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The insanity defense is a legal construct that excuses certain mentally ill defendants from legal responsibility for criminal behavior. This practice guideline has delineated the forensic psychiatric evaluation of defendants raising the insanity defense. The document describes acceptable forensic psychiatric practices. Where possible, standards of practice and ethical guidelines have been specified. And where appropriate, the practice guideline has emphasized the importance of analyzing the individual case, the jurisdictional case law and the state (or federal) statute. This practice guideline is limited by the evolving case law, statutory language and legal literature. The authors have emphasized the statutory language of current legal standards, as well as the state or federal courts' interpretation of those standards because the same statutory language has been interpreted differently in different jurisdictions. Similarly, this practice guideline has reviewed the state and federal trends that determine which diagnoses meet the criteria for mental disease or defect. These trends yield to jurisdictional court interpretations. Finally, the authors hope this practice guideline has begun the dialogue about formulating a forensic psychiatric opinion by surveying the various approaches used to analyze case data. The forensic psychiatrist's opinion in each case requires an understanding of the current jurisdictional legal standard and its application, as well as a thorough analysis of the individual case. The psychiatrist's analysis and opinion should be clearly stated in the forensic psychiatric report. It should be noted that the role of a psychiatric expert witness in the criminal justice system is predicated on the law's interest in individualizing the criteria of mitigation and exculpation. Forensic psychiatric analyses and formulations of opinions are, therefore, subject to change as the legal guidance changes.

Classification of Mental Retardation (MR) into severe and profound is based on IQ threshold (<35 and 20% respectively) and on quite generic descriptions of deficits in adaptive behavior. The LAPMER scale (after Level of Activity in Profound/severe Mental Retardation) was developed as a measure of severity through observed behavior in adult patients. The Rasch analysis (RA, in its rating scale model) was adopted as a guide for selection of items, conceptualization of item levels, and validation of the overall instrument. The RA provides estimates on a continuum measure corresponding to the discrete cumulative score. A model prescribes the expected scores on each subject-item interaction. Discrepancies between observed and expected scores allow diagnostic procedures on coherence (fit) of both subjects and items. The final version included 8 items: Feeding, Sphincters, Communication, Manipulation, Dressing, Locomotion, Spatial Orientation and Praxiae, scored 0/1 or 0/1/2 (cumulative range for the total set of items was 0-13) the higher the score, the better the performance. The test can be administered in 15 minutes through observation or inquiry from proxies and personnel. A psychologist rated 146 permanent hosts of a large Institute for mentally retarded adults (51 profound and 95 severe, 91 male, age 18-63, median 36). Median score was 6/13, IQR 1-9, range 0-12, 19% of cases scored 0. Cronbach a for internal consistency was 0.90. Fifty-seven patients were also independently scored by another psychologist. Between-rater Cohen's k reliability index ranged from 0.77-0.96 across items. Median raw scores were 1 and 8 in profound and severe cases, respectively (p<0.001). Rasch person reliability coefficient, a 0 to 1 index of internal consistency analogous to Crohnbach a, was 0.92. For each item the standardized differences between observed and model-expected scores (residuals) were c2 tested (a level 0.05) across sub-groups of patients. These were: profound vs. severe cases, and classes of motor impairment (tetra-,hemi-,para-plegic and unimpaired), matched for overall ability measure. For 6 items some residuals were found to be statistically significant. Absolute differences ranged from 0 to 0.7 raw score points, with no systematic patterns. Gender, age group and rater did not bias the measure. Residuals did not correlate meaningfully across pairs of items (r<(0.5)), further supporting the unidimensionality of the measure. The scale seems a valid tool for classification of adult severe and profound MR cases.

A boy with an unspecific symptomatology consisting of mental retardation, strabismus, hypotonia and mild ataxia was diagnosed with a congenital disorder of glycosylation (CDG). Neither cerebellar atrophy nor dysmorphic features were present. The serum transferrin band pattern obtained by isoelectric focusing(IEF) showed a strongly elevated disialotransferrin band together with only slightly elevated asialotransferrin, thus a type I pattern. This is a new CDG classified CDG-x since CDG-la, -b, -c, -d and -e were excluded. Quantitative differences to the type 1 pattern of a CDG-la patient with a moderate to severe course were confirmed by densitometric evaluation of the gels and by SDS gel electrophoresis. Liver biopsy showed lysosomal inclusions suggesting a pre-Golgi defect. This patient's case supports the approach to include isoelectric focusing of serum transferrin in the diagnostic work-up of patients with unexplained symptoms.

