1/28. Evaluation of facilitated communication.Two case studies are presented to demonstrate the effectiveness of Facilitated communication (FC). A wide variety of sources of data have been examined and consistent behaviour found within FC interactions. There are indications that changes in behaviour of both people resulted at least in part from the use of FC.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/28. Psychological management of intractable seizures in an adolescent with a learning disability.Psychological interventions aimed at seizure management are described with a 14-year-old boy with a learning disability and intractable epilepsy. Baseline records suggested that a majority of tonic seizures and 'drop attacks' were associated with going off to sleep and by environmental 'startles'. Psychological formulation implicated sudden changes in arousal levels as an underlying mechanism of action. Cognitive-behavioural countermeasures were employed to alter arousal levels and processes in different ways in different 'at-risk' situations. A multiple baseline design was used to control for non-specific effects of interventions on non-targeted seizures. Results suggested significant declines in the number of sleep onset and startle-response seizures were attained by these methods. Gains were maintained at 2-month follow-up.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
3/28. Psychiatric inpatients and chromosome deletions within 22q11.2.Velocardiofacial syndrome (VCFS) is a congenital disorder characterised by multiple dysmorphisms, cleft palate, cardiac anomalies, and learning disabilities due to a microdeletion of chromosome 22q11.2. Although VCFS is often associated with psychiatric symptoms, its prevalence among psychiatric patients is unknown. A total of 326 patients admitted in September and October 1997 to a Japanese psychiatric hospital were screened for the clinical features of VCFS. Twelve patients with minor facial dysmorphia were identified; chromosomal analysis with fluorescent in situ hybridisation (FISH) was performed in six patients who, further assessment suggested, were most likely to have VCFS. Chromosome 22q11.2 deletion was identified in a 41 year old woman who had symptoms of schizophrenia but no major dysmorphia, such as cardiovascular anomalies and cleft palate. Her behavioural and neuropsychological profiles were similar to those previously reported in VCFS. She was hemizygous for the FISH probe N25 (GDB locus D22S75) and also for probes N72H9 (D22S181), sc11.1a, C443 (D22S941), sc4.1 (D22S134), sc11.1b, N19B3 (D22S264), N122B5 (D22S934), and N77F7 (D22S939). The size of the deletion was about 3 Mb. Our patient had only some features of VCFS including a square nasal root, hypernasal speech, and hypoparathyroidism. She did, however, have the common larger deletion of type A. This finding suggests that psychiatric symptoms in VCFS can occur without major developmental symptoms such as cardiovascular anomalies and cleft palate. Additional patients with schizophrenia may have subtle features of VCFS which are unrecognised on routine medical examinations.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
4/28. The spectrum of acquired cognitive disturbances in children with partial epilepsy and continuous spike-waves during sleep. A 4-year follow-up case study with prolonged reversible learning arrest and dysfluency.We report a longitudinal study (7-11 years) of a previously normal boy (MR) who presented from the age of 5 years with rare partial motor seizures and atypical 'absences'. The history revealed a stagnation in development and speech difficulties a few months before onset of his epilepsy. The first waking electroencephalogram (EEG) showed rare generalized discharges during hyperventilation. magnetic resonance imaging revealed an arachnoid cyst in the frontotemporal region. Although his epilepsy never became severe, he experienced important learning difficulties. Subsequent EEGs became increasingly active with left focal epileptic activity and continuous spike-waves during sleep (CSWS) present from the first sleep EEG. The first neuropsychological evaluation (7 years) showed a speech dysfluency, word finding and naming problems, inattention and low intelligence quotient. carbamazepine was changed to clobazam and later ethosuximide was added with a rapid improvement (within 1 month) in linguistic and cognitive performances as well as in behaviour. Furthermore, the patient showed considerable progress in acquisition over the next months whereas learning to read had previously been very difficult. The epileptic activity gradually disappeared and he was able to follow regular school at an age-appropriate level. This case adds a new facet to the already recognized more obvious acquired neuropsychological disturbances known to occur in some partial childhood epilepsy syndromes with CSWS (aphasia, dementia). It manifested as prolonged insidious stagnation in learning and subtle language disability. This study documents rapid specific language improvement with change in anti-epileptic drugs and a restored immediate and long-term learning capacity, suggesting a direct but 'hidden' role of epilepsy.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
5/28. Oral midazolam for adults with learning disabilities.This paper demonstrates how oral midazolam can be employed as an alternative method of behaviour management to general anaesthesia for the dental treatment of people with learning disabilities. A range of treatments, from scaling to root canal therapy, can be carried out successfully using the sedation technique outlined. The advantages of sedation include reduced morbidity and mortality. Treatment outcomes are also likely to be improved as root canal therapy and periodontal care can be carried out over a number of visits rather than a single treatment session under general anaesthesia. Oral sedation with midazolam should improve the scope of dental treatment available to patients with disabilities.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
