Owens PL, Kerker BD, Zigler E
… +1 more, Horwitz SM
Ment Retard Dev Disabil Res Rev
· 2006 · PMID 16435325
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Over the past 20 years, there has been an increased emphasis on health promotion, including prevention activities related to vision and oral health, for the general population, but not for individuals with intellectual d...Over the past 20 years, there has been an increased emphasis on health promotion, including prevention activities related to vision and oral health, for the general population, but not for individuals with intellectual disability (ID). This review explores what is known about the prevalence of vision problems and oral health conditions among individuals with ID, presents a rationale for the increased prevalence of these conditions in the context of service utilization, and examines the limitations of the available research. Available data reveal a wide range of prevalence estimates for vision problems and oral health conditions, but all suggest that these conditions are more prevalent among individuals with ID compared with the general population, and disparities exist in the receipt of preventive and early treatment for these conditions for individuals with ID. Recommendations for health improvement in these areas include better health planning and monitoring through standardized population-based data collection and reporting and increased emphasis on health promotion activities and early treatment in the healthcare system.
Ment Retard Dev Disabil Res Rev
· 2006 · PMID 16435324
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This literature review describes the physical activity behavior of adults with mental retardation consistent with the U.S. Surgeon General's recommendation of 30 minutes of moderate intensity physical activity on 5 or mo...This literature review describes the physical activity behavior of adults with mental retardation consistent with the U.S. Surgeon General's recommendation of 30 minutes of moderate intensity physical activity on 5 or more days per week. The proportion of participants achieving this criterion ranges from 17.5 to 33%. These data are likely to be generous estimates of activity as individuals included in physical activity studies to date have been relatively young and healthy volunteers with mild to moderate limitations. Major sources of physical activity were walking and cycling for transport, chores and work, dancing, and Special Olympics. There is a pressing need to conduct studies using appropriately powered representative samples and to validate measures that assess physical activity less directly; including methodologies in which proxy respondents are used. Accurate information about existing patterns of behavior will enhance the development of effective strategies to promote physical activity among persons with mental retardation.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240413
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Positron emission tomography (PET) is a technique that enables imaging of the distribution of radiolabeled tracers designed to track biochemical and molecular processes in the body after intravenous injection or inhalati...Positron emission tomography (PET) is a technique that enables imaging of the distribution of radiolabeled tracers designed to track biochemical and molecular processes in the body after intravenous injection or inhalation. New strategies for the use of radiolabeled tracers hold potential for imaging gene expression in the brain during development and following interventions. In addition, PET may be key in identifying the physiological consequences of gene mutations associated with mental retardation. The development of high spatial resolution microPET scanners for imaging of rodents provides a means for longitudinal study of transgenic mouse models of genetic disorders associated with mental retardation. In this review, we describe PET methodology, illustrate how PET can be used to delineate biochemical changes during brain development, and provide examples of how PET has been applied to study brain glucose metabolism in Rett syndrome, serotonin synthesis in autism, and GABAA receptors in Angelman's syndrome and Prader-Willi syndrome. Future application of PET scanning in the study of mental retardation might include measurements of brain protein synthesis in fragile X syndrome and tuberous sclerosis complex, two common conditions associated with mental retardation in which cellular mechanisms involve dysregulation of protein synthesis. Mental retardation results in life-long disability, and application of new PET technologies holds promise for a better understanding of the biological underpinnings of mental retardation, with the potential to uncover new treatment options.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240412
