bluuebunny/crossref_metadata_2025 huggingface dataset:
Cervical spinal cord injury following cephalic presentation and delivery by Caesarean section
Authors: Morgan C , Newell S J
Published: July 2010

We describe a term infant with an acute spinal cord injury following emergency Caesarean section. Foetal movements were normal on the day that the mother was admitted for postterm induction of labour. Caesarean section was performed because of foetal distress and failure to progress during labour. The initial clinical picture suggested acute birth asphyxia. The presence of a high cervical spine injury became more obvious as the clinical picture evolved over the next 7 days. A discontinuity of the cervical spinal cord at C4–5 was confirmed on MRI. Spontaneous respiration failed to develop and intensive care was withdrawn on day 15. No evidence of trauma, or a vascular, neurological, or congenital anomaly of the cervical spinal cord was found at post mortem. The absence of a similar case following cephalic presentation and Caesarean section made bereavement couselling of the parents especially difficult.
Effects of ketogenic diet on development and behavior: preliminary report of a prospective study
Authors: Pulsifer Margaret B , Gordon Julie M , Brandt Jason , Vining Eileen PG , Freeman John M
Published: July 2010

The ketogenic diet is increasingly used for the management of difficult‐to‐control seizures in children. Here, we describe the first prospective study of the effects of the diet on development, behavior, and parenting stress. Participants were 65 children (36 males, 29 females) with intractable seizures, ages 18 months to 14 years 6 months, enrolled in a prospective study at the Johns Hopkins Hospital, Baltimore, MD, USA, to study the diet's efficacy. Children were assessed before diet initiation and at 1‐year follow‐up. At follow‐up, 52% (34 of 65) children remained on the diet. Mean seizure frequency decreased from 25 per day before diet initiation to less than two per day 1 year later. At follow‐up, mean developmental quotient showed statistically significant improvement (p<0.05), with significant behavioral improvements in attention and social functioning. Parental stress was essentially unchanged. No baseline factor examined predicted diet adherence, and the primary reason for diet discontinuation was insufficient seizure control. These preliminary results support prior anecdotal reports of the beneficial effects of the diet on cognition and behavior.
Effects of Johnstone pressure splints combined with neurodevelopmental therapy on spasticity and cutaneous sensory inputs in spastic cerebral palsy
Authors: Kerem Mintaze , Livanelioglu Ayse , Topcu Meral
Published: July 2010

The purpose of this study was to investigate the effectiveness of Johnstone pressure splints (JPSs) on spasticity and cutaneous sensory inputs in children with spastic cerebral palsy (CP). Thirty‐four children with spastic diplegic CP participated in this study. Children whose motor development levels were similar were divided into a treatment and a control group. Each group consisted of 17 participants (six females and 11 males). Mean age of the treatment group was 48.82 months (SEM 4.42), and the control group, 47.52 months (SEM 5.27). The treatment group underwent Bobath's neurodevelopmental therapy (NDT) combined with JPSs. The control group underwent NDT alone five days a week for three months. Before and after treatments, lower‐extremity passive range of motion (ROM) by goniometric measurements, spasticity by Modified Ashworth Scale (MAS), and somatosensory evoked potentials (SEPs) were measured. Passive ROM showed significant improvements in both groups (p<0.01). In the treatment group, all MAS scores increased. In the control group, the difference was significant except for values of internal rotator muscles. Improvements in passive ROM in the treatment group were significantly higher than the control group except in hip abduction and external rotation (p<0.05). MAS scores of the treatment group were significantly higher than the control group (p<0.05). SEP values increased in both groups but values of the treatment group were significantly higher than the control group (p<0.05).
Visual function in children with hemiplegia in the first years of life
Authors: Guzzetta Andrea , Fazzi Barbara , Mercuri Eugenio , Bertuccelli Barbara , Canapicchi Raffaello , Duin Jackie van Hof‐van , Cioni Giovanni
Published: July 2010

The aim of this study was to evaluate the incidence of visual function abnormalities in children with infantile hemiplegia, and the relation between visual abnormalities and type of lesion, as shown by brain MRI. Visual function was tested (grating acuity, visual field size, binocular optokinetic nystagmus [OKN], and ocular movements) in a group of 47 children with congenital or early acquired hemiplegic cerebral palsy (mean age 25 months, range 8 to 52 months). The cohort was subdivided into four groups according to MRI findings: brain malformations (n=5), abnormalities of the periventricular white matter (n=20), cortical‐subcortical lesions (n=16), and non‐progressive postnatal brain injuries (n=6). More than 80% of the children showed abnormal results in at least one visual test: acuity was the least impaired function, while visual field and OKN were abnormal in more than 50% of the cohort. No specific correlation could be identified between the type and timing of the lesions and visual function. Unlike adults with stroke, visual field defects were not always related to contralateral damage in the optic radiations or in the visual cortex. These results indicate that visual abnormalities are common in children with hemiplegia, and that they cannot always be predicted by MRI. All children with hemiplegia need a detailed assessment of visual function.
Autistic spectrum disorders in Möbius sequence: a comprehensive study of 25 individuals
Authors: Johansson Maria , Wentz Elisabet , Fernell Elisabeth , Strömland Kerstin , Miller Marilyn T , Gillberg Christopher
Published: July 2010

