bluuebunny/crossref_metadata_2025 huggingface dataset:
Proximal femoral resection for subluxation or dislocation of the hip in spastic quadriplegia
Authors: Ackerly Steve , Vitztum Coley , Rockley Brent , Olney Brad
Published: July 2010

Management of a painful or contracted hip dislocation in individuals with severe spastic quadriplegia is difficult. Clinical and radiographic results of 12 proximal femoral resection‐interposition operations performed in seven non‐ambulatory persons (five males, two females; mean age 14 years, 8 months; age range 6 years 11 months to 19 years 8 months) with severe spasticity were reviewed to determine if pain relief and restoration of motion were maintained. At a mean follow‐up of 7 years 7 months (median 9 years 6 months) all participants maintained a good sitting position and a functional range of motion with improved hygiene. Hip pain was improved in all participants compared with their preoperative status. Proximal femur migration occurred causing slight pain in one person. Heterotopic ossification was observed but was not clinically significant. Complications included traction pin loosening and infection and a late supracondylar femur fracture 3 months after the operation. Proximal femoral resection effectively decreased pain and restored hip motion in those with severe spastic quadriplegia leading to improved sitting and perineal care.
Brainstem dysgenesis: report of five patients with congenital hypotonia, multiple cranial nerve involvement, and ocular motor apraxia
Authors: Roig Manuel , MD Margarida Gratacòs , Vazquez Elida , Del Mireia , FoguetMD Anton , Ferrer Isidre , Macaya Alfons
Published: July 2010

This paper reports three females and two males with a distinctive congenital syndrome characterized by severe congenital hypotonia, facial diplegia, jaw ankylosis, velo‐pharyngeal incoordination, pyramidal tract signs, and ocular motor apraxia. Patients were followed up at ages ranging from 20 months to 16 years. All cases of this syndrome are sporadic, without dysmorphological features, chromosomal, or MRI brain abnormalities. Electrophysiological studies indicate the brainstem as the site of the neurological dysfunction. Postmortem CNS study of one of the patients demonstrated neuronal depletion of the IV, VII, VIII, and IX cranial nerve nuclei and intact morphology of the cerebral hemispheres. A vascular accident, early in foetal life, is the most likely cause of the clinical picture. The extent of brainstem involvement and its related clinical findings distinguishes these patients from those with Moebius, Pierre Robin, or Cogan syndromes. Outcome is better than what could be anticipated during the first few months of life given the severity of symptoms. Intelligence or developmental quotients are within the normal range for their age. Facial hypomimia, feeding, and speech articulatory performance difficulties are the main disabilities observed in these patients at follow‐up.
Polymicrogyria in monozygous twins and an elder sibling
Authors: MD Po‐Cheng Hung , MD Huei‐Shyong Wang
Published: July 2010

Monozygous twin births have been associated with brain lesions such as hydranencephaly, multicystic encephalomalacia, and porencephaly. Prenatal circulatory injury has been considered to be the cause. Polymicrogyria is rare but has been reported in autopsied cases. The sibship in this case report, comprising monozygotic male twins and their elder sister from the same non‐consanguineous parents, all had global developmental delay. Brain MRI showed polymicrogyria. We suggest that, apart from circulatory compromise, genetic etiology must be implicated as the cause of polymicrogyria.
Outcome after prolonged convulsive seizures in 186 children: low morbidity, no mortality
Authors: Metsäranta Piia , Koivikko Matti , Peltola Jukka , Eriksson Kai
Published: July 2010

Prolonged convulsive seizures are a common neurological emergency and a potential cause of neuronal damage and functional sequelae. We explored the role of seizure duration and various background factors for neurological sequelae in children with prolonged convulsive seizures. The population‐base of this study was all children (age < 16 years) who had been admitted to the Tampere University Hospital, Finland between 1993 and 1999 with convulsive seizures lasting more than 5 minutes. Patients were followed up individually (mean length of follow‐up 2 years 1 month, range 0 to 7 years 8 months). All available data on the prolonged seizure episodes and clinical follow‐up were analyzed retrospectively by a detailed review of all medical charts and records. In 186 children (94 males, 92 females; mean age 4 years 5 months, SD 3 years 10 months, range 1 month to 15 years 4 months) there were 279 separate convulsive seizure episodes lasting over 5 minutes, yielding an annual incidence of 47.5 out of every 100000 episodes. Seizure aetiology was idiopathic in 26.2% of episodes, febrile in 41.9%, remote symptomatic in 28%, and acute symptomatic in 3.9% of episodes. Mean duration of all seizure episodes was 42.5 minutes (SD 46.1 minutes) and was significantly correlated with the aetiology: shortest in the febrile group (mean 35.4 minutes) and longest in the acute symptomatic group (mean 88.6 minutes; p < 0.001). There was no mortality related directly to these acute seizure episodes. The most common sequela was an onset of epilepsy in 40 children (22%). Permanent neurological sequelae were noted in only four patients (2.2%; mean seizure duration 16 minutes) and non‐permanent sequelae in six patients (3.2%; mean seizure duration 38 minutes). Neurological sequelae of prolonged convulsive seizures in children are rare and are related to aetiological factors rather than the duration of a single seizure. The role of acute seizures in the evolution of epilepsy in children remains obscure.
Effectiveness of the Innsbruck Sensori‐motor Activator and Regulator in improving saliva control in children with cerebral palsy
Authors: Johnson Hilary M , Reid Susan M , Hazard Catherine J , Lucas James O , Desai Mala , Reddihough Dinah S
Published: July 2010

