Neurocognitive Rehabilitation of Down Syndrome :Early Years

Publication subTitle :Early Years

Author: Jean-Adolphe Rondal; Juan Perera; Donna Spiker  

Publisher: Cambridge University Press‎

Publication year: 2011

E-ISBN: 9781139119993

P-ISBN(Paperback): 9781107400436

Subject: R74 Neurology and Psychiatry

Keyword: 神经病学与精神病学

Language: ENG

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Neurocognitive Rehabilitation of Down Syndrome

Description

Down syndrome is one of the most commonly occurring developmental disorders and it is now possible to conceptualize and define opportunities for neurocognitive rehabilitation for those with the condition. This book describes how early cognitive intervention in children with Down syndrome can be carried out, and can reduce, or compensate for, the major deficits characteristic of the condition. This comprehensive account relates the neurocognitive approach to the major therapeutic endeavors in the neighboring fields of neurogenetics, experimental environmental enrichment, molecular genetics, pharmacology, pediatrics and cardiology for infants with Down syndrome. Neurocognitive Rehabilitation of Down Syndrome provides the guidance required to establish effective rehabilitation programs, and is essential reading for developmental clinicians, pediatricians, neuropsychologists and other health professionals.

Chapter

Conclusion

Summary

References

2 The history of early intervention for infants and young children with Down syndrome and their families

Goals of early intervention

Changing expectations for children with Down syndrome

Research about early intervention: past, present, and future

Early efficacy studies

More recent efficacy studies

Early education policy and practice for children with disabilities

Promoting inclusive educational programming

Child characteristics that impact on inclusion

Strategies for promoting language, communication, and social development

Changing perspectives about parent participation in early intervention

US national data on early intervention and preschool special education

Changes in assessment: research and practice and future directions

Accountability in early childhood

Functional child outcomes in an accountability system

Family outcomes in an accountability system

Expectations and information about Down syndrome

Follow-up studies of adults with Down syndrome

Conclusions and looking to the future

Summary

References

3 Advances in clinical endpoints for neurocognitive rehabilitation in Down syndrome

Key properties of clinical endpoint assessments: development of the Arizona Cognitive Test Battery for Down syndrome

The cognitive and behavioral profile of Down syndrome: key clinical endpoints

Hippocampal memory

Verbal short-term memory

Frontal functions

Cerebellar functions

Language

Adaptive behavior

Maladaptive behavior

Intelligence quotient

Key issues in efficacy assessment of neurocognitive intervention in Down syndrome

Translation from rodents to humans

Which age?

What constitutes significant change?

Discussion

Summary

Acknowledgments

References

Section 2 – Genetics, brain, and animal models

4 New perspectives on molecular and genic therapies in Down syndrome

Down syndrome and phenotypes

Murine models

Candidate genes

Criteria used to define candidate genes

Chromosomic localization

Functions or potential functions

Territories of expression

Level of expression in Down syndrome or in transgenic mice

Associated phenotypic changes in murine models

Gene-based corrective strategies

RNA targets

Protein targets

Protein activities as targets

Cell cycle pathways

Sonic hedgehog pathway

Prozac and neurogenesis

Synaptic plasticity and memory pathways

Gamma-aminobutyric acid pathways

N-methyl-D-aspartate receptor antagonist memantine

DYRK1A pathway

Future prospects

Summary

References

5 Brain plasticity and environmental enrichment in Ts65Dn mice, an animal model for Down syndrome

The concept of neuronal plasticity and enriched environment

Mouse models of Down syndrome

The effect of an enriched environment on Ts65Dn mice

Synaptic plasticity

Neurogenesis

Proteins encoded by triplicated genes

Summary

References

6 Development of the brain and metabolism

Introduction

Early studies of nutrition in Down syndrome and attempts to improve brain development and ameliorate intellectual disability in Down syndrome

Genes on HSA21 relevant to metabolism and nutrition

Mouse models and the study of metabolism, brain development, and Down syndrome

Methyl group metabolism and Down syndrome

Oxidative stress and Down syndrome

Inositol metabolism and Down syndrome and brain development

“Omics” studies of fetal development in Down syndrome and future directions

Conclusions and future directions

Summary

Acknowledgments

References

Section 3 – Pharmacological and medical management and treatment

7 Pharmacotherapy for children with Down syndrome

Introduction

Intelligence: cognition, memory, and learning

Molecular pharmacology

Neurobiology

Thinking about clinical trials

Previous strategies

Recent strategies

How clinical trials are designed presently

Measuring outcomes

Overlooked physiological variables

Cognitive medication trials

Acetylcholine and the cholinergic system

Cholinergic medications

Donepezil in adults

Donepezil in children

Rivastigmine in adults

Rivastigmine in children

Macrocircuits and pyramidal neurons: why all the excitement?

