associated with premature sexual development/precocious puberty. Thirty‐two individuals with premature sexual development were identified, with the earliest changes seen in one girl at 1 year 7 months of age. In this group, the mean age at onset was 7 years 2 months in boys and 5 years 11 months in girls. Central precocious puberty, which was the most common cause of onset of early pubertal changes, was present in 15 of the 32 children. The results of this study suggest that children with a neurodevelopmental disability are at increased risk of premature pubertal changes when compared to children without a neurodevelopmental disability. This study indicates the need for health‐care providers to be vigilant in screening for early pubertal changes in children with neurodevelopmental disabilities.
Age‐related walking in children with spina bifida
Authors: Williams Elizabeth N , Broughton Nigel S , Menelaus Malcolm B
Published: July 2010

The aim of this study was to examine children with myelomeningocele (spina bifida) who were investigated according to the level of neurosegmental lesion and to determine the number who walked and their average age at commencement/cessation of walking. The data have been collected since 1978 at the Royal Children's Hospital, Melbourne during annual evaluations of patients with spina bifida and high‐level paralysis. After screening for established criteria and assigning to a neurosegmental lesion level, 173 children were examined for their ability to walk, with or without aids, in a community setting. Thirty‐five children had lesions at the thoracic level, and seven walked at a mean age of 4 years 6 months. Three children with a lesion at the thoracic level ceased walking at a mean age of 7 years 6 months. Ten children had lesions at the high‐lumbar level (L1/2), and five walked at a mean age of 5 years 2 months. Three children who had lesions at the high‐lumbar level ceased walking at a mean age of 6 years 11 months. Fifteen children had lesions at the mid‐lumbar level (L3) and nine walked at a mean age of 5 years. Three ceased walking at a mean age of 7 years. Forty‐five children had lesions at the low‐lumbar level (L4/5) and 38 walked at a mean age of 3 years 10 months. Five ceased walking at a mean age of 9 years 1 month. Sixty‐eight children had lesions at the sacral level; all of whom walked at an average age of 2 years 2 months. None had ceased walking by the end of the study period. Delay in achieving ambulation can be expected in all children with spina bifida, including those with low neurosegmental level lesions. Furthermore, some children with high lesion level, given the opportunity, might be expected to ambulate effectively in the community but may cease walking after 3 to 4 years of this activity, which is earlier than previously recorded.
Sturge–Weber syndrome: cerebral haemodynamics during seizure activity
Authors: Aylett S E , Neville B G R , Cross J H , Boyd S , Chong W K , Kirkham F J
Published: July 2010

The aim of this study was to examine the haemodynamic response to seizures in three infants with Sturge–Weber syndrome by measuring regional cerebral blood flow using transcranial Doppler sonography and 99mTc HMPAO SPECT. Time‐locked video/digital EEG recording was carried out for ictal studies. MRI was performed in all subjects. SPECT showed hemispheric hypoperfusion interictally in all three patients and also ictally in one of the three; a small region of hyperperfusion was seen on the same ictal scan in the latter, ie. the patient with interictal and ictal hypoperfusion. In the two older children middle cerebral artery velocity (MCAV) was reduced by between 29 and 62% in the middle cerebral artery of the predominantly affected hemisphere compared with the contralateral side. During seizures, increases of 6 to 30% in MCAV were recorded for the clinically seizing hemisphere compared with 24 to 170% for the contralateral side in four of the seizures recorded. In one infant, MCAV fell bilaterally during a seizure that generalized (–18 and –43% in the predominantly affected and contralateral side respectively). Sequential recordings in one infant suggested that, with time, the haemodynamic response to seizures of the unaffected hemisphere may decrease. These findings suggest that the venous malformation in SWS is associated with an impairment of the cerebral haemodynamic response to seizure activity.
Causes of excess mortality in cerebral palsy
Authors: Strauss David , Cable William , Shavelle Robert
Published: July 2010