The prevalence of autistic disorder was analysed in 25 individuals with Möbius sequence, a disorder with brain‐stem dysfunction. The sample consisted of 18 males and seven females (20 participants were aged 2 to 22 years, and five were aged 1, 19 and 23 months, and 55 years old). Participants were recruited after a nationwide call and were part of a multidisciplinary study of individuals with Möbius sequence. They were given a meticulous neuropsychiatric examination including standardized autism diagnostic interviews. Ten individuals had an autistic spectrum disorder. Six of these met all diagnostic criteria for autism. In 23 individuals cognitive development could be assessed. Eight of those 23 patients had clear learning disability and six individuals were functioning in the normal but subaverage range. Autistic spectrum disorder and learning disability occurred in more than a third of the examined patients. Considering the hospital‐based nature of the sample, these findings may be overestimates. Nevertheless, awareness of this coexistence is important in the diagnosis and habilitation care of children with Möbius sequence. Moreover, the results provide further support for the notion of a subgroup of autistic spectrum disorders being caused by first trimester brain‐stem damage.
Comparison of three ankle‐foot orthosis configurations for children with spastic hemiplegia
Authors: Buckon Cathleen E , Thomas Susan Sienko , Jakobson‐Huston Sabrina , CPO Michael Moor , Sussman Michael , Aiona Michael
Published: July 2010

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.
Hip dysplasia in bilateral cerebral palsy: incidence and natural history in children aged 18 months to 5 years
Authors: Scrutton David , Baird Gillian , Smeeton Nigel
Published: July 2010

Children with bilateral cerebral palsy (CP) born during 1989 to 1992 (n=346) to a geographically defined population were ascertained and followed up to age 5 years. The aims of the study were to monitor hip development by serial X‐rays; to record gross locomotor development, aspects of physical management, and any hip‐related orthotics or surgery; to learn more of the natural history of hip development in this condition; and to provide guidelines for a surveillance protocol for those clinically managing children with bilateral CP. Children were X‐rayed at 18, 24, 30, 48, and 60 months. X‐rays were taken in a standardized position and measured to record migration percentage, acetabular index, Sharp's angle, and Smith's diaphyseal ratios. Relationships between the measures were investigated and a natural history of the children's hip development suggested. At as early as 18 months, migration percentages were significantly greater than in the normally developing population, although no measured CP factor could be identified to be the cause of this early change. Two possible factors affecting early hip development are described, neither of which is clinically apparent: the first separates the bilateral CP population as a whole from the normal population; but there is an additional presdisposing factor affecting some children only which in the presence of the motor disorder, has a significant influence on early hip development and subsequent dysplasia. The study confirms that, when measured correctly, migration percentage is the best guide to hip surveillance and the need for treatment. It is suggested that all children with bilateral CP should be X‐rayed in a standardized position at 30 months (corrected for gestational age).
Diagnosing children presenting with asymmetric pendular nystagmus
Authors: Shawkat Fatima S , Kriss Anthony , Russell‐Eggitt Isabelle , Taylor David , Harris Christopher
Published: July 2010

Horizontal asymmetric nystagmus usually occurs in one of three situations: secondary to an intracranial lesion, with monocular visual loss, or as part of the triad that constitutes the diagnosis of spasmus nutans (asymmetric nystagmus, abnormal head posture, head shake). Clinical records of 277 children, presenting with congenital nystagmus over an 8‐year period were reviewed. Nystagmus was asymmetric in 24 of 277 cases. Seven of these patients were diagnosed with spasmus nutans. This is a rare condition that is only diagnosed retrospectively based on the absence of any abnormal neuroimaging or electrophysiological findings. Twelve of 24 patients had intracranial pathology and all had abnormal visual evoked potentials (VEPs). Five patients were diagnosed with congenital sensory defect nystagmus including one with albinism, three with congenital cone dysfunction, and one with cone‐rod dystrophy. This paper stresses that although neuroimaging is necessary in all patients presenting with asymmetric nystagmus, such nystagmus can also occur with retinal disease or albinism and indicates the importance of non‐invasive VEP/ERG testing in all forms of nystagmus.
Classification criteria and distinction between migraine and tension‐type headache in children
Authors: Rossi Livia N , Cortinovis I , Menegazzo L , Brunelli G , Bossi A , Macchi M
Published: July 2010