Glutamate-based strategies

Nootropic medications

Piracetam in children

Microcircuits and interneurons: inhibitory tendencies

Targeting the prefrontal cortex in Down syndrome

Psychotropic medication in persons with Down syndrome

Physiologically impairing symptoms

Strategies to reduce physiologically impairing symptoms

The urgency of reducing physiologically impairing symptoms

Concerns about moving forward

Futuristic notions of biological therapy

Merging biological and educational strategies

The Ts65Dn mouse model of Down syndrome

Summary

References

8 Early medical caretaking and follow-up

Introduction

Congenital malformations

Sensory defects

Immune disorders and autoimmune diseases

Cancer

Musculoskeletal disorders

Short stature

Sleep problems

Seizure disorders

Normal and pathological ageing

Nutritional problems

Summary

References

9 Evaluation and management of cardiovascular diseases in Down syndrome

Introduction

Pathophysiology of cardiovascular disease in Down syndrome

Congenital heart diseases in Down syndrome

Atrioventricular septal defect

Ventricular septal defect

Tetralogy of Fallot

Atrial septal defect

Patent ductus arteriosus and aortic arch malformations

Surgical experience at the Cliniques universitaires Saint-Luc, Brussels

Results

Discussion

Summary

References

Section 4 – Early development and intervention

10 Developmental models as frameworks for early intervention with children with Down syndrome

Werner and the universality of development

Developmental sequences

Universality and uniqueness

The notion of a whole child

Cicchetti and an expanded developmental approach

Ecological theory

Complex developmental trajectories

Implications of developmental models

Summary

Acknowledgments

References

11 Aspects of motor development in Down syndrome

Introduction

Fundamental perceptual–motor processes

Development of reach-to-grasp

Development of walking

Early intervention

Program planning

Generalization of skills

Recent research from neuroscience

Conclusions

Summary

Author notes

References

12 Memory development and learning

Introduction

Short-term memory and Down syndrome

Long-term memory in Down syndrome: a diffuse and pervasive impairment?

Memory, Down syndrome, and brain development

Conclusions

Summary

References

13 Prelinguistic and early development, stimulation, and training in children with Down syndrome

Language before birth

Prelanguage in the first year

Prelanguage intervention

Orofacial physical therapy

Lexical development and intervention

Grammatical development

Conclusions

Summary

References

14 Speech perception, stimulation, and phonological development

Phonological difficulties in children with Down syndrome

An uneven profile

Inconsistency and non-developmental errors

Possible causes of phonological difficulties

Speech production

Speech perception

The importance of early speech perception in typical development

The impact of early auditory deprivation – the case of children with cochlear implants

Phonological difficulties

Early auditory deprivation and higher-level speech processing abilities

Questions to consider for intervention

Summary

References

15 Goal-directedness as a target for early intervention in Down syndrome

Goal-directedness as a target for early intervention in Down syndrome

Motor exploration, cognitive representations, and goal-directed behavior

Early motor exploration

Cognitive consequences of motor exploration

Object affordances

Relevance for development in Down syndrome

Causality

Relevance for development in Down syndrome

Goal-directedness and development in Down syndrome

Implications for goal-directed behavior

Clinical interpretation and intervention implications

Targeting motor exploration

Strategies to enhance goal-directedness

Summary

References

16 The role of parents of children with Down syndrome and other disabilities in early intervention

The parenting model of child development

The parenting model and developmental intervention

Parenting and intervention effectiveness

Parenting as intervention

Long-term effects of responsive parenting

Making sense of the role of parents in intervention

Why parents are so important to childrens development

Why responsiveness is so important to childrens development

Summary

References

Section 5 – Therapeutic perspectives

17 Perspectives of hybrid therapeutic strategies in intellectual disabilities and Down syndrome

Summary

References

Conclusions

Index

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