It is well known that individuals with cerebral palsy (CP) are subject to higher mortality than the general population but the causes of this have not been systematically analysed. This study investigates mortality in a Californian population of 45 292 individuals with CP, 4028 of whom died during the 1986 to 1995 study period. The aims of this study were to identify diseases that may be causally linked with CP, and diseases whose diagnosis and/or treatment need improvement. Overall, the standardized mortality ratio (SMR) was 8.4. Mortality from breast cancer was three times that of the general population, suggesting poorer detection and/or treatment. The dramatic elevation of mortality due to brain cancer, especially in children (SMR=24), raises the possibility of a link between this and CP. Cause of death was non‐specific in some individuals. Therefore, these SMRs are conservative. As expected, SMRs due to respiratory diseases were very high but, contrary to anecdotal reports, such diseases did not account for most deaths. High SMRs were also found for diseases of the circulatory and digestive systems. Finally, a marked elevation of deaths was due to external causes, including drowning and being hit by motor vehicles.
Quantitative assessment of mirror movements in children and adolescents with hemiplegic cerebral palsy
Authors: Kuhtz‐Buschbeck Johann P , Sundholm Lena Krumlinde , Eliasson Ann‐Christin , Forssberg Hans
Published: July 2010

Mirror movements in individuals with hemiplegic cerebral palsy (CP) may result from a reorganization of the central sensorimotor system. Motor performances of both hands were measured to characterize mirror activity (or mirroring) and hand functions in 22 participants (6 to 18 years) with hemiplegic CP and in 17 control participants. During a unimanual repetitive squeezing task, contractions of the active hand and fingertip forces of the opposite hand were recorded simultaneously. In the control group, slight mirror activity (or mirroring) was found that decreased with age. In participants with CP, mirror activity was 15 times stronger than in the control group, and was found at all age levels. Mirroring was more prominent in the unaffected hand of the CP group. The amount of mirror activity was not related to the degree of hemiplegia, which was assessed with measures of spasticity, strength, and dexterity. Mirror movements disturbed functional bimanual skills, although to some extent they could be suppressed by voluntary effort.
Serum NGF levels in children and adolescents with either Williams syndrome or Down syndrome
Authors: Calamandrei Gemma , Alleva Enrico , Cirulli Francesca , Queyras Armelle , Volterra Virginia , Capirci Olga , Vicari Stefano , Giannotti Aldo , Turrini Paolo , Aloe Luigi
Published: July 2010

The neurotrophin nerve growth factor (NGF) is a major regulator of peripheral and central nervous system development. Serum NGF was measured in normally developing control children (n=26) and in individuals affected by congenital syndromes associated with learning disability: either Williams syndrome (WS; n=12) or Down syndrome (DS; n=21). Participants were assessed at three distinct developmental stages: early childhood (2 to 6 years), childhood (8 to 12 years), and adolescence (14 to 20 years). A sample was taken only once from each individual. Serum NGF levels were markedly higher in participants with WS, than DS and control participants. In addition, different developmental profiles emerged in the three groups: while in normally developing individuals NGF levels were higher in early childhood than later on, children with WS showed constantly elevated NGF levels. When compared to control participants, those with DS showed lower NGF levels only during early childhood. Neuropsychological assessment confirmed previously reported differences among the three groups in the development of linguistic/cognitive abilities. Some features of individuals with WS, such as hyperacusis and hypertension, could be related to high‐circulating NGF levels.
Kinaesthetic acuity in adolescent boys: a longitudinal study
Authors: Visser J , Geuze R H
Published: July 2010

The Kinaesthetic Sensitivity Test (KST) was used to measure the development of kinaesthetic acuity in adolescent boys. Thirty boys were tested longitudinally, at intervals of 6 months, between the ages of 111/2and 14 years. A second group of 20 boys was tested at the ages of 14 and 161/2years. The findings were compared with existing normative data on 5‐ to 12‐year‐old children and young adults, and they indicated improvement in kinaesthetic acuity with age. Although the age effect is statistically significant only in the older group, confidence intervals show that the rate of improvement in both groups is comparable to improvement between the ages of 5 and 12 years. The reliability of the test is rather poor. The conclusion is that kinaesthetic development continues throughout adolescence. Further, development is quite robust and detectable even with a fairly unreliable measurement instrument. However, individual assessments should be interpreted with caution.
The feasibility of universal screening for primary speech and language delay: findings from a systematic review of the literature
Authors: Law James , Boyle James , Harris Frances , Harkness Avril , Nye Chad
Published: July 2010