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Studies on human patients and animal models of disease have shown that disruptions in prenatal and early postnatal brain development are a root cause of mental retardation. Since proper brain development is achieved by a...Studies on human patients and animal models of disease have shown that disruptions in prenatal and early postnatal brain development are a root cause of mental retardation. Since proper brain development is achieved by a strict spatiotemporal control of neurogenesis, cell migration, and patterning of synapses, abnormalities in one or more of these events during prenatal development can lead to cognitive dysfunction after birth. Many of underlying causes of mental retardation must therefore be studied in developing brains. To aid in this research, live imaging using laser scanning microscopy (LSM) has recently allowed neuroscientists to delve deeply into the complex three-dimensional environment of the living brain to record dynamic cellular events over time. This review will highlight recent examples of how LSM is being applied to elucidate both normal and abnormal cortical development.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240411
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The human and mouse genome projects elucidated the sequence and position map of innumerous genes expressed in the central nervous system (CNS), advancing our ability to manipulate these sequences and create models to inv...The human and mouse genome projects elucidated the sequence and position map of innumerous genes expressed in the central nervous system (CNS), advancing our ability to manipulate these sequences and create models to investigate regulation of gene expression and function. In this article, we reviewed gene targeting methodologies with emphasis on applications to CNS development and neurodevelopmental disorders.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240410
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As researchers have examined the so-called "behavioral phenotypes" of different genetic mental retardation conditions, the measurement of behavior becomes increasingly important. From earlier, more global examinations of...As researchers have examined the so-called "behavioral phenotypes" of different genetic mental retardation conditions, the measurement of behavior becomes increasingly important. From earlier, more global examinations of noteworthy behaviors or profiles, recent researchers have moved from reliance on a single, all-encompassing questionnaire to more multimethod, multireporter approaches. This paper summarizes strengths and weaknesses in utilizing parent-report questionnaires and checklists; psychiatric nosology and specific psychiatric questionnaires; observational studies; reports from individuals with mental retardation themselves; profile analyses of psychometric test and subtest scores; and "grouping" analyses of items (factor analysis) and people (cluster analysis). Although we advocate a mixture of approaches to behavioral assessment, in future work researchers will require more theoretically driven methods for studying behavior, greater acknowledgment that the child's behavior is simply one event in ongoing interactions-transactions with other people, and more attention to age-related changes in behavioral phenotypes.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240409
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Mental retardation affects 2 to 3% of the US population. It is defined by broad criteria, including significantly subaverage intelligence, onset by age 18, and impaired function in a group of adaptive skills. A myriad of...Mental retardation affects 2 to 3% of the US population. It is defined by broad criteria, including significantly subaverage intelligence, onset by age 18, and impaired function in a group of adaptive skills. A myriad of genetic and environmental causes have been described, but for approximately half of individuals diagnosed with mental retardation the molecular basis remains unknown. Genomic microarrays, also called array comparative genomic hybridization (array CGH), represent one of several novel technologies that allow the detection of chromosomal abnormalities, such as microdeletions and microduplications, in a rapid, high throughput fashion from genomic DNA samples. In one early application of this technology, genomic microarrays have been used to characterize the extent of chromosomal changes in a group of patients diagnosed with one particular type of disorder that causes mental retardation, such as deletion 1p36 syndrome. In another application, DNA samples from individuals with idiopathic mental retardation have been assayed to scan the entire genome in attempts to identify chromosomal changes. Genomic microarrays offer both a genome-wide perspective of chromosomal aberrations as well as higher resolution (to the level of approximately one megabase) compared to alternative available technologies.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240408
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Studying the biological mechanisms underlying mental retardation and developmental disabilities (MR/DD) is a very complex task. This is due to the wide heterogeneity of etiologies and pathways that lead to MR/DD. Breakth...Studying the biological mechanisms underlying mental retardation and developmental disabilities (MR/DD) is a very complex task. This is due to the wide heterogeneity of etiologies and pathways that lead to MR/DD. Breakthroughs in genetics and molecular biology and the development of sophisticated brain imaging techniques during the last decades have facilitated the emergence of a field called Behavioral Neurogenetics. Behavioral Neurogenetics focuses on studying genetic diseases with known etiologies that are manifested by unique cognitive and behavioral phenotypes. In this