Association of cerebral palsy with epilepsy

Fifty patients of various types of cerebral palsy were studied to find out an association between cerebral palsy, EEG abnormalities and development quotient. Seventy-six per cent patients had spastic cerebral palsy. Hypotonic cerebral palsy was the next common type (14%). Athetosis and ataxic forms were found to be rare (2% each). Epilepsy was associated with 56% patients. Clinical types of seizures observed were: Generalised tonic-clonic (43%), myoclonic (17.9%), generalised tonic (10.7%), partial simple (10.7%) and partial complex (17.9%). The incidence of seizures was highest in hypotonic type in which 85.7% had epilepsy. Mean developmental quotient of cerebral palsy patients was 34.9% with maximum retardation in hypotonic cerebral palsy (25.14%). Sixty per cent of patients had abnormal EEG, out of these hypotonic patients had maximum (70%) chances of EEG abnormality followed by spastic patients (55%). Developmental retardation was more severe statistically in the patients with abnormal EEG than normal EEG.     

Active epilepsy in mentally retarded children. I. Prevalence and additional neuro-impairments

A population-based study of active epilepsy was conducted in 6-13-year-old mentally retarded children born between 1975 and 1986. The population at risk comprised 48,873 children. Ninety-eight children were identified, 35 mildly and 63 severely retarded. The prevalence was 2.0 per 1000; 0.7 per 1000 for mildly and 1.3 per 1000 for severely retarded children. Sixty-nine children had at least one additional neuroimpairment. Cerebral palsy was found in 42 children with a majority of spastic/dystonic tetraplegias; visual impairment was present in 24 and autism in 24. Thirty-three children had only a mild or no gross motor disability and mild mental retardation, while 23 had IQs < 20 and a very severe gross motor disability. This study underlines the fact that active epilepsy in mentally retarded children is often associated with additional neuroimpairments, especially a combination of severe cerebral palsy and severe visual impairment.     

Antithrombin, protein C and protein S levels in 127 consecutive young adults with ischemic stroke

Children with spina bifida, cerebral palsy, mental retardation, developmental delays, and seizure states are handicapped with sensorimotor deficits, including gait or coordination instability, temperature insensitivity, and mental simplicity. These handicaps make this distinct and unpretentious population more susceptible to lethal burns. A 30-year review was conducted in a pediatric burn center to examine the relationships between pediatric sensorimotor deficit and burn injury. Of the 4874 acute burn admissions, 66 children were identified with preexisting sensorimotor deficits. Data indicate that children with sensorimotor deficit are more prone to burn injury from both their physical impairment and poorly supervised environments. In addition to extended hospitalizations, these children bear significantly higher mortality risks. Had the special supervisions and protection required by such handicapped children been provided, 80% of the burn injuries could have been prevented. Results emphasize that the future of these special children with sensorimotor deficits relies on health care providers playing a greater role in educating parents and caregivers.     

Classification of learning disabilities: Old problems and new approaches.

There is an increasing awareness of problems with traditional classifications and definitions of learning disabled children. This article briefly reviews the conceptual and operational limitations of classic approaches. New classification research with learning disabled populations is investigating alternative classification schemes through systematic and empirical hypothesis-testing paradigms. Such research has sparked renewed interest in learning disability subtypes, developmental factors in learning, and remediation strategies. Only through the development of more reliable and valid classification will many of the present problems in clinical and research endeavors with learning disabled children be addressed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

An impairment model of learning disability diagnosis.

This review of the literature and available evidence attempts to clarify the debate surrounding the learning disability (LD) diagnosis by offering a diagnostic model based on the principles of academic and functional impairment. The authors briefly review the strengths and weaknesses of current LD diagnostic approaches. Next, they propose a method for diagnosing LD that presumes core symptoms of below average academic achievement and associated impairment in other domains of functioning, including behavior and emotion, interpersonal relations, and self-care and fulfillment, which is modeled on the successful decades old approach to mental retardation diagnosis. The authors contend that this approach may help ameliorate the diagnostic problems plaguing the LD field. Finally, they present a rationale for future research efforts aimed at determining a level of low achievement that is associated with functional impairment. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Significance of intrapartum asphyxia for the onset of fetal brain damage