The aim of this study was to determine the effectiveness of an intraoral appliance, the Innsbruck Sensorimotor Activator and Regulator (ISMAR), in improving drooling and eating skills in a group of children with cerebral palsy, and to determine which factors might indicate good candidates for this type of treatment. Eighteen children (13 males, five females; mean age 7 years 10 months, range 4 to 13 years) were selected. Measures of drooling and feeding skills were taken at baseline, at the completion of a 6–month control phase, and at two more 6–monthly time points after the ISMAR was fitted. Children varied greatly in both the length of time taken to tolerate wearing the ISMAR and duration for which the appliance was worn. Only six children (four females, two males) completed the full study. Their motor disabilities were athetosis (n=3), spastic quadriplegia (n=2), and hypotonia (n=1); four of the six children used a wheelchair for locomotion. None had epilepsy and none had greater than mild cognitive impairment. For these children drooling severity scores and eating and drinking skills improved significantly over the treatment period in comparison with the control phase. We conclude that the ISMAR remains a valid option in improving drooling in children and merits further study.
Drooling, saliva production, and swallowing in cerebral palsy
Authors: Senner Jill E , Logemann Jerilyn , Zecker Steven , Gaebler‐Spira Deborah
Published: July 2010

Fourteen participants (six females, eight males) ranging in age from 7 years 11 months to 18 years 2 months (mean 11y 7mo) with a confirmed diagnosis of spastic cerebral palsy (CP) were included in the study. Participants included those who drooled (CP+,n=14); age‐ and sex‐matched children with spastic CP who were dry to mild and never to infrequent droolers (CP−,n=14) as well as typically developing peers (CTRL,n=14) served as controls. Frequency of swallowing was measured by using simultaneous cervical ausculation and videotaping of the head and neck. Saliva production was measured with the Saxon test, a simple gauze‐chewing procedure. In addition, Pediatric Evaluation of Disability Inventory (PEDI), Test of Nonverbal Intelligence‐3 (TONI‐3), dysarthria severity scale, and Gross Motor Function Classification System (GMFCS) scores were obtained for each participant. Both groups of participants with CP tended to swallow less frequently than typically developing participants and tended to produce less saliva than typically developing controls; however, these differences were not statistically significant. No correlation was found between amount of saliva produced and amount drooled (r=0.245). An analysis of variance (ANOVA) conducted on the PEDI functional skills mean scores indicated significant differences between the three groups (F((2,39))=23.522,p<0.0001). Likewise, an ANOVA conducted on the TONI‐3 scores revealed statistically significant differences between the three groups (F(2,39)=31.761,p<0.0001). A Spearman's rho correlation indicated that GMFCS scores were not significantly correlated with drooling severity (Spearman's rho correlation=0.3951,p=0.037). Drooling severity was found to be positively correlated with dysarthria severity (Spearman's rho correlation=0.82,p<0.0001). These findings suggest that drooling in patients with CP is related to swallowing difficulties rather than hypersalivation.
Efficacy of botulinum toxin A, serial casting, and combined treatment for spastic equinus: a retrospective analysis
Authors: Glanzman Allan M , Kim Heakyung , Swaminathan Kartik , Beck Teresa
Published: July 2010

Botulinum toxin type A (BTX‐A), serial casting, and combined treatment for spastic equinus were compared by chart review. Fifty‐five patients with diplegia (n=21), hemiplegia (n=17), quadriplegia (n=6), and other diagnoses (n=11) were reviewed. Thirty‐one patients had Gross Motor Function Classification System (GMFCS) scores of I or II, 10 had a GMFCS score of III, and eight had GMFCS scores of IV or V (for six there were no available data). Mean age was 7 years 1 month (SD 4 y 8mo). The combined group showed a significantly greater increase in passive range of motion (ROM) of the ankle joint in comparison with BTX‐A alone (p=0.0002), with a mean of 17° (SD 11.2) versus 5° (SD 6.7), and casting alone showed a significantly greater increase in comparison with BTX‐A alone (p=0.0001), with a mean of 15° (SD 4.5) versus 5° (SD 6.7). With cast number controlled, change in ROM after casting with and without BTX‐A was not significantly different (p=0.37). There was no significant difference between casting with or without BTX‐A, and both improved ROM to a greater degree than BTX‐A alone. Casting demonstrated a significantly more robust impact on range of motion than BTX‐A alone.
Lead exposure and attention regulation in children living in poverty
Authors: Davis Deborah Winders , Chang Florence , Burns Barbara , Robinson Julia , Dossett Dena
Published: July 2010