The International Headache Society (IHS) classification system (1988) was developed primarily for headache disorders in adults and its validity for paediatric age is currently under discussion; in 1995 Seshia et al. proposed a revision of the criteria for migraine without aura to make diagnostic criteria more applicable to children. The purposes of the current study were to: (1) compare the IHS classification with the Seshia proposal, (2) compare the children affected by migraine without aura (MO) with the children affected by tension headache (TH) as defined by Seshia, for characteristics which are not included in the classification. The patients are a series of 320 children (mean age 9.9, SD 2.6 years; 144 males, 176 females) with recurrent or chronic headaches referred to a headache clinic in Milan, Italy. Using the Seshia criteria instead of the IHS criteria a higher number of children were included in the MO category: bilateral pain and family history of migraine were the most important factors which allowed a shift of children into this category. However, with the Seshia classification there was no reduction in the number of unclassifiable children. The reason why some children could not be classified was a short duration of attacks; the majority of unclassifiable children were 6 years old or less. No relevant difference was found between children with MO and children with TH for the following variables: occurrence of attacks in the afternoon or evening after school, reduction of attacks during school holidays, full‐time schooling, after‐school activities on school days, disordered daily life. On the contrary children with MO when compared with those with TH showed a higher number of precipitating factors and for the following factors a significant difference was found: exposure to TV or a computer, sleep deficiency, and strong emotions. Furthermore, children with MO showed a greater severity of attacks.
Visual impairment due to a dyskinetic eye movement disorder in children with dyskinetic cerebral palsy
Authors: Jan James E , Lyons Christopher J , Heaven Roberta KB , Matsuba Carey
Published: July 2010

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.
Developmental delay and multiple congenital anomalies in a child with a unique combination of partial monosomy 18 and partial trisomy 16
Authors: Schmidt Nicole , Dyke Don C Van , Keppler‐Noreuil Kim , Muilenburg Ann , Patil Shivanand , Kanis Adam B
Published: July 2010

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.
Neuromotor development from 5 to 18 years. Part 1: timed performance
Authors: Largo Remo H , Caflisch Jon A , Hug Franziska , Muggli Kathrin , Molnar Attila A , Molinari Luciano , Sheehy Anne , Gasser Theo
Published: July 2010

Timed performance in specific motor tasks is an essential component of a neurological examination applied to children with motor dysfunctions. This article provides centile curves describing normal developmental course and interindividual variation of timed performances of non‐disabled children from 5 to 18 years. In a cross‐sectional study (n=662) the following motor tasks were investigated: repetitive finger movements, hand and foot movements, alternating hand and foot movements, sequential finger movements, pegboard, and dynamic and static balance. Intraobserver, interobserver, and test‐retest reliability for timed measurements were moderate to high. Timed performances improved throughout the entire prepubertal period, but differed among various motor tasks with respect to increase in speed and when the‘adolescent plateau' was reached. Centile curves of timed performance displayed large interindividual variation for all motor tasks. At no age were clinically relevant sex differences noted, nor did socioeconomic status significantly correlate with timed performance. Our results demonstrate that timed motor performances between 5 and 18 years are characterized by a long‐lasting developmental change and a large interindividual variation. Therefore, a well standardized test instrument, and age‐specific standards for motor performances are necessary preconditions for a reliable assessment of motor competence in school‐age children.
Neuromotor development from 5 to 18 years. Part 2: associated movements
Authors: Largo Remo H , Caflisch Jon A , Hug Franziska , Muggli Kathrin , Molnar Attila A , Molinari Luciano
Published: July 2010

Associated movements (AMs) are the most frequently assessed parameters of movement quality in children with motor dysfunctions. In this article, reference curves of duration and degree of AMs from 5 to 18 years are provided. In a cross‐sectional study of non‐disabled children (n=662) duration and degree of AMs were estimated at six specific ages while children performed repetitive finger, hand, and foot movements, alternating hand and foot movements, diadochokinesis, sequential finger movements, pegboard, stress gaits, and dynamic balance. Moderate‐to‐high intraobserver and interobserver reliability for the assessment of AMs were noted. Duration and degree of AMs displayed a non‐linear developmental course that was a function of the motor task's complexity. AMs decreased most with age in repetitive movements, less in alternating and sequential movements, and least in the pegboard and dynamic balance. Reference curves demonstrated large interindividual variations for duration and degree of AMs. Both the variable developmental course and large interindividual variation need to be taken into account in the assessment of movement quality of school‐age children. In contrast to timed performance, considerable sex differences for AMs were observed.
Tethering of the vertebral artery in the congenital arcuate foramen of the atlas vertebra: a possible cause of vertebral artery dissection in children
Authors: Cushing K E , Ramesh V , Gardner‐Medwin D , Todd N V , Gholkar A , Baxter P , Griffiths PD
Published: July 2010