review, we describe the principles of magnetic resonance imaging (MRI) techniques, including structural MRI, functional MRI, and diffusion tensor imaging (DTI), and how they are implemented in the study of Williams (WS), velocardiofacial (VCFS), and fragile X (FXS) syndromes. From WS we learn that dorsal stream abnormalities can be associated with visuospatial deficits; VCFS is a model for exploring the molecular and brain pathways that lead to psychiatric disorders for which subjects with MR/DD are at increased risk; and finally, findings from multimodal imaging techniques show that aberrant frontal-striatal connections are implicated in the executive function and attentional deficits of subjects with FXS. By deciphering the molecular pathways and brain structure and function associated with cognitive deficits, we will gain a better understanding of the pathophysiology of MR/DD, which will eventually make possible more specific treatments for this population.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240407
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New knowledge of the perceptual, discriminative, and memory capabilities of very young infants has opened the door to further evaluation of these abilities in infants who have risk factors for cognitive impairments. A ne...New knowledge of the perceptual, discriminative, and memory capabilities of very young infants has opened the door to further evaluation of these abilities in infants who have risk factors for cognitive impairments. A neurophysiologic technique that has been very useful in this regard is the recording of event-related potentials (ERPs). The event-related potential (ERP) technique is widely used by cognitive neuroscientists to study cognitive abilities such as discrimination, attention, and memory. This method has many attractive attributes for use in infants and children as it is relatively inexpensive, does not require sedation, has excellent temporal resolution, and can be used to evaluate early cognitive development in preverbal infants with limited behavioral repertories. In healthy infants and children, ERPs have been used to gain a further understanding of early cognitive development and the effect of experience on brain function. Recently, ERPs have been used to elucidate atypical memory development in infants of diabetic mothers, difficulties with perception and discrimination of speech sounds in infants at risk for dyslexia, and multiple areas of cognitive differences in extremely premature infants. Atypical findings seen in high-risk infants have correlated with later cognitive outcomes, but the sensitivity and specificity of the technique has not been studied, and thus evaluation of individual infants is not possible at this time. With further research, this technique may be very useful in identifying children with cognitive deficits during infancy. Because even young infants can be examined with ERPs, this technique is likely to be helpful in the development of focused early intervention programs used to improve cognitive function in high-risk infants and toddlers.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16240406
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Drosophila melanogaster is emerging as a valuable genetic model system for the study of mental retardation (MR). MR genes are remarkably similar between humans and fruit flies. Cognitive behavioral assays can detect redu...Drosophila melanogaster is emerging as a valuable genetic model system for the study of mental retardation (MR). MR genes are remarkably similar between humans and fruit flies. Cognitive behavioral assays can detect reductions in learning and memory in flies with mutations in MR genes. Neuroanatomical methods, including some at single-neuron resolution, are helping to reveal the cellular bases of faulty brain development caused by MR gene mutations. Drosophila fragile X mental retardation 1 (dfmr1) is the fly counterpart of the human gene whose malfunction causes fragile X syndrome. Research on the fly gene is leading the field in molecular mechanisms of the gene product's biological function and in pharmacological rescue of brain and behavioral phenotypes. Future work holds the promise of using genetic pathway analysis and primary neuronal culture methods in Drosophila as tools for drug discovery for a wide range of MR and related disorders.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161098
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A wide variety of tests are available to assess the central nervous system (CNS) function of the toddler and preschool-aged child. These tests vary as to function; qualities and abilities tapped; facility with which they...A wide variety of tests are available to assess the central nervous system (CNS) function of the toddler and preschool-aged child. These tests vary as to function; qualities and abilities tapped; facility with which they can be learned, administered, and scored; availability of test materials and manuals or training videos; and strength of standardization and validation data. Some were developed to screen development of large numbers of children. Others were developed to evaluate a child for diagnosis of disability or delineation of a child's strengths and weaknesses. Some broadly screen or assess multiple aspects of development, while some focus on specific abilities. A limitation of all these tests is that they tap only a small portion of a child's abilities at a given point in time. Administration of a variety of different tests provides a more complete evaluation of a child's abilities. Tests that can follow a child's development over time tap into the continuum of human development. The ability to measure various aspects of CNS functional development is a first step in addressing our greatest challenge, how to promote and support a child's development.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161097
