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A cluster-randomised trial of screening for language disorders in toddlers.

OBJECTIVE: To assess the screening performance of a specific language-screening instrument at 18 and 24 months of age and to assess its effect on the early detection and prognosis of language delay. DESIGN: Child health care physicians were randomised to the intervention group, in which specific language screening was conducted twice (at age 18 months and 24 months), or to the control group (usual care). The specific screening instrument consisted of a uniform set of questions for the parents and test elements for the child, with scaled scores to assess responses. SETTING: Child health care in the Netherlands and referral of screen-positive children. SUBJECTS: 5734 children in the intervention group and 4621 in the control group. MAIN OUTCOME MEASURES: Test characteristics and disorders at 24 months, and confirmed diagnoses of a language disorder before 36 months in both groups. Gold standard based on reports of parents, specialists and expert panel. Prognosis estimated from two diagnostic language development performance scores at 36 months (in questionnaire). RESULTS: In the intervention group, 3147 of the 5734 children (55%) were screened with the specific screening instrument and 73 of the screened children (2.3%) were screen-positive. Of the screen-positive children, 41 (55%) had confirmed language delay (diagnostic assessment and/or reported treatment). The estimated sensitivity of the test ranged between 24-52% depending on the severity of language disorders. The prevalence of language disorders in three-year olds was estimated to be 2.4-5.3%. In the intervention group, 1.25-2 times more children with language delay had been diagnosed before 36 months. The assessment of language development at 36 months showed no statistically significant differences between the intervention and the control groups. CONCLUSIONS: The inclusion of a specific language-screening instrument in child health centre activities resulted in the earlier detection of children with language delay. Short-term health benefits could not be demonstrated. Large-scale introduction cannot be recommended on the basis of this information alone.

Case-Control Studies↗

Language lateralization development in children with autism: insights from the late field magnetoencephalogram.

Left hemisphere dominance represents the typical language lateralization profile for the majority of neurologically healthy, right-handed individuals. We investigated hemispheric dominance for language in language-impaired children with autism and typically developing controls to investigate the hypothesis that atypical functional specialization for language represents one component of developmental language impairment in autism. Late field magnetoencephalography (MEG) recordings were used to calculate a hemispheric Lateralization Index from the neuromagnetic activity evoked by passive auditory presentation of vowel stimuli. Results indicate that children with autism and typically developing children follow opposite maturational trajectories in language lateralization; while leftward lateralization (i.e. left hemisphere dominance) emerged from bilaterally symmetric neuronal activation as age increased in our sample of typically developing children, rightward lateralization emerged from bilaterally symmetric activity as age increased in our sample of children with autism.

Acoustic Stimulation↗

Language development in children with congenital strokes.

The congenital lesion population provides an excellent forum to investigate the issues of innate specialization and plasticity. Effects of early lesions on left and right hemisphere function reflect both the cognitive process under study and biological/neurological factors of lesion parameters and hemispheric maturation rates. In general, toddlers with both congenital left and right lesions show mild to moderate delays in language acquisition. School-age children with left hemisphere lesions have more problems with language and language-based academic skills than those with right hemisphere lesions, but the problems are subtle and do occur in both groups. This pattern stands in sharp contrast with the adult, who shows striking language deficits with acquired lesions only in the left perisylvian region. Thus, there is evidence in these studies of the immature nervous system for both innate specialization and plasticity, as well as a right hemisphere contribution to language acquisition and verbal cognition.

Adult↗

Non-progressive congenital ataxia with or without cerebellar hypoplasia: a review of 34 subjects.

Information on the long-term development of larger series of children with non-progressive congenital ataxia (NPCA) is scarce. We have updated a personal cohort of subjects previously diagnosed as having NPCA. Children with brain malformations, acquired neurological illness, or defined syndromes were excluded. From 58 subjects, 34 were available for review (including three pairs of siblings). All our subjects had delayed motor and speech development. Truncal ataxia persisted but became less significant. Two subjects developed spasticity and three a focal dystonia. Epilepsy was a feature in 10 of the subjects. Cognitive impairment was present in 22 of 34 subjects. MRI was normal in 15 of 27. There were no obvious correlations between degree of motor delay, severity of ataxia, cognitive impairment, and neuroimaging. Although genetically and clinically not a homogeneous entity, NPCA is a helpful diagnostic label. Major problems arise in the majority of subjects related to cognitive impairment and less to neurological symptoms. Early individual prognosis is not possible from early developmental milestones, neurological signs, or neuroimaging.

Adolescent↗

A summary of recent research into the development of children with Down's syndrome.

Research over the last 5 to 6 years has indicated quite clearly that Down's syndrome children are a distinct, somewhat delayed group of people, the delays being a result of both physical and cognitive problems. What is considerably more positive is that much may apparently be done to help ameliorate the problems. However, the older Down's syndrome child has the most serious problems, and comparatively little research is being performed with this age group. What is being done tends to be less integrated than the work being done with younger children, leading to less generalisability across areas of development. Finally, research also shows that parents are a vital resource, as they are willing and able, particularly with guidance, to stimulate and encourage their children in all aspects of growth and development.