This study examined the relationship between lead exposure and attention regulation in children from low‐income families. Children with lead exposure were expected to have lower attention regulation abilities than children who were not exposed. The 57 participants (39% male, 61% female) were children aged 4 and 5 years (mean age 4y 7mo [SD5mo]); 24 children were lead‐exposed (10–29μg/dl) and 33 were non‐exposed comparisons (1–6μg/dl). Both groups qualified to be enrolled in programs for low‐income families. A puzzle‐matching task was completed in parent‐child and child‐alone conditions. Attention allocation and puzzle‐matching performance were assessed in the two conditions. Children who were lead‐exposed demonstrated less self‐regulated attention than comparisons and had poorer puzzle performance in the child‐alone condition. Attention patterns did not differ in the parent–child condition. This study extends previous knowledge concerning effects of lead exposure on preschool children from disadvantaged environments and suggests that lead exposure affects specific attention regulation abilities.
Familial cancer and developmental dyslexia: an observational pilot study
Authors: Taylor Kathleen E
Published: July 2010

The aim of the study was to test the hypothesis that raised platelet‐activating factor (PAF) may contribute to the aetiology of developmental dyslexia. PAF is a potent proinflammatory mediator which signals cell damage and facilitates natural killer cell activity. Raised PAF may help protect against tumourigenesis. As dyslexia has a partial genetic basis, the PAF hypothesis predicts that dyslexia may be negatively associated with a family history of cancer. To test this prediction, children with dyslexia (n=163) and children without dyslexia (n=154), with (n=152) and without (n=165) a family history of cancer (total n=317; mean age 11 years 5 months, SD 2 years 11 months), were compared on standard psychometrics (British Ability Scales subtests). Results showed that proportionately fewer children with dyslexia (38%) than controls (58.4%) had a family history of cancer, and there was some evidence of a‘dose’effect: children who had more relatives with cancer showed better reading and spelling. It was concluded that children at genetic risk of dyslexia who have a family history of cancer have better reading and spelling than those without a family history of cancer, confirming the prediction of the PAF hypothesis.
Neuropathic bladder and intermittent catheterization: social and psychological impact on families
Authors: Borzyskowski †Malgorzata , Cox Antony , Edwards Melinda , Owen Amanda
Published: July 2010

Clean intermittent catheterization (CIC) is the mainstay of management in neuropathic vesicourethral dysfunction, both to improve continence and, more importantly, to preserve renal function. We looked at the effects of this procedure on children, adolescents, and their families. In particular, we wished to see if there were any differences between those who successfully catheterized and those who did not. Forty families were enrolled into the study. Ages of children and adolescents (23 females, 17 males) ranged from 1 to 20 years. Most participants (n=31) had spina bifida. Other causes of bladder dysfunction included transverse myelitis, spinal cord injury, and spinal neuroblastoma. Parents were assessed using the Effects of Handicap on Parents semi‐structured interview, the Socioemotional Functioning Interview, and a semi‐structured interview, specifically designed for the study, which looked at family characteristics and experience related to diagnosis and catheterization. In addition, the Rutter Parental‘A’Scale Questionnaire was used to screen for emotional and behavioural disorders in the child. Results showed that CIC by carer or self‐ catheterization itself did not cause major emotional and behavioural problems but the bladder problem may act as a focus that puts considerable strain family relationships. Although most parents disliked CIC they complied with the suggested management. It is important that all those involved understand the aims of management and success can be achieved by combined input from medical, psychological, and specialist nursing staff. The problem is lifelong and continued support from a multidisciplinary team is essential.
Outcome of craniopharyngioma in children: long‐term complications and quality of life
Authors: Poretti Andrea , Grotzer Michael A , Ribi Karin , MD Eugen Schönle , Boltshauser Eugen
Published: July 2010

Childhood craniopharyngiomas are histologically benign tumours arising from remnants of Rathke's pouch in the hypothalamic—pituitary region. The two common treatment approaches are primary total resection or limited resection followed by radiotherapy. To study the outcome after a primary surgical approach, we followed 25 consecutive patients (10 females, 15 males) under 16 years of age who were treated in a single institution with a management policy of radical tumour excision (mean age at diagnosis 9 years 2 months, SD 4 years 3 months; range 2 years 9 months to 15 years 11 months). Mean follow‐up after primary surgery was 11 years 3 months (SD 7 years 7 months). Tumour control, and neurological, endocrine, and hypothalamic complications and their impact on health‐related quality of life were assessed (medical follow‐up, semi‐structured interview, and questionnaires). Results of tumour control were generally good, however, local failure was observed in 6 of 25 patients, and severe late‐treatment complications decreased quality of life for many long‐time survivors. Endocrine deficiency occurred in 24/25, visual complications in 16/24, neurological complications in 8/24, obesity in 14/23, increased daytime sleepiness in 6/21, and significant school problems in 10/20. Patients with craniopharyngioma rated their health‐related quality of life as considerably lower than healthy controls; the domains of social and emotional functioning were particularly affected. Parents’ratings were considerably lower than those of the patients. Poor functional outcome was associated with large tumours infiltrating or displacing the hypothalamus, the occurrence of hydrocephalus, and young age at diagnosis, but also with multiple operations due to tumour recurrence. Alternative treatment strategies should be considered, especially in very young patients with large tumours.
Autism spectrum disorders in children with normal intellectual levels: associated impairments and subgroups
Authors: Sturm Harald , Fernell Elisabeth , Gillberg Christopher
Published: July 2010