Purpose: The purpose of this investigation was to describe and assess the disparities, if any, in parental perceived cost barriers to oral health care among developmentally disabled children using a national data set., Methods: Data from the 1997 National Health Interview Survey (NHIS) were analyzed using a SUDAAN statistical package., Results: After adjusting for age and sex, parental perception of unmet need was significantly associated with developmentally disabled children 2-17 years in lower socioeconomic groups., Conclusions: Though most children from lower socioeconomic groups are eligible for Medicaid coverage, parents of these children perceive cost barriers to dental care. Children with developmental disabilities face even more perceived barriers to care based on family income.

The Wechsler Intelligence Scale for Children--Third Edition and the Cognitive Assessment System were compared for a sample of 78 White and Black students in special education programs for children with mental retardation. Results showed that the WISC-III identified more children as having mental retardation than did the Cognitive Assessment System. More important, however, the WISC-III classified disproportionately more Blacks than Whites as having mental retardation as compared to the Cognitive Assessment System. Results imply that the problem of disproportionate representation of Black children in special education classes for children with mental retardation may be addressed if the Cognitive Assessment System were used instead of the WISC-III.

Objectives: To review patterns of physical and sexual abuse in cohorts of sexual offenders and nonsexual offenders with intellectual disability., Method: Forty-six sexual offenders were compared with 48 male nonsexual offenders in relation to their experiences of sexual and physical abuse in childhood. Comprehensive assessments were taken over a period of at least one year, and were conducted independently by a range of professionals., Results: Thirty-eight percent of the sexual offenders and 12.7% of the nonsexual offenders had experienced sexual abuse, while 13% of the sexual offenders and 33% of the nonsexual offenders had experienced physical abuse., Conclusions: Sexual abuse seems a significant variable in the history of sexual offenders, while physical abuse seems a significant variable in the history of nonsexual offenders. The results support the view that the "cycle of abuse" is neither inevitable nor an adequate explanation of future offending.

This article provides the dentist with a background on developmental disabilities, services, and issues related to statutory and regulatory requirements, with a focus on mental retardation and Down syndrome. Down syndrome has mental retardation as a component, yet requires additional emphasis because of its prevalence and associated craniofacial manifestations. Also discussed are treatment considerations.

People with profound learning and multiple disabilities are at risk of pain because they experience complex and severe health conditions and frequently undergo surgical procedures that cause pain. Pain assessment in this client group requires a skilled approach, although there is little evidence available to guide nursing practice. In the absence of pain assessment measures specifically designed for use with people with profound learning disabilities, the nurse will need to rely on careful observation and interpretation of the client's communicative behaviour, clinical judgement and knowledge of the person during assessment. Intuition is also an important attribute and can be a valuable source of knowledge when assessing clients who are unable to verbalize their experience of pain. These issues, along with implications for nursing practice, are explored.

Eighteen preschool children diagnosed according to the Diagnostic and Statistical Manual of Mental Disorders Third Edition Revised (DSM III-R) as having Pervasive Developmental Disorder-Not Otherwise Specified (PDD-NOS) were compared to 176 children with DSM III-R Autistic Disorder (AD), and to 311 non-autistic children with developmental language disorders (DLD) (N = 201) or low IQ (N = 110). All children were partitioned into "high" and "low" cognitive subgroups at a nonverbal IQ of 80. Within cognitive subgroups, the 18 PDD-NOS children did not differ significantly from either the DLD or the AD children in verbal and adaptive skills and obtained scores intermediate between those of these groups. The PDD-NOS did not differ from the AD children in maladaptive behaviors. Both the PDD-NOS and AD children had many more of these behaviors than the non-autistic comparison groups. Children in the "high" and "low" cognitive subgroups of AD, but not of PDD-NOS, differed substantially on most measures, with the children with lower cognitive scores significantly more impaired on all measures. Similarity of PDD-NOS children to AD children in maladaptive behaviors and an intermediate position between autistic and non-autistic groups on virtually all measures explains the difficulty clinicians encounter in classifying children with PDD and raises questions about the specificity of these diagnostic subtypes of the autistic spectrum.