This paper reports on a systematic review of the literature commissioned to examine the feasibility of universal screening for speech and language delay. The results, based on an examination of productivity figures, including positive predictive ability and likelihood ratio, indicate that a number of screening tests are adequate. Sensitivity was generally lower than specificity, and study quality was inversely related to both sensitivity and likelihood ratio, suggesting that it is easier to identify accurately children who do not have language and speech problems than those who do. The review concluded that there is insufficient evidence to warrant the introduction of universal screening at this stage. This paper discusses the type of data that would be needed to address this issue further and recommendations are made for alternative approaches to early identification.
Comparison of the Gross Motor Function Measure and Paediatric Evaluation of Disability Inventory in assessing motor function in children undergoing selective dorsal rhizotomy
Authors: Nordmark Eva , Jarnlo Gun‐Britt , Hägglund Gunnar
Published: July 2010

This study was designed to compare assessment with the functional outcome measures Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) over time, in children with cerebral palsy (CP) undergoing selective dorsal rhizotomy combined with individualised physiotherapeutic interventions. Using the Gross Motor Function Classification System (GMFCS), 18 children with spastic diplegia were divided into two groups according to age‐related severity of motor function impairment. Data were collected preoperatively, and at 6 and 12 months postoperatively. Both instruments were sensitive to changes in function over time in the series as a whole and in the group with milder impairment, although the PEDI detected significant changes earlier. In the group with more severe impairment, changes in function were detected only with the PEDI, not with the GMFM. Thus, the instruments are to be considered complementary tests, because they measure different aspects of function.
Behavioural treatment of urinary incontinence and encopresis in children with learning disabilities: transfer of stimulus control
Authors: Smith Linda , Smith Paul , Yi Lee Susan Kwok
Published: July 2010

Urinary and faecal incontinence present a considerable problem in people with learning disabilities, despite the general effectiveness of behavioural techniques in continence training. Children with learning disabilities and obsessional behaviour may be particularly resistant to toilet training, even where relatively cognitively able, and often despite a substantial degree of control over their eliminatory functions. Their resistance may be more appropriately regarded as a challenging behaviour and their incontinence better explained by factors other than a simple failure to learn. A 'stimulus‐control’hypothesis proposes that the child's nappy (diaper) /potty/underwear has developed strong stimulus control over the elimination response. This report describes three case studies in which treatment‐resistant children, aged between 8 and 12 years, with mild or moderate learning disabilities, were successfully treated for nappy‐dependent nocturnal encopresis or diurnal urinary incontinence. The children were routine case referrals for whom previous attempts to train bowel or bladder control had failed. Behavioural techniques, such as 'shaping’(gradually increasing the proximity to the toilet),‘fading’(reducing the presence of the nappy), and rewards for eliminating, effected successful transfer of stimulus control over elimination from nappy to toilet. Treatment times varied, depending on the degree of the child's obsession and resistance to change.
The Gross Motor Function Classification System for Cerebral Palsy: a study of reliability and stability over time
Authors: Wood Ellen , Rosenbaum Peter
Published: July 2010

Children with cerebral palsy (CP) experience a change in motor function with age and development. It is important to consider this expected change in offering a prognosis, or in assessing differences in motor function after an intervention. The Gross Motor Function Classification System for CP (GMFCS) has been developed for these purposes. This study was based on a retrospective chart review of 85 children with CP followed from ≤2 to ≥12 years of age. The GMFCS was applied to clinical notes by two blinded raters four times throughout the study. Interrater reliability was high (G=0.93). Test‐retest reliability was high (G=0.79). The positive predictive value of the GMFCS at 1 to 2 years of age to predict walking by age 12 years was 0.74. The negative predictive value was 0.90. The GMFCS can validly predict motor function for children with CP. The results are discussed in terms of their implications for clinical practice and future research.
Epileptic syndromes, cognitive assessment and school placement: a study of 251 children
Authors: Bulteau C , Jambaque I , Viguier D , Kieffer V , Dellatolas G , Dulac O
Published: July 2010