In order to define potential subgroups pertaining to the spectrum of‘high‐functioning’pervasive developmental disorders (PDDs) the medical and psychiatric records of 101 children with PDD were reviewed. Ninety‐one children had a diagnosis of Asperger syndrome, nine had a PDD not otherwise specified, and one had‘high‐functioning’autism. Mean age of the children (71 males, 30 females) was 9 years 8 months (age range 5 to 12 years). Apart from the core dysfunctions of the PDD, i.e. deficient social interaction, communication and repertoires, and restricted interests, 95% had attentional problems, 75% had motor difficulties, 86% had problems with regulation of activity level, and 50% had impulsiveness. About three‐quarters had symptoms compatible with mild or severe attention‐deficit‐hyperactivity disorder (ADHD) or had deficits in attention, motor control, and perception (DAMP), indicating a considerable overlap between these disorders and high‐functioning PDD in children of normal or near‐normal intelligence. A combination of affective dysregulation, thought disturbance, and severely restricted social interaction, referred to as a multiple complex developmental disorder (MCDD; a condition possibly related to schizoaffective disorder), was recorded in about 8% of the children. Seventeen percent had another major medical diagnosis or medical syndrome, which highlights the importance of completing a neurological assessment of all children with PDDs.
Depressive pseudodementia in a child with autism
Authors: Pollard Andrew J , Prendergast Michael
Published: July 2010

Depression is rare in early childhood and unusual in autism in this age group. We describe a female child aged 6 years with autism who presented with regression of developmental skills previously gained. Her sleep and appetite were poor, her affect was sad, and she had morbid speech content. She responded to treatment with antidepressant medication. When this clinical picture occurs in adults it is called depressive pseudodementia; paediatric neurologists and neuropsychiatrists need to be aware of it in children.
One hundred males with Asperger syndrome: a clinical study of background and associated factors
Authors: Cederlund Mats , Gillberg Christopher
Published: July 2010

The objective of this study was to investigate the background and associated factors in a representative group of young males with Asperger syndrome (AS) presenting at a specialized autism clinic. One hundred males aged 5 years 6 months to 24 years 6 months, with a mean age of 11 years 4 months (SD 3y 10mo), who had a clinical diagnosis of AS were included in the study. An in‐depth review of their medical records and neuropsychological test data was performed. There was a high rate (51%) of non‐verbal learning disability (defined as Verbal IQ more than 15 points higher than Performance IQ), but otherwise there was little or no support for the notion of right‐hemisphere brain dysfunction being at the core of the syndrome. There was a very high rate of close relatives with autism spectrum problems, but also high rates of prenatal and perinatal problems, including prematurity and postmaturity. In comparison with general population data, those with AS very often had a combination of genetic and prenatal and perinatal risk factors. Non‐verbal learning disability test results applied in about half the group. There was a subgroup of individuals with AS who had macrocephalus. However, there was no support for an association of AS with low body mass index.
Muscle responses to seated perturbations for typically developing infants and those at risk for motor delays
Authors: Washington Kathleen , Shumway‐Cook Anne , Price Robert , Ciol Marcia , Kartin Deborah
Published: July 2010

This study examined spatial and temporal organization of muscle activity in response to seated forward platform perturbations in typically developing (TD) infants and in infants at risk for ongoing neuromotor delays. Twelve high‐risk (HR) infants (six males, six females) and 12 TD infants (nine males, three females) aged 8 to 10 months (corrected for prematurity), who were all independent sitters, participated in this study. Surface electromyograms recorded muscle responses to forward perturbation in the neck, trunk, and leg (6cm amplitude, 12cm/s). Center of pressure measures were also recorded. The TD infants demonstrated significantly more phasic responses than the HR infants, who had more trials with tonic activity and more trials with no burst of muscle activity (Mann‐Whitney U test, two‐tailed, p=0.006). The TD infants demonstrated caudalcephalo, directionally appropriate recruitment patterns more often than the HR infants. There were some similarities between the two groups, with no significant difference between any of the center of pressure measures or onset latencies. Results suggest that postural development in HR infants with motor delays is complex. Some elements of seated postural control appear to be developmentally appropriate, e.g. presence of phasic activity in directionally appropriate muscles sequenced in a caudalcephalo pattern. However, frequency of these age appropriate responses was significantly lower in HR infants, who demonstrated many normal, albeit immature, responses, such as cephalocaudally sequenced muscle activity.
Bimanual coordination during a goal‐directed task in children with hemiplegic cerebral palsy
Authors: Hung Ya‐Ching , Charles Jeanne , Gordon Andrew M
Published: July 2010

Most studies of impaired hand function in children with hemiplegic cerebral palsy (CP) have focused on either the involved or the non‐involved extremity in isolation. Coordination of the involved and non‐involved hand during bimanual tasks in these children is not well understood. The present study examined bimanual coordination using a drawer‐opening task under speed and hand constraints in 10 children with hemiplegic CP (5 males and 5 females, mean age 13y 5mo, range 8y to 16y) and 10 age‐matched right‐handed developing typically children (6 males and 4 females, mean age 13y 1mo). Children were asked to reach forward and open a drawer with one hand and then activate a light switch inside the drawer with the contralateral hand. The role of the two hands (open drawer and activate switch) and speed (self‐paced vs fast‐as‐possible) were varied. The children with hemiplegic CP were slower (p<0.001) and less coordinated in this task, with reduced movement overlap of the two hands (p<0.001) and sequential completion of the two movement objectives (p<0.001). Moreover, the hand used for each task subcomponent affected task performance for the children with hemiplegic CP (p<0.05). Interestingly, faster speed facilitated better bimanual coordination for the children with hemiplegic CP (p<0.001). Results highlight the importance of movement constraints on task performance and suggest that movement speed might facilitate better bimanual coordination.
Is treatment with growth hormone effective in children with cerebral palsy?
Authors: Shim Melanie L , Moshang Thomas , Oppenheim William L , Cohen Pinchas
Published: July 2010