Recent discussions about changing the term mental retardation to a different term may be considered in the broader framework of three distinct but related processes: naming (terminology), defining, and classifying. The three processes are analyzed according to their purposes and functions: In naming, a term is assigned; in defining, the term is explained; and in classifying, the group is divided into subgroups according to stated principles. The current status of each process is described, especially as represented in the 1992 AAMR manual, Mental Retardation: Definition, Classification, and Systems of Supports. We suggest three sets of guiding questions that may help evaluate proposed changes in naming, defining, or classifying.

One of the most important tasks of a physician who has patients with delayed development is to assess the cause of the problem. To help with this work, an aetiological classification based on timing and type of the injury to the central nervous system (CNS), has been created. The main divisions are: genetic causes; CNS malformations and multiple malformation syndromes of unknown origin; external prenatal factors; paranatally acquired disorders (-1 to +4 weeks from delivery); postnatally acquired disorders; and untraceable or unclassified causes. In the classification, the earliest factor injuring the CNS is the primary diagnosis. The first stage, which consists of a quite simple workup, reveals the timing of the injury and allows the possibility for family counselling. The overview of the second stage assessment is also given. The 'aetiological tree' illustrates the classification method and serves as a reference and teaching aid. It can also be used for genetic counselling to demonstrate the situation. This method has been used for almost 20 years and has been proven to enhance diagnostic activity and family counselling.

Aim of this review is to present the latest advances in the identification of the genetic determinants of intellectual deficiency. Mental retardation (MR) is often associated with other neurologic symptoms, metabolic disorders, or malformation syndromes. The purpose of the review is to subdivide the large field of MR into categories that may help professionals in making a diagnosis. Nonspecific MR can also segregate in families and the mapping and cloning of corresponding mutant genes will eventually advance our understanding of normal and abnormal brain functioning. Several genes responsible for nonspecific X-linked mental retardation have been identified in the last 12 to 24 months and are being intensively investigated. This will hopefully lead to new possibilities of either genetic or pharmacological therapy.

Although it is assumed that genes that influence cognitive function are ubiquitous in the human genome, to date, more such genes have been found on the X chromosome than on any other comparable segment of the autosomes. This is in large measure because of the power of hemizygosity in exposing mutations of X-linked genes in males. Clinical manifestations, mapping of gene loci by linkage analysis or chromosome rearrangements, and gene identification by positional cloning or mutational analysis of candidate genes have permitted extensive lumping and splitting within the large and heterogeneous category of X-linked mental retardation (XLMR). Approximately 130 XLMR syndromes have been identified, 25 gene loci have been mapped and cloned, and 55 other loci have been mapped but not cloned. Well-recognized syndromes (e.g., Fragile X and Coffin-Lowry syndromes) and syndromes represented by only a single family (e.g., Arena and monoamine oxidase-A syndromes) are among these more or less well-defined entities. In addition, more than 75 families with nonsyndromal XLMR have been regionally mapped and 7 causative genes have been identified.

Whether the Psychopathology Inventory for Mentally Retarded Adults (PIMRA) could detect specific psychopathological disorders was investigated in 652 subjects with different levels of mental retardation living in the community or in residential facilities. An exploratory factor analysis was carried out to check the scale organization of PIMRA. The Anxiety, Adjustment Disorder, Somatoform Disorder, and Soundness Scales were confirmed by 4 corresponding factors; the Psychosexual Disorder Scale was replaced by a factor specific to gender identity problems, and the Schizophrenia Scale by two factors concerning isolation and bizarre behaviors, respectively. The items of the Depression Scale were distributed over the three factors concerning anxiety, adjustment, and psychosomatic disorders, while the items of the Personality Disorder Scale were scattered over almost all the factors. Moreover, 55 subjects with anxiety disorders and 49 with depression were compared to 50 control subjects of the same age, intelligence level, and gender ratio but without dual diagnosis and obtained significantly higher factorial scores both on the overall scale and on the factors specifically related to their disorders. PIMRA has been found to show good construct validity. These results could be considered particularly valid as they were obtained from a large sample comprising different levels of mental retardation and thus showing all possible psychopathological behaviors.

