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Metabolic correlates of learning disability.

To summarize, the neuropsychologic findings in MSUD and MMA children in both groups demonstrated deficits in cognitive/language areas, but interesting individual differences existed. For example, a marked contrast in abilities existed between NT and GV, even taking into account the age difference between these children with MSUD. While NT's general intellectual functioning was within the low-average range, GV was severely impaired. In more specific areas of cognitive functioning, NT had an uneven performance profile with areas of strengths and weaknesses, while GV evidenced a flat profile with significant impairment in all areas. NT demonstrated mild delay in visual-spatial processing, articulation development, motor-speech abilities, and selective attention and concentration; however, his language and psychosocial development were within the broad range of normality. In contrast, GV exhibited severe dysfunction in speech, language, and perceptual/conceptual development. The three children with MMA also exhibited a range of disabilities in cognitive functioning. TJ and SH contrasted in the degree of developmental delay across language and cognitive domains. Both were distractible and exhibited very short attention spans but there was a measurable difference in the extent of their speech, language, visual-spatial development. CH was markedly delayed in all areas of cognitive development, which is consistent with global retardation. The severity of her disease and its probable impact on brain development were undoubtedly related to her intellectual impairment. These datasets illustrate the challenges we face, because within each group we see a range of impairment. Nevertheless, we are optimistic that our efforts to relate metabolic, anatomic and neuropsychologic findings for such children will lead to a better understanding of these disorders. We are hopeful that this will result in new advances in diagnosis and intervention, which will ultimately improve the prognosis for cognitive development. In summary, a multidisciplinary center has been established at UCSD to study the neurologic basis of disorders of language, learning and behavior in infants and children. The center draws together a group of researchers from many fields including neurology, biochemistry, pediatrics, cognitive and developmental neuropsychology, psycholinguistics, neurophysiology and communicative disorders. Because of the diverse interests and expertise of our group, it is hoped to forge a synthesis of the behavioral and neurosciences to study populations of children with a variety of neurologic, metabolic, and language/learning disorders. Disorders currently under study include Lesch-Nyhan disease, oculocutaneous tyrosinemia, propionic acidemia, carnitine palmityl transferase deficiency, Schwachman-Diamond syndrome, histidinemia, Hartnup disease, citrullinemia, galactosemia, maple syrup urine disease, and methylmalonic acidemia.(ABSTRACT TRUNCATED AT 400 WORDS)

Child↗

[Cognitive and linguistic indexes in children with early hemispheric lesion].

Prospective studies of development following early focal brain injury have identified deficits across a range of cognitive, linguistic and affective domains. In most cases these deficits are quite subtle and often not detected on standardized behavioral tests. Nonetheless, more detailed tests of neuropsychological functioning have documented subtle, persistent profiles of impairment. These contrasting findings have been cited in defense of two conflicting beliefs: plasticity and hemispheric equipotentiality theories on one side and early hemispheric specialization on the other. This paper shows some experimental data concerning studies from children with an early focal brain lesion. Our aim is to describe the effects determined by the lesion on the cognitive development and possible diagnostic and rehabilitative strategies.

Brain Diseases↗

Learning disorders. Some medical aspects.

The first skills are motor ones and these depend on perceptual development and the organisation of movement. If development is deviant the child will be clumsy. The clinical picture is considered, and the effects this may have in the school situation. Disorders of language development may be secondary to other factors such as deafness or brain injury, or may be a specific disability. The various grades of the latter are described. Both perceptual and language disorders can underlie reading retardation and the analysis of a particular child's difficulties is stressed. The etiology of these conditions is discussed with particular reference to the failure of integration. Intracerebral connections may not form, be destroyed, or not be used. The role of the Doctor is an important one, and this includes not only diagnosis and assessment but also helping the child in the home and school, especially when emotional and behaviour complications occur. The doctor must also act as a questioner and co-ordinator.

Child↗

[What are specific performance weaknesses?].

The term "specific developmental disorder" means localized deficits in very different functions contrasting fit in the general otherwise normal performance level of a child. These disorders are mostly looked upon in a developmental context and differentiated from acquired neuropsychological syndromes (e.g. aphasia, apraxia). The most important clinically relevant disorders are: the specific reading disability, the specific dyscalculia, other specific learning disabilities, the specific developmental language disorder, the specific retardation in the motor development, and multiple developmental retardations. In a population of all patients treated in institutions for children and adolescents, 30% show specific developmental disorders with a predominance of boys. There is no correlation between dyslexia and social class but it does exist for specific developmental language disorder and motor retardation. As to the etiology of specific developmental disorders, genetic factors, congenital or acquired cerebral dysfunctions, maturation or developmental retardation and cognitive variables are discussed. The multifactor-approach seems to be the best way of understanding specific developmental disorders, which are difficult to prove.