Children with cerebral palsy (CP) often have poor linear growth during childhood, resulting in a diminished final adult height. Here we report a female with CP and short stature but without growth hormone (GH) deficiency who exhibited increased growth during treatment with GH. We also report two other children with CP who were treated with GH: one female with a history of leukemia, and a male with Klinefelter syndrome. These two children were both found to be GH‐deficient by insulin provocative GH testing and responded to treatment with increased growth rate. Growth improved to a greater extent in the two children with apparent GH deficiency. In summary, it is felt that GH therapy might be beneficial for children with CP and warrants further investigation.
Non‐verbal development of children with deafness with and without cochlear implants
Authors: Schlumberger Emilie , Narbona Juan , Manrique Manuel
Published: July 2010

Deprivation of sensory input affects neurological development. Our objective was to explore clinically the role of hearing in development of sensorimotor integration and non‐verbal cognition. The study involved 54 children (15 males, 39 females; 5 to 9 years old) with severe or profound bilateral prelocutive deafness but without neurological or cognitive impairment. Of these, 25 had received an early cochlear implant (CIm). Patients were compared with 40 children with normal hearing. All were given a battery of non‐verbal neuropsychological tests and a balance test, and were timed for simple and complex movement of limbs. Deafness, whether treated by CIm or not, resulted in a delay in development of complex motor sequences and balance. Lack of auditory input was also associated with lower, but non‐pathological, scores in visual gnoso‐praxic tasks and sustained attention. Such differences were not observed in children with CIm. Hearing contributes to clinical development of spatial integration, motor control, and attention. An early CIm enables good verbal development and might also improve non‐verbal capacities.
Sonographic appearance of the normal and abnormal insula of Reil
Authors: Govaert Paul , Swarte Renate , De Vos Anniek , Lequin Maarten
Published: July 2010

Three planes are discerned during ultrasonographic screening of the insula in parasagittal view: opercular, insular, and fissural. Six newborn infants with normal brain anatomy, including two each of 28, 34, and 40 weeks’gestation, were selected for a description of the evolution of these parasagittal planes. Opercularization of the insula begins to be detected on a sonogram at about the 24th gestational week and progresses cranially. On coronal section the insular space forms a shallow groove at 24 weeks, becoming a slit at 28 weeks that grows longer and develops branches after 32 weeks. At 28 weeks the ascending anterior branch of the circular groove and the lateral fissure at the bottom of the insula are clearly seen in parasagittal section. Subsequent change consists of undulation and bifurcation of the lateral fissure, together with elongation of the anterior margin. Secondary gyri become visible in the insular dome between 28 and 34 weeks, forming short anterior and long posterior insular gyri. Five term newborn infants with perisylvian polymicrogyria were observed with both neonatal ultrasound and magnetic resonance imaging: two of unknown cause, and one each due to cytomegalovirus, bifunctional peroxisomal protein deficiency, and monozygous twinning. With polymicrogyria, on parasagittal sonographic examination at fissural level the anterior margin of the circular groove is a short rudiment. In insular view, only rudimentary sulci ascend from the lateral fissure; short and long gyri are not seen. In opercular view, no secondary branches are seen from the lateral fissure, an image akin to that seen at 28 weeks. A complete insular triangle is not recognized in any of these sections. A deep abnormal sulcus may prolong the lateral fissure when schizencephaly is associated with polymicrogyria. The insula in glutaric aciduria type II has no secondary gyri at term and is reduced to a simple and small triangle. Not all instances of polymicrogyria lead to macroscopically recognizable alteration of insular gyri.
Cognitive competence at the onset of West syndrome: correlation with EEG patterns and visual function
Authors: Randò Teresa , Baranello Giovanni , Ricci Daniela , Guzzetta Andrea , Tinelli Francesca , Biagioni Enrico , La Torre Giuseppe , Epifanio Roberta , Signorini Sabrina , Fazzi Elisa , Mercuri Eugenio , Cioni Giovanni , Guzzetta Francesco
Published: July 2010

The aim of this study was to evaluate cognitive development at the onset of West syndrome (WS) with regard to electroencephalogram (EEG) patterns and visual function. Twenty‐five patients (14 males, 11 females) at the onset of spasms (T0) in WS and 2 months later (T1) underwent a full clinical evaluation, including neuroimaging, cognitive assessment, video‐EEG, and visual function. Mean age of the patients at spasm onset was 5.9 months (SD 2.5; range 2 to 13mo). Cognitive development, assessed with Griffiths Mental Development Scales (GMDS), was generally impaired at T0 and was significantly related to visual function (p<0.001) at both T0 and T1 In general, there was a specific major impairment in the eye–hand coordination scale of the GMDS which tended to disappear after 2 months in less severe cases. At the onset of spasms, sleep EEG organization seemed to be better related to cognitive abilities than awake hypsarrhythmia. These results support a close link between visual function and cognitive competence in WS and provide additional information to improve the understanding of possible mechanisms underlying cognitive impairment.
Post‐streptococcal autoimmune disorders of the central nervous system
Authors: Dale Russell C
Published: July 2010

