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Infants with congenital heart disease are at risk of impaired neurodevelopment, which frequently manifests as motor delay during their first years of life. This delay is multifactorial in origin and environmental factors, such as a limited experience in prone, may play a role. In this study, we evaluated the motor development of a prospective cohort of 71 infants (37 males) with congenital heart disease at 4 months of age using the Alberta Infant Motor Scales (AIMS). We used regression analyses to determine whether the 4-month AIMS scores predict the ability to walk by 18 months. The influence of demographic and clinical variables was also assessed. Fifty-one infants (71.8%) were able to maintain the prone prop position (AIMS score of ≥3 in prone) at 4 months. Of those, 47 (92.2%) were able to walk by 18 months compared to only 12/20 (60%) of those who did not maintain the position. Higher AIMS scores were predictive of a greater likelihood of walking by 18 months ( P < .001), with the scores in prone having a higher predictive ability compared to those in other positions (Exp(B) 15.2 vs 4.0). Shorter hospital stays and female gender were also associated with an earlier onset of walking. In conclusion, our study demonstrates that early ventral performance in infants with congenital heart disease impacts the age of acquisition of walking and could be used to guide referral to rehabilitation.

Purpose: The purpose was to examine whether children diagnosed with motor problems in the preschool period still exhibit motor problems at school age and the impact of these difficulties on participation. Method: The study group comprised 60 children 7-12 years old who were referred to occupational therapy 4-6 years prior to study initiation due to motor difficulties. The control group comprised 28 age-matched children with typical development. Parents completed the Participation, Developmental Coordination Disorder and Performance Skill Questionnaire 4-6 years after treatment. Results: Significant differences were found between children with and without motor difficulties in motor function, but not in any of the participation domains except for parental satisfaction. Children in the control group had significantly higher scores than children with motor difficulties in motor and process skills. Conclusions: Motor difficulties persist into school years. Children with motor difficulties manifest performance skill problems, however they succeed in narrowing the gap and participate similar to their peers.

This study aimed to investigate the integrity of on-line control of reaching in congenital spastic hemiplegia in light of disparate evidence. Twelve children with and without spastic hemiplegia (11-17 years old) completed a double-step reaching task requiring them to reach and touch a target that remained stationary for most trials (viz nonjump trial) but unexpectedly displaced laterally at movement onset for a minority of trials (20%: known as jump trials). Although children with spastic hemiplegia were generally slower than age-matched controls, they could account for target perturbation at age-appropriate levels shown by a lack of interaction effect on movement time and nonsignificant group difference for time to reach trajectory correction on jump trials. Our data suggest that at a group level, on-line control of reaching may be age-appropriate in spastic hemiplegia. However, our data also highlight the need to experimentally acknowledge the considerable heterogeneity of the spastic hemiplegia population when investigating motor cognition.

We report the first Palestinian child manifesting with 3-methylglutaconic aciduria psychomotor delay, muscle hypotonia, sensori-neural deafness, and Leigh-like lesions on brain magnetic resonance imaging (MRI), a clinical phenotype that is characteristic of MEGDEL syndrome. MEGDEL syndrome was recently found to be caused by mutations in SERAC1, encoding a protein essential for mitochondrial function, phospholipid remodeling, and intracellular cholesterol trafficking. We identified a novel homozygous mutation in SERAC1 gene (c.1018delT) that generates frame shift and premature termination of protein translation. Plasma and cerebrospinal fluid lactate, plasma alanine, and respiratory chain complexes in fresh muscle were normal. This report further expands the genetic spectrum of MEGDEL syndrome and adds to the evidence that it is associated with variable patterns of respiratory chain abnormalities.

This study aimed to investigate any differences between the motor skills and sensory processing abilities of children between the ages of 4 and 8, who do and do not have an idiopathic toe walking gait. Children in each cohort were tested with a number of norm referenced assessments. A total of 60 children participated, 30 within each cohort. Those with an idiopathic toe walking gait were found to have different Sensory Profile quadrant scores ( P = .002), poorer performance on the Bruininks–Oseretsky Test of Motor Proficiency ( P ≤ .001), a lower vibration perception threshold ( P = .001), and poorer performance on the Standing Walking Balance subtest of the Sensory Integration and Praxis Test ( P = .047), compared with non–toe walking peers. Although this research does not give a causative factor for toe walking gait, it provides a number of theories as to why this gait may not be idiopathic in nature.