Child↗

Autism and pervasive developmental disorders: concepts and diagnostic issues.

The purpose of this report is to bring up to date available information on the defining features and diagnostic issues relating to autism and related disorders. We review the validity of the syndrome based on our last review (Rutter, 1978; Schopler, 1978). Subsequent data have produced refinement in our understanding of both diagnostic criteria and the nature of the basic deficit. Controversies over both the boundaries and the heterogeneity within the autism syndrome are evaluated according to available evidence. Diagnostic rating instruments for expediting systematic sample selection are critiqued, and leads for new research directions are suggested.

Autistic Disorder↗

Did Asperger's cases have Asperger disorder? A research note.

With publication of the fourth edition of the Diagnostic and statistical manual of mental disorders (DSM-IV), standardized criteria for Asperger Disorder, a putative subtype of Pervasive Developmental Disorder, are now available. This paper examines the four cases Asperger originally presented in his seminal paper (1991/1994), using DSM-IV criteria to determine whether a diagnosis of Autistic or Asperger Disorder is most appropriate. We found that all four cases met DSM-IV criteria for Autistic Disorder, rather than Asperger Disorder. This suggests that the syndrome Asperger originally described may not be captured by present diagnostic criteria. Implications for future research are discussed.

Autistic Disorder↗

[Diagnostic and etiopathogenic aspects of autism].

The Pervasive Developmental Disorders are a very complex and discussed subject both from a clinical point of view and about the etiopathogenesis. Different opinions have generated, in the past, different classification systems based on the underlying theoretical hypothesis. Nowadays the most popular classification systems are the ones contained in the DSM-IV and in the ICD-10. They define these diseases as "conditions characterized by a distortion of base development regarding verbal and non-verbal communication, the reciprocal and social ability of interaction and the imaginative activity." Inside this scenery are also compromised the motion activity, the awareness, the sensorial perception, the humour and the knowledge ability. About the pathogeny, nowadays many authors think autism is based on particular biological conditions, nevertheless they are not well-known or general. The exact origins of the rise of autistic disease are, in fact, still hypothetical. In the same way the concatenation of pathological events determining the arise of such a complex and wide clinical condition is also hypothetical. The discussion is mainly about the classification of childhood autism as a pure organic pathology or as a final expression of organic, psychic, familiar and social factors correlating with wrong operating structures, differentiated from an anatomic and functional point of view. So it seems quite plausible to take into account the possibility of a multisystemic compromise likely generated by a multifactor condition.

Autistic Disorder↗

Disorders of higher cerebral function in preschool children. 1.

In preschoolers, disorders of higher cerebral function are most likely to present as inadequate development of language, while in school-age children, learning disabilities and attention deficit predominate. The main considerations in the differential diagnosis in preschoolers with inadequate language are hearing loss, mental deficiency, dysphasia, and autistic spectrum disorders. Attention to the child's ability to engage in symbolic play and communicate meaningfully is the key to this often baffling differential diagnosis. With the exception of definitive assessment of hearing, classic medical investigations are seldom informative because structural brain lesions and metabolic errors are much rarer etiologies than prenatal and genetic influences on brain development. While many children improve with age, the underlying deficit(s) usually persists. The role of the physician is to detect the developmental problem, give the parents a correct diagnosis, refer the child for appropriate investigation and intervention, and provide follow-up and counseling. Early diagnosis is essential for effective remediation.

Attention Deficit Disorder with Hyperactivity↗

Otitis media and child development. Design factors in the identification and assessment of hearing loss.

Otitis media with effusion (OME) is the major cause of auditory dysfunction in preschool and early-grade schoolchildren. At especially high risk are children with cleft palate/lip; some groups of mentally retarded children; children from underprivileged communities and children with severe or profound sensorineural hearing loss. In neonates the major concern is to identify those with severe sensorineural hearing loss but awareness of the likelihood of OME is also vital. The hearing loss associated with OME in its early stages may be as small as 10--15 dB and in consequence conventional hearing tests employing pure tones are inadequate is screening procedures. Acoustic impedance testing has proved to be a practicable, acceptable, sensitive and efficient method of identification of OME in infants and children. Impedance measurement cannot, as yet, provide accurate assessment of hearing loss, but this is probably not a serious drawback. The primary requirement is to detect OME in order that swift and effective treatment can be instituted.

Acoustic Impedance Tests↗

Verbal and cognitive sequelae following unilateral lesions acquired in early childhood.