Twelve children with vertebrobasilar artery stroke are reported (seven males, five females; aged 6 months to 15 years). Patient 1 showed an arcuate foramen in the posterior arch of the atlas, an anatomical variant occurring in 3 to 15% of the population. It was hypothesized that the presence of the arcuate foramen might cause tethering of the vertebral artery and lead to its dissection by repetitive trauma. Lateral plain films of the cervical spine in cases of posterior circulation stroke were taken. Eight of 11 patients showed aberrant arcuate foramina. Of the remaining three patients, one had normal cervical spine X‐rays, one had an absent right posterior arch of the atlas following previous surgery for a cervical meningocele, and one patient had incomplete ossification of the vertebrae. Seven of the nine patients with arcuate foramina had vertebral angiograms. In all cases this showed the vertebral artery passing through the arcuate foramen before entering the brain and an appearance consistent with arterial dissection and occlusion at the same site below the foramen. Most documented cases of posterior circulation stroke in children follow trauma, which may be minimal or repetitive, with thrombotic occlusion of the artery at C1‐C2 level. The association with an arcuate foramen and its possible causative role in the genesis of posterior circulation stroke in children has not been previously recognized. There may be a causal association between the presence of an arcuate foramen, tethering of the vertebral artery in the foramen, and dissection from repetitive trauma with movement of the neck.
Possible strategies to protect the preterm brain against the fetal inflammatory response
Authors: Dammann Olaf
Published: July 2010

The accruing evidence that a fetal inflammatory response is the link between antenatal infection and white matter damage in the preterm newborn infant offers room for speculation how this harmful sequence could be interrupted. Enhancement of endogenous protection, response modification, and damage limitation downstream could be helpful strategies for intervention design. Appropriate observational and experimental studies are needed before clinical interventions can be initiated.
Selective dorsal rhizotomy: meta‐analysis of three randomized controlled trials
Authors: McLaughlin John , Bjornson Kristie , Temkin Nancy , Steinbok Paul , Wright Virginia , Reiner Ann , Roberts Theodore , Drake James , O'Donnell Maureen , Rosenbaum Peter , Barber Jason , Ferrel Anne
Published: July 2010

This study is a comparative analysis and meta‐analysis of three randomized clinical trials. Children with spastic diplegia received either 'selective’dorsal rhizotomy (SDR) plus physiotherapy (SDR+PT) or PT without SDR (PT‐only). Common outcome measures were used for spasticity (Ashworth scale) and function (Gross Motor Function Measure [GMFM]). Baseline and 9‐ to 12‐month outcome data were pooled (n=90). At baseline, 82 children were under 8 years old and 65 had Gross Motor Function Classification System level II or III disability. Pooled Ashworth data analysis confirmed a reduction of spasticity with SDR+PT (mean change score difference ‐1.2; Wilcoxon p<0.001). Pooled GMFM data revealed greater functional improvement with SDR+PT (difference in change score +4.0, p=0.008). Multivariate analysis in the SDR+PT group revealed a direct relationship between percentage of dorsal root tissue transected and functional improvement. SDR+PT is efficacious in reducing spasticity in children with spastic diplegia and has a small positive effect on gross motor function.
Postural balance in children with cerebral palsy
Authors: Rose Jessica , Wolff Don R , Jones Vincent K , Bloch Daniel A , Oehlert John W , Gamble James G
Published: July 2010

Postural control deficits have been suggested to be a major component of gait disorders in cerebral palsy (CP). Standing balance was investigated in 23 ambulatory children and adolescents with spastic diplegic CP, ages 5 to 18 years, and compared with values of 92 children without disability, ages 5 to 18 years, while they stood on a force plate with eyes open or eyes closed. The measurements included center of pressure calculations of path length per second, average radial displacement, mean frequency of sway, and Brownian random motion measures of the short‐term diffusion coefficient, and the long‐term scaling exponent. In the majority of children with CP (14 of 23) all standing balance values were normal. However, approximately one‐third of the children with CP (eight of 23) had abnormal values in at least two of the six center of pressure measures. Thus, mean values for path length, average radial displacement, and diffusion coefficient were higher for participants with CP compared with control individuals with eyes open and closed (p<0.05). Mean values for frequency of sway and the long‐term scaling exponent were lower for participants with CP compared with control participants (p<0.05). Increased average radial displacement was the most common (nine of 23) postural control deficit. There was no increase in abnormal values with eyes closed compared with eyes open for participants with CP, indicating that most participants with CP had normal dependence on visual feedback to maintain balance. Identification of those children with impaired standing balance can delineate factors that contribute to the patient's gait disorder and help to guide treatment.
Fluoxetine response in children with autistic spectrum disorders: correlation with familial major affective disorder and intellectual achievement
Authors: DeLong G Robert , Ritch Chad R , Burch Sherri
Published: July 2010