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Approximately 400,000 preschool children have a major neurodevelopmental disorder impacting on mobility, cognitive-adaptive, or communicative skills. As many as 1 in 3 children live at psychosocial disadvantage because o...Approximately 400,000 preschool children have a major neurodevelopmental disorder impacting on mobility, cognitive-adaptive, or communicative skills. As many as 1 in 3 children live at psychosocial disadvantage because of poverty, parental mental illness or substance misuse, or low parental educational (i.e. less than high school). In the past decade over 500,000 preschool children have survived being born with very low birth weight (1001-1500 g) or extremely low birth weight status (< or =1000 g). Given the scope of these risks and the importance of optimizing outcomes for vulnerable children, this review will highlight advances in functional assessment using adaptive and multiattribute health-related quality of life measures. A framework based on the International Classification of Functioning, (ICF) World Health Organization and the Dynamic Kaleidoscope Model of the Institute of Medicine (IOM) will be described and illustrated with examples of children receiving new biomedical technologies. Assessment scales were chosen for review if they measured adaptive skills or multiattribute health status and had been used in child disability populations. Instruments reviewed include the Infant and Toddler Quality of Life Questionnaire (ITQOL), The Netherlands Office of Prevention Assessment of Preschool Quality of Life (TAPQOL), the Health Status Classification System-PreSchool (HSCS-PS), the Pediatric Evaluation of Disability Inventory (PEDI), the Vineland Adaptive Behavior Scale (VABS), the Warner Inventory of Developmental and Emerging Adaptive and Functional Skills (Warner IDEA-FS), the Scales of Independent Behavior Revised (SIB-R) Early Development Form, the Pediatric Functional Independence Measure (WeeFIM), and the Pediatric Quality of Life Inventory Version 4 (PedsQL 4.0). By measuring functional and adaptive skills and health-related quality of life, we can help devise intervention strategies that optimize developmental independence, family supports, and community participation among children who are at risk for neurodevelopmental disabilities or who have evolving established neurodevelopmental disabilities.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161096
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The present paper reviews the methods available for neurological or neuromotor evaluation at preschool age. General textbooks on pediatric neurology describe the neurological examination at preschool age in terms of the...The present paper reviews the methods available for neurological or neuromotor evaluation at preschool age. General textbooks on pediatric neurology describe the neurological examination at preschool age in terms of the assessment of the evaluation of cranial nerves, muscle tone, muscle power, reflexes, and the presence of abnormal movements. They stress the fact that assessment at preschool age is difficult because of the time needed to achieve the child's cooperation. Noncooperation at the preschool neurological exam is associated with an increased risk for learning and behavioral problems at school age. At present three age-specific and standardized test for neurological or neuromotor evaluation at preschool age are available. The method of Amiel-Tison and Gosselin, of which information can be accessed easily, has the drawback that it focuses on muscle tone and reflexes. It pays little attention to the quality of spontaneous motor behavior. The other two methods, i.e., the neuromotor behavioral inventory (NBI) and the Hempel assessment, are probably more promising in terms of assessment of minor neurological dysfunction as these methods pay ample attention to the child's quality of motor behavior. All methods have in common that information in terms of concurrent validity is scarce, with no information on predictive validity. This means that further research on applicability and validity of preschool neuromotor assessment is urgently needed.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161095