Two‐hundred and fifty‐one children (98 girls and 153 boys, aged from 3 to 17 years) with documented diagnosis of epileptic syndrome, IQ measurement, and information on school placement were included in this retrospective study. The relations between these three parameters as well as effects of age at onset and duration of epilepsy, seizure frequency, and number of antiepileptic drugs (AEDs) were analysed. Both IQ and schooling were univariately related to epileptic syndrome, age at onset and duration of epilepsy, and number of AEDs; seizure frequency was related to IQ but not to school placement. Multiple regression showed that IQ was independently related to epileptic syndrome and AED; multiple logistic regression showed that type of school (mainstream versus adapted or special) was independently related to IQ and AED. Children with idiopathic generalised or with localisation‐related epilepsy had higher IQ scores and higher probability of mainstream schooling than those with symptomatic or cryptogenic generalised epilepsies or epileptic syndromes which were undetermined. Subtests profile of intelligence scale in localisation‐related epilepsies showed different specific cognitive deficits, according to the location of the epileptic focus.
Congenital skull fracture as a presentation of Menkes disease
Authors: Ubhi Tim , Reece Ashley , Craig Adam
Published: July 2010

We report the rare presentation of Menkes disease with a congenital skull fracture, intracerebral bleeding, and seizures. The diagnosis was made at 3 months of age based on the characteristic features of the syndrome, by which time the child experienced uncontrollable seizures. Following progressive neurodegeneration, death occurred at 3 years of age. The prognosis in Menkes disease is dependent on early copper‐histidine therapy. Effective treatment has led to children surviving into adulthood. Diagnosing the syndrome during the neonatal period is difficult. There are no published reports of congenital skull fracture as a presenting sign of Menkes disease. It is concluded that Menkes disease should be considered in any child who presents with congenital skull fracture as early diagnosis and treatment significantly improve the outcome.
Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy
Authors: Terjesen Terje , Lange Johan E , Steen Harald
Published: July 2010

To evaluate the clinical results of the treatment and to assess the factors that influenced the rate of scoliosis progression, a retrospective study of spinal orthosis in 86 patients with spastic quadriplegic cerebral palsy was performed. The mean age of the patients was 13.8 years (range 5 to 33 years). Their scoliotic deformities were treated with custom‐moulded, polypropylene thoraco‐lumbar‐sacral orthoses. Cobb angles were measured on radiographs taken in a sitting position before treatment, in orthosis, and during follow‐up. The mean initial Cobb angle was 68.4° (range 25 to 131°). The mean correction in orthosis was 25° (range 3 to 60°). Seventy‐two patients had a follow‐up period of more than 2 years. At the latest follow‐up, average 6.3 years (range 2 to 14 years) after the start of treatment, the mean Cobb angle without orthosis was 93.1° (range 40 to 145°). The mean progression per year was 4.2° (range ‐3 to 21°). Linear multiple regression revealed that age and initial correction in orthosis were the only variables that significantly influenced the rate of progression. Twenty‐two patients had no progression or progression <1.0° per year. Correction in orthosis was the only variable that predicted progression <1.0° per year in both age groups (<15 years and ≤15 years). Of the 57 patients who were still alive and had not undergone surgical fusion, 72% used their orthoses at a mean age of 22 years. Parents and caregivers expressed satisfaction with the use of orthosis, mainly because of improved sitting stability which gave better overall function.
Acquired aphasia in children after surgical resection of left‐thalamic tumors
Authors: Nass Ruth , Boyce Leslie , Leventhal Fern , Levine Beth , Allen Jeffrey , Maxfield Carol , Salsberg David , Sarno Martha , George Ajax
Published: July 2010

