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1.
Aim  This study examines the overlap between children with bulbar cerebral palsy (Worster–Drought syndrome [WDS]) and perisylvian polymicrogyria.
Method  A total of 121 children (81 males, 40 females; mean age 5y 5mo, SD 3y 6mo; age range 1mo–15y 4mo) were studied using retrospective clinical data and magnetic resonance imaging. In all, 70 children had WDS with normal perisylvian imaging, 31 had congenital bilateral perisylvian polymicrogyria (CBPP), and 20 had congenital unilateral perisylvian polymicrogyria (CUPP).
Results  All groups shared aetiological markers (male sex, congenital contractures, low familial incidence, excess antenatal events). There was a common phenotype of pseudobulbar palsy with mild limb pyramidal signs in all children with WDS, 90% of those with CBPP, and one-third of those with CUPP, often also associated with learning disability * , epilepsy, and behavioural difficulties. A further 15% of children with CUPP acquired this phenotype through an epileptic encephalopathy. Pseudobulbar palsy rather than polymicrogyria was more predictive of additional impairments other than epilepsy.
Interpretation  We propose that congenital perisylvian dysfunction is a spectrum encompassing the WDS phenotype and perisylvian polymicrogyria imaging abnormalities. As with other prenatal brain abnormalities, there is not necessarily concordance between imaging and clinical findings, although the phenotype is often more severe to manifest imaging abnormality. Clinical phenotype is the best indicator of prognosis. Epileptic encephalopathy can cause an acquired form of perisylvian dysfunction where there is.  相似文献   

2.
Aim To report the prevalence, clinical associations, and trends over time of oromotor dysfunction and communication impairments in children with cerebral palsy (CP). Method Multiple sources of ascertainment were used and children followed up with a standardized assessment including motor speech problems, swallowing/chewing difficulties, excessive drooling, and communication impairments at age 5 years. Results A total of 1357 children born between 1980 and 2001 were studied (781 males, 576 females; median age 5y 11mo, interquartile range 3–9y; unilateral spastic CP, n=447; bilateral spastic CP, n=496; other, n=112; Gross Motor Function Classification System [GMFCS] level: I, 181; II, 563; III, 123; IV, 82; IV, 276). Of those with ‘early‐onset’ CP (n=1268), 36% had motor speech problems, 21% had swallowing/chewing difficulties, 22% had excessive drooling, and 42% had communication impairments (excluding articulation defects). All impairments were significantly related to poorer gross motor function and intellectual impairment. In addition, motor speech problems were related to clinical subtype; swallowing/chewing problems and communication impairments to early mortality; and communication impairments to the presence of seizures. Of those with CP in GMFCS levels IV to V, a significant proportion showed a decline in the rate of motor speech impairment (p=0.008) and excessive drooling (p=0.009) over time. Interpretation These impairments are common in children with CP and are associated with poorer gross motor function and intellectual impairment.  相似文献   

3.
Aim  To investigate whether drooling in children with cerebral palsy (CP) in general and in CP subtypes is due to hypersalivation.
Method  Saliva was collected from 61 healthy children (30 males, mean age 9y 5mo [SD 11mo]; 31 females, mean age 9y 6mo [1y 2mo]) and 100 children with CP who drooled (57 males, mean age 9y 5mo [3y 11mo], range 3–19y; 43 females, mean age 10y 1mo [4y 9mo], range 4–19y), of whom 53 had spastic, 42 had dyskinetic, and five had ataxic CP. Almost all children were affected bilaterally, and 90 of them were at Gross Motor Function Classification System levels III or higher. The saliva was collected by the swab saliva collection method. The intensity of drooling was evaluated using the drooling quotient.
Results  No difference was found in the flow rates, age, or sex between healthy children and children with CP who drooled. On additional subgroup analysis, the flow rates of children with dyskinetic CP differed statistically from those of healthy children (submandibular p =0.047, parotid p =0.040).
Interpretation  This study supports the finding in previous studies that no hypersalivation exists in children with CP who drool. Dysfunctional oral motor control seems to be responsible for saliva overflow from the mouth, whereas increased unstimulated salivary flow may occur in children with dyskinetic CP as a result of hyperkinetic oral movements.  相似文献   