A central part of Pediatric Neurology is currently dominated by the search for genetic factors involved in developmental disorders of the nervous system, including cases where the cytogenetic examination remains uncontributive. The prerequisite for a good definition of the malformative phenotypes leads to distinguish: 1 cerebral malformations that can be identified at the macroscopic scale, by imaging. 2 polymalformative syndromes including mental retardation where cerebral imaging is not contributive, thus the syndromatic definition is based on associated somatic anomalies. 3 Non-syndromatic mental retardation, where a genetic origin is clear only in the familial forms. Various methodological approaches have included genetic linkage studies, search for inframicroscopic chromosomal rearrangements in the critical region and investigation of candidate genes. A great number of syndromes have been connected with a great diversity of genetic mechanisms, whose many examples are presented: genopathies with regular or variable expression, unstable mutations, contiguous gene syndromes or other complex infracytogenetic rearrangements, chromosomal or genic mosaicisms, mutations submitted to parental imprinting. New methods of genomic screening will be necessary to progress in this field, given the great number of genes involved in cerebral development. As for the early developmental disorders of the PNS and muscle, their diagnosis becomes frequent during the intrauterine life, raising the problem of a better definition of the fetopathological phenotypes.

The objective of this investigation was to generate a medical and dental profile of patients attending the Mount Sinai Hospital Dental Program for Persons with Disabilities, to determine if certain selected criteria could identify patients likely to require dental care in that setting. The need for dental care under general anesthesia was used as the prime indicator that care should be provided in a hospital setting. A retrospective review of all the charts of the patients enrolled in this hospital program was undertaken. Results indicated that patients who were treated in this hospital-based dental program had the following characteristics: moderate to profound mental retardation (39.5%), moderate to severe behavioral problems (31.1%), and/or a history of seizure activity (29.1%). Behavioral criteria appear to be the predominant reason for the provision of hospital-based dental care for persons with disabilities.

Differences between patients being treated in two different medium secure units, one for those of normal intelligence and one for those with learning disability, were investigated. Sociodemographic, psychiatric, medical and medicolegal data were recorded for all inpatients in both secure units. Patients in the medium secure unit for those of normal intelligence were older at the time of the study and at conviction for the index offence, more likely to have been admitted from the penal system, and more likely to suffer from a psychotic disorder, particularly schizophrenia or a mood disorder. Their index offence was more likely to be homicide, attempted murder, manslaughter or grievous bodily harm, while that of the patients with learning disability was more likely to be a sexual offence. A need for separate medium secure unit facilities for those of normal intelligence and those with learning disability is supported by differing psychiatric and behavioural requirements.

Several states and the United States have laws that exempt persons who have mental retardation from the death penalty and other severe sentences. Two recent murder cases in Indiana, which has such a law, illustrate some of the problems in applying it. The characteristics of the two defendants were quite similar, but one defendant was found to have mental retardation and was exempted from the death penalty and the other was not. The disparity was attributed to differences in the assessment of adaptive behavior and to general stereotypes of people who have mental retardation. Equal application of sentencing limitation laws requires greater involvement of professionals with specialized training and experience in mental retardation.

Functional assessment is a method to identify the relationships between a behavior of interest and an individual's environment. Traditional methods for functional assessment have relied on experimental techniques in which analog sessions are designed to replicate conditions in the individual's environment. However, these techniques can be time-consuming, require advanced training, and rely on the availability of extensive resources in the individual's setting. Development of a brief functional assessment checklist would circumvent these difficulties and meet clinical needs for efficient assessment methods. The current study provides psychometric data for the Questions About Behavioral Function. These data include test-retest, inter-rater, and internal consistency.