Congenital muscular torticollis is a common condition, but long-term neurodevelopmental follow-up is lacking. This study reports on neurodevelopmental outcome of 68 children, aged 7 to 9 years, with a history of congenital muscular torticollis, excluding children with torticollis due to other conditions. Thirty-eight children were examined for presence of neurodevelopmental disorders. Telephone interview data were available for an additional 30 children. Of those examined, 22/38 (57.9%) had or were at risk for a developmental disorder (attention-deficit hyperactivity disorder (ADHD), developmental coordination disorder, language impairment, autistic spectrum disorder) on at least 1 of the assessments administered, 23/38 (60.5%) had received developmental treatment during childhood. One child, based on a telephone interview, had a history of developmental treatment. Therefore, 30/68 (44.1%) children of the total sample demonstrated a developmental delay/disorder, currently (22/68) or previously (8/68). Our findings suggest congenital muscular torticollis to be a significant risk factor for later neurodevelopmental conditions with disorders presenting at different stages of development.

Prior case reports have identified neurodevelopmental abnormalities in children with PHACE syndrome, a neurocutaneous disorder first characterized in 1996. In this multicenter, retrospective study of a previously identified cohort of 93 children diagnosed with PHACE syndrome from 1999 to 2010, 29 children had neurologic evaluations at ≥ 1 year of age (median age: 4 years, 2 months). In all, 44% had language delay, 36% gross motor delay, and 8% fine motor delay; 52% had an abnormal neurological exam, with speech abnormalities as the most common finding. Overall, 20 of 29 (69%) had neurodevelopmental abnormalities. Cerebral, but not posterior fossa, structural abnormalities were identified more often in children with abnormal versus normal neurodevelopmental outcomes (35% vs. 0%, P = .04). Neurodevelopmental abnormalities in young children with PHACE syndrome referred to neurologists include language and gross motor delay, while fine motor delay is less frequent. Prospective studies are needed to understand long-term neurodevelopmental outcomes.

Dependent on criteria used, between 35% and 53% of the participants with cerebral palsy fulfilled the criteria of clinically relevant executive function problems as defined by Conners’ (1994) Continuous Performance Test. Executive function problems were noticed mainly in participants with bilateral brain lesions and who had been born preterm. Findings highlight the need to check for attention problems in children with cerebral palsy.

The specific aims of this study were to collect and analyze detailed symptom descriptions from patients with pediatric restless legs syndrome, ages 6 to 17 years, as well as assess symptom impact and the usefulness of drawings. Trained qualitative interviewers conducted face-to-face audio-recorded interviews of children and adolescents who met criteria for definite restless legs syndrome. Thirty-three patients in 3 age groups used 16 different categories of descriptors for restless legs sensations, with a mean of 3 or more categories used per patient in each age group. “Need to move/kick,” “pain/hurts,” “uncomfortable/cannot get comfortable,” and “like bugs or ants/crawling” were the most common descriptors. Two-thirds reported daytime sensations, and nearly half had arm involvement. They described impact on sleep, cognitive function, and affect. Drawings provided useful diagnostic information. These detailed empirical data will be useful in clinical practice, as well as in the development of formal diagnostic tools and severity measures.

Developmental coordination disorder affects 5% to 8% of the general population, and about 50% to 60% of these children have a comorbid attention-deficit disorder with hyperactivity and learning disorders. Left-handedness is relatively common among children with dyslexia, learning disabilities, and autism; however, its frequency in children with developmental coordination disorder is less clear. The present study investigated the distribution of hand dominance in 98 children (age range, 5.5-17 years) with developmental coordination disorder compared with their parents or siblings. Thirty children (30.6%) were left-handed and 13 (13.3%) were ambidextrous. The prevalence of left-handedness among their parents and siblings was similar to that of the general population. The results suggest that children with developmental coordination disorder, like children with learning disorders and deficit disorder with hyperactivity, present with higher frequency of left-hand dominance compared with the general population.