4.
Nephropathic cystinosis is a genetic disorder in which the amino acid cystine accumulates in lysosomes, resulting in multiorgan dysfunction. Progressive neuromuscular dysfunction, with bulbar and upper extremity weakness, has been described in adults with this disorder. The purpose of the present study was to determine whether there was evidence of early bulbar involvement, suggested by feeding difficulties or oral motor dysfunction in these patients, and whether the feeding and oral motor problems were associated with other evidence of neurologic dysfunction. Twenty-two children and adolescents with nephropathic cystinosis were studied. Parents completed questionnaires on feeding history and oral motor problems. Eighteen patients were given an oral motor examination, and 14 received a complete neurologic examination. The majority of children had a history of feeding difficulties. Seven children required a gastrostomy tube. Abnormalities on oral motor examination included hypotonia, abnormal gag reflex, and throaty or congested voice. Abnormalities on neurologic examination included hypotonia, muscle weakness, gross and fine motor dysfunction, and ataxia. The results indicate that feeding difficulties and oral motor dysfunction are common in children with cystinosis and appear to correlate with the general degree of neurologic dysfunction. Long-term follow-up is necessary to determine whether the early oral motor problems predict the later development of the progressive myopathy observed in adults with cystinosis.  相似文献   

5.
Participation and activity performance (motor and cognitive or behavioural) were examined in 148 children with cerebral palsy (CP; 87 males, 61 females; mean age 9y 8mo, SD 1y 11mo; range 6y 1mo to 13y 7mo), mainstreamed in fully inclusive (n=100) and in self-contained classes (n=48) within general schools in Israel, using the School Function Assessment. Differences in participation within these groups were analyzed in relation to the type of CP (mainly spastic hemiplegia, spastic diplegia, and spastic tetraplegia), the level of impairment according to the Gross Motor Function Classification System (GMFCS; level II 55%, level III 37%, and level IV 8%), and additional neuroimpairments. Univariate analyses of variance revealed significant differences in levels of participation and levels of activity performance between different types of CP and GMFCS levels. With regard to additional neuroimpairments, significant differences in participation were found for fully included children with speech and language impairments and those with learning disability within the self-contained group. Physical activity performance partly accounted for differences in participation between children with different types of CP and different levels of motor impairment. These findings suggest that within the mainstreamed environment, participation and activity performance increase as motor disability and/or additional neuroimpairments (speech and language impairments and learning disability) decrease.  相似文献   

6.
Purpose: To evaluate cortical auditory function, including speech recognition, in children with benign rolandic epilepsy (BRE).
Methods: Fourteen children, seven patients with BRE and seven matched controls, underwent audiometric and behavioral testing, simultaneous EEG recordings, and auditory-evoked potential recordings with speech and tones. Speech recognition was tested under multiple listening conditions.
Results: All participants demonstrated normal speech recognition abilities in quiet, as well as normal peripheral and subcortical auditory function. BRE patients performed significantly worse than controls when speech recognition was tested under adverse listening conditions, including background noise. Five BRE patients who were impaired on two or more tests had centrotemporal spiking on awake EEG. There were no significant group differences in the latency or amplitude of early N100 cortical responses to speech or tones. Conversely, the mismatch negativity, a preattentive index of cortical processing that is elicited passively, was absent or prolonged for speech, but not tones, in BRE patients as compared to controls.
Discussion: Children with BRE demonstrated specific speech recognition impairments. Our evoked potential findings indicate that these behavioral impairments reflect dysfunction of nonprimary auditory cortex and cannot be attributed solely to attention difficulties. A possible association between auditory impairments and centrotemporal spiking (>1/min) on awake EEG was identified. The pattern of speech recognition impairments observed is a known risk factor for academic difficulties in school-age children. Our results underscore the importance of comprehensive auditory testing, using behavioral and electrophysiological measures, in children with BRE.  相似文献   