Eight left-hemisphere lesioned children and eight right-hemisphere lesioned children between 18 months and 8 years of age were compared to control subjects on a battery of intelligence and language measures. Both left- and right-lesioned subjects had lower IQ scores than their controls, yet most functioned within the normal range or higher. Lexical comprehension and production were depressed in both subject groups and appeared to be depressed to a greater degree in right-lesioned subjects than in those with left lesions. In contrast, syntactic production in left-lesioned subjects was markedly deficient in comparison to controls as well as right-lesioned subjects. Although both subjects and controls included children with articulation errors, the number of misarticulating children and misarticulated sounds was greatest in the left-lesioned group. Finally, fluency disorders were observed in both right- and left-lesioned subjects but were not observed in controls. The study provides further evidence that the right and left hemispheres are not equipotential for language and that left-hemisphere lesions acquired early in childhood impair syntactic development to a greater degree than do right-hemisphere lesions.

Articulation Disorders↗

Causes and associations of severe and persistent specific speech and language disorders in children.

Eighty-two school-age children with severe and persistent specific speech and language disorders were studied. 71 had specific developmental language disorders, three had structural malformations (cleft palate) and eight had disorders acquired after a period of normal language development, including five with Landau-Kleffner syndrome. The sex ratio was 3.8 boys to one girl. Nearly half had a family history of speech-language disorder, with one in 5.2 affected siblings. Aetiological factors were found in 26 per cent: 11 per cent prenatal, 3 per cent perinatal and 12 per cent postnatal. 21 per cent had had a seizure and 7 per cent had had seizures after the age of eight. 29 per cent were left-handed, 90 per cent were clumsy and 22 per cent first walked after 18 months. The complex origins of specific speech and language disorders are discussed.

Brain Damage, Chronic↗

Children's language disorders: recent research advances.

Between 3 and 8% of preschool children in Britain and the United States are delayed in their language development by more than a year below the normal range. Evidence suggests that such children are particularly impaired in acquiring syntactic and morphological rules, although well-defined syndromes of impaired language development have not yet been determined. The effects of preschool impairments in language development on subsequent school achievement are significant: 60% of these children are in special classes for the learning impaired at 9 years of age. Auditory processing deficits have been demonstrated in language-impaired children, but the contributions of such deficits to language development difficulties have yet to be determined. While autistic children have severe cognitive disorders and visual and auditory perceptual disturbances, the difficulties of nonautistic language-impaired children seem confined more to language expression and comprehension. Recent neuropsychological evidence concerning brain organization for language behavior suggests that these children have deficiencies in language functions which normally depend on left hemisphere functioning.

Achievement↗

[Haptic form discrimination. Group comparison of children with normal speech development and former speech development disordered patients].

The importance of the neurobiological basis of developmental language disorders includes somatosensory modalities. Twenty-five children were diagnosed as having specific language-impairment at preschool age. All were examined with regard to their manual haptic form discrimination without visual control at a mean age of 8.7 years +/- 7.1 months. This study group was compared to age- and gender-matched normal children of equal non-verbal intelligence (control group). Haptic discrimination was measured with the Seguin formboard on which the children were required to place ten geometrical forms in appropriate holes. Both groups differed significantly in their mean quantitative performances in favor of the control group (P < 0.05). The difference in their mean performance and their mean discrimination times did not reach statistical significance. All results were not age-dependent. The control group on average performed significantly better with their left hands than the study group (P < 0.05). Qualitative analysis revealed a significant difference in haptic discrimination of the pointed forms and was probably caused by inadequate exploration procedures and/or cognitive representation deficits. The results of the children with previous developmental language disorders were interpreted as an expression of an impaired cerebral maturation.

Child↗

Procedures for teaching appropriate gestural communication skills to children with autism.

Four children with autism were taught to use gestures in combination with oral communication. Using a multiple-baseline across-responses design, intervention was introduced successively across three response categories containing gestures representative of attention-directing/getting, affective, and descriptive behavior. Although none of the participants displayed appropriate gestural and verbal responses during baseline, all participants acquired this skill with the systematic implementation of modeling, prompting, and reinforcement. Generalization measures indicated that the children learned to respond in the presence of novel stimuli and a novel setting. Social validity measures revealed that the participants' behavior appeared more socially appropriate at the completion of the study than at the start of the study, and that the participants' behavior was indistinguishable from that of their typically developing peers.

Autistic Disorder↗

Early intervention critical to autism treatment.

It is still not universally accepted within the scientific community that the habilitation of autistic children is possible, or that their ability to function without supports in regular education by third, fourth, or fifth grade happens as a direct result of EIBI. However, using the outcome studies that have been reported, the rate of children reaching a best-outcome status appears to be between about 10% and 47%. There is a more global way to look at the effects of EIBI or behavioral intervention. Even if the child retains many characteristics of autism, the usual outcome of treatment is that the child learns useful skills. Behavioral intervention results in effective and efficient learning, which is precisely what it aims to accomplish and what behavioral techniques have been developed to do. Children and families have been able to achieve much more than many would ever have believed before EIBI became a realistic possibility.

Age Factors↗