When reaching, children with dystonia exhibit movements that are slower and more variable than normal children. We hypothesize that in dystonia there is an increase in signal-dependent noise so that there is increased variability with increasing speed. This hypothesis predicts that slower movement in children with dystonia is at least partly due to a compensatory strategy to reduce variability by decreasing speed. To test this hypothesis, we measured the speed of arm movement while children attempted to contact buttons of different sizes. We tested 23 control children and 15 children between the ages of 4 and 16 years with dystonia owing to either cerebral palsy, idiopathic dystonia not due to the DYT1 (torsin A) mutation, or other identified causes. A consistent inverse relationship between movement time and button size was seen for both the control children and the children with dystonia. The variance of movement speed increased with the average speed for all subjects. Children with dystonia moved significantly more slowly at all button sizes, and their movement speed was more sensitive to changes in button size. Therefore, part of the reduction in speed in dystonia is due to relatively greater difficulty in contacting small targets. This finding is consistent with the hypothesis of increased signal-dependent noise in children with dystonia, and we present a simple computational model that provides a possible explanation for the origin of this noise. ( J Child Neurol 2005;20:489—496).

Pelizaeus-Merzbacher disease is a rare X-linked disease characterized by defective central nervous system myelination owing to a mutation in the proteolipid protein 1 gene. Few studies report detailed clinical findings in children with genetic confirmation of mutations in the proteolipid protein 1 gene. We reviewed the records of 10 boys with Pelizaeus-Merzbacher disease and one symptomatic carrier girl. Their median age was 2½ years (range 10 months to 20 years). Nine had proteolipid protein 1 gene duplications, one had a point mutation, and one had a single codon deletion. The families of eight patients reported perinatal complications, including maternal hypertension (three patients) and meconium aspiration (three patients). All of the patients were social and interactive, but all had difficulty with expressive speech. All patients presented with nystagmus and had hypotonia that progressed to spasticity, affecting the legs more than the arms; ataxia also contributed to motor impairment. Additional problems reported regarded feeding (eight patients) and sleep (three patients). Further work is needed to clarify the variations in disease course and the relationship of genotype to phenotype. (J Child Neurol 2004;19:328-331).

Children with impaired motor coordination (or developmental coordination disorder) have difficulty representing internally the visuospatial coordinates of intended movements. We have proposed that this deficit reflects impairment in the generation of forward models of the efference copy of intended movements―the efference-copy-deficit hypothesis. In this study, we challenged this hypothesis by examining the efficacy of an imagery intervention designed specifically to train the forward modeling of purposive actions. A pre-post design was adopted. Fifty-four children referred with motor coordination problems were assigned randomly to one of three groups: imagery training, traditional perceptual-motor training, and wait-list control. The imagery protocol―delivered by an interactive CD―ROM―was shown to be equally effective to perceptual-motor training in facilitating the development of motor skill in the referred children. These results support the efference-copy-deficit hypothesis in explaining the cause of motor clumsiness in most children. Directions for future intervention studies and remediation in the field of developmental clumsiness are discussed. (J Child Neurol 2002;17:491-498).

The aim is to assess repetition-based learning of procedures in children with developmental coordination disorder (DCD), reading disorder (RD) and attention-deficit hyperactivity disorder (ADHD). Participants included 187 children, studied in 4 groups: ( a) DCD comorbid with RD and ADHD (DCD+RD+ADHD) (n = 30); ( b) RD comorbid with ADHD (RD+ADHD) (n = 48); ( c) ADHD (n = 19); and typically developing children (control group) (n = 90). Two procedural learning tasks were used: Assembly learning and Mirror drawing. Children were tested on 4 occasions for each task: 3 trials were consecutive and the fourth trial was performed after an interference task. Task performance by DCD+RD+ADHD children improved with training ( P < .05); however, the improvement was significantly lower than that achieved by the other groups (RD+ADHD, ADHD and controls) ( P < .05). In conclusion, children with DCD+RD+ADHD improve in their use of cognitive-motor procedures over a short training period. Aims of intervention in DCD+RD+ADHD should be based on individual learning abilities.

