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Neuropsychological evaluation of preschoolers with fetal alcohol syndrome.

Children with Fetal Alcohol Syndrome (FAS) manifest a variety of central nervous system deficits which may include mental retardation, perceptual, linguistic and fine motor deficits, as well as behaviour problems. The neuropsychological performance of to preschool-aged children with FAS was compared to age, sex, and race-matched controls. Profile analysis revealed significant deficits in the FAS group on measures of intelligence and linguistic abilities. Comparison of group profiles revealed that the FAS group displayed impaired visual-motor integration in the presence of average visual-perceptual matching. The FAS children were also reported to exhibit a greater frequency of behaviour problems compared to controls. Although the FAS group were significantly growth delayed, the often reported association between cognitive abilities and skeletal growth was not evident. These results indicate that preschoolers with FAS display a distinct pattern of impairments, and understanding of this unique ability profile is important for the development of support and intervention programs for these patients and their families.

Body Height↗

[Assessment of disability and quality of life in patients with cognitive disorders].

OBJECTIVE: To identify disability, handicap and quality of life questionnaires available for patients with cognitive impairment. MATERIALS AND METHODS: We systematically reviewed the literature in Medline using the keywords assessment, evaluation, deficiency, disability, disadvantage, handicap, quality of life, scale, index, questionnaire, ICIDH-1, and ICIDH-2, combined with the cognitive deficits dysexecutive syndrome, memory deficits, attention deficits, neglect, apraxia, aphasia, agnosia and mood disorders. We focused on validated scales and distinguished scales dedicated to assess disability, handicap and quality of life. RESULTS: At the level of disability, global and specific scales are available. Specific scales exist for dysexecutive syndrome, memory deficits, attention deficits, unilateral neglect, aphasia and mood disorders. French adaptations of foreign language tests and original tests developed in French have been validated in these areas. No specific tool is available for isolated apraxia or agnosia. Generic scales and pathology-specific scales (for stroke, traumatic brain injury, and multiple sclerosis) are available for quality of life. For aphasia, specific tools are available for incapacity handicap and quality of life. CONCLUSION: Previous results show the impact of the ICIDH-1 framework on functional outcome assessment of cognitive impairments. This approach is often limited by the lack of theoretical background and by the difficulty to assess the involvement of environment and anosognosia.

Cognition Disorders↗

Lateralizing value of postictal automatisms in temporal lobe epilepsy.

PURPOSE: To describe clinical characteristics and lateralizing value of postictal automatisms in patients with temporal lobe epilepsy (TLE). METHODS: One hundred and ninety-three videotaped seizures of 55 consecutive patients with refractory TLE and postoperatively seizure-free outcome were analyzed. Ictal as well as postictal (manual, oral and speech) automatisms were monitored. RESULTS: Thirty-four (62%) of the 55 patients showed PA at least once during their seizures. Postictal automatism was observed in 70 (36%) attacks as manual (21%), oral (13%) or speech (9%) automatisms. Fifteen seizures contained a combination of two different postictal automatisms. The presence of postictal oral automatisms did not lateralize the seizure onset zone (p=0.834). Speech automatisms (repetitive verbal behavior) occurred more frequently after left-sided seizures (p=0.002). Postictal unilateral manual automatism showed no lateralizing value occurring by the ipsilateral hand in 10 and the contralateral upper limb in 6 seizures (p=0.454). CONCLUSION: : Postictal automatism is a relatively frequent phenomenon in TLE. Postictal speech automatism lateralizes the seizure onset zone to the left hemisphere. Our observation can help the presurgical evaluation of TLE because verbal perseveration frequently occurs spontaneously, even in seizures without appropriate postictal language testing.

Adolescent↗

Selective impairment as evidence for mental organisation of arithmetical facts: BB, a case of preserved subtraction?

We report here the case of a patient (BB), suffering from a precocious evolving dementia with impaired arithmetic performance, who showed specific and theoretical pertinent dissociations in basic mental arithmetic. First, in a task involving production of answers to simple arithmetic problems, a strong dissociation was found among operation: while multiplication was severely impaired, addition was moderately and subtraction only slightly impaired. A second dissociation was found between problems potentially solvable by rules and the others, with the former being better preserved. Finally, in multiplication verification tasks, the rate and distribution of errors among problems were not different from those observed in the multiplication production task. This pattern of performance like the one presented by the patient RG (Dagenbach and McCloskey, 1992), suggests first that stored arithmetical fact representations are segregated by arithmetic operation and second that a distinction has to be drawn between arithmetical rules and arithmetical facts. Last, the parallelism of performance observed here in verification and production tasks suggests that the same deficit(s) is (are) responsible for errors in both tasks.

Adult↗

Impairment of right brain-damaged patients on a linguistic cognitive task.