One hundred and twenty‐nine children, 2 to 8 years old, with idiopathic autistic spectrum disorder diagnosed by standard instruments (Childhood Austim Ratings Scale and Autism Diagnostic Observation Schedule) were treated with fluoxetine (0.15 to 0.5mg/kg) for 5 to 76 months (mean 32 to 36 months), with discontinuation trials. Response criteria are described. Family histories were obtained using the family history method in repeated interviews. Fluoxetine response, family history of major affective disorder, and unusual intellectual achievement, pretreatment language, and hyperlexia were used to define a coherent subgroup of autistic spectrum disorder. Statistical analyses were post hoc. Of the children, 22 (17%) had an excellent response, 67 (52%) good, and 40 (31%) fair/poor. Treatment age did not correlate with response. Fluoxetine response correlated robustly with familial major affective disorder and unusual intellectual achievement, and with hyperlexia in the child. Family history of bipolar disorder and of unusual intellectual achievement correlated strongly. Five children developed bipolar disorder during follow‐up. Fluoxetine response, family history of major affective disorder (especially bipolar), unusual achievement, and hyperlexia in the children appear to define a homogeneous autistic subgroup. Bipolar disorder, unusual intellectual achievement, and autistic spectrum disorders cluster strongly in families and may share genetic determinants.
Botulinum toxin treatment of spasticity in diplegic cerebral palsy: a randomized, double‐blind, placebo‐controlled, dose‐ranging study
Authors: Baker R , Jasinski M , Maciag‐Tymecka I , Michalowska‐Mrozek J , Bonikowski M , Carr L , MacLean J , Lin J‐P , Lynch B , Theologis T , Wendorff J , Eunson P , Cosgrove A
Published: July 2010

This study evaluated the efficacy and safety of three doses of botulinum toxin A (BTX‐A; Dysport) in 125 patients (mean age 5.2 years, SD 2; 54% male)with dynamic equinus spasticity during walking. Participants were randomized to receive Dysport (10, 20, or 30 units/kg) or placebo to the gastrocnemius muscle of both legs. Muscle length was calculated from electrogoniometric measurements and the change in the dynamic component of gastrocnemius shortening at four weeks was prospectively identified as the primary outcome measure. All treatment groups showed statistically significant decreases in dynamic component compared with placebo at 4 weeks. Mean improvement in dynamic component was most pronounced in the 20 units/kg group, being equivalent to an increase in dorsiflexion with the knee extended at 1920, and was still present at 16 weeks. The safety profile of the toxin appears satisfactory.
Fracture prevalence in Duchenne muscular dystrophy
Authors: McDonald Denise G M , Kinali Maria , Gallagher Andrew C , Mercuri Eugenio , Muntoni Francesco , FRCPCH Helen Roper , FRCPCH Philip Jardine , Jones David Hilton , Pike MG
Published: July 2010

The objective of this study was to determine the prevalence, circumstances, and outcome of fractures in males with Duchenne muscular dystrophy (DMD) attending neuromuscular clinics. Three hundred and seventy‐eight males (median age 12 years, range 1 to 25 years) attending four neuromuscular centres were studied by case‐note review supplemented by GP letter or by interview at the time of clinic attendance. Seventy‐nine (20.9%) of these patients had experienced fractures. Forty‐one percent of fractures were in patients aged 8 to 11 years and 48% in independently ambulant patients. Falling was the most common mechanism of fracture. Upper‐limb fractures were most common in males using knee‐ankle‐foot orthoses (65%) while lower‐limb fractures predominated in independently mobile and wheelchair dependent males (54% and 68% respectively). Twenty percent of ambulant males and 27% of those using orthoses lost mobility permanently as a result of the fracture. In a substantial proportion of males, the occurrence of a fracture had a significant impact on subsequent mobility.
Evaluation of therapeutic electrical stimulation to improve muscle strength and function in children with types II/III spinal muscular atrophy
Authors: Fehlings Darcy L , Kirsch Susan , McComas Alan , PhD Kent Campbell
Published: July 2010

The study aimed to evaluate the effect of low‐intensity nighttime therapeutic electrical stimulation (TES) on arm strength and function in children with intermediate type spinal muscular atrophy (SMA). The design was a randomized controlled trial with a 6‐month baseline control period. Children were evaluated at baseline, 6, and 12 months. TES was applied from 6 to 12 months to the deltoid and biceps muscle, of a randomly selected arm with the opposite arm receiving a placebo stimulator. Thirteen individuals with SMA between 5 to 19 years of age were recruited into the study and eight completed the 12‐month assessment. No statistically significant differences between the treatment and control arm were found at baseline, 6, and 12 months for elbow flexors, or shoulder abductors on quantitative myometry or manual muscle testing. There was no significant change in excitable muscle mass assessed by M‐wave amplitudes, nor function on the Pediatric Evaluation of Disability Inventory (self‐care domain). Therefore, in this study there was no evidence that TES improved strength in children with SMA.
Early postnatal Doppler assessment of cerebral blood flow velocity in healthy preterm and term infants
Authors: Pezzati Marco , Dani Carlo , Biadaioli Roberto , Filippi Luca , Biagiotti Roberto , Giani Teresa , Rubaltelli Firmino F
Published: July 2010