External hydrocephalus in an infant is a condition in which the rate of growth of head circumference exceeds the expectations of the Nellhouse curve together with increased size of the subarachnoid spaces. Developmental milestones of 20 infants (aged 0-16 months) with external hydrocephalus were studied by the Mullen Scales of Early Learning. The areas of development that were studied were visual reception, fine motor, expressive language, receptive language, and total Mullen score. There were no significant differences between the study group and the general population in all 5 scores. The only prominent feature of our external hydrocephalus patients was hypotonia (transient in 9 [45%] and persistent in 2 [10%]). Although the term external hydrocephalus describes a roentgenographic presentation, the term benign enlargement of subarachnoid space is preferred in infants similar to our group by better describing the benign nature of the phenomenon in appropriately selected infants.

The prognosis for recovery from brachial plexus injury sustained at or before birth is generally favorable. However, roughly 10% of these infants remain profoundly weak and later exhibit functional disability in the affected arm. Early identification of these at-risk infants would be helpful in selecting patients for surgical management. In our prospective study, 80 infants with brachial plexus injury were examined on a monthly basis. Complete recovery occurred in 53 (66%); in 9 (11%), mild weakness persisted. In each child, recovery to antigravity strength in the biceps, triceps, and deltoid was noted by 6 months of age. Moderate arm weakness persisted in 7 children (9%); none had antigravity strength in the deltoid at age 6 months. Eleven children (14%) had severe permanent weakness (mean follow-up: 4.4 years). At age 6 months, these individuals exhibited at best 2/5 strength proximally and typically 0-1/5 strength in the wrist and finger extensors. Our results demonstrate that detailed strength testing up to 6 months of age predicts not only complete recovery of neonatal brachial plexus injury but also those children destined for long-term severe disability. (J Child Neurol 2001;16:488-492).

Because of inadequate response to or intolerable side effects of oral medication, nine patients with segmental, generalized, and focal myoclonus were treated with intramuscular botulinum toxin type A. All patients were evaluated with neuroimaging, routine and limb-monitored electroencephalography, electromyography, evoked potentials and appropriate biochemical studies. Patients were aged 2 to 22 years, with duration of myoclonus from 1 month to 10 years. Multiple medication trials included antiepileptic drugs, benzodiazepines, tryptophan, L-dopa/carbidopa, baclofen, and dantrolene. Patients were injected with botulinum toxin in their affected area with electromyographic guidance to affected muscles with different doses (8 to 20 units/kg), except two patients who were injected with 32 and 45 units/kg, respectively, at 4- to 8-month intervals. One patient did not complete botulinum toxin treatment because of subjective weakness, although there were virtually no side effects reported in patients completing therapy. Patients reported a dramatic reduction in painful myoclonus. In addition, patients exhibited improved functional skills, as demonstrated by markedly improved use of affected extremities and improvements in ambulation. One patient, who was nonambulatory prior to treatment, was able to walk afterward. Long-term benefits could be related to higher dosage used or negative feedback effect. (J Child Neurol 1999;14:781-786).

We prospectively acquired clinical data regarding the presentation, evaluation, and developmental progress of all patients identified with dissociated motor maturation to define their clinical outcomes. Children ( N = 8) referred for evaluation of suspected cerebral palsy because of delayed sitting or walking and identified to have dissociated motor maturation were followed with serial clinical examination. All displayed the characteristic “sitting on air” posture while held in vertical suspension and had otherwise normal developmental assessments. This posture is composed of the hips held in flexion and abduction with the knees extended and feet plantar or dorsiflexed. Three children were initially evaluated at 10 months of age owing to absence of sitting and five other children were evaluated at a mean of 14 months (range 12—19 months) owing to inability to stand. Follow-up evaluations were conducted over a mean of 10.5 months (range 5—34 months). Five children were born prematurely at 34 to 36 weeks gestation. Denver Developmental Screening Test and general and neurologic examinations were normal except to note hypotonia in six children and the “sitting on air” posture in all of the children. Four children have older siblings or parents who “walked late” (after 15 months). On average, the children attained sitting by 8 months (range 7—10 months). One child did not crawl prior to independent walking, two children scooted rather than crawled, and five children crawled at an average of 13.5 months (range 10—16 months). All children cruised by a mean of 18 months (range 16—21.5 months) and attained independent walking by 20.1 months (range 18—23.5 months). Neuroimaging and serum creatine kinase enzyme testing were normal in two children who were tested. These eight children conform to the syndrome of dissociated motor maturation. The “sitting on air” posture serves as a diagnostic sign and anticipated excellent prognosis, but follow-up is required to ensure a normal outcome. ( J Child Neurol 2003;18:391—393).