The clinical genetic diagnosis was reviewed in 429 subjects with intellectual disability in the Australian Child and Adolescent Development (ACAD) study of behavioural problems. With minor differences, the overall "general distribution by causation" was similar to that to that found by the Consensus Conference of the American College of Medical Genetics in 1995. There was a significant male excess in the whole series which was shown to reside in those with "autism," those with undiagnosed nonsyndromic mental retardation (NSMR) and those with X-linked monogenic disorders. It is argued that a substantial proportion of undiagnosed NSMR is caused by genes on the X chromosome. Some of the practical problems of assigning individuals to diagnostic groups are discussed., (Copyright 2000 Wiley-Liss, Inc.)

Purpose: To determine if using more stringent criteria for cryptogenic Lennox-Gastaut syndrome (LGS) would result in an improved prognosis for that group. Cryptogenic, symptomatic, and non-cryptogenic LGS patients without etiology (indeterminate) were compared with respect to seizure and cognitive outcome., Methods: Retrospective chart review was performed on 245 patients seen at the Mayo Clinic Rochester from 1976 to 1997, with a diagnosis of either LGS or slow spike wave on EEG. LGS was confirmed in 107 (64 male, 43 female) patients. This group was divided into cryptogenic, symptomatic, and indeterminate groups containing 23, 47, and 37 patients, respectively. In this study, cryptogenic patients all had normal development before onset of LGS, absence of dysmorphic features, normal neurologic examination, and normal magnetic resonance (MRI) brain imaging. Of the 107 patients, 74 had >/=3 years of follow-up., Results: LGS onset in the 107 patients occurred at a median age of 4.0 years (range, 0.6-28.9 years). When last seen, 63% of those with symptomatic LGS had more than three seizures a day compared with 50% of cryptogenic and 34% of indeterminate patients. The most common seizure types were tonic (77%), atypical absence (61%), and generalized tonic-clonic (56%). Only three patients, all part of the indeterminate group, were seizure free at last follow-up., Conclusions: Using stringent criteria in defining the cryptogenic subgroup resulted in no significant difference in seizure outcome. Individuals with a normal cognitive outcome did not segregate into one etiologic subgroup, but did have LGS onset at an older age.

In order to clarify the characteristics of epilepsy in patients with severe mentally retarded (SMR) subjects, we analyzed 52 SMR subjects with epilepsy from the institute for SMR subjects at Kikuchi National Hospital, Kumamoto, Japan. A total of 61.5% patients had uncontrolled seizures which were resistant to treatment. The most common combinations of seizure types in those not responding to conventional anticonvulsants were generalized tonic-clonic seizures (GTCS) with tonic seizure and GTCS with atypical absence. Their clinical features were characterized by spastic paralysis associated with a slower background electroencephalogram and abnormal computed tomography scans of the head, suggesting the involvement of cortical damage. These findings suggest that a large proportion of epilepsy in SMR subjects does not respond to treatment and that the severity of organic brain damage may therefore affect the natural course of epilepsy in such patients.

The utility of the Dementia Rating Scale (DRS) when administered to individuals with mental retardation (MR) was examined. Our sample was comprised of individuals residing in an intermediate care facility in the southeastern United States, included individuals diagnosed with mild, moderate, or severe MR, and consisted of both Caucasians (50%) and individuals of African-American descent (50%). Descriptive statistics for the DRS Total Score and five subtests (e. g., Attention, Initiation/Perseveration, Construction, Conceptualization, and Memory) obtained from our sample of individuals with mild MR compared favorably with previously published values. The group with mild MR performed significantly better than the group with moderate MR on the Total Score and all subtests except Construction, and the group with moderate MR performed significantly better than individuals with severe MR on all measures. These results show that the DRS can provide information about the cognitive strengths and weaknesses of individuals with mental retardation, and they show that the DRS can be administered to a wide range of individuals diagnosed with MR.