The aim of the present study was to generate normal reference data for anterior and middle cerebral artery blood flow velocity and resistance index in preterm and term infants during the first 8 hours of life. The study population longitudinally included 120 healthy preterm and term infants (gestational age 24 to 41 weeks), all of appropriate weight for gestational age. The following parameters were studied: peak‐systolic velocity, end‐diastolic velocity, mean velocity, and resistance index. All parameters were measured in the anterior cerebral artery, in the left middle cerebral artery, and in the right middle cerebral artery with the use of Doppler colour ultrasonography. In addition, we studied the ratio of mean arterial blood pressure to mean velocity in the three cerebral arteries as a further estimate of cerebral relative vascular resistance. We found that cerebral blood flow velocities increased significantly with increasing gestational age and birth weight, both in the anterior cerebral artery and in the right and left middle cerebral arteries. Resistance index, both in the anterior cerebral artery and in the middle cerebral arteries, increased significantly only with increasing gestational age. Relative vascular resistance decreased significantly with increasing gestational age and birthweight in the three cerebral arteries. Significant differences were found (p<0.05) in these values between the anterior cerebral artery and the middle cerebral arteries. The narrow time frame (2 to 8 hours) that we used to evaluate cerebral blood flow velocity often represents a significant moment at which decisions are made that can be fundamental for the outcome of the newborn infant.
MRI findings in children with school problems who had been exposed prenatally to alcohol
Authors: Autti‐Rämö Ilona , Autti Taina , Korkman Marit , Kettunen Satu , Salonen Oili , Valanne Leena
Published: July 2010

This study examined 17 children (nine males, eight females; mean age 13 years) with prenatal alcohol exposure of various durations. The aim of the study was to detect specific brain morphological alterations by means of MRI and to see if findings correlated with particular cognitive deficits. Of the 17 children, five had been exposed to heavy maternal consumption of alcohol (over 10 drinks/week) during the first trimester only; four had been exposed during the first and second trimester; and eight had been exposed throughout pregnancy. Five children had alcohol related neurobehavioural disorder, seven were diagnosed as having foetal alcohol effects and five were diagnosed as having foetal alcohol syndrome. Hypoplasia of the vermis was observed in 10 children and malformed posterior vermis in one additional child. Five children had hypoplastic cerebellar hemispheres. Hypoplasia of the corpus callosum was observed in two children. Small hippocampi were observed in three children and wide cortical sulci in six. No specific structural anomaly correlated with a particular neuropsychological deficit. In this study, deviations in the development of the vermis was the most sensitive morphological indicator of the effects of prenatal alcohol exposure. It was seen in every diagnostic group including children who had been exposed during only the first trimester of pregnancy.
Prognostic value of positron emission tomography in cryptogenic West syndrome
Authors: MD Kazuya Itomi , MD Akihisa Okumura , MD Tamiko Negoro , MD Kazuyoshi Watanabe , MD Jun Natsume , Takada Hiroyuki , MD Masanori Tadokoro , Ishigaki Takeo
Published: July 2010

The relationship between positron emission tomography (PET) findings and developmental or seizure outcome was examined in 17 infants (11 males, six females; mean age at onset of spasms 7 months, range 3 to 26 months) with newly diagnosed cryptogenic West syndrome. The predictive value of PET in these infants was assessed. PET was performed in the infants at the onset of spasms and 3 months after initial therapy using 18F‐labelled 2‐deoxy‐2‐fluoro‐D‐glucose. A third PET was performed at 18 months of age if the second scan was abnormal. All infants were followed up until at least 3 years of age. Cortical hypometabolism was detected in 11 infants on the first PET and in five infants on the second. Rate of developmental delay at the last follow‐up was significantly higher in infants with hypometabolism on the second PET than in those without PET abnormalities (p<0.05). Rate of seizure occurrence after initial treatment was higher in infants with cortical hypometabolism on the second PET, but the difference was not statistically significant. Results suggest that when PET after the initial treatment shows no abnormalities, even though the first PET shows hypometabolism, infants with cryptogenic West syndrome may have a favourable developmental or seizure outcome. PET may be a useful tool in evaluating the prognosis in infants with cryptogenic West syndrome.
Evaluation of the functional effects of a course of Bobath therapy in children with cerebral palsy: a preliminary study
Authors: Knox Virginia , Evans Andrew Lloyd
Published: July 2010

This study aimed to evaluate functional effects of Bobath therapy in children with cerebral palsy (CP). Fifteen children with a diagnosis of CP were recruited (9 males, 6 females; mean age 7 years 4 months, SD 2 years 8 months; age range 2 to 12 years). Types of motor disorder were as follows: spastic quadriplegia (n=9); spastic diplegia (n=4); athetoid quadriplegia (n=1), and ataxia (n=1). Participants were distributed across the following Gross Motor Function Classification levels: level I, n=1; level II, n=4; level III, n=5; level IV, n=4; and level V, n=1. Children awaiting orthopaedic intervention were excluded. A repeated measures design was used with participants tested with the Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) at 6‐weekly intervals (baseline, before and after Bobath therapy, and follow‐up). As the data were of ordinal type, non‐parametric statistics were used, i.e. Wilcoxon's test. Participants showed a significant improvement in scores in the following areas following Bobath therapy compared with the periods before and after Bobath therapy: GMFM total score (p=0.009); GMFM goal total (p=0.001); PEDI self care skills (p=0.036); and PEDI caregiver assistance total score (p=0.012). This demonstrates that in this population, gains were made in motor function and self care following a course of Bobath therapy.
Impact of feeding problems on nutritional intake and growth: Oxford Feeding Study II
Authors: Sullivan P B , Juszczak E , Lambert B R , Rose M , Ford‐Adams M E , Johnson A
Published: July 2010