Our objective was to examine the association of attention deficit and disorganization in boys with and without specific developmental disorder of motor function. Four groups of boys between the age of 7 and 12 years—(1) Disorganization + coordination disorder (n = 30); (2) Coordination disorder (n = 33); (3) Disorganization (n = 28); and (4) Control (n = 29)—were included. Teachers completed the Questionnaire for Assessing the Students’ Organizational Abilities for the Teacher and the Conners’ Teachers Rating Scale–Revised. The Movement Assessment Battery for Children and 2 subscales of an intelligence test (vocabulary and similarities) were administered. A significantly increased rate of attention deficit in children with organizational deficit was identified. Attention deficit in children with specific motor disorder was exclusively associated with an organizational deficit. Organizational deficit in childhood is highly associated with attention deficit, and this association is particularly relevant in children with specific coordination disorder.

A retrospective cohort study was conducted to describe the relationship between gross motor function and manual ability in children with cerebral palsy and explore differences between cerebral palsy subtypes and associated comorbidities. Children with cerebral palsy born between 1999 and 2008 were included from the Registre de la Paralyse Cérébrale de Québec identifying 332 children. The overall agreement between Gross Motor Function Classification System and Manual Ability Classification Scale Levels was moderate (kappa 0.457, standard error 0.034) with a strong positive correlation (Spearman rho of 0.820, standard error 0.023). This agreement was moderate among children with spastic quadriparesis and dysketic cerebral palsy, fair in children with spastic diplegia, and poor in children with spastic hemiplegia. Children with cognitive impairment showed a higher correlation than those without cognitive impairment. The correlation between gross motor function and manual ability in children with CP varies based on neurologic subtype and cognitive level.

Developmental coordination disorder affects a relatively large proportion (5%-6%) of the childhood population. Severity of the disorder varies but there is a great need for therapeutic intervention. We propose a method for the training of manual actions in children with developmental coordination disorder. Our solution is achieved by applying haptic virtual reality technology to attack the difficulties that children with developmental coordination disorder evidence. Our results show that children with developmental coordination disorder are able to learn complex motor skills if proper training methods are employed. These findings conflict with reports of impaired motor learning in developmental coordination disorder because of underactivation of cerebellar and parietal networks.

Weakness resulting from spinal muscular atrophy causes severe limitations in functional mobility. The early introduction of power mobility has potential to enhance development and mitigate disability. These outcomes are achieved by simulating normal skill acquisition and by promoting motor learning, visuospatial system development, self-exploration, cognition, and social development. There are few reports on early power mobility in spinal muscular atrophy, and it is typically not prescribed until school age. The authors evaluated 6 children under age 2 years with neuromuscular disease (5 spinal muscular atrophy, 1 congenital muscular dystrophy) for power mobility. Parents recorded the practice hours necessary to achieve independence using the Power Mobility Skills Checklist. Four children achieved independence in all items on the checklist by 7.9 months (range: 73-458 days). Introduction of early power mobility is feasible in spinal muscular atrophy patients under age 2 years and should be introduced in late infancy when children typically acquire locomotor skills.

Abilities among school-aged children with cerebral palsy with different patterns and levels of motor function were evaluated. Children within spasticity patterns (33 with quadriplegia, 25 with hemiplegia, 19 with diplegia) and Gross Motor Function Classification System levels were compared (level I, walking = 47%; level II-III, restricted ambulation = 18%; level IV-V, wheelchair needs = 34%,). Outcomes included measures of intelligence, behavior, motor, and functional limitations (communication, daily living, socialization). Motor performance and prosocial behaviors were lower for children with quadriplegia (F = 16.13, 12.71; P < .0001), with no differences for behavioral difficulties between spasticity groups. Prosocial behaviors were different between level IV-V and other groups (F = 16.25, P < .0001). Functional limitations were more likely for children with quadriplegia (P < .0001), but not diplegia or hemiplegia, and for children in level IV-V, but similar for level I and level II-III. Children with quadriplegia, or level IV-V, are more likely to exhibit limitations, whereas children with better motor function (I-III), hemiplegia, or diplegia, exhibit diverse capabilities. A holistic assessment approach is essential to ensure that limitations are addressed comprehensively.