The prevalence of cerebral palsy is around 0.2% and has remained constant during the last 30 years. Retrospective case-control studies do not show a clear correlation between perinatal asphyxia and the development of cerebral palsy. Less than 10% of all cerebral palsy cases show signs of severe asphyxia during labour and delivery as the major pathological and likely cause for the brain damage. Severe cases of birth asphyxia with multiorgan defects and signs of hypoxic-ischaemic encephalopathy have a high mortality and the risk of permanent brain damage is increased by a factor of 10 to 30. Inspite of this, 90% of the survivors show normal development. The association between perinatal asphyxia and neuromotor developmental disturbances does not provide proof of a causal connection. Intrapartal abnormalities of foetal heart rate monitoring are not specific for foetal asphyxia and show only a limited correlation with the apgar and the cord blood pH. Foetal heart rate recording with pathological changes does not imply an elevated risk of later problems with neuromotor development and the widespread use of foetal heart rate monitoring during labour and delivery did not result in a significant reduction in the frequency of cerebral palsy. In addition to malformations, various forms of antinatal pathology like prematurity, intrauterine growth retardation and congenital infections are related to the development of brain damage. In each case of birth asphyxia, additional pathology like congenital infections or malformations in addition to changes in brain structure as a result of asphyxia must be ruled out using specific diagnostic methods like ultrasound, computed tomography and magnetic resonance. Furthermore, a careful documentation of the developmental phases is of fundamental importance for a final evaluation. In otherwise unremarkable deliveries at term, four conditions must be fulfilled to postulate a causal relationship between asphyxia and the development of cerebral palsy: The asphyxia must be severe. During the early neonatal period, clinical symptoms of moderate to severe hypoxic-ischaemic encephalopathy with functional impairment of other organs must be present. The neurological symptoms must be typical for intrapartal asphyxia. Documentation of diagnostic evaluation to rule out other forms of pathology must be complete (21).     

Shaken baby syndrome

Shaken baby syndrome is a serious form of physical child abuse, which is frequently overlooked. It should be suspected in all children younger than one year of age, who present with drowsiness, coma, seizures or apnoea. A combination of subdural haematomas and retinal haemorrhages with minimal or no trauma and no coagulopathy is almost pathognomonic of the syndrome. The findings are caused by shaking with or without impact. Physical signs of violence are often absent and the syndrome may easily be mistaken for serious infection or seizure disorder. Many cases are fatal or lead to severe disability including blindness, cerebral palsy, mental retardation or epilepsy in about 60% of the children. There are many unresolved problems regarding diagnosis, pathophysiology, treatment, prognosis, prophylaxis and legal actions. We discuss these problems and in addition present eleven children with shaken baby syndrome.     

The lifestyle assessment questionnaire: an instrument to measure the impact of disability on the lives of children with cerebral palsy and their families

Suitable measures of health and morbidity are less readily available for children than they are for adults. We present a measure, which is used to describe the impact of impairment and disability on the lives of children with cerebral palsy and their families. The development of this measure involved data collected from 691 children with cerebral palsy contained within the North-East England Cerebral Palsy Register and born between 1960 and 1985. Uniquely, multidimensional scaling techniques were used to derive dimensions analogous with those described in the International Classification of Impairments, Disabilities, and Handicaps. We present the analyses undertaken to test the properties of the tool, which show that it is a reliable and valid measure of the disadvantages experienced by children with cerebral palsy.     

The use of an algorithmic method for small molecule superimpositions in the design of antiviral agents

The new American Association on Mental Retardation (AAMR) definition of mental retardation (Luckasson et al., 1992) represents a radical departure from previous definitions. In the present paper we examined the extent to which the new definition provides decision rules to guide clinicians and researchers in classification efforts. We concluded that the IQ criterion of 75 will increase the proportion of the general population eligible near the cut-off score. Moreover, the 10 adaptive skill areas adopted fail to consider developmental factors and cannot be assessed reliably with current scales. Proposed differentiation by levels of needed supports is also challenged as being imprecise and not amenable to reliable measurement. Implications of this new definition for diagnostic practices and research endeavors were explored.     