6/28. Neuropsychiatric aspects of sotos syndrome. A review and two case illustrations.sotos syndrome, also known as cerebral gigantism, is a rare growth disorder first described by Sotos and colleagues in 1964. Since then, a majority of the literature pertaining to sotos syndrome has focussed on the medical aspects of the syndrome, while neuropsychiatric issues have been given little attention. The present article presents a review of the current knowledge concerning neuropsychiatric aspects of sotos syndrome. Information is provided relating to physical appearance, intellectual impairment, learning disabilities, language impairments, behavioural disorders and outcome. It is emphasised that careful delineation of neuropsychiatric aspects within a diagnostic syndrome may turn out to be the first step towards molecular characterisation of behaviour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/28. A 'positive' approach to supporting a pupil with down syndrome during 'dedicated numeracy time'?This exploratory study has been designed to consider how mainstream staff can most effectively support pupils with down syndrome in numeracy. The study is partly based on the work of Wishart (1996, 2001), looking at motivation and learning styles in young children with down syndrome. An individual case study was carried out during 'dedicated numeracy time', a central part of the National Numeracy Strategy framework, in a reception class. Background information was collected from the educational records of the pupil, initial unstructured observations and semi-structured interviews with the class teacher and learning Support Assistant. Structured observations were carried out across individual, group and whole class settings. The findings, from this single case study, revealed that, contrary to the expectations of staff, the pupil demonstrated less inappropriate behaviour in the whole class setting, in comparison to individual and group settings. However, the pupil was more successful in his independent attempts at tasks in individual and group settings, in comparison with the whole class setting. The report concludes that it is important to provide pupils with appropriate support to avoid failure, particularly during the early stages of learning. It also recommends that the pupil is able to work within the mainstream classroom and participate, with the appropriate support, in the whole class 'mentral maths' session and plenary on a more regular basis. This is a small-scale study and the findings tentative. It does however indicate the need for further research in this area.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
8/28. learning and behavioural difficulties but not microcephaly in three brothers resulting from undiagnosed maternal phenylketonuria.Universal screening introduced in the 1960s has reduced the incidence of learning disability resulting from phenylketonuria (PKU), which is a treatable condition. Nonetheless, PKU may still be having an impact on the paediatric-age population. We report a woman with previously undiagnosed PKU who was born before the onset of universal screening. She is of normal intelligence, and so the diagnosis was not suspected until after the birth of her three children. Her serum phenylalanine concentration was found to be in excess of 1 mmol/L, well into the toxic range. She has had three sons, all of whom have a significant degree of learning disability resulting from intrauterine exposure to toxic levels of phenylalanine. None of the sons had microcephaly, a physical sign that, if present, might have helped to point towards the correct diagnosis. We suggest that maternal PKU should be suspected where there is sibling recurrence of cognitive impairment, particularly where the mother was born before the initiation of the neonatal screening programme for PKU.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
9/28. Conditional use of a request for assistance.PURPOSE: To establish the conditional use of requests for assistance as an alternative to escape maintained challenging behaviour. METHOD: The study involved a 12-year-old boy with a behaviour disorder. Experiment 1 involved the implementation of a reversal design comparing the learner's performance during "easy" and "difficult" addition problems. Experiment 2 involved the implementation of a quasi-experimental design to establish the conditional use of requests for assistance. RESULTS: Conditional use of requests for assistance successfully competed with escape maintained challenging behaviour. CONCLUSIONS: Our results suggest that for a newly acquired response to be displayed conditionally, two events must occur. First, the individual must learn the skill that has been associated with requests for assistance. Secondly, the contingencies must be such that reinforcement value is greater for an independent response than for a response that involves a request for assistance.- - - - - - - - - - ranking = 1.5keywords = behaviour (Clic here for more details about this article) |
10/28. Ring chromosome 20 syndrome with intractable epilepsy.Ring chromosome 20 (r[20]) syndrome is characterized by mild to moderate learning disability*, behavioural disorders, epilepsy, and various dysmorphic features. Although still considered rare, r (20) syndrome is being increasingly diagnosed. More than 30 cases have been described in the literature since 1976. Here we report an additional case of a 14-year-old male with r (20). He had moderate to severe learning disability and epileptic seizures manifesting at about 18 months of age. During the 13 years' follow-up period he showed intractable epileptic seizures, behavioural disorders, and mild dysmorphological features including microcephaly, strabismus, micrognathia, down-slanting eyelids, and ear abnormalities. Frequent episodes of atypical absence or non-convulsive status associated with electroencephalogram changes were seen in follow-up. He was treated with several classical and new antiepileptic drugs, including intravenous immunoglobulin, corticotropin, and vagal nerve stimulation, with unsuccessful control of seizures. Finally, surgical treatment (corpus callosotomy) was performed at the age of 13 years; severity of tonic seizures was diminished, but frequency was unchanged. Although his behavioural problems, e.g. hyperactivity, were mild in early childhood they became more severe when he was 11 years old. Aggressiveness, compulsiveness with self-injury, and panic attacks developed at the age of 13 years, and were more pronounced after callosotomy. This case report provides the first description of deterioration in psychological situation in patients with r(20) intractable epilepsy. The patient was diagnosed with r(20) syndrome after 13 years of clinical follow-up. karyotype analysis should, therefore, be performed in every patient with intractable epilepsy of unknown aetiology.- - - - - - - - - - ranking = 1.5keywords = behaviour (Clic here for more details about this article) |
| | Next -> |