Studies have suggested disparate variables affecting long-term outcomes in patients with infantile spasms. Using a retrospective chart review, the authors identified 109 patients who had follow-up data for at least 1 year since the onset of spasms. Patient and treatment variables were recorded, in addition to neurodevelopmental and seizure outcomes. Etiology was strongly associated with motor and cognitive status but not with long-term seizure control. Lag time to initiation of treatment was not predictive of any outcome, nor for need to use a second agent to resolve spasms, even when controlling for etiology. However, patients who responded to the first medication achieved superior seizure and cognitive outcomes. The delayed impact of individual medications could not be analyzed because many patients received multiple agents. While etiology and response to first medication predict better outcomes, the majority of patients with infantile spasms continue to have epilepsy with long-term motor and cognitive disabilities.

Affecting 5% to 6% of school-age children, developmental coordination disorder is characterized by a marked impairment of motor coordination that significantly interferes with activities of daily living and academic achievement. Little is known about the etiology of developmental coordination disorder, but the disorder often coexists with attention-deficit hyperactivity disorder (ADHD), speech/language impairment, and/or reading disability. This comprehensive review examines the literature supporting or refuting hypothesized neural correlates of developmental coordination disorder and suggests directions for future research. Potential sources of neuropathology include the cerebellum, parietal lobe, corpus callosum, and basal ganglia. Comorbidities and deficits associated with developmental coordination disorder are highly suggestive of cerebellar dysfunction; yet, given the heterogeneity of this disorder, it is likely that the cerebellum is not the only neural correlate. Neuroimaging studies and behavioral investigations of learning-related change in motor behavior are the next critical step in enhancing our understanding of developmental coordination disorder.

Isolated cerebral folate deficiency was detected in a 13-year-old girl with cognitive and motor difficulties and juvenile rheumatoid arthritis. Her serum contains autoantibodies that block membrane-bound folate receptors that are on the choroid plexus and diminish the uptake of folate into the spinal fluid. Whereas her serum folate exceeded 21 ng/mL, her spinal fluid contained 3.2 ng/mL of 5-methyltetrahydrofolate as a consequence of the autoantibodies diminishing the uptake of this folate.

The later developmental trajectory of young children diagnosed early with global developmental delay was determined. Using a prospective study, preschool children diagnosed with global developmental delay were systematically reassessed during the early school years with standardized developmental and functional outcome measures (Battelle Developmental Inventory and Vineland Adaptive Behavior Scale). Of an original cohort of 99 children assessed and diagnosed at a mean age of 3.4 ± 1.1 years, 48 were reassessed at a mean age of 7.3 ± 0.9 years. Group performance on the Battelle Developmental Inventory overall was 66.4 ± 4.3 (mean 100 ± 15). Between 75% and 100% of the cohort performed at least 1.5 SD below the normative mean on the individual domains of the Battelle Developmental Inventory. Similarly, the group mean on the Vineland Adaptive Behavior Scale overall was 63.5 ± 20.8 (mean 100 ± 15), with between 61% and 76% of the cohort scoring more than 1.5 SD below the mean on each of the domains. Univariate and multivariate analyses on potential predictor variables identified a lack of an underlying etiology as predictive of poorer performance on the Battelle Developmental Inventory fine motor and motor domains and increasing severity of initial delay as predictive of poorer performance on the Vineland Adaptive Behavior Scale communication domain and overall score. Similarly, maternal employment and paternal postsecondary education improved Vineland Adaptive Behavior Scale communication scores, whereas paternal postsecondary education alone predicted better socialization and total scores on the Vineland Adaptive Behavior Scale. Children with early global developmental delay demonstrate persistent and consistently poor performance across all developmental and functional domains. Few variables are apparent at intake to predict later performance. ( J Child Neurol 2005;20:648—654).