Unlabelled: During the last decades, a new policy involving normalisation, integration, equality and deinstitutionalisation of mentally retarded (MR) individuals has been accepted in Sweden. Consequently, many institutions have been closed and an increased number of people with MR are now integrated into the Swedish community. The general aim of this thesis was to investigate if a greater degree of independence for MR adults influenced the prevalence of oral diseases., Material and Methods: The caries incidence and prevalence, number of remaining teeth, incidence of tooth mortality and interproximal bone loss were investigated in a group of MR adults, aged 21-40 years, all with protracted, regular preventive dental care. The studied variables were related to living arrangements and degree of MR, and were followed during 8.5 years (Papers I, II and VI). Incidence and reasons for tooth mortality were studied during 10 years in institutionalised MR individuals (Paper III). In connection with the closing-down of an institution for MR adults, the changes of oral hygiene habits, sucrose intake and oral microbiological flora were studied in the new integrated living (Papers IV and V)., Results: Less restrictive living arrangements and mild forms of MR were correlated to a high caries prevalence in persons with MR. After 8.5 years, the caries incidence had decreased in all subjects, but persons with mild MR and those who lived integrated still had a higher caries prevalence than other MR adults. With the exception of subjects with Down syndrome, the interproximal bone loss during 8.5 years was similar or lower compared to normal Swedish populations. Institutionalised MR subjects had lost an average of 3.72 teeth during 10 years. Periodontitis was the main reason for tooth mortality. Persons who cooperated well with dental treatment and those who used neuroleptics lost fewer teeth during the 10-year period compared to other MR individuals. The proportion of subjects with high levels of mutans streptococci and subjects who showed growth of P. intermedia/P. nigrescens decreased after deinstitutionalisation, and less subjects consumed sucrose frequently. The oral hygiene routines, and the levels of lactobacilli, P. gingivalis and A. actinomycetemcomitans were not influenced by the change in living arrangements. A simplified saliva sampling method, involving licking directly on the bacteria culture, was developed to count lactobacilli in saliva when delivering whole saliva was impossible., Conclusions: Less restrictive living and a mild degree of MR were correlated to a high caries prevalence. Subjects with poor ability to cooperate with dental treatment and those with Down syndrome have an obvious risk of deteriorated oral health. However, compared to normal Swedish populations, the MR subjects studied in this thesis had, after several years of regular dental care, satisfactory oral health.

X-linked mental retardation has recently become one of the most interesting genetic anomalies. Studying this group of conditions has led to many insights into the mechanisms involved in normal and abnormal gene actions in humans. Since the early 1980s, the number of disease entities for which the responsible genes could be localized on the X chromosome has increased from year to year; at the Ninth International Workshop on Fragile-X-Syndrome and X-linked Mental Retardation, 199 such disease units were counted (Hamel, 1999). Conventionally, these units were subdivided into two groups: syndromal and non-syndromal types. The syndro- mal types are characterized by external features, neurological signs, and/or metabolic anomalies. The non-syndromal types do not show such specific features; here, the X-linked mode of inheritance is the only indicator. Due to the reduced reproduction of mentally severely retarded males, a relatively high fraction of new mutants among cases of a specific type must be expected. It cannot be the purpose of the present short article to review sufficiently well the entire field; this would require a complete book. Rather, it is our intention to point to some open problems and possible ways for their solution., (Copyright 2001 S. Karger AG, Basel)

Recent health legislation in the Netherlands makes specific provisions for patients who are not (fully) capable of acting reasonably in their own interests. If this legislation is actually to improve the legal position of incompetent people, it must be clear to whom these provisions apply. The description in modern legislation is that the person 'is regarded as not being able to make a reasonable assessment of his interests in the issue in question'. The law does not provide criteria according to which competence can be assessed in practice. In the present study, data on the competence assessment of 133 clients were collected by means of a poll held among those people who decide on competence in practice and a total of 485 assessors were polled. Client files were also examined with the intention of gaining insight into the carers' motivation to involve or exclude clients from the decision-making process. Finally, interviews were conducted with clients who have a mild or moderate intellectual disability. Large differences appeared to exist between the various assessors in the assessment of the general competence of clients. A general assessment of a client's competence is not sufficient in daily practice; therefore, partial competence was also assessed. Opinions about the partial competence of clients varied considerably among assessors. A ranking was compiled of discriminating characteristics in the qualification of competence or incompetence.

