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Journal of Intellectual Disability - Diagnosis and Treatment

Contextual Integration of Causal Coherence in People with Down Syndrome: Evidence from Figurative Comprehension
Pages 55-62
Ching-Fen Hsu

DOI: http://dx.doi.org/10.6000/2292-2598.2016.04.01.7

Published: 14 March 2016

 


Abstract: Contextual integration is seldom explored in people with Down syndrome (DS). This study aimed to investigate this ability by comparing causal inferences made by people with DS who were presented with homonyms embedded within sentences and asked to choose which of three interpretations (figurative meaning, literal meaning, or unrelated meaning) was correct. Accuracy was the dependent variable. Each homonym was presented in a scenario familiar to the participants. The results revealed that the participants with DS were the least accurate in responding to figurative meanings and erred most compared to matched controls in chronological age and mental age. It was concluded that people with DS were developmentally delayed in causal inferences and weak central coherence is indeed a syndrome-general phenotype across populations with developmental disorders.

Keywords: Down syndrome, causal inference, weak central coherence.
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Journal of Intellectual Disability - Diagnosis and Treatment

Mucopolysaccharidosis II, IV-A and VI: First Colombian Neuropsychological Characterization 
Pages 63-7388x31
Nolly Nataly Castañeda-Ibáñez and María Rocio Acosta-Barreto

DOI: http://dx.doi.org/10.6000/2292-2598.2016.04.01.8

Published: 14 March 2016

 


Abstract: The Mucopolysaccharidosis [MPS] are a group of orphan or rare genetic diseases characterized by lysosomal storage disorders which are recognized by bone malformations and neuropsychological implications that have not been studied so far. For this reason, the first cross-sectional descriptive study of neuropsychological nature was conducted on variants of the disease, Hunter Syndrome [MPS II], Morquio A Syndrome [MPS IV A] and Maroteaux-Lamy syndrome [MPS VI] in 21 children and adolescents aged 3 to 19 years old, at the Colombian Association of Patients with Lysosomal Storage Diseases [ACOPEL, for its Spanish acronym] in Bogotá, Colombia. Results indicate that for the different types of MPS tested is not easy to make a neuropsychological characterization and generalize these results to other populations. However, MPS type II shows moderate to severe cognitive deficit with a compromise in psychomotor development. Morquio A presents average intelligence, and MPS type VI points to deficits partially related to sensory impairment, implying significant differences between them. It is important to continue carrying on this type of studies to achieve a better classification of these diseases according to their cognitive functioning from the neuropsychological perspective.

Keywords: Mucopolysaccharidosis, Hunter Syndrome, Morquio A Syndrome, Maroteaux-Lamy Syndrome, Neuropsychological characterization.
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Journal of Intellectual Disability - Diagnosis and Treatment

Early Intervention of Kindergarten Children at Risk for Developmental Disabilities: A Greek Paradigm
Pages 238-246
Maria Tzouriadou, Constantinos Vouyoukas, Eleni Anagnostopoulou and Lito E. Michalopoulou

DOI: http://dx.doi.org/10.6000/2292-2598.2015.03.04.10

Published: 15 January 2016

 


Abstract: Internationally most of the children at risk for developmental disabilities (intellectual disabilities, learning disabilities and language impairments) are detected within preschool settings. In Greece there are no early intervention services mandated nationwide. In practice, not detailed guidance and support is given to preschool teachers regarding the assessment and intervention of children at risk. This study provides research evidence from the implementation of an early intervention program in inclusive kindergartens, which is part of the practical training of students from the Faculty of Preschool Education Sciences, Department of Psychology and Special Education, Aristotle University of Thessaloniki, Greece. Response to intervention, as applied to kindergartens, was used to detect children not responding to core kindergarten curriculum. An inclusive early intervention educational program was implemented based on children’s profiles of strengths and weaknesses. At risk children participated in large group classroom activities with all children. For these children a specialized inclusive program with emphasis on school readiness was implemented. For children with developmental disabilities, an intensive individualized program was implemented, which put emphasis on the improvement of their cognitive abilities and socialization.

Keywords: Kindergarten, early intervention, inclusive curricula, developmental disabilities, at risk children, response to intervention, dynamic assessment.
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Journal of Intellectual Disability - Diagnosis and Treatment

Cognitive Consequences in Children with Epilepsy 
Pages 74-78
Hideaki Kanemura and Masao Aihara

DOI: http://dx.doi.org/10.6000/2292-2598.2016.04.01.9

Published: 14 March 2016

 


Abstract: Some epilepsy is, in general, often associated with cognitive problems that can also affect a patient’s adjustments. Epileptic seizures result from an excessive, synchronous discharge of cerebral neurons. Interictal paroxysmal electroencephalogram (EEG) abnormalities are regarded as a correlate of persistent pathological neuronal discharges. Thus, correlation between cognitive deterioration and seizure severities/EEG paroxysmal abnormalities should be investigated. We have previously measured frontal/prefrontal lobe volumes using three-dimensional (3D)-magnetic resonance imaging (MRI) in children with benign childhood epilepsy with centrotemporal spikes, epilepsy with continuous spike-waves during slow sleep, frontal lobe epilepsy, and Panayiotopoulos syndrome, and confirmed that longer active seizure period as frequent spike-waves coupled with the occurrence of frequent seizures and presence of status epilepticus may be associated with prefrontal lobe growth disturbance, which relates to cognitive impairments. These findings suggest that seizure severities such as repeated seizures and presence of status epilepticus, and the subclinical paroxysmal EEG abnormalities may induce prefrontal lobe growth disturbance, which leads to intellectual impairments. Achieving better seizure control and remission for paroxysmal EEG abnormalities is a key to improve quality of life (QOL) in children with epilepsy. From the perspective of decreased cognitive problems and improving QOL, management may be required to remit seizures and paroxysmal EEG abnormalities as soon as possible to achieve optimal prognosis in epilepsy.

Keywords: Epilepsy, Seizure severity, Electroencephalogram (EEG), Intellectual impairments, Children.
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Journal of Intellectual Disability - Diagnosis and Treatment

Reaching the Hard to Reach: Mobile Development Screening Van to the Rescue
Pages 247-256
Joannie Busillo-Aguayo, Wendy Murawski and Ivor Weiner

DOI: http://dx.doi.org/10.6000/2292-2598.2015.03.04.11

Published: 15 January 2016

 


Abstract: Currently more than 15% of children have an emotional, behavioral, or developmental concern. In spite of recommendations by the American Academy of Pediatrics and the Centers for Disease Control and Prevention to conduct universal developmental surveillance and screening with children at 9, 18, and 24 months of age, fewer than 30% of children under 6 years of age ever receive a developmental screening. Children in low-income and diverse communities are even less likely to be screened by a medical provider and/or referred for further diagnostic evaluation when predictive concerns are identified. As part of a cross-agency collaborative effort involving a family resource center, a child care resource and referral agency, a regional center for developmental disabilities, and a master’s degree program in early childhood education, the Mobile Developmental Screening Van Project conducted outreach to provide free developmental screening with families of children 0-8 years of age in diverse and low-income communities within the greater Los Angeles County. Using the Parent Evaluation of Developmental Status (PEDS) screening tool, 94 children were screened over the course of 6 months, with 33% showing 2 or more predictive concerns that resulted in referrals for further diagnostic evaluation. The feasibility of reaching families in hard to reach communities using a mobile screening van, as well as study limitations and recommendations for next steps, are discussed.

Keywords: Developmental screenings, low-income, mobile screening, early childhood, community collaboration.
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