Fifteen posthemispherectomy children were examined to assess residual motor function of the paretic side using the 74-point Fugl-Meyer Assessment of Motor Recovery scale. The degree of residual motor control differed for upper and lower extremities, with hand function being most severely impaired. Posthemispherectomy motor outcomes also differed as a function of etiology: cortical dysplasia, perinatal infarct, and Rasmussen's encephalitis. Children whose intractable seizures resulted from perinatal middle cerebral artery stroke demonstrated the most spared motor function. To detect cortical areas that represented motor control of the hemiparetic side, we focused on voluntary control of the affected lower extremity. Seven of our patients were able to carry out a foot dorsiflexion paradigm during functional magnetic resonance imaging, and these results were compared with activations found in normal controls. All children showed activations in the sensorimotor network ipsilateral to the affected side. The perinatal infarct group demonstrated greater activity in the cingulate cortex, whereas the Rasmussen's encephalitis group had significant activations in the insula, suggesting etiology-specific differences in reorganization. These findings are discussed in the framework of our understanding of mechanisms of cortical plasticity in the injured brain and its relevance to neurologic rehabilitation. We suggest that imaging techniques are important tools in identifying cortical regions underlying functional reorganization. Furthermore, detection of such areas might become a basis for specific training promoting the optimal reorganization of cortical networks to enhance motor control. ( J Child Neurol 2005;20:64—75).

The spectrum of clinical disease in juvenile Huntington's disease differs from that seen in adults. Younger patients often present with seizures, dystonia and rigidity. The mechanism and type of seizures, timing of onset and electrographic features have not been well characterized in either adults or children. We describe the electroencephalographic findings observed in a young child with Huntington's disease who presented with motor regression and seizures. Recordings demonstrated bilateral posterior quadrant epileptiform discharges and occipital intermittent rhythmic delta activity, generally considered a nonspecific abnormality. These findings have not been reported in adult or juvenile Huntington's disease. Its presence in proximity to bilateral posterior spikes suggests that occipital intermittent rhythmic delta activity is an epileptiform abnormality, on a continuum with the posterior spike-and-wave discharges. Intermittent rhythmic delta activity can occur secondary to either the subcortical gray-matter disease or the associated seizure disorder. Huntington's disease should be considered in the differential diagnosis of young children who present with seizures and developmental regression.

The purpose of this study was to clarify the neurophysiologic basis of oromotor deficits in a patient with atypical rolandic epilepsy. We investigated magnetoencephalographic analysis of rolandic discharges with right predominance before and during clonazepam therapy. Before clonazepam administration, current sources of rolandic discharges were broadly distributed in the secondary sensory cortex, superior temporal gyrus, and parietal association area in addition to hand and orofacial division of the primary somatosensory cortex. During clonazepam therapy, oromotor deficits were improved, along with a decrease in rolandic discharge, and current sources of residual right-sided rolandic discharges were shifted to the right superior parietal lobule. Taking the clinical course and magnetoencephalographic findings together, the distributed rolandic discharge focus might be closely related to oromotor deficits, and clonazepam was effective for the disorder. ( J Child Neurol 2004;19:456-459).

Residual hand use in functional tasks, extent of laterality, and right or left preference were studied in 145 2- to 24-year-old, postregression Australian subjects with Rett syndrome via parent questionnaire. Hand use was markedly restricted, more for complex than simple and for external (touching food and objects) than internal tasks (scratching, rubbing eyes), suggesting a deficit in cerebral control of external, goal-oriented hand use, which is perhaps genetically determined because there is significantly greater restriction of external tasks in subjects with demonstrated MECP2 mutations. Overall, 33.6% of patients were reported with a left-hand preference, 40.7% with a right-hand preference, and 25.7% with an equal hand preference. Extent of laterality was greater for external than internal and for complex than simple external tasks. Older subjects showed less functional hand use and possibly more overall laterality. However, their hand preference was similar to younger subjects. The anomalous pattern of hand preference in Rett syndrome may be linked to the primary apraxic deficit in this disorder rather than to late manifestation of laterality. ( J Child Neurol 2003;18:481-487).