Child↗

Psychometric scatter in retarded, autistic preschoolers as measured by the Cattell.

To determine whether psychometric scatter is characteristic of the developmental profile of young autistic children, the performance of 38 autistic children, as measured by the Cattell, was compared with the performance of MA matched samples of normal, Down's Syndrome and non-Down's Syndrome children with mental retardation. Results indicated significantly more psychometric scatter in the autistic group than in the other groups. Similarly, 66% of the autistic children vs 13, 26 and 32% of the normal, Down's and non-Down's samples, respectively, had significant scatter. Further analyses revealed that autistic children showed consistent relative strength in non-language and weakness in language.

Autistic Disorder↗

Are phonological processing deficits part of the broad autism phenotype?

Two tests of phonological processing, nonword repetition, and nonsense passage reading, were administered to 80 probands with autistic disorder or PDDNOS (index cases) and 59 typically developing controls, together with their parents and siblings. In addition, parents completed a questionnaire about history of language and literacy problems, and all participants were given tests of verbal (VIQ) and performance IQ (PIQ). Parents also completed the Autism-Spectrum Quotient, which was used to index the broad autism phenotype. Index probands scored well below control probands on the two phonological tests. However, on neither phonological measure did index relatives differ from control relatives. Within the index group, there was no relationship between the proband's level of VIQ, or age at achieving phrase speech, and phonological score of relatives. VIQ was the only measure to show any familiality within the index group. Reported history of language and literacy problems did not differentiate index parents from control parents overall, but those who were categorized as cases of the broad phenotype reported more history of language and literacy problems than did other index parents. However, they did not have poorer scores on the phonological measures. It is concluded that phonological processing deficits are not part of the broad autism phenotype.

Articulation Disorders↗

[Asperger syndrome--an overview of diagnostic criteria].

Asperger syndrome is an autistic disorder which was first described in 1944 without further acceptance in the literature over almost four decades. Following several publications in the "80's, the disorder became more widely known. Asperger syndrome was introduced into ICD-10 and DSM-IV as a new diagnosis in 1988 and 1994, respectively. Several authors developed own criteria. Until now, some of the diagnostic criteria of Asperger syndrome remain controversial. We present a survey and a comparison of the criteria in the classification of DSM-IV, ICD-10 and of other authors. Six criteria are widely accepted, but there are divergent opinions about the criteria "intelligence" and "speech development".

Autistic Disorder↗

Are there "autistic-like" features in congenitally blind children?

Twenty-four congenitally blind children between 3 and 9 years of age were studied for the prevalence of "autistic-like" features, as assessed by teacher reports and by systematic observations of the children's behaviour. A comparison between the 15 blind children who had IQs over 70 and 10 sighted children group-matched for age and verbal ability revealed that a number of autistic-like features were more common in the blind. When the nine blind children who had IQs less than 70 were compared with nine group-matched autistic children, the picture that emerged was of substantial overlap in clinical presentation, despite subtle differences on clinical impression. Similar results were obtained when blind subgroups were reconstituted according to the children's nonautistic or autistic-like clinical presentation, rather than IQ. These findings are discussed in relation to competing theories concerning the development of autism and "theory of mind".

Autistic Disorder↗

Inattention, hyperactivity and speech delay at 2-4 years of age as a predictor for ADD-ADHD syndrome.

In the Jerusalem Institute for Child Development children with various developmental disorders at ages of 0-5 years are examined. Thirty-six children aged 2-4 years were examined by us and were found to have inattention, hyperactivity and speech delay with an IQ or DQ above 70 and were reexamined at 7-14 years of age. They were compared to a group of 27 control children. All children had a complete neurodevelopmental examination using the Touwen & Prechtel examination for Minor Neurological Dysfunction. They also had a Pollack tapper test for the identification of learning disabilities and the Conners parent's and teacher's hyperactivity rating scales. Of the 36 children from the research group 20 studied in special education classes because of behavioral disorders, inattention, and severe learning disabilities. They all had ADD-ADHD. There were 16 children in regular schools, of whom 9 had ADD-ADHD. In the control group only one child had ADD-ADHD. A very high number of the research group children failed in 2 or all 3 tests used in this study in comparison to controls. It seems that "soft" neurological signs with hyperactivity, inattention and speech delay may be early clinical signs of ADD-ADHD as 80% of the children with these clinical features developed ADD-ADHD during early school age.

Attention↗

Hyperserotonemia in adults with autistic disorder.

Hyperserotonemia is the most consistent serotonin-related finding in autism. The basis of this phenomenon, and its relationship to the central serotonergic dysfunction remains unclear. Platelet serotonin level (PSL) in 53 autistic adults and 45 healthy controls was measured. Mean PSL in autistic group (75.7 +/- 37.4 ng/microL) was significantly higher than the control sample (59.2 +/- 16.2 ng/microL) due to a presence of hyperserotonemic subjects which comprised 32% of the patients. PSL of autistic subjects did not correlate with the severity of symptoms, as measured by total CARS score, or the degree of mental retardation. However, significant negative relationship was observed between PSL and speech development, indicating the relationship between the peripheral 5HT concentrations and verbal abilities in autistic subjects.