The prevalence, stability and significance of developmental language delay in preschool children

A sample of 937 children in Dunedin, New Zealand, was assessed at ages three and five years in order to estimate the nature, prevalence, stability and significance of developmental language delays in three-year-olds. Assessments included language development, intelligence, and fine and gross motor-co-ordination. Detailed results are given which described three types of language delay at age three: delayed verbal comprehension only, delayed verbal expression only, and delayed development in both aspects of language. Follow-up testing at age five indicated that the specific language delays were not highly stable, whereas delays in both aspects were highly stable. A large proportion of the latter children gained very low scores on the measures at age five, and they accounted for 84 per cent of all children with very low intelligence. The implications of the results for a screening programme to identify three-year-old children at high risk of later developmental delays are considered.     

Developmental lag versus deficit models of reading disability: A longitudinal, individual growth curves analysis.

Individual growth curves were used to test whether the development of children with reading disabilities is best characterized by models of developmental lag or developmental deficit. Developmental changes in reading ability were modeled by using 9 yearly longitudinal assessments of a sample of 403 children classified into three groups representing (a) deficient reading achievement relative to IQ expectations (RD-D), (b) deficient reading achievement consistent with IQ expectations (LA), and (c) no reading deficiency (NRI). Using a model of quadratic growth to a plateau, the age and level at which reading scores plateaued were estimated for each child. Reading-disabled children differed on average from nondisabled children in the level but not in the age at which reading skills plateaued. The RD-D and LA groups did not differ in reading plateau or age at plateau. The subgroup of RD-D children scoring below the 25th percentile in reading differed from LA children only in reading plateau. Results suggest that the developmental course of reading skills in children with reading disability is best characterized by deficit as opposed to lag models. In addition, no support for the validity of classifications of reading disability based on IQ discrepancies was apparent. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Social competency in young children.

Discusses the tenacity of IQ measurement and problems of societal values and goal definition in the light of a discouraging history of attempts to cope with the complexities of human competence. 29 statements defining "social competency" in young children are presented, which involve many important conceptual problems. These problems relate to ecological and population generality of constructs, distinctions between proficiency and performance and between positive components of competency and negative characteristics that obstruct development, discontinuities in the meaning of variables at different scale points, classification of variables by developmental trends, and the importance of dynamic as opposed to static assessment. The implications of the 29 competency statements for measurement, research, and policy relating to young children are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Using the ICF and psychological models of behavior to predict mobility limitations.

Objective: To test the ability of a model that integrates the theory of planned behavior (TPB) into the International Classification of Functioning, Disability and Health (ICF), to predict walking limitations in adults awaiting hip or knee replacement surgery. Study Design and Participants: Cross-sectional structural equation modeling study of activity limitations in 190 adults. Method: A postal questionnaire measuring TPB, ICF, and walking limitations. Results: The integrated model accounted for more variance in activity limitations (57%) than did either TPB or ICF alone. Control beliefs (TPB) significantly mediated the relationship between impairment (ICF) and activity limitations. Conclusions: The integrated model provides an interdisciplinary, theoretical framework that identifies intervention targets to effect reductions in disability without the need for concomitant reductions in impairment. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Disability in geriatric depression

OBJECTIVE: The authors' purpose was to identify the relationship of disability to clinical measures that are part of a comprehensive psychiatric examination of depressed elderly patients. METHOD: The disability of 75 elderly inpatients and outpatients with major depression whose cognitive function ranged from normality to mild dementia was assessed with the Philadelphia Multilevel Assessment Instrument. Age at onset of depression, chronicity of depression, severity of depression, cognitive impairment, medical burden, social support and living environment were assessed with standardized instruments. RESULTS: Impairment in instrumental activities of daily living was significantly associated with advanced age, severity of depression, and medical burden. The relationship of depressive symptoms to impairment in instrumental activities of daily living was not influenced by age or medical burden. Anxiety and depressive ideation as well as retardation and weight loss were significantly associated with impairment in instrumental activities of daily living. Interviewer-rated global disability was associated with advanced age at onset of depression, medical burden, and overall cognitive impairment. Specifically, a disturbance in initiation and perseveration was significantly related to global disability. CONCLUSIONS: Impairment in instrumental activities of daily living appears to be a relatively independent dimension of health status that is related to depressive symptoms, particularly anxiety and depressive ideation as well as retardation and weight loss. Global disability may be associated with impairment in initiation and perseveration and with late onset of depression. These findings provide a basis for studies investigating whether psychotherapy aimed at depressive ideation and rehabilitation efforts focused on instrumental activities of daily living can improve the outcome of geriatric depression.     