Forty right hemisphere-damaged patients and 35 normal controls were given five language tests, the Token test, a naming to description test, the Reporter's test, a word fluency test and a sentence anagram test. The sentence anagram test was the only measure on which right hemisphere patients performed significantly more poorly than control patients. It is surmised that this impairment may be indicative of a direct right hemisphere participation in linguistic performances.

Brain Damage, Chronic↗

Defective pantomime of object use in left brain damage: apraxia or asymbolia?

Disturbance of pantomime of object use in patients with left brain damage (LBD) and aphasia has been firmly established but its nature remains controversial. It may be due to an inability to perform movements from memory without external support by objects (apraxia) or to an inability to produce signs referring to absent objects and actions (asymbolia). The need to perform movements without external support is shared with imitation of gestures, and the demand to designate absent objects with drawing from memory. Both of these tasks have been found to be impaired in LBD. We examined pantomime of object use, drawing objects from memory, imitation of meaningless gestures, and aphasia in 40 patients with LBD and aphasia and compared them to healthy controls and to patients with right brain damage (RBD). Whereas drawing showed comparable sensitivity to LBD and RBD, pantomime was distinctly more disturbed in LBD than in RBD patients. Pantomime was worse than drawing in LBD but better than drawing in RBD. In the LBD group scores on pantomime showed significant correlations of very similar strength to drawing, imitation, and all language tests. Multidimensional scaling of the correlational structure placed pantomime in an intermediate position between verbal and non-verbal tests. We conclude that neither apraxia nor asymbolia can satisfactorily explain our results. It seems as if pantomime of object use taps a central aspect of left hemisphere function which is compromised by any LBD. We propose that this may be the ability to select and combine distinctive features of objects and actions.

Adult↗

Psycholinguistic aspects of learning disorders: identification and assessment.

This article examines in detail the relationship between language and learning disorders in children of school age and the behavioral characteristics of children with language and learning disabilities. Guidelines are offered for screening for identification of language disorders and for differential diagnosis.

Child↗

Prospective follow-up of intellectual development in children with a recent onset of epilepsy.

In this study the intellectual development of children with a recent onset of epilepsy was compared to that of children without epilepsy. Eleven children aged 7-15 years and with mean duration of epilepsy of 2 years and 38 children without epilepsy performed three times the dutch Wechsler intelligence scale for children-revised and the Beery developmental test for visuo-motor integration. The children with epilepsy did show a significantly less gain in FSIQ score over the 11 years of follow-up. Performance intelligence scores and Beery standardized scores showed a trend towards this effect. Findings partly support the hypothesized cascadic model of deterioration, in which children with epilepsy are supposed to show a process of mental deterioration shortly after the onset of epilepsy. The lack of evidence of an absolute mental deterioration are discussed in terms of methodology, mental age and premorbid intelligence level.

Adolescent↗

Bright spots: correlations of gray matter volume with IQ in a normal pediatric population.

The localization of brain areas related to cognitive functions has yet to be thoroughly explored in children. We therefore set out to apply volumetric, voxel-based, and structural connectivity analyses to magnetic resonance images from a large sample of healthy children. We could confirm a strong correlation of whole-brain gray matter volume and the individual intelligence quotient; however, this correlation only developed with age in our sample, in that it was not present in the younger children. With the application of an optimized protocol for voxel-based morphometry, the anterior cingulate was shown to be directly correlated with a measure of human intelligence. Furthermore, an analysis of structural connectivity identified gray matter volume in several distinct brain areas to be related to cognitive functions. The implications of our findings for normal development, pathological processes, and our understanding of cognition are discussed and related to previous findings.

Aging↗

Consonant clusters in disordered speech: constraints and correspondence patterns.

Comparison of patterns of cluster realization from 47 children ranging in age from 3;4 to 6;8 with functional (non-organic) speech disorders with those reported in the literature for normal acquisition reveals that these patterns are essentially the same for both groups. Using a two-level generative phonology for children's independent systems, further analysis of cluster realizations by means of feature geometry and underspecification theory reveals that there are systematic and principled relationships between adult representations of clusters and children's underlying representations and between children's underlying representations and their phonetic representations. With special emphasis on coalescence phenomena, it is suggested that the apparent diversity in children's cluster realizations can be reduced to four constraints on the form of underlying and phonetic representations.

Child↗

Long-term neuropsychological outcomes of very low birth weight: associations with early risks for periventricular brain insults.

Few follow-up studies of children with very low birth weight (VLBW, <1,500 g) have examined neuropsychological sequelae at later ages or neonatal risks as predictors of these outcomes. The present study assessed cognitive skills at mean age 16 years in 48 participants with <750 g birth weight, 47 with 750-1,499 g birth weight, and 52 term-born controls. Our major objectives were to delineate the long-term cognitive consequences of VLBW, and to determine if risks for periventricular brain insults accounted for variations in outcomes. Analysis revealed poorer outcomes for the <750 g group than for term-born controls on nearly all measures, with specific impairments in visual-motor skills, spatial memory, and executive function. Predictors of outcome for participants with VLBW included lower birth weight, lower weight for gestational age, and a longer period of oxygen requirement for chronic lung disease. The longer-term consequences of VLBW are consistent with expectations based on early brain pathology and suggest limitations to functional plasticity.