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This paper orients the reader to social communication assessment and reviews methods for assessing social communication behavior in children from toddlerhood through the preschool years. Most standardized, normed tests o...This paper orients the reader to social communication assessment and reviews methods for assessing social communication behavior in children from toddlerhood through the preschool years. Most standardized, normed tests of language in this age range focus on morpho-syntactic and semantic comprehension and production abilities. While social communication is perhaps one of the most important skills for peer acceptance, these skills are often overlooked in language evaluation with children. However, there are a number of caregiver questionnaires, interviews, or direct social-communication sampling methods that are available to assist clinicians or researchers in documenting social-communication skills or behaviors. Since assessment of social communication is essential in clinical work with children with an autism spectrum disorder, some of the tools described below are outgrowths of autism research or provide autism-related scores. While many children receiving social communication assessments do not have autism, the need to assess social communication skills in children with language impairment is highlighted by the growing literature documenting social and pragmatic difficulties in this population (Bishop 2000 Causes, Characteristics, Intervention, and Outcome. Hove, UK: Psychology press). Regardless of whether the measures presented herein were initially designed for children with autism or not, they will provide insights into the social communicative behaviors or tendencies in young children.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161094
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After providing a brief review of three other approaches to assessment of preschool children (DSM-IV diagnoses, "Zero to Three" diagnoses, and temperament scales), this paper focuses on the Achenbach System of Empiricall...After providing a brief review of three other approaches to assessment of preschool children (DSM-IV diagnoses, "Zero to Three" diagnoses, and temperament scales), this paper focuses on the Achenbach System of Empirically Based Assessment (ASEBA). The empirically based assessment paradigm provides user-friendly, cost-effective, reliable, and valid procedures for assessing children's behavioral/emotional problems from the perspectives of multiple informants. The ASEBA preschool forms, the Child Behavior Checklist for ages 1.5-5 (CBCL/1.5-5) and the Caregiver-Teacher Report Form (C-TRF), are usable by many different kinds of professionals in diverse settings. The CBCL/1.5-5 also includes the Language Development Survey (LDS), which provides a quick screen for delays in vocabulary and word combinations. The problem items of the CBCL/1.5-5 and the C-TRF are scored on both empirically based syndromes and DSM-oriented scales, which are normed on the same general population sample. Variations in children's functioning across contexts and interaction partners make it essential to obtain and integrate data from multiple sources. Therefore, ASEBA software provides side-by-side comparisons of item and scale scores from up to eight assessment forms per child. Clinical and research applications of ASEBA preschool forms are summarized in the paper, and strengths and limitations are discussed.
Isquith PK, Crawford JS, Espy KA
… +1 more, Gioia GA
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161093
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Assessment of the overarching self-regulatory mechanisms, or executive functions, in any age group is challenging, in part due to the complexity of this domain, in part due to their dynamic essence, and in part due to th...Assessment of the overarching self-regulatory mechanisms, or executive functions, in any age group is challenging, in part due to the complexity of this domain, in part due to their dynamic essence, and in part due to the inextricable links between these central processes and the associated domain-specific processes, such as language, motor function, and attention, over which they preside. While much progress has been made in clinical assessment approaches for measuring executive functions in adults and to some extent in adolescents and school-aged children, the toolkit for the preschool evaluator remains sparse. The past decade, however, has seen a substantial increase in attention to executive functions in very young children from a developmental neuropsychological perspective. With this has come a necessity for better, more specific, and more internally valid performance measures, many of which are now described in the experimental literature. Few such tasks, however, have adequately demonstrated psychometric properties for clinical application. We present two performance tasks designed to tap selective aspects of executive function in preschoolers that are emerging from the experimental laboratory and hold promise of appropriate reliability and validity for the clinical laboratory. Performance tests alone, however, are insufficient to develop a comprehensive picture of a child's executive functioning. Thus, we present a rating scale of preschoolers' executive function in the everyday context, and advocate a model of executive function assessment that incorporates both controlled performance tasks that target specific aspects of executive function and parent/teacher ratings that target more global aspects of self-regulation in the everyday context.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161092