Poor nutritional status and growth failure are common in children with cerebral palsy (CP). The aim of this study was to assess, within a subgroup of a large and clearly defined population of children with disabilities, the impact of feeding difficulties on (1) the quality (micronutrient intake) and quantity (macronutrient intake) of their diet and (2) their growth. One hundred children with disabilities (40 females, 60 males; mean age 9 years, SD 2 years 5 months; range 4 years 6 months to 13 years 7 months) underwent a detailed dietetic analysis and a comprehensive anthropometric assessment. Diagnostic categories of disability were: CP (n=90); global developmental delay (n=3); Marfan syndrome (n=1); intractable epilepsy (n=2); agenesis of the corpus callosum (n=2); methyl malonic aciduria (n=1); and congenital rubella (n=1). Neurological impairment was classified according to difficulty with mobility which was graded as mild (little or no difficulty walking), moderate (difficulty walking but does not need aids or a helper), and severe (needs aids and/or a helper or cannot walk). Results confirmed the significant impact of neurological impairment in children on body growth and nutritional status becoming worse in those with a greater degree of motor impairment. The major nutritional deficit was in energy intake, with only one fifth reportedly regularly achieving over 100% estimated average requirement (EAR), whilst micronutrient intake was less markedly impaired and protein intake was normal in this group (96% above EAR). Many children with neurological impairment would benefit from individual nutritional assessment and management as part of their overall care.
Comparing reliability and validity of pediatric instruments for measuring health and well‐being of children with spastic cerebral palsy
Authors: McCarthy Melissa L , Silberstein Charles E , Atkins Eileen A , Harryman Susan E , Sponseller Paul D , Hadley‐Miller Nancy A
Published: July 2010

This study sought to examine the reliability and validity of three generic instruments for measuring the health of children with cerebral palsy (CP) and to compare them with a disease‐specific measure, the Gross Motor Function Measure (GMFM). The Pediatric Evaluation and Disability Inventory (PEDI), the Pediatric Outcomes Data Collection Instrument (PODCI), and the Child Health Questionnaire (CHQ) were completed by the primary caregivers of 115 young children with spastic CP. The GMFM was administered to the children. The mean age of the sample was 5 years 8 months (range 3:1 to 10:4) and consisted of more males (58%) than females. The PEDI scales demonstrated higher internal consistency than the PODCI and CHQ scales. In comparison with the GMFM, the PODCI transfer and mobility scale (relative validity, 62%) and the PEDI mobility scale (relative validity, 53%) detected the most significant health differences between children with hemiplegia, diplegia, and quadriplegia. The PEDI social function scale detected the largest differences in cognitive function between children with an IQ of less than 70 compared with those with an IQ of 70 or greater. The reliability and validity of these different instruments varied significantly in this patient population.
Bilateral lesions of thalamus and basal ganglia: origin and outcome
Authors: Krägeloh‐Mann Ingeborg , Helber Alexandra , Mader Irina , Staudt Martin , Wolff Markus , Groenendaal Floris , DeVries Linda
Published: July 2010

Twenty‐seven MRI examinations from 17 children (7 females, 10 males) with bilateral lesions of the basal ganglia and thalamus, presenting over a period of 8 years, were reevaluated, and correlated with the type of cerebral palsy (CP) as well as motor and cognitive impairment. Children were between 1 year 6 months and 17 years old at last examination (mean 5 years 9 months). Brain damage had occurred as a consequence of birth asphyxia in nine patients and of neonatal shock in four patients. No adverse event could be identified in four children. In these, late prenatal compromise is assumed, as extensive screening (including MR spectroscopy in two patients) did not yield an underlying metabolic disorder. Three different degrees of MRI lesion patterns could be defined: a mild pattern (involvement of nucleus lentiformis and ventro‐lateral thalamus only; n=7), an intermediate pattern (involvement of nucleus lentiformis, ventro‐lateral thalamus, and pericentral region; n=3), and a severe pattern (involvement of nucleus lentiformis, entire thalamus, pericentral region, and hippocampus; n=7). This grading of MRI findings correlated significantly with the severity of both cognitive and motor impairment and type of CP. Normal cognitive development and mild motor delay was only seen with the mild pattern. All children developed CP: purely dyskinetic CP was only seen with the mild pattern, whereas the dyskinetic‐spastic or spastic CP types could be seen in all three lesion patterns, with dyskinetic‐spastic CP more related to the moderate, and purely spastic CP more related to the severe pattern.
Benign paroxysmal torticollis of infancy: four new cases and linkage to CACNA1A mutation
Authors: Giffin N J , Benton S , Goadsby P J
Published: July 2010