Mental retardation (MR) is a heterogeneous condition defined by significantly subaverage intellectual and adaptive functioning and onset before age 18 years. With an approach underscored by principles of normalization and the availability of appropriate education and habilitation, persons with MR generally live, are educated, and work in the community. Mental disorders occur more commonly in persons with MR than in the general population. However, the disorders themselves are essentially the same. Clinical presentations can be modified by poor language skills and by life circumstances, so a diagnosis might hinge more heavily on observable behavioral symptoms. The diagnostic assessment considers and synthesizes the biological, psychological, and psychosocial context of mental disorders. Comprehensive treatment integrating various approaches, including family counseling, pharmacological, educational, habilitative, and milieu interventions is the rule.

Characteristics influencing employment of individuals with mental retardation were analyzed to evaluate employment rates for county providers. Participants in the sample (N = 11,743) earned at least $50 per week for a 6-month period. Good physical and emotional health, being male, age between 18 and 49, IQ between 50 and 75, and good adaptive skills were associated with employment. Eighteen percent of the variability in employment rates was explained by the ratio of individuals served by the Boards to the county population and the percentage of the county population living in rural settings. Median income, unemployment rates, and poverty explained 22% of the variance in noncompetitive employment rates. Results suggest that health promotion programs could help individuals with mental retardation obtain and sustain employment.

The present authors made an attempt to ease the diagnostic work of physicians who have patients with intellectual disability by creating an aetiological classification system based on the time and mechanism of injury to the central nervous system (CNS). The current paper presents the work-up needed for understanding at least the timing of the causative factor/factors. The timing principle opens a direct course to family counselling. This method has been very well accepted during its 18 years of use in Finland, and therefore, it was felt that it would be helpful to organize the relevant ICD-10 diagnoses according to the timing principle. This method has been published as a manual. An image of a tree became the obvious metaphor for this system. The genetic category forms the main root and stem, from which multiple branched roots and limbs emerge. The individual diagnoses appear as root nodules and leaves. The system is flexible, making it possible to add new branches for groups of diagnoses when improved diagnostic methods create these options (e.g. microdeletions). The aetiological diagnoses change accordingly. Over the course of further development, new incidents damaging the CNS may affect the functional level of an individual and require additional diagnoses. It is a constant challenge for physicians to keep the diagnoses of their patients up to date. The image of the tree helps professionals to think in terms of timing, and thus, makes family counselling easier. It is also helpful in the education of medical professionals.

Relations among instruments used in community mental health services for people with developmental disabilities were explored with 284 individuals. Correlation coefficients among the instrument subscales were interpreted in terms of statistical significance and effect size. Of the 157 coefficients, 44% were significant, p < .001, and 35% represented large effects, r > .50. Reiss Screen subscale scores correlated with Irritability, Lethargy, and Hyperactivity on the Aberrant Behavior Checklist (ABC) and with Social Behavior and Disturbing Interpersonal Behavior on the ABS Part II. Stepwise regression analyses predicting Reiss Screen scores from the ABS and ABC resulted in a significant regression, with an overall adjusted R2 of .67. Variance was largely accounted for by two ABS domains and two ABC subscales.

Objective: To report the phenotypic spectrum and management issues of children with Kabuki syndrome (Niikawa-Kuroki syndrome) from North America., Design: A case series of children (n = 18) with clinical findings of Kabuki syndrome., Setting: Medical genetics clinics in Washington, Alaska, and Arizona., Results: Most patients had postnatal growth retardation, and all had developmental delay and hypotonia. Feeding difficulties, with or without cleft palate, were common; 5 patients required gastrostomy tube placement. Developmental quotients/IQs in all but 2 were 60 or less. Seizures were seen in less than half of the patients, but ophthalmologic and otologic problems were common, particularly recurrent otitis media. Congenital heart defects were present in 7 (39%); 3 patients underwent repair of coarctation of the aorta. Other features included urinary tract anomalies, malabsorption, joint hypermobility and dislocation, congenital hypothyroidism, idiopathic thrombocytopenic purpura, and in one patient, autoimmune hemolytic anemia and hypogammaglobulinemia. All patients had negative family histories for Kabuki syndrome., Conclusions: Kabuki syndrome is a mental retardation-malformation syndrome affecting multiple organ systems, with a broad spectrum of neuromuscular dysfunction and mental ability. Given that 18 ethnically diverse patients were identified from 2 genetics programs, it appears that this syndrome is more common in North American non-Japanese patients than previously appreciated.