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Early detection of developmental and behavioral/mental health problems is greatly facilitated when quality instruments are deployed. This article describes how to identify accurate measures and presents standards for scr...Early detection of developmental and behavioral/mental health problems is greatly facilitated when quality instruments are deployed. This article describes how to identify accurate measures and presents standards for screening tests. Included is a table delineating accurate tools for primary care: typically those relying on information from parents (e.g., PEDS, ASQ, PSC, etc.) as well as measures useful in settings where providers have more time and skill at eliciting behaviors from children (e.g., Brigance screens). Screening measures should be used in the context of developmental surveillance; the longitudinal process of incorporating professional observations into decision-making about children's developmental needs.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161090
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The literature on the importance of early identification and early intervention for children with developmental disabilities such as autism continues to grow. The increased prevalence of autistic spectrum disorders has f...The literature on the importance of early identification and early intervention for children with developmental disabilities such as autism continues to grow. The increased prevalence of autistic spectrum disorders has fostered research efforts on the development and validation of autism-specific screening instruments for use with young children. There are currently several such autism-specific screening tools meant to be used with young children in various stages of development. Data from a few of these screening instruments have been published, and they include the Checklist for Autism in Toddlers (CHAT), Pervasive Developmental Disorders Screening Test (PDDST), Screening Tool for Autism in Two year olds (STAT), Checklist for Autism in Toddlers-23 (CHAT-23), and the Modified Checklist for Autism in Toddlers (M-CHAT). In this review, these five tools designed for use with children under three years old will be highlighted. In particular, the Modified Checklist for Autism in Toddlers (M-CHAT) will be discussed.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161089
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The PLS-4 (Preschool Language Scale, 4th edition) is a psychometrically sound instrument constructed to assess language skills in children from birth to 6 years 11 months. It is a useful diagnostic and research tool that...The PLS-4 (Preschool Language Scale, 4th edition) is a psychometrically sound instrument constructed to assess language skills in children from birth to 6 years 11 months. It is a useful diagnostic and research tool that can be used to identify current comprehension and expressive language skills and can measure changes in language skills over time. The test is individually administered and includes tasks that assess skills in the areas of preverbal behaviors, as well as linguistic skills in the areas of semantics, morphology, syntax, integrative language skills, and preliteracy skills. Extensive reliability and validity evidence are presented. The test has been used extensively in research examining language development of young children from different home environments, children with identified medical conditions, and children participating in a variety of habilitation and intervention programs. Both the previous edition (PLS-3) and the new PLS-4 English and Spanish editions are used extensively in clinical, medical, and research settings to examine developmental language patterns of infants, toddlers, and preschoolers.
Ment Retard Dev Disabil Res Rev
· 2005 · PMID 16161088
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The goal of this review of the clinical and research literature is to identify, describe, and critique published methods for assessment of attention and related functions in preschool children (ages 6 and younger). The n...The goal of this review of the clinical and research literature is to identify, describe, and critique published methods for assessment of attention and related functions in preschool children (ages 6 and younger). The need for valid assessment of preschool children has grown dramatically in the past two decades following the implementation of special education services for infants, toddlers, and preschoolers under Public Law 99-457. At the same time, the number of preschool children presenting to clinicians for assessment of attention problems has grown at a steady rate. In contrast to assessment methods for school-age children, the availability of reliable and valid measures of attention for preschool children is much more limited. The majority of instruments developed to measure attention in preschoolers are described in the experimental literature, with fewer available commercially. Attention tests that can be appropriately used with children aged 3 and younger are scarce. Most preschool tests of attention report validity data involving sensitivity in contrasting clinical groups to controls, while the literature describing the utility of these measures in differentiating between clinical groups is much more limited. The review provides information on performance based tests (e.g., continuous performance, cancellation, auditory/visual span), as well as parent and teacher rating scales. The review concludes that valid assessment of attention and related skills can be effectively achieved in preschool children, with appropriate cautions.