Group A Streptococcus can induce autoimmune disease in humans with particular involvement of the heart, joints, and brain. The spectrum of post‐streptococcal disease of the central nervous system (CNS) has been widened recently and includes movement disorders (chorea, tics, dystonia, and Parkinsonism), psychiatric disorders (particularly emotional disorders), and associated sleep disorders. Neuroimaging and pathological studies indicate that the most vulnerable brain region is the basal ganglia. The immunopathogenesis of the disease is incompletely defined, and although there is some support for autoantibody‐mediated disease, several conflicting studies cast doubt on the autoantibody hypothesis. It has been speculated that post‐streptococcal autoimmunity has a role in common neuropsychiatric disease but the evidence is conflicting and routine screening of patients with Tourette syndrome and obsessive‐compulsive disorder for post‐streptococcal autoimmune abnormalities is not be recommended at present. However, post‐streptococcal disorders of the CNS remain a useful model of neuropsychiatric disease, which may improve our understanding of abnormal movements and behaviours in children.
Attention deficits in children with 22q.11 deletion syndrome
Authors: Niklasson Lena , Rasmussen Peder , Óskarsdóttir Sólveig , Gillberg Christopher
Published: July 2010

This study examined attention abilities of children with 22q.11 deletion syndrome. Thirty children (14 males, 16 females; age range 7 to 13y) were given comprehensive neuropsychological and neuropsychiatric assessments. Learning disability* was found in 13 children. Superiority in verbal over performance IQ was very common. Attention‐deficit‐hyperactivity disorder (mainly of inattentive subtype) was diagnosed in 13 children. There appeared to be a relation between low IQ and presence of autism spectrum problems. The presence of attention deficits was clearly supported by the scores on the Child Behavior Checklist and the Conners Questionnaire. On the Becker attention tests the reaction times were significantly longer in the two visual and auditory tests, indicating that the ability to sustain attention is critically impaired in this group. A tendency of inferiority on auditory compared with visual tests was noted but there were no specific problems with the focus‐execute aspect of attention.
Medical, functional, and social determinants of health‐related quality of life in individuals with myelomeningocele
Authors: Bier Jo‐Ann Blaymore , Prince Anthony , Tremont Michelle , Msall Michael
Published: July 2010

The purpose of this study was to identify factors associated with health‐related quality of life (HRQOL) in individuals with myelomeningocele. Eighteen female and 16 male patients with myelomeningocele (mean age 13y, SD 6y; range 4 to 27y) were prospectively enrolled. Twenty‐four of the patients had lesions at or above the L4 level, and 11 were wheelchair dependent. Twenty‐five patients had shunted hydrocephalus; mean IQ of the cohort on testing was 85 (SD 18; range 36 to 111). Functional independence was measured in three areas (self‐care, mobility, and social cognition) using the Functional Independence Measure for Children (WeeFIM). The Support Function Scale (social supports available to the family), the Amount of Assistance Questionnaire (assistance required for activities of daily living), and the Health Utilities Index II (HUI‐II; perceived health status measure) were completed by participants or their parents. Each patient's HRQOL was quantified using a‘feeling thermometer’, which is a plastic‘thermometer’scaled from 0 (least desirable health state) to 100 (perfect health). Results of multiple regression analysis using HRQOL as the dependent variable revealed that the Amount of Assistance Questionnaire, WeeFIM self‐care, and HUI‐II were significantly correlated with quality of life ratings (p<0.025). Maximizing functional independence should be a priority in improving HRQOL in individuals with myelomeningocele.
Book Review
Authors: Gordon Andrew M
Published: July 2010

Book Review in this Article

The Brain's Sense of Movement
The use of ergonomic spoons by people with cerebral palsy: effects on food spilling and movement kinematics
Authors: Van Dominique , Steenbergen Bert
Published: July 2010

The effects of adapted spoons on food spilling and movement kinematics (movement duration, dysfluency) were examined in people with tetraparetic cerebral palsy (CP). We varied the thickness of the spoon's handle (diameters of 1, 3, and 5cm), the substance with which the spoon was filled (sugar or water), and the size of the bowl into which the spoon had to be emptied (diameter 5 or 11cm). Ten adolescents with tetraparesis (five males, five females; mean age 17y 2mo [SD 1y 3mo]; range 14y 7mo‐18y 6mo) and 12 healthy comparison participants (four males, eight females; mean age 25y 6mo [SD 3y 0mo]; range 20y 0mo‐30y 0mo) participated. Four participants with tetraparesis could walk independently, while the other six sat unstrapped in a wheelchair. The task consisted of picking up and transporting the filled spoon and emptying it into a bowl. Movements of the hand were recorded by a three‐dimensional motion tracking device. When the spoon's handle was thickened participants with CP were faster in picking up the spoon and transporting it to the bowl, in particular when the spoon was filled with water. Importantly, this faster performance did not lead to increased spilling. These results are the first systematic test of the supposed beneficial effect of adapted spoons on movement performance in people with CP.
Reliability of the V‐scope system in the measurement of arm movement in children with obstetric brachial plexus palsy
Authors: Bialocerkowski Andrea E , Wrigley Tim , Galea Mary
Published: July 2010