Memory, the ability to store and retrieve information, is essential for learning in children. Modern neurobiology research is revealing some of the fundamental steps that encode memories within networks of neuronal synaptic connections in the brain. Somewhat different networks store verbal declarative memories and habit or procedural memories. Several biochemical steps convert short-term memories into permanent memories. These changes include activation of neurotransmitter and growth factor receptors, intracellular protein kinases, and nuclear transcription factors that stimulate gene expression of memory proteins. The proteins strengthen synaptic connections and stabilize long-term memories. Genetic defects in those pathways appear to be responsible for several human retardation and learning disability syndromes, including Coffin-Lowry syndrome and neurofibromatosis.

The use of the Kaufman-Assessment Battery for Children (K-ABC) was evaluated with a sample of 50 mentally disabled adults in a wide age range, as there exist no special tests for this group of patients. Intercorrelation--and factor analyses, and correlations with the German adaptation of the Wechsler Adult Intelligence Scale (HAWIE-R) proved the use of the K-ABC to be valid. As an assessment battery for children designed for the wide age range from 2-6 to 12-5 years is more adequate to the capability of intellectually impaired adults, the K-ABC delivers results on individual strengths and weaknesses, based on a neuropsychological theory. Thus the individual cognitive abilities can be assessed with more differentiation, and that gives an important base for intervention, partial remediation, and placement.

Forty-two children (ages 6 to 12 years old) with moderate mental retardation to borderline intellectual functioning were studied in a laboratory playroom setting to determine whether children identified as ADHD (attention deficit hyperactivity disorder) or controls differed on activity and attentional measures. Children with ADHD were further divided into ADHD + conduct problems (ADHD + CD) and ADHD-only subgroups (with an ADHD-combined group comprising children of both subgroups). An interval recording system was used to code observations of independent play and a restricted academic task. Results indicated that the ADHD-combined group was significantly more vocal and engaged in a significantly greater number of toy changes than controls during independent play. Significant group differences were also noted during the restricted academic task, with the ADHD-combined and ADHD + CD groups more off-task and engaging in a greater number of toy touches than controls. Discriminant analyses found independent play measures to predict group membership in 70 percent of cases (ADHD-combined vs. controls), but in only 64 percent of cases using measures from the restricted academic task. No significant findings resulted when the ADHD subjects were further divided into two subgroups. Despite some inconsistent findings, such laboratory-based observations may be of value in the diagnosis of ADHD in children with moderate mental retardation to borderline intellectual functioning.

Stereotypies are high-frequency, highly repetitive, nonfunctional behaviors that are also often characterized as rhythmic. Rhythmicity suggests that the behavior is periodic, occurring at fixed intervals. Few studies, however, have rigorously demonstrated periodicity in stereotypy. This study examined various topographies of stereotypy in 9 participants and used spectral methods to detect existence of periodicties. Two general patterns emerged in the spectral analysis. Participants who engaged in stereotypic rocking showed peaks in their power spectra; participants who engaged in other topographies of stereotypy did not show peaks. Thus, it appears that although some stereotypies--notably, rocking--have a periodic component, rhythmicity does not appear to be a characteristic of stereotypy in general.

In accordance with 20 CFR 402.35(b)(1), the Commissioner of Social Security gives notice of Social Security Ruling, SSR 98-1p. This Ruling results from the "top-to-bottom" review of the implementation of changes to the Supplemental Security Income childhood disability program necessitated by the Personal Responsibility and Work Opportunity Reconciliation Act of 1996 (Pub. L. 104-193). It provides a policy interpretation that children who have a "marked" limitation in cognitive functioning and a "marked" limitation in speech have an impairment or combination of impairments that medically equals Listing 2.09. It also provides guidance for determining when a child has a "marked" or an "extreme" limitation in each of these areas.