The association method of teaching children with disorders of language development.
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Biomedical subjects
Publications and source records attributed to N Gordon.
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Learning disorders of various types are relatively common, and the reaction of the affected child can lead to social problems. The higher the children's intelligence the more frustrated they can become. Lack of self-esteem can cause unacceptable behaviour in trying to counteract this, and boost the child's confidence. There is evidence that learning disabilities are prevalent among those who are delinquent. Theories suggest this may be due directly to the learning difficulties, or to failure at school, starting a series of events leading to delinquency, or to the idea that those with learning difficulties are treated differently, especially when it comes to legal proceedings. Epilepsy can easily lead to learning disorders; particularly if the temporal lobes are involved, there are associated emotional disorders, or side effects of treatment. Also this association is often found amongst children with attention deficit disorders. If the link between learning disorders and delinquency is recognised there is considerable scope for prevention.
Many of the neurons which migrate to the cortex die at an early stage of development. In certain animals many cells die when their task is done; a phenomenon known as programmed cell death, or apoptosis. In humans a far commoner cause is the competition hypothesis: if the axon of the cell does not make contact with the dendrites of the cell in its target area it will die. Then the complexity and number of these synapses is more essential to the function of the brain than the actual number of neurons. Examples are given of the effect of sensory deprivation on the survival of neurons. The withdrawal of neuronal growth factor, and subsequent loss of the axon or cell can be a factor in the fine tuning of neural circuits, and therefore in an improvement in learning and the development of skills. However, it may also account for the diminished plasticity of the nervous system, and its ability to adapt to injury. It has been postulated that these changes contribute to the development of personality. Factors which may be involved include differences in brain development between the sexes; temperamental traits which may have a genetic or prenatal origin; and the effects of sensory perception, including language. Surely there can be no doubt that the results of experience represent the fundamental mechanisms by which the organism adapts to its environment.
Loss of consciousness in childhood may be due to cardiovascular causes, and the Long Q-T syndromes can present with seizures. The Romano-Ward syndrome is of autosomal dominant inheritance, and the Jervell and Lange-Nielson syndrome, with associated deafness, of autosomal recessive inheritance. The diagnosis is often delayed, but a careful history can avoid this. The syndromes can appear to be due to an imbalance in the sympathetic nerve to the ventricular myocardium, and precipitating causes such as stress suggest a CNS influence on this. The electrocardiogram can confirm the prolonged Q-T interval, but this is not always present, at least without an exercise test. Treatment with beta-blockers is often successful. If a wrong diagnosis of epilepsy is made a chance may be missed of avoiding sudden death, quite apart from all the medical, and social consequences that can result from such a diagnosis.
Neural tube defects may due to a number of factors acting on a susceptible embryo. The most widely accepted is a lack of folic acid at a critical time of development. Studies in which folic acid supplements have been given are reviewed. The results of these support the policy of giving folic acid, starting before conception. The metabolism of folate and vitamin B12 is discussed, including specific metabolic disorders; although there is no evidence that these play any part in causing neural tube defects. The special problems that occur among women taking anti-epileptic drugs are also considered. There are slight differences in the recommendations that have been made for supplementation, and further studies are needed before there can be complete agreement.
Reflex sympathetic dystrophy presents with pain out of proportion to the cause, loss of function, and significant evidence of an autonomic disorder. These findings are often accompanied by psychological disturbances, which can dominate the condition. There are differences in the symptoms and signs during childhood. It is more frequent among girls than boys, and the legs are more often affected than the arms; and trophic changes may be absent. There may be no history of trauma, and the response to treatment is often satisfactory. There are a number of theories on etiology. A disorder of the sympathetic nervous system with increased activity has been suggested, but on the evidence available super-sensitivity to neurotransmitters is more likely. Also there may be a spinal, as well as a peripheral, component to the sensitivity. Other suggestions include the release of a pain substance, a disturbance of natural opioid metabolism, and an exaggerated inflammatory response. The diagnosis is mainly clinical, supported by X-ray examination, bone scans with Technetium 99m labelled diphosphonates, and a characteristic scintograph pattern. Laser Doppler flowmetry can also, be useful. The most effective preventative measure is control of pain and early mobilisation. Many treatments have been tried, but the response is variable. Drugs include analgesics, non-steroid anti-inflammatory drugs, anti-depressants, and steroids. Betablockers, with gradually increasing doses may help; as may vasodilators. Calcitonin, by intramuscular injection has been given a particularly favourable report. Physiotherapy is of prime importance. Some treatments are only likely to be considered in older children. Blocking of sympathetic pathways can be tried with paravertebral or epidural injections of local anesthetics. Regional intravenous injections of alpha adrenergic blocking agents distal to a tourniquet can relieve pain, but usually only transiently; and ketanserin may act favourably as a serotonin antagonist. Surgical sympathectomy can also be considered.
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Records of 244 preschool medicals were studied retrospectively. The value of the examination to the children, their parents and teachers, and the use of clinical medical officer time was considered. Fifty-seven per cent had previously undetected problems deemed to require follow-up. This number was subsequently corrected to 50% in the light of current practice. Fourteen per cent warranted referral to other professionals and 4.5% received treatment. The high number of vision defects noted suggests further study. Fifteen per cent of the parents wished to discuss concerns about their children with the clinical medical officer. Clinical medical officer practice varied, but showed the incidence of follow-up to be inversely proportional to the referral rate. The number of previously undetected problems recorded in the survey supports medical examination of all children at this age. However, review of criteria and further study is indicated to make the best use of resources.