This study reports on a novel methodology using the V‐scope to quantify elbow and shoulder movement in young children with obstetric brachial plexus palsy (OBPP), and the intra‐and interreliability of this method. The V‐scope, a portable, inexpensive movement analysis system, was configured in an L‐shape, with two transmitting towers placed on the floor and one 1.35m off the ground. These towers received ultrasonic pulses from buttons that were placed over standardized landmarks of the child's trunk, chest, and upper limb. Two physiotherapists (a paediatric and a generalist) facilitated the maximum range of active elbow flexion/extension and shoulder abduction/flexion in 30 children with OBPP (18 females, 12 males; age range 6mo‐4y 7mo; mean age 2y 6mo [SD 1y 2mo]). Assessments were conducted on two occasions, one week apart. The V‐scope was found to be feasible to use by a specialist and a generalist physiotherapist, demonstrating moderate to high reliability coefficients, small measurement errors, and lack of missing data. The pediatric physiotherapist was more reliable in measuring elbow and shoulder movement compared with the generalist physiotherapist, which suggests that the same experienced, pediatric physiotherapist should assess elbow and shoulder movement across all occasions of testing.
Gross motor abilities in children with Hurler syndrome
Authors: Dusing Stacey C , Thorpe Deborah , Rosenberg Angela , Mercer Vicki , Escolar Maria L
Published: July 2010

Hurler syndrome is the most severe form of mucopolysaccharidosis type I. There is a paucity of literature reporting the gross motor abilities of children with untreated Hurler syndrome. The purpose of this case series is to describe the gross motor abilities of one male and three female children (mean age 11.4mo [SD 3.1]; range 9.5–16mo) diagnosed with Hurler syndrome. The children were assessed using the Peabody Developmental Motor Scales, 2nd edition. Gross motor delays were present in all four children at the time of assessment, and were most evident in locomotor abilities for three of the children. All four children had range of motion limitations at multiple joints. This case series provides evidence for early gross motor delays in this population, as well as evidence for specific gross motor abilities of children with untreated Hurler syndrome. It is recommended that children diagnosed with Hurler syndrome be referred to physical therapy services upon diagnosis and that physical therapists be part of the interdisciplinary team involved in the care of children with Hurler syndrome.
Development of hand‐arm bimanual intensive training (HABIT) for improving bimanual coordination in children with hemiplegic cerebral palsy
Authors: Charles Jeanne , Gordon Andrew M
Published: July 2010

Constraint‐induced (CI) movement therapy is a physical intervention that has been receiving increasing attention in pediatric rehabilitation. So far, the evidence suggests that practice associated with CI therapy may improve impaired unimanual hand function in some children with hemiplegic cerebral palsy (CP). However, CI therapy has several important limitations. Most importantly, children with hemiplegia have impairments in bimanual coordination beyond their unilateral impairments. Thus, an intervention approach to increase functional independence during activities of daily living by using both hands in cooperation is needed. Here we briefly review the etiology of hemiplegic CP, describe studies of pediatric CI therapy efficacy in relation to the etiology, discuss the conceptual and practical limitations of CI therapy for this population, and describe bimanual coordination impairments in children with hemiplegia. Finally, we introduce a new intervention for children with hemiplegia, hand‐arm bimanual intensive training (HABIT), to address the limitations of CI therapy and to improve bimanual coordination. HABIT retains the two major elements of pediatric CI therapy (intensive structured practice and child‐friendliness). The proposed methodology demonstrates that extensive targeted practice can be provided in a child‐friendly manner without using a physical restraint, although the efficacy of such an approach remains to be determined.
Terminology used in research reports of developmental coordination disorder
Authors: Magalhães Lívia C , Missiuna Cheryl , Wong Shirley
Published: July 2010

A systematic search was performed of all articles from January 1995 to December 2005 published in peer‐reviewed journals on children with developmental coordination disorder (DCD). Criteria were applied to ensure that articles included DCD so 319 articles were included in the analysis. Since the publication of a systematic search conducted in 1994, the number of publications in this field has greatly increased across countries and disciplines. The term DCD was used in 52.7% of the articles. Other terms were used less frequently: clumsy children (7.2%), developmental dyspraxia (3.5%), handwriting problems (3.1%), hand‐eye coordination problems (2.8%), sensory integration dysfunction (2.5%), deficits in attention, motor control, and perception (2.5%), minor neurological dysfunction (2.2%), and several other scattered terms (23.5%). The data indicated that progress has been made in the usage of the term DCD, but a standardized approach has not yet been achieved. Without consistent use of the term DCD, there is limited communication of research results internationally, slowed progress in understanding the condition, and limited development of intervention and management programmes for children with DCD.
Developmental dissociation, deviance, and delay: occurrence of attention‐deficit‐hyperactivity disorder in individuals with and without borderline‐to‐mild intellectual disability
Authors: Voigt Robert G , Barbaresi William J , Colligan Robert C , Weaver Amy L , Katusic Slavica K
Published: July 2010