Adolescent↗

Effectiveness of contextual repetition priming treatments for anomia depends on intact access to semantics.

Naming of two semantically impaired aphasic patients was treated with the contextual repetition priming technique, which involves repeated repetition of names of pictures that are related semantically, phonologically, or are unrelated. Our previous studies using this technique have suggested that patients with impaired access to lexical-semantic representations benefit in the short-term from this treatment technique, but show no long-term improvement in naming. In contrast, patients with good access to semantics show short- and long-term benefits from this treatment. Here we report two studies of treatment for two individuals with aphasia affecting access to lexical semantics and anomia but spared access to input and output phonology and spared conceptual semantics. We predicted that they would show short-term facilitation from the contextual priming, but no long-term improvements in naming. The results confirmed the prediction. An account of this pattern is offered within the framework of an interactive activation model of word retrieval. Additionally, we discuss alternative techniques for addressing naming deficits when access to semantics is impaired.

Adult↗

Association of autism severity with a monoamine oxidase A functional polymorphism.

A functional polymorphism (the upstream variable-number tandem repeat region, or uVNTR) in the monoamine oxidase A (MAOA) promoter region has been reported to be associated with behavioral abnormalities as well as increased serotonergic responsivity. We examined the relation between MAOA-uVNTR alleles and the phenotypic expression of autism in 41 males younger than 12.6 years of age. Children with the low-activity MAOA allele had both lower intelligence quotients (IQ) and more severe autistic behavior than children with the high-activity allele. In follow-up testing of 34 of the males at the 1-year time-point, those with the low-activity allele showed a worsening in IQ but no change in the severity of their autistic behavior. We conclude that functional MAOA-uVNTR alleles may act as a genetic modifier of the severity of autism in males.

Activities of Daily Living↗

The role of neuroscience in the remediation of students with dyslexia.

Dyslexia is a specific learning disability that is neurobiological in origin. It is characterized by difficulties with accurate and/or fluent word recognition, spelling and decoding abilities. Research findings agree that these and other observed behavioral manifestations largely result from a deficit in the phonological component of language. However, conflicting theories on the exact nature of the phonological deficit have given rise to divergent treatment approaches. Recent advances in functional brain imaging and genetics have allowed these theories to be examined more closely. If implemented appropriately, commercial programs can be effective in identifying dyslexia. Treatment of dyslexia has been advanced through neuroscience, yet further study is needed to provide rigorous, reproducible findings that will sustain commercial approaches.

Brain↗

The impact of aided language stimulation on symbol comprehension and production in children with moderate cognitive disabilities.

Over the past decade, aided language stimulation has emerged as a strategy to promote both symbol comprehension and symbol production among individuals who use graphic mode communication systems. During aided language stimulation, an interventionist points to a graphic symbol while simultaneously producing the corresponding spoken word during natural communicative exchanges. The purpose of this study was to determine the impact of aided language stimulation on children with moderate cognitive disabilities. Three preschool children with moderate cognitive disabilities who were functionally nonspeaking participated in the investigation. The investigator implemented a multiple-probe design across symbol sets/activities. Elicited probes were used to determine whether the children increased their comprehension and production of graphic symbols. Results indicated that all 3 children displayed increased symbol comprehension and production following the implementation of aided language stimulation.

Child, Preschool↗

The efficacy of treatment for children with developmental speech and language delay/disorder: a meta-analysis.

A meta-analysis was carried out of interventions for children with primary developmental speech and language delays/disorders. The data were categorized depending on the control group used in the study (no treatment, general stimulation, or routine speech and language therapy) and were considered in terms of the effects of intervention on expressive and receptive phonology, syntax, and vocabulary. The outcomes used in the analysis were dependent on the aims of the study; only the primary effects of intervention are considered in this review. These were investigated at the level of the target of therapy, measures of overall linguistic development, and broader measures of linguistic functioning taken from parent report or language samples. Thirty-six articles reporting 33 different trials were found. Of these articles, 25 provided sufficient information for use in the meta-analyses; however, only 13 of these, spanning 25 years, were considered to be sufficiently similar to be combined. The results indicated that speech and language therapy might be effective for children with phonological or expressive vocabulary difficulties. There was mixed evidence concerning the effectiveness of intervention for children with expressive syntax difficulties and little evidence available considering the effectiveness of intervention for children with receptive language difficulties. No significant differences were found between interventions administered by trained parents and those administered by clinicians. The review identified longer duration (>8 weeks) of therapy as being a potential factor in good clinical outcomes. A number of gaps in the evidence base are identified.

Child↗