7.
Impairment in movement skills of children with autistic spectrum disorders   总被引:1,自引:0,他引:1  
Aim  We undertook this study to explore the degree of impairment in movement skills in children with autistic spectrum disorders (ASD) and a wide IQ range.
Method  Movement skills were measured using the Movement Assessment Battery for Children (M-ABC) in a large, well defined, population-derived group of children ( n =101: 89 males,12 females; mean age 11y 4mo, SD 10mo; range 10y–14y 3mo) with childhood autism and broader ASD and a wide range of IQ scores. Additionally, we tested whether a parent-completed questionnaire, the Developmental Coordination Disorder Questionnaire (DCDQ), was useful in identifying children who met criteria for movement impairments after assessment ( n =97 with complete M-ABCs and DCDQs).
Results  Of the children with ASD, 79% had definite movement impairments on the M-ABC; a further 10% had borderline problems. Children with childhood autism were more impaired than children with broader ASD, and children with an IQ less than 70 were more impaired than those with IQ more than 70. This is consistent with the view that movement impairments may arise from a more severe neurological impairment that also contributes to intellectual disability and more severe autism. Movement impairment was not associated with everyday adaptive behaviour once the effect of IQ was controlled for. The DCDQ performed moderately well as a screen for possible motor difficulties.
Interpretation  Movement impairments are common in children with ASD. Systematic assessment of movement abilities should be considered a routine investigation.  相似文献   

8.
The aim of the present study was to describe the prevalence and associated factors of pervasive developmental disorders (PDD), including autistic disorder and PDD not otherwise specified (NOS), in a clinical sample of 126 children and adolescents (75 males, 51 females; age range 4–18y, mean 8y 8mo, SD 3y 8mo) with tetraplegic, hemiplegic, diplegic, dyskinetic, or mixed types of cerebral palsy (CP); 28% could not crawl or walk even with support, 29% could move with support, and 43% walked independently. Participants were examined for PDD in two stages. In the first stage, probable participants were determined by direct observation, Autism Behavior Checklist score, and medical reports. In the second stage, those with 'probable' symptoms underwent psychiatric examination and their autistic symptoms were scored on the Childhood Autism Rating Scale. The final diagnosis of autistic disorder or PDD-NOS was given according to DSM-IV criteria. Fourteen (11%) and five (4%) of the participants met the criteria for autistic disorder and PDD-NOS respectively. Children with CP and PDD differed from those without PDD in terms of type of CP ( p =0.02), presence of epilepsy ( p <0.001), intellectual level ( p <0.001), and level of speech ( p <0.001). PDD was more common in children with tetraplegic, mixed, and hemiplegic CP, and in children with epilepsy, learning disability, * and low level of speech. The findings corroborate the notion that CP is a complex disorder, often associated with additional impairments. PDD is not rare in CP and should be considered in patients with comorbid conditions such as epilepsy, learning disability, and language delay and in the presence of tetraplegic, mixed, and hemiplegic CP types.  相似文献   

9.
Aims  Motor dysfunction is common to both autism and Asperger syndrome, but the underlying neurophysiological impairments are unclear. Neurophysiological examinations of motor dysfunction can provide information about likely sites of functional impairment and can contribute to the debate about whether autism and Asperger syndrome are variants of the same disorder or fundamentally distinct neurodevelopmental conditions. We investigated the neurophysiology of internally determined motor activity in autism and Asperger syndrome via examination of movement-related potentials (MRPs).
Method  We used electroencephalography to investigate MRPs, via an internally cued movement paradigm, in the following three groups: (1) individuals with high-functioning autism (14 males, one female; mean age 13y 1mo, SD 4y 2mo, range 7y 8mo to 20y 9mo; mean Full-scale IQ 93.40, SD 20.72); (2) individuals with Asperger syndrome (10 males, two females; mean age 13y 7mo, SD 3y 9mo, range 8y 11mo to 20y 4mo; mean Full-scale IQ 103.25, SD 19.37), and (3) a healthy control group (13 males, seven females; mean age 14y 0mo, SD 3y 11mo; range 8y 4mo to 21y 0mo; mean Full-scale IQ 114.25, SD 11.29).
Results  Abnormal MRPs can reflect disruption of motor-related neural networks involving the basal ganglia, thalamus, and supplementary motor area. There was evidence for abnormal MRPs in autism (e.g. increased post-movement cortical activity, abnormal peak time) but not in Asperger syndrome.
Interpretation  The results support basal ganglia, thalamus, and supplementary motor area involvement as a likely source of motor dysfunction in autism, and provide further evidence for the neurobiological separateness of autism and Asperger syndrome.  相似文献   