Five children (three males, two females; four right‐, one left‐handed; age range 6 to 14 years) who developed aphasia after gross‐total excision of left predominantly thalamic tumors are reported. Three patients had Broca aphasia, one had mixed transcortical aphasia, and one patient had conduction aphasia. In the months after surgery, three children improved while receiving radiation and/or chemotherapy, although none recovered completely. Two patients with malignant tumors developed worsening aphasia when the tumor recurred, and later died. Two of three patients tested had visuospatial difficulties in addition to language deficits. Attention and executive functioning were affected in three of three patients tested. Memory, verbal and/or visual functioning, were affected in four of four patients tested. Both patients who were tested showed transient right hemineglect. Two of two patients tested were probably apraxic. The wide range of deficits in these children highlights the importance of the thalamus and other subcortical structures in developing cognition.
Preliminary validation of an observational pain checklist for persons with cognitive impairments and inability to communicate verbally
Authors: Breau Lynn M , McGrath Patrick J , Camfield Carol , Rosmus Christina , Finley G Allen
Published: July 2010

To obtain a preliminary validation of the Non‐Communicating Children's Pain Checklist for individuals with an inability to communicate verbally, 32 caregivers of individuals with cognitive impairments aged 3 to 44 years retrospectively completed the Non‐Communicating Children's Pain Checklist and rated item usefulness (0 to 10). In the second part of the study 33 caregivers completed the Checklist after two painful (e.g. burns, falls, surgery), one distressful (e.g. unwanted grooming, feared animal/noise), and one calm event (e.g. watching television). Checklist scores did not correlate with the age, sex, or physical limitations of individuals with an inability to communicate verbally. In the first part of the study Cronbach's alpha was 0.66; all mean usefulness ratings exceeded 5 out of 10. In the second part of the study after four items were removed, Cronbach's alpha was 0.79. Checklist scores during pain correlated with numerical ratings of pain intensity did not differ between the two pain events, and differed significantly from calm scores. Differences in Checklist scores during pain and distress were found for two subscales. The Checklist exhibits internal consistency, and preliminary evidence suggests it can detect pain and is reliable over time.
Prevalence and severity of feeding and nutritional problems in children with neurological impairment: Oxford Feeding Study
Authors: Sullivan PB , Lambert B , Rose M , Ford‐Adams M , Johnson A , Griffiths P
Published: July 2010

The aim of this study was to estimate the prevalence and severity of feeding and nutritional problems in children with neurological impairment within a defined geographical area. In a cross‐sectional study, a validated questionnaire was sent to 377 parents of children (aged 4 to 13 years) on the Oxford Register of Early Childhood Impairments with oromotor dysfunction. The return rate was 72%. Of these, 93% had cerebral palsy; 47% were unable to walk; 78% had speech difficulty; and 28% continuous drooling of saliva. Gastrointestinal problems were commonly encountered: 59% were constipated; 22% had significant problems with vomiting, and 31% had suffered at least one chest infection in the previous 6 months. Feeding problems were prevalent: 89% needed help with feeding and 56% choked with food; 20% of parents described feeding as stressful and unenjoyable. Prolonged feeding times (3h/day) were reported by 28%. Only 8% of participants received caloric supplements and 8% were fed via gastrostomy tube. Even though 38% of respondents considered their child to be underweight, 64% of children had never had their feeding and nutrition assessed. The results highlight that feeding problems in children with neurological impairment are common and severe, causing parental concern. Many of these children would benefit from nutritional assessment and management as part of their overall care.
Semen retrieval and analysis in men with myelomeningocele
Authors: Hultling C , Levi R , Amark P , Sjöblom P
Published: July 2010

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.
Effectiveness of lamotrigine in children with paroxysmal kinesigenic choreoathetosis
Authors: Überall Michael A , Wenzel Dieter
Published: July 2010

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare movement disorder, characterized by recurrent, brief involuntary dystonic attacks that are provoked by sudden movements. Pathophysiology is uncertain, but a channelopathy is discussed. Treatment recommendations favour antiepileptic drugs (AEDs) acting on voltage‐gated neuronal ion channels. This report summarizes the history of three children (6, 8, and 10 years of age) with idiopathic PKC successfully treated with low doses of lamotrigine, an AED acting primarily via neuronal voltage‐sensitive sodium channels.
Cerebral arteriovenous malformation presenting as visual deterioration in a child
Authors: Kaye Lesley C , Kaye Stephen B , Lagnado Ruby , Boothroyd Anne , Morton Clare , May Paul
Published: July 2010