Adolescent↗

Unexpected sex-ratios in families of language/learning-impaired children.

There is a well-documented propensity of males affected with developmental language/learning impairment. Results from this study demonstrate, unexpectedly, that this sex-ratio difference of males to females with developmental language/learning disorders was found to occur significantly only in families with a language/learning-impaired mother. In addition, a remarkably aberrant offspring sex-ratio was found in families of language/learning-impaired children who had an affected mother, but not father. Mothers who were developmentally language/learning-impaired had three times as many sons as daughters, and five times as many language/learning-impaired sons as daughters. Genetic and hormonal influences that might affect both sex-ratio and neuroanatomical development and disorders are discussed.

Child, Preschool↗

[Objective determination of auditory threshold in the child].

In children who are difficult to test with dubious behavioural-audiometric results, brainstem electric response audiometry (BERA) is today the method of choice for accurate determination of hearing threshold. Oral sedation with the shortacting neuroleptic chloroprothixene allows BERA to be performed on an outpatient basis. Thirty-six girls and 41 boys aged between 11 weeks and 12 years (median age 40 months) with inconsistent behavioural-audiometric findings were examined. Frequency-following responses were searched for in flat fast response curves. BERA proved to be a very sensitive method compared with behavioural audiometry and was reliable even for children who are difficult to evaluate clinically, and for asymmetrical auditory thresholds. In conjunction with the standard pedaudiological test battery, BERA improves diagnostic accuracy and causes little disturbance as an outpatient procedure.

Audiometry, Evoked Response↗

A follow-up study of auditory sequential memory abilities in children with histories of preschool language impairment.

Auditory sequential memory abilities of 15 subjects with histories of preschool language impairment were compared to 15 non-language impaired matched controls on an auditory processing screening battery. The battery included assessment of symbols, syllables, words, sentences and commands in auditory sequential memory tasks. All five subtests significantly differentiated the subjects from their matched controls at the 1% level. Information from teacher and parent questionnaires revealed that many of the subjects presented with some areas of academic difficulty. These subjects seem predisposed to develop persistent communication difficulties. A dynamic continuum of language-learning disabilities is proposed. Clinical and theoretical implications and directions for research are discussed.

Achievement↗

Autism families with a high incidence of alcoholism.

To determine the significance of neuropsychiatric disorders in autism families, we analyzed 167 pedigrees ascertained through an autistic child; 39% had alcoholism in patterns consistent with transmission of a genetic trait. Children from high alcoholism families were more likely to have the onset of their autistic behavior occur with a loss of language (52.5% vs. 35.8%, p = 0.04). This occurred primarily in families where the mother was alcoholic (80% vs. 40%, p = 0.05), suggesting an association between maternal alcoholism and regressive onset autism. Children from high alcoholism families were less likely to be macrocephalic (14.7% vs. 40.6%, p = 0.0006). Children from high alcohol and low alcohol families did not differ in dysmorphology status, IQ, sex ratio or sib recurrence risk.

Adolescent↗

Autistic spectrum disorder: evaluating a possible contributing or causal role of epilepsy.

The onset of epilepsy in brain systems involved in social communication and/or recognition of emotions can occasionally be the cause of autistic symptoms or may aggravate preexisting autistic symptoms. Knowing that cognitive and/or behavioral abnormalities can be the presenting and sometimes the only symptom of an epileptic disorder or can even be caused by paroxysmal EEG abnormalities without recognized seizures, the possibility that this may apply to autism has given rise to much debate. Epilepsy and/or epileptic EEG abnormalities are frequently associated with autistic disorders in children but this does not necessarily imply that they are the cause; great caution needs to be exercised before drawing any such conclusions. So far, there is no evidence that typical autism can be attributed to an epileptic disorder, even in those children with a history of regression after normal early development. Nevertheless, there are several early epilepsies (late infantile spasms, partial complex epilepsies, epilepsies with CSWS, early forms of Landau-Kleffner syndrome) and with different etiologies (tuberous sclerosis is an important model of these situations) in which a direct relationship between epilepsy and some features of autism may be suspected. In young children who primarily have language regression (and who may have autistic features) without evident cause, and in whom paroxysmal focal EEG abnormalities are also found, the possible direct role of epilepsy can only be evaluated in longitudinal studies.

Adult↗

The Autism Diagnostic Observation Schedule: revised algorithms for improved diagnostic validity.

Autism Diagnostic Observation Schedule (ADOS) Modules 1-3 item and domain total distributions were reviewed for 1,630 assessments of children aged 14 months to 16 years with an autism spectrum disorder (ASD) or with heterogeneous non-spectrum disorders. Children were divided by language level and age to yield more homogeneous cells. Items were chosen that best differentiated between diagnoses and were arranged into domains on the basis of multi-factor item-response analysis. Reflecting recent research, the revised algorithm now consists of two new domains, Social Affect and Restricted, Repetitive Behaviors (RRB), combined to one score to which thresholds are applied, resulting in generally improved predictive value.

Adolescent↗