10.
The aim of this study was to evaluate the clinical features and frequency of autistic disorder or Asperger syndrome (AS; according to Diagnostic and Statistical Manual of Mental Disorders, 4th edition [DSM-IV] criteria) in children exposed to anticonvulsant medication in utero. During a 20-year study period, 626 children were born in Aberdeen to mothers taking antiepileptic drugs (AEDs). The study examined long-term effects of prenatal exposure to AEDs in 260 children (122 males, 138 females). Of these, 26 (16 males) were reported by parents to have social or behavioural difficulties. Eleven children (6 males, 5 females) fulfilled the DSM-IV criteria for autistic disorder and one (female) fulfilled the DSM-IV criteria for AS. These children comprised 4.6% of the exposed children studied, and 1.9% of all exposed children born during the study period. Mean age of these children at diagnosis was 5 years 4 months (SD 2y 11mo) and 9 years 10 months (SD 3y 10mo) at the time of this study. Other children from the group of 26 had difficulties in areas of speech and language development and social communication but did not meet the criteria for an autism spectrum disorder (ASD). Sodium valproate was the drug most commonly associated with autistic disorder, five of 56 (8.9%) of the study children exposed to sodium valproate alone had either autistic disorder or AS. It was concluded that prenatal exposure to anticonvulsant medication is a risk factor for the development of an ASD. Fetal anticonvulsant syndrome associated autistic disorder is characterized by an even sex ratio, absence of regression or skill loss, and language delay in the absence of global delay.  相似文献   

11.
The aims of the study were to describe the functional ability, health status, and health-related quality of life (HRQL) of young children with a vision impairment or blindness (VI/BL) and to examine the effect of different types of ophthalmic condition and the presence of other impairments or systemic disorders. A cross-sectional community survey of children aged 3 to 8 years with VI/BL was conducted in four areas of England using the Health Utilities Index Mark 3 system. Seventy-nine children (47 males, 32 females; mean age 6 y 2 mo [SD 1 y 6 mo]) met the selection criteria: 43% had a visual pathway condition, 38% a condition of the eye, and 19% nystagmus alone; and 61% had additional impairments/disorders. Eighty per cent had functional limitations on at least two of the following attributes: vision, hearing, speech, cognition, ambulation, dexterity, emotion, and pain. Forty-four per cent had functional limitations on four or more attributes. Children with nystagmus alone had significantly higher mean HRQL utility scores (0.80 [SD 0.26]) than children with a condition of the eye (0.45 [SD 0.33]), who, in turn, had higher scores than children with a visual pathway condition (0.05 [SD 0.33], p=0.002). Children with an isolated VI/BL had significantly higher mean scores (0.73 [SD 0.21]) than those with additional impairments/disorders (0.09 [SD 0.34], p<0.001). These findings underline the need for a broad assessment of each child with VI/BL and a multidisciplinary approach to care.  相似文献   

12.
The objective of this study was to characterize participation in leisure activities in children with cerebral palsy (CP) and identify determinants of greater involvement. Ninety-five children of school age (9y 7mo [SD 2y 1mo]) with CP were recruited, and participation was evaluated with the Children's Assessment of Participation and Enjoyment in a subset (67/95; 42 males, 25 females) who could actively participate in completion of the assessment. Most had mild motor dysfunction (Gross Motor Function Classification System: 59% level I, 23% level II, 18% levels III-V) and had a spastic subtype of CP (23 hemiplegia, 17 diplegia, 16 quadriplegia, 11 other). Biomedical, child, family and environmental predictor variables were considered in the analysis. Results demonstrated that these children were actively involved in a wide range of leisure activities and experienced a high level of enjoyment. However, involvement was lower in skill-based and active physical activities as well as community-based activities. Mastery motivation and involvement in rehabilitation services enhanced involvement (intensity and diversity) in particular leisure activities, whereas cognitive and behavioral difficulties, activity limitations, and parental stress were obstacles to participation.  相似文献   