The difficulties in giving even relative independence to handicapped children are reviewed. A compromise has to be worked out between too much and too little independence. The personality of the child will be a governing factor, but there are many environmental influences. Not least among these are the attitudes of the parents, and to what extent they accept the handicapped child and encourage self-reliance. Education must be realistically based on the child's ability and likely potentials on leaving school. Acquiring daily-living skills may be as important as academic qualifications. More needs to be learnt about the balance between independence and over-protection, and the success of management for an individual child can only be judged when maturity is reached with adequate self-confidence, and a maximal ability to compete in the adult world.
Questionnaires were sent to 76 schools in the Macclesfield district. Seventy-four of these were returned by head teachers and/or reception teachers. Replies concerned the school entry medical examination, information on individual children, general health information and access to professionals. Additional comments on any aspects of the service were also solicited. The results have been analysed. The majority of teachers considered that the school entry medical examination is of value, but the quality of information reaching teachers is viewed more critically. Meetings were the most favoured means of communication, but there are obvious defects in the system. Some teachers did not even know of access channels. If improvements do not occur some of the value of the examination will be lost. Suggestions for better communications are made. With local management of schools, teacher opinion must be considered if a better school health service is to be provided for the children of the district.
A number of syndromes included under this rubric are considered, and their main features discussed. The congenital muscular dystrophy of the Fukuyama type as it occurs in Japan and in the western world are almost certainly the same condition. The muscle disorder is associated with cerebral lesions which may be due to an arrest of neural migration or to demyelination. Muscle, eye and brain disease, or Santavuori's syndrome, shows ocular abnormalities, as well as those of the muscle and brain, as does the Walker-Warburg syndrome. In the latter disorder the cerebral lesions tend to be more severe, and it is more rapidly fatal. The manifestations of all these syndromes undoubtedly overlap, but there has been controversy on the question of their identity. Are they separate entities, or are they different expressions of a similar genetic disorder? The genes for all these conditions will have to be isolated to see if the different phenotypes are alleles of the same gene, or not. Some of the arguments, for and against, are presented.
Juvenile myoclonic epilepsy does not seem to be recognized as often as it should be, accounting as it does for about one in 10 of those with epilepsy. In addition to the myoclonus, absence seizures and tonic-clonic fits can occur. The interictal EEG shows polyspike and wave discharges, and during the myoclonus, medium to high amplitude 16 Hz spikes. Patients may not be seen by a doctor until a major seizure occurs, and if a history of myoclonus is not obtained, inappropriate treatment may be given. The myoclonus may attributed to clumsiness. The inheritance of the condition is most probably polygenic, although it is claimed that juvenile myoclonic epilepsy may be determined by a single autosomal recessive gene. The most effective treatment is with sodium valproate, and this may have to be life-long. In the presence of major seizures carbamazapine should be used with caution as it may exacerbate minor attacks.
The possible causes of attention deficit hyperactivity disorder are considered, and the drugs that may be of benefit in treating it. Accurate diagnosis is essential for effective management. This includes the syndrome itself and associated conditions. Theories of causation are discussed. These involve the anatomical substrate but, equally importantly, the role of neurotransmitters; and the use of tests such as positron emission tomography will undoubtedly increase understanding. Genetic factors also play a part. Options for treatment are considered. Evidence supports the effectiveness of stimulant drugs, such as methylphenidate. Among the justifications for such treatment are the excessive strains placed on family relationships by the condition, and the disruption of the child's schooling. It must be carefully planned, and a constant watch kept for adverse effects. These can include difficulty in sleeping, poor appetite, loss of weight and tics. Drugs alone, however, are unlikely to help, and attention must first be given to the situation at home and at school, and how this can be improved. Treatment is often successful, and it can be a rewarding condition to treat.
OBJECTIVE: To examine the performance of individuals with vascular dementia (VaD) on the Hooper Visual Organization Test (HVOT) and to determine the influence of naming on HVOT performance in this population. BACKGROUND: The HVOT is commonly administered to assess visuospatial perception among neurologic patients, but the test requires verbal identification of stimuli as well as spatial ability. Previous studies have examined the influence of naming on the HVOT, but the issue has not been systematically addressed in individuals with subcortical VaD. METHOD: Individuals with a diagnosis of VaD were administered the HVOT, three additional measures of visuospatial function (Block Design and Picture Completion subtests of the Wechsler Adult Intelligence Scale-Revised, Rey-Osterrieth Complex Figure Test copy trial), and the Boston Naming Test (BNT). RESULTS: On average, the VaD patients performed poorly compared with normative data on each of the cognitive measures, with the most pronounced deficit evident on the BNT. Regression analyses revealed that more than 60% of the variance in performance on the HVOT was accounted for by performance on the Block Design subtest of the Wechsler Adult Intelligence Scale-Revised; performance on the BNT did not contribute significantly. CONCLUSIONS: The results suggest that VaD patients perform below expectations on the HVOT and that the measure is robust to naming deficits in this population.
OBJECTIVE: The purpose of this study is to investigate associations between executive dysfunction, neuroimaging findings, and functional impairment in patients with vascular dementia (VaD). METHODS: Twenty-nine VaD patients completed the Dementia Rating Scale and underwent MRI scanning to generate quantitative ratings of subcortical hyperintensities (SH) and cortical volume. Patients' caregivers completed items from the Lawton and Brody Activities of Daily Living Questionnaire, designed to measure instrumental activities of daily living (IADLs). The authors hypothesized that performance on the Initiation/Perseveration (IP) subscale, a measure of executive abilities, and SH would significantly predict levels of IADLs. RESULTS: A hierarchical multiple-regression analysis revealed that IP and SH accounted for 42% of the variance in IADLs; IP alone accounted for 28%, and SH accounted for 14% beyond the contribution made by IP. CONCLUSION: Findings indicate that specific cognitive and neuropathological factors are associated with functional impairment in VaD.
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