A neurodevelopmental model for understanding the spectrum of developmental disabilities postulates that developmental delay, dissociation, and deviance reflect underlying central nervous system dysfunction. In this study, we determined the occurrence of the dissociated and developmentally deviant behavioral profile of attention‐deficit‐hyperactivity disorder (ADHD) among children with and without developmental cognitive delays (borderline‐to‐mild intellectual disability) in a population‐based birth cohort (n=5718). Among the 70 children identified to have borderline‐to‐mild intellectual disability, 30% (n=21; 7 females, 14 males) met research criteria for ADHD, compared to only 6.4% of children without intellectual disability (n=358; 88 females, 270 males). The odds ratio for comorbid ADHD for children with versus without borderline‐to‐mild intellectual disability was 6.3 (95% confidence interval: 3.7–10.6; p < 0.001). These data illustrate that developmental dissociation and/or deviance, as represented by ADHD, are more likely to occur in the context of developmental delay, represented by borderline‐to‐mild intellectual disability.
Magnetic resonance spectroscopic characteristics of glutaric aciduria type II
Authors: Kemal Ahmet , Karakas Hakki Muammer , Yakinci Cengiz
Published: July 2010

Magnetic resonance spectroscopy (MRS) of a 12‐year‐old female patient with glutaric aciduria type II was compared with data obtained from four healthy age‐ and sex‐matched volunteers. In the clinically active phase, conventional magnetic resonance imaging showed mild ventricular dilatation. Frontal lobe choline/creatine (Cho/Cr) ratio (1.98) was higher than the ratios reported for the comparison participants (1.64 [SD 0.21]). The N‐acetylaspartate/creatine (NAA/Cr) ratio (1.95) was lower than normal limits (2.66 [SD 0.23]). After successful riboflavin treatment and dietary restriction for proteins, the NAA/Cr ratio was within the normal range (2.44) and Cho/Cr ratio was below the normal range (1.15), suggesting riboflavin‐responsive multiple acyl‐coA dehydrogenase deficiency. An elevated Cho/Cr ratio and decreased NAA/Cr ratio is consistent with a demyelinating process in the active phase of glutaric aciduria type II. MRS helps to monitor the progress of the disease and the efficacy of treatment by revealing changes in NAA/Cr and Cho/Cr ratios.
The effectiveness of passive stretching in children with cerebral palsy
Authors: Pin Tamis , Dyke Paula , Chan Michael
Published: July 2010

Passive stretching is widely used for individuals with spasticity in a belief that tightness or contracture of soft tissues can be corrected and lengthened. Evidence for the efficacy of passive stretching on individuals with spasticity is limited. The aim of this review was to evaluate the evidence on the effectiveness of passive stretching in children with spastic cerebral palsy. Seven studies were selected according to the selection criteria and scored against the Physiotherapy Evidence Database scale. Effect size and 95% confidence intervals were calculated for comparison. There was limited evidence that manual stretching can increase range of movements, reduce spasticity, or improve walking efficiency in children with spasticity. It appeared that sustained stretching of longer duration was preferable to improve range of movements and to reduce spasticity of muscles around the targeted joints. Methods of passive stretching were varied. Further research is required given the present lack of knowledge about treatment outcomes and the wide use of this treatment modality.
Reliability of the Manual Ability Classification System for children with cerebral palsy
Authors: Morris Christopher , Kurinczuk Jennifer J , Fitzpatrick Raymond , Rosenbaum Peter L
Published: July 2010

The aim of this study was to determine the reliability of family and professional assessment of manual ability using the Manual Ability Classification System (MACS) for children with cerebral palsy (CP) in the UK. Families who were taking part in a study measuring the activities and participation of children with CP were invited to classify their child's manual ability using the MACS. Postal surveys were conducted with the families and health professionals nominated by the families. Perfect agreement was assessed as a percentage; chance‐corrected agreement was measured using Cohen's kappa (κ), and reliability was determined using the intraclass correlation coefficient (ICC). Families of 91/128 (71%) children responded to the survey (53 males, 38 females; mean age 9y 11mo [SD 1y 11mo], range 6–12y) out of whom 88 indicated a single MACS level. Seventy‐two children (82%) were classified with spastic C P, 12 (14%) with dyskinesia, two (2%) with ataxia, and two (2%) were not classified. There were 21, 27, 11, 10, and 19 children who were classified by their families in Gross Motor Function Classification System Levels I to V respectively; 14, 30, 18, 13, and 13 children classified by their families in MACS levels I to V. The survey of health professionals generated 60/71 (85%) responses from physiotherapists, 55/58 (93%) responses from paediatricians, and 21/24 (88%) responses from occupational therapists. There was perfect agreement between families and professionals for more than 50% of children; the indices of chance‐corrected agreement ranged from κ=0.3 to 0.5, and the reliability coefficients ranged from ICC 0.7 to 0.9. Indices of agreement and reliability between families and professionals were equivalent to those between different professionals. The MACS, therefore, offers a valid and reliable method for communicating about the manual ability of children with C P. Families and professionals may not always agree precisely on a MACS level, particularly if children's performance of manual tasks varies in different environments.