13.
Development of literacy skills was studied in six children (one male, five females) with severe speech impairments and cerebral palsy (CP). These skills were related to intellectual development, phonological abilities, and short-term memory. Three of the children were diagnosed with dystonia, and three with diplegia. They had no, or severely restricted, independent mobility (Gross Motor Function Classification System Level IV for four children and Level V for two), and severe fine motor problems, including difficulty with pointing. As they had no intelligible speech, the Bliss system was the primary communication mode. Assessments were made at approximately 6, 9, and 12 years of age. The results revealed that the children had difficulties acquiring literacy skills, although intellectual level and phonological ability predicted otherwise. Positive development during the first 3 years was followed by an arrest. A conspicuous decrease in IQ points was also found. Thus, phonological ability does not seem to have the same predictive power for literacy development in children with severe speech impairments and CP as in typically developing children. Further studies are needed to clarify the role of phonological abilities, working memory, and strategies used in literacy acquisition in these children. Such studies might also clarify the importance of articulatory abilities in early literacy acquisition.  相似文献   

14.
We studied expressive and receptive language, oral motor ability, attention, memory, and intelligence in 20 6-year-old children with epilepsy (14 females, six males; mean age 6y 5mo, range 6y-6y 11mo) without learning disability, cerebral palsy (CP), and/or autism, and in 30 reference children without epilepsy (18 females, 12 males; mean age 6y 5mo, range 6y-6y 11mo). Ten children had partial, six primarily generalized, and four unclassified epilepsy. Fourteen were having monotherapy and six were taking two or more antiepileptic drugs; 13 children were free from seizures 3 months before the assessment. Results show no statistically significant difference between the groups concerning Verbal IQ, expressive and receptive grammar, and receptive vocabulary. The children with epilepsy had a significantly lower Performance IQ and lower scores in tests of oral motor ability, articulation, emerging literacy, auditory attention, short-term memory, and rapid word retrieval. Parent ratings revealed no significant difference in communicative ability. Polytherapy and early onset of epilepsy influenced some results. Preschool children with epilepsy without learning disability, CP, and/or autism may have receptive verbal ability within the normal range but visuoperceptual, auditory attentional, and speech-language difficulties that could affect school achievement. Careful testing of children with epilepsy who appear to be functioning within the normal range is needed because this may reveal specific impairments that require appropriate professional input.  相似文献   

15.
Executive functions (EFs), seizure-related factors, and school performance were studied in a population-based sample of children with epilepsy (n=117; 71 males, 46 females; mean age 10y 5mo [SD 2y]; range 6y-12y 11mo) and a comparison group (n=124; 71 males, 53 females; mean age 10y 1mo [SD 2y 1mo]; range 6y-12y 11mo). EF, cognitive function, depression, socioeconomic status, and school performance were examined. Patients with epilepsy performed significantly lower than the comparison group on all EF measures except incidental memory. Intellectual dysfunction and depression accounted for 43% of EF problems. All epilepsy syndrome groups (except Rolandic epilepsy) were associated with decreased EF in addition to early epilepsy onset, high seizure frequency, and polytherapy. Patients had more school performance problems than comparison children which were attributed partly to EF difficulties. All aspects of EF were affected in children with epilepsy and all epilepsy syndrome groups, except Rolandic epilepsy, influenced EF negatively. EF problems contributed to patients' school difficulties beyond intellectual dysfunction.  相似文献   

16.
Aim  Botulinum toxin type A (BoNT-A) injections were used in the treatment of lower-limb spasticity in children with cerebral palsy (CP). Anecdotal evidence suggests a reduction in pain after this treatment in children who had pain localized to a displaced hip joint. We report on our current clinical practice.
Method  Twenty-six children with non-ambulant quadriplegic CP (Gross Motor Function Classification System level V) were assessed as having significant spasticity and pain at the hip level. Twelve were males and 14 females, with an age range of 2 to 19 years (mean age 11y 6mo, SD 4y 9mo). Ten had functional difficulties secondary to predominant spasticity and 16 had a mix of a high-background peripheral tone with superimposed dystonia. Of the 26 children assessed, 10 had at least one hip which was dislocated and three had at least one hip which was subluxed. As part of their spasticity management programme they received targeted BoNT-A injections to the adductor magnus, medial hamstrings and iliopsoas muscle groups. The Paediatric Pain Profile was used as the primary outcome measure.
Results  All had highly significant improvement in their recorded pain profile scores measured at 3 months after treatment ( p <0.001). There was equal efficacy in response to treatment in the children with subluxed or dislocated hips. In addition, families commented on improved quality of life for the children across several areas, including sleep, postural management, and activities of daily living.
Interpretation  This report demonstrates that targeted BoNT-A injections reduced pain in children with significant spasticity and pain at the hip level. They may also improve quality of life of non-ambulant children with CP and a hip problem.  相似文献   