Disability and cognitive impairment in the elderly

Disability and cognitive impairment show similar patterns of increasing frequency with ageing. A review of the published literature shows that there is a cross-sectional relationship between cognitive impairment and disability, independent of demographic, medical, and lifestyle factors. Some instrumental activities of daily living (IADL) items appear more specifically related to cognitive impairment, but cognition and functional impairment are distinct concepts requiring separate assessments. Subjects with low cognitive performances are at higher risk of functional impairment in the following years. Cognitive impairment as well as disability increase the risk of death and institutionalization. Preventive strategies could be directed against the risk factors of cognitive impairment and disability, and would aim to delay the onset of dementia. Prevention of disability associated with cognitive impairment needs further assessment in elderly community-dwellers. Further research is needed to better identify the specific areas cognition involved in the disablement process.     

Evidence for a central cholinergic deficit in congenital ornithine transcarbamylase deficiency

Congenital ornithine transcarbamylase (OTC) deficiency is the most common inborn error of urea cycle enzymes in humans. A large percentage of survivors of neonatal OTC deficiency suffer severe developmental disorders, including seizures, mental retardation and cerebral palsy. Neuropathological studies reveal ventricular enlargement, cerebral atrophy and delayed myelination, as well as Alzheimer type II astrocytosis. Using the sparse-fur (spf) mouse model of congenital OTC deficiency, studies of central cholinergic integrity revealed a developmental delay in choline acetyltransferase activity and of high-affinity [3H]-choline uptake in several brain structures. Subsequent studies of muscarinic cholinergic binding site distribution showed a widespread loss of M1 sites, consistent with cholinergic cell loss. These alterations are similar to those reported in Alzheimer's disease, suggesting that the severe cognitive dysfunction in congenital OTC deficiency may at least partly result from a muscarinic cholinergic lesion. Possible mechanisms involved in the pathogenesis of cholinergic cell loss in congenital OTC deficiency include ammonia-induced inhibition of pyruvate and alpha-oxoglutarate oxidation, resulting in decreased synthesis of acetyl CoA and a cerebral energy deficit, as well as NMDA receptor-mediated excitotoxicity. Treatment of spf mice with acetyl-L-carnitine (ALCAR) results in partial recovery of the developmental choline acetyltransferase deficit, suggesting a potential therapeutic benefit of ALCAR in congenital OTC deficiency. Other therapies currently used include ammonia-lowering strategies (using sodium benzoate or sodium phenylacetate) and, in severe cases, liver transplantation.     

A case study in communication disorders

Often, children with language disorders exhibit other more subtle delays or deviance in social, communicative, and cognitive development. By focusing on these three areas, the clinician can understand the developmental context in which the language delay manifests. This approach includes the following: gathering information, psychological and communicative examinations, psychiatric examination, and medical evaluation.     

Evaluation of oligonucleotide probes for simple tandem repeats (STR) to produce informative DNA fingerprints of the chicken

Early intervention programs are designed to enhance the developmental competence of participants and to prevent or minimize developmental delays. Children targeted for early intervention may either include environmentally or biologically vulnerable children, or those with established developmental deficits. There is growing consensus based on the best available evidence that early interventions can exert moderate positive effects. However, this literature is limited by substantial methodological weaknesses in most studies. Therefore further randomized clinical trials are needed to ascertain which programs best meet the needs of children with or at risk for developmental disability.     

Social disability.

Considers social disability as a form of deviance which refers to a broad class of social adaptations based on failures in the development of competence. The concept is related to R. White's biological conceptualization of effectance and competence motivation and to L. Phillips's developmental view of the origins of symptomatology. Empirical evidence is summarized in support of the position that the developmental failures of social disability in all their forms are linked to identifiable, broad, noninterpersonal dimensions of the physical as well as the social environment. Implications are discussed in terms of a reconceptualization of views of a variety of forms of deviance and related consequences for the present system of classification. Suggestions are made for a complex, multidimensional theoretical model which would accommodate the testing of etiological hypotheses linking the development of competence to the environment. It is also shown that the concept is useful as a unifying rationale for specifications of therapeutic and preventive environments and for providing a conceptual and organizing framework for training in the community mental health fields. (114 ref.) (PsycINFO Database Record (c) 2010 APA, all rights reserved)