A rare case of visual loss as the presenting feature of a central arteriovenous malformation involving the vein of Galen is reported. A 5‐year‐old girl with a history of deteriorating vision for the past 6 months was examined. Ocular examination showed a left hemianopia, left optic atrophy, and dilated vessels of the right optic disc. MRI revealed a massive deep‐seated central arteriovenous malformation involving the vein of Galen. The mechanism of visual loss is likely to be a combination of ischaemic optic atrophy associated with a steal phenomenon and direct compression of the right optic radiation.
Effect of secretin on children with autism: a randomized controlled trial
Authors: Dunn‐Geier Jennifer , Ho Helena H , MD Edward Auersperg , MD David Doyle , PhD Linda Eaves , Orrbine Elaine , Whiting Sharon
Published: July 2010

To determine the effect of intravenous porcine secretin on autistic behaviours in children aged 2 to 7 years, the effects of secretin on (1) performance on a standardized language measure, and (2) autistic behaviours, as rated by parents and child development professionals was examined. Employing a randomized, double‐blind, placebo‐controlled design, 95 participants were assigned to one of two groups and administered a single dose of either secretin or placebo. A follow‐up assessment was conducted 3 weeks after the injection. No significant differences in language or autistic behaviour measures were observed at the 3‐week follow‐up between the groups. Also, there was no significant difference in the proportion of individuals who improved by 6 points on the language measure at follow‐up. This study showed no significant effects of secretin on children with autism. Our results are consistent with a systematic review of randomized controlled trials evaluating the effect of secretin in children with autism.
Somatosensory evoked potentials as a means of assessing neurological abnormality in congenital talipes equinovarus
Authors: Nadeem Rana Dilawaiz , Brown J Keith , Lawson Gillian , Macnicol Malcolm F
Published: July 2010

Somatosensory evoked potentials (SSEPs) are a very sensitive measure of the functional integrity of the neuroaxis, including peripheral and central structures. When used in diagnostic mode they can provide additional information regarding the probable areas of dysfunction. SSEPs were recorded from 44 children (64 feet with congenital talipes equinovarus, CTEV), between the ages of 2 to 15 years, who had structural CTEV deformity previously treated by surgery, with no clinical evidence of neurological deficit. SSEPs were elicited after sequential and bilateral stimulation (0.1 ms/5 Hz/10 to 20 mA) of the posterior tibial nerve and the common peroneal nerve and were recorded cortically (P40). In half the children, additional recordings were conducted at the knee (N5), the first lumbar spinous process (N14), and the seventh cervical spinous process (N20). Eighteen children had abnormal responses, four children had non‐reproducible responses, and 22 children had normal responses. Analysis of the data at different levels of the nervous system showed that eight children had abnormality at the spinal level. The surgical outcome was influenced by the neurological abnormality, with an excellent or good outcome in 34 of 36 feet with normal neurology and 19 of 28 feet where a deficit was present (p<0.05). These findings support the neurological theory as an etiological factor in CTEV deformity.
Detrimental neural control of precision grip lifts in children with ADHD
Authors: Pereira Heloisa S , Eliasson Ann‐Christin , Forssberg Hans
Published: July 2010

The aim was to investigate the performance of children with attention‐deficit‐hyperactivity disorder (ADHD) in tasks involving motor‐memory representations. A special grip object recorded forces generated by the fingertips during a precision grip‐lift task. Common objects were lifted from a linear scale. Twenty‐five boys with ADHD were evaluated and grouped according to the presence (ADHD+) or absence (ADHD) of movement dysfunction using the Movement Assessment Battery for Children (Henderson and Sugden 1992). Mean group ages were 11.4 years(range 9.0 to 11.0 years) and 11.7 years (9.0 to 15.6 years), respectively. They were compared to a control group of 25 age‐matched boys, mean group age 11.8 years(range 9.0 to 13.0 years). Variability of motor performance was predominant in the ADHD+ group. Several of these participants presented a higher grip‐force output during the gripping movement. They also had difficulties in adapting the motor output to target different weights, suggesting deficient anticipatory parameter control based on memory representations. The results suggest that in some children motor problems are due to detrimental neural control functions rather than core symptoms of ADHD.
Intrathecal baclofen withdrawal simulating neuroleptic malignant syndrome in a child with cerebral palsy
Authors: Samson‐Fang Lisa , Gooch Judith , Norlin Chuck
Published: July 2010