Benign paroxysmal torticollis of infancy (BPTI) is a disorder characterized by recurrent episodes of head tilt secondary to cervical dystonia. Attacks are often accompanied by vomiting, pallor, and ataxia, settling spontaneously within hours or days. Episodes begin within the first 12 months of life and resolve by 5 years. We report four patients with BPTI. Symptoms started from 3 months of age, with head tilting lasting between 10 minutes and 2 months; the shorter episodes were followed by vomiting, apathy, and unsteadiness. Head tilt became less prominent after infancy, replaced by vertigo and eventually by migraine headaches. Two patients came from a kindred with familial hemiplegic migraine linked to CACNA1A mutation. BPTI may be regarded as a migraine aura equivalent. The syndrome poses interesting questions regarding varying phenotypic expression of calcium channelopathies at different stages of development.
Use of a lying hip abduction system in children with bilateral cerebral palsy: a pilot study
Authors: Hankinson J , Morton R E
Published: July 2010

Fourteen children aged 4 to 14 years with hip subluxation due to cerebral palsy (CP) were considered for a trial in a lying hip abduction system (Jenx Dreama, Jenx Limited, Sheffield, UK). Baseline data were recorded for 6 months, then assessments of the system were made for one year at 6 and 12 months. Assessments consisted of hip radiographs in the standard position, a parental questionnaire, and a sleep chart, which was completed by parents every Friday night during the trial. Three children could not enter the trial because of general sleep problems, and three could not complete it because they were unable to sleep with the system. One further child withdrew from the study just before the end because of unacceptable deterioration for which surgery was needed. The remaining seven children completed the trial and there was an overall improvement in rate of hip migration percentage on the right from 7% per annum in the baseline period to ‐4% with the system (p<0.05). On the left, changes were ‐3% and 0% respectively (ns). Average sleep at night changed from 9 to 9.4 hours (ns) and wakenings/night from 1 to 1.3 (ns). On the parental questionnaire, there were significant improvements noted with the system in position for seating and sleeping, ease of hip abduction for washing, and with pain reduction. This pilot study supports the use of this type of lying system but further studies are needed to establish the acceptability and efficacy of these systems, particularly in children aged 2 to 5 years when irreversible bony deformities of the hip tend to occur.
Intermittent intensive physiotherapy in children with cerebral palsy: a pilot study
Authors: Trahan Johanne , Malouin Francine
Published: July 2010

The aims of the study were: (1) to determine the feasibility of a rehabilitation program combining intensive therapy periods (4 times/week for 4 weeks) with periods without therapy (8 weeks) over a 6‐month period in severely impaired children with cerebral palsy (CP); and (2) to measure changes in gross motor function after intensive therapy periods (immediate effects) and rest periods (retention). A convenient sample included five children (two females, three males; mean age 22.6 months [SD 9.9]) with severe forms of CP with impairment of four limbs and trunk (GMFCS levels IV and V). A multiple‐baseline design was used. Changes in motor performance were assessed by a blind evaluator using the Gross Motor Function Measure. Visual and statistical analyses followed. Level of compliance during intensive therapy was 93.1%. Children received a mean of 30 treatments over the 24 weeks of the experimental phase compared with the 48 treatments they would have received routinely. Increases in GMFM scores (mean 9.2%; range 3 to 15%) were significant in three children (p<0.05) and all participants maintained their motor performance during the two 8‐week rest periods. Results showed that four treatments per week over a 4‐week period were well tolerated when separated by rest periods. The intermittent program led to improvements in motor function that were maintained over the rest periods. Results underline the need to reconsider the organization of physical rehabilitation programs. A regime that is intensive enough without being tiring and one that provides practice conditions for consolidating motor skills learned during the intensive therapy period may best optimize motor training.
Health status of school‐aged children with cerebral palsy: information from a population‐based sample
Authors: Kennes Janneke , Rosenbaum Peter , Hanna Steven E , Walter Stephen , Russell Dianne , Raina Parminder , Bartlett Doreen , Galuppi Barbara
Published: July 2010

In this study parents’systematic accounts of the health status of 408 school‐aged children with cerebral palsy (CP) are reported (221 males, 187 females; mean age 8 years 5 months, SD 1 year 11 months; range 5 to 13 years), as are relations between severity of functional motor impairment and eight functional health status domains. Data were collected as part of a longitudinal study of the motor development of a population‐based, stratified, random sample of children with CP from across Ontario, Canada. The Gross Motor Function Classification System (GMFCS) was used to classify severity of CP and functional health status was described with the eight‐level Health Utilities Index ‐ Mark 3. Rates of functional limitations in Mobility, Dexterity, Speech, and Vision were statistically significantly associated with GMFCS levels (all p<0.01), with correlation values (tau‐b) of 0.82,0.58,0.46, and 0.36, respectively. Functional limitations in hearing (tau‐b=0.16; p=0.04) and cognition (tau‐b=0.27;p<0.01) were both statistically significantly associated with GMFCS levels, though correlations were low. Neither emotion (tau‐b=0.03;p=0.24) nor pain (tau‐b=0.07;p=0.37) was associated with degree of functional limitation as described by the GMFCS. Clinical and epidemiological implications of findings are discussed.