This prospective study was designed to characterize the neurodevelopmental and cognitive difficulties specific to children with intrauterine growth retardation and to detect early clinical predictors of these difficulties. Eighty-one children with intrauterine growth retardation were monitored up to 6 to 7 years of age using biometric parameters, perinatal risk questionnaires, and detailed neurodevelopmental and cognitive assessments. Forty-one children served as age-matched, appropriate for gestational age controls. A significant difference in growth parameters (P < .001), neurodevelopmental score (P < .05), and IQ (P < .05) was found between the children with intrauterine growth retardation and controls. A specific profile of difficulties in coordination, lateralization, spatial and graphomotor skills, and abundance of associated movements is typical of the children with intrauterine growth retardation and hints at possible later learning disabilities. The clinical parameters best predicting neurodevelopmental outcome were the neonatal risk score (P < .05) and the weight and height at 6 years of age (P < .05). The children with intrauterine growth retardation with neonatal complications had lower neurodevelopmental scores than the controls but no difference in IQ. Intrauterine growth retardation children diagnosed prenatally had the same neurodevelopmental and IQ scores as those diagnosed at birth, probably due to the careful perinatal and obstetric care provided. Children with intrauterine growth retardation demonstrate a specific profile of neurodevelopmental disabilities at preschool age. Early diagnosis and intervention could probably reduce these difficulties to a minimum. (J Child Neurol 2000;15:781-786).

Clinical, computed tomographic (CT), and magnetic resonance imaging (MRI) correlations of subacute sclerosing panencephalitis with an atypical onset are presented in three children. In all three patients, the disease began similarly, with unilateral neurologic deficit followed by gnosis, praxis, and memory dysfunction corresponding to massive one-sided lesions. The first patient demonstrated right frontal-lobe syndrome and polymorphic extrapyramidal hyperkinesias; MRI showed a large high-signal lesion in the right frontal lobe, while CT was normal. The second patient displayed a disease onset with left-sided hemiparkinsonism and involuntary movements correlating to the MRI finding, ie, a massive right-sided occipitotemporoparietal subcortical lesion. An acute, stroke-like episode represented the first symptom in the third child. CT visualized cerebral, mainly left-sided cortical atrophy. In all three children, CT and MRI revealed significantly progressing brain atrophy at the disease's latest stages. We discuss the role of MRI in detecting early pathologic changes in children with subacute sclerosing panencephalitis. ( J Child Neurol 2000;15:258-260).

The purpose of this study is three-fold: First, to determine the levels of gross and fine motor functions in children before and after hemidecortication, using standardized measurement tools; second, to investigate if predictors of these outcomes can be characterized; and third, to explore if both clinical measures and functional magnetic resonance imaging (MRI) potentially can identify optimal timing of surgery. The Gross Motor Function Measure and the Quality of Upper Extremity Skills Test are shown to be comprehensive, standardized outcome measures of movement performance in affected children. Age at surgery and interval between age at seizure onset and age at surgery could be the most reliable predictors of clinical outcomes. Functional MRI studies are valuable in exploring the potential of the cortical reorganization that sustains residual sensorimotor function. The combination of clinical measures with functional MRI is a promising method of inquiry into developmental and plasticity processes. (J Child Neurol 1999;14:304-315).

Thirty-seven infants with severe central nervous system injury or extreme prematurity were randomly assigned to a multisensory (auditory-tactile-visual-vestibular) intervention or control group. Intervention began in the hospital at 33 weeks' postconceptional age and continued twice daily in the home until 2 months' corrected age. Mother-infant interactions during feedings were videotaped, and the Bayley Scales of Infant Development were administered. Control mothers stimulated their infants more during feeding, but these significant differences dissipated by 4 months. The presence of periventricular leukomalacia was associated with significantly poorer mental development, regardless of group assignment. Experimental infants tended to exhibit better motor and mental performance and had 23% fewer cerebral palsy diagnoses at 1 year, but these trends were not statistically significant. The type of brain injury was more important in determining 1-year developmental outcome than type of postnatal experience, suggesting that periventricular leukomalacia presents a major challenge for infant development. (J Child Neurol 2001;16:493-498).