Intrathecal baclofen infusion (IBI) is being used with increasing frequency in children to treat spasticity and dystonia. In this report, we summarize the clinical course of a 9–year‐old boy with quadriplegic cerebral palsy with mixed tonal abnormalities (spasticity and dystonia) experiencing withdrawal from intrathecal baclofen. His clinical course is compared to that of adults experiencing withdrawal from IBI and to neuroleptic malignant syndrome. If unrecognized, this disorder may have significant potential for morbidity and mortality. Clues to diagnosis, appropriate evaluation, and potential treatments are discussed. When a child treated with IBI presents with unexplained multiorgan system dysfunction, particularly if accompanied by evidence of rhabdomyolysis, the integrity of the IBI system must be evaluated. In some cases, evaluation might necessitate surgical exploration. Caregivers most commonly seek urgent evaluation and treatment from their primary care provider when their child experiences fever or acute illness. Primary care providers of children treated with IBI should be made aware of this clinical scenario to prevent delays in diagnosis.
Reliability of the Melbourne Assessment of Unilateral Upper Limb Function
Authors: Randall Melinda , Carlin John B , Chondros Patty , Reddihough Dinah
Published: July 2010

This study examines the reliability of the Melbourne Assessment of Unilateral Upper Limb Function: a quantitative test of quality of movement in children with neurological impairment. The assessment was administered to 20 children aged from 5 to 16 years (mean age 9 years 10 months, SD 2 years 10 months) who had various types and degrees of cerebral palsy (CP). The performances of the 20 children during assessment were videotaped for subsequent scoring by 15 occupational therapists. Scores were analyzed for internal consistency of test items, inter‐ and intrarater reliability of scorings of the same videotapes, and test‐retest reliability using repeat videotaping. Results revealed very high internal consistency of test items (α=0.96), moderate to high agreement both within and between raters for all test items (intraclass correlations of at least 0.7) apart from item 16 (hand to mouth and down), and high interrater reliability (0.95) and intrarater reliability (0.97) for total test scores. Test‐retest results revealed moderate to high intrarater reliability for item totals (mean of 0.83 and 0.79) for each rater and high reliability for test totals (0.98 and 0.97). These findings indicate that the Melbourne Assessment of Unilateral Upper Limb Function is a reliable tool for measuring the quality of unilateral upper‐limb movement in children with CP.
Powered wheelchairs and independence in young children with tetraplegia
Authors: Bottos Michele , Bolcati Cristina , Sciuto Lucia , Ruggeri Claudia , Feliciangeli Alessandra
Published: July 2010

The aim of this study was to assess the effect of early provision (≦ 8 years) of a powered wheelchair (PWC) in children with tetraplegia. Twenty‐nine children (15 males, 14 females; mean age 6 years 3 months, age range 3 to 8 years) with spastic or dystonic tetraplegia were studied. All participants had severe motor impairment. Treatment outcomes were investigated in several dimensions of disablement: Impairment, Functional Limitation/Activity, Disability/Participation. It was found that the level of independence improved significantly after PWC provision, while motor impairment, IQ, and quality of life did not. The majority of children (21 of 27) reached a level of driving competence which allowed them to move around with or without minimal (i.e. verbal) adult support. Achievement of this competence was not statistically related to IQ or motor impairment but correlated to the time spent in the PWC. The majority of parents (21 of 25) were not in favour of the PWC when the study started but after PWC provision, 23 of 25 parents expressed positive feelings about it. Reactions of the majority of children (23 of 25) were positive from the beginning of the study and did not change over time. The authors concluded that PWCs can aid independence and socialization and the majority of children can achieve a good‐enough driving competence, even those with severe learning disability or motor deficit. PWCs should not be viewed as a last resort but as a means of providing efficient self‐locomotion in children with a severe motor deficit.
Homozygous thermolabile variant of the methylenetetrahy‐drofolate reductase gene: a potential risk factor for hyperhomo‐cysteinaemia, CVD, and stroke in childhood
Authors: Prengler Mara , Sturt Natalie , Krywawych Steve , Surtees Robert , Liesner Raina , Kirkham Fenella
Published: July 2010