17.
Myotonic dystrophy (DM) is a neuromuscular disorder caused by an expansion of a CTG repeat sequence on chromosome 19q13. The aim of the present study was to describe the characteristics and prevalence of oral motor dysfunction in a cohort of children and adolescents with DM and to correlate different aspects of oral motor function with the type of DM and sex. Fifty-six individuals with DM (30 males, 26 females; median age 13y 2mo; range 2y 6mo-21y 5mo) were compared with healthy controls. They were divided into four subgroups: severe congenital DM (n=18); mild congenital DM (n=18); childhood DM (n=18); and classical DM (n=2). A speech-language pathologist assessed different variables of oral motor function, intelligibility, and lip force. The families used a questionnaire to report on eating difficulties and drooling. All individuals with DM had impaired facial expression. Intelligibility was moderately or severely reduced in 30 patients (60%), excluding six patients without speech. Most had a moderate or severe impairment of lip motility (76.0%), tongue motility (52.2%), and lip force (69.2%), causing deviant production of bilabial and dental consonants. The families reported problems with eating (51.9%) and drooling (37.0%). Oral motor dysfunction was most prominent in congenital DM, and males were more affected than females.  相似文献   

18.
19.
Aim The aim of this study was to study systematically social, communication, and repetitive/restrictive (SCRR) behavioural difficulties and clinical autism spectrum disorder (ASD) in children with optic nerve hypoplasia (ONH) and/or septo‐optic dysplasia (SOD), and to investigate the relationship between visual impairment, SCRR difficulties, ASD, and cognition. Method A case‐note study of clinic records from a specialist developmental vision service was completed. Standardized assessments of vision and development and clinician judgements about SCRR difficulties and clinical ASD were made by a multidisciplinary team. Results A total of 45 females and 38 males (mean age 3y 5mo; range 10mo–6y 10mo) with ONH or SOD and profound visual impairment (PVI) or severe visual impairment (SVI) were assessed. A total of 58% of children had at least one SCRR difficulty, and 31% had a clinical diagnosis of ASD. The prevalence of ASD was slightly higher in children with SOD than in children with ONH (36% vs 26%) also slightly more frequent in children with PVI than in children with SVI (36% vs 27%). The prevalence of SCRR difficulties was statistically higher in children with PVI than in children with SVI (p=0.003). Clinical ASD was most likely to be diagnosed between 2 years 4 months and 4 years 6 months. Development was significantly delayed in children with ASD compared with children without social communication difficulties (p=0.001). Interpretation Children with SVI or PVI are at risk of SCRR difficulties and clinical ASD. Children with ONH and/or SOD and visual impairment have a similar risk of developing clinical ASD as other visual impairment groups. However, ASD prevalence data from this study are a minimum estimate, as some young children may have developed ASD behaviours in later childhood. Developmental surveillance for children with ONH and/or SOD should continue until at least the age of 4 years 6 months.  相似文献   

20.
Using prospectively collected data from 13971 births enrolled in a large population-based cohort study (Avon Longitudinal Study of Parents and Children: ALSPAC), the prevalence of feeding difficulties at 4 weeks and 6 months of age in 33 children subsequently diagnosed with cerebral palsy (CP) were investigated. It was also assessed whether early feeding difficulties could be predictive of functional and growth outcomes at age 8 years. Weak sucking at 4 weeks of age was reported in 11 of 23 children with CP and in 2206 of 12299 (18%) of the remaining population (p<0.0001), and great difficulties feeding at 6 months of age was reported in two of 21 (10%) of the group with CP and in 373 of 10941 (3.3%) of control participants (p=0.017). Feeding difficulties at 4 weeks of age were associated with the pattern of functional impairment at age 4 years (p=0.009) and at 8 years (p=0.068), being clinically underweight (p=0.01), and having speech and swallowing difficulties (p=0.005) at 8 years of age. Early, persistent, and severe feeding difficulties are a marker for subsequent poor growth, feeding, and developmental outcomes and can identify children with CP who will benefit from gastrostomy feeding.  相似文献   

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