In this study of 118 children (median age 5.1 years; range 6 months to 17 years) with ischaemic stroke or transient ischaemic attack (TIA), 22 children (19%) were homozygous for the thermolabile variant of the methylenetetrahydrofolate reductase allele (t‐MTHFR), compared with nine of 78 (12%) of a reference population (p=0.18, OR 1.76, 95% CI 0.76 to 4.04). Of those with cerebrovascular disease (CVD), 17 of 84 were homozygous for the t‐MTHFR allele (p=0.13 compared with the reference population (OR 1.95, 95% CI 0.81 to 4.65). There was a significant (p<0.025) increment of plasma total homocysteine concentration in homozygotes for the t‐MTHFR allele compared with heterozygotes, negatives for the t‐MTHFR allele, and control children with no history of stroke. In four of 12 homozygotes for the t‐MTHFR allele, plasma homocysteine levels were raised, compared with three of 38 of those who were negative or heterozygous (p=0.047; OR 5.8, 95% CI 1.1 to 31.2). Homozygotes for the t‐MTHFR allele were significantly more likely to have a recurrent event than those who were negative or heterozygous (Cox regression p=0.031, hazard ratio 2.18, 95% CI 1.08 to 4.42). These data suggest that homozygosity for the t‐MTHFR allele is associated with raised homocysteine levels in children and is a risk factor for primary and secondary stroke and TIA.
Neuropsychological outcome at adolescence of very preterm birth and its relation to brain structure
Authors: Rushe T M , Rifkin L , Stewart A L , Townsend J P , Roth S C , Wyatt J S , Murray R M
Published: July 2010

Neuropsychological outcome at 14 to 15 years of age of a cohort of 75 participants (39 male, 36 female) born at <33 weeks’gestation was investigated. Research was conducted parallel to a recent MRI study by Stewart and colleagues which reported that 55% of this cohort had evidence of brain abnormality. One aim of the study was to compare neuropsychological function in those very preterm children with and without MRI abnormality. Compared to a control sample of term adolescents, very preterm participants had impairment only on a measure of word production. On measures of attention, memory, perceptual skill, and visuomotor and executive function, the adolescents born very preterm performed in the normal range, whether or not they had evidence of MRI abnormality. Our findings are encouraging as the neuropsychological consequences of damage to the very preterm brain, still evident on MRI at 14 to 15 years of age, appear to be minor.
Landau‐Kleffner syndrome: course and correlates with outcome
Authors: Robinson Richard O , Baird Gillian , Robinson Gary , Simonoff Emily
Published: July 2010

The presenting characteristics of 18 (11 female, seven male) children with Landau‐Kleffner syndrome (LKS) were studied with respect to course and outcome of their condition at a mean length of 67 (SD 46) months’follow‐up. All had regression of receptive language (mean age of onset 4 years 9 months) and electrical status epilepticus in sleep (ESES). Length of ESES correlated strongly with length of period between onset of illness and onset of recovery (p<0.006) and also with eventual receptive (p<0.001) and expressive (p<0.007) language. Behaviour during the acute phase was severely affected in nine children and associated with frontal lobe discharges in awake EEGs (p<0.004). Age at onset was not correlated with outcome. All children had impaired short‐term memory at follow‐up. Three children had language outcome within the normal range. No child with ESES lasting longer than 36 months had normal language outcome. These data lend support for intervention in ending ESES by 36 months using multiple subpial transection (MST) if steroids are ineffective or cause unacceptable side effects.