Worster-Drought syndrome, a mild tetraplegic perisylvian cerebral palsy. Review of 47 cases.
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A retrospective case-note analysis was undertaken of 47 children with a congenital upper motor neurone bulbar palsy (excluding pure speech dyspraxia) to clarify the phenotype of Worster-Drought syndrome (WDS) and to record its associated features and complications. The results revealed that the study children had significant bulbar problems (with 80% still needing a modified diet and a similar number using augmentative communication methods at last review). There were also high rates of predictable bulbar complications (86% had dribbling, 60% had glue ear, gastro-oesophageal reflux in 40%, history of poor nutrition in 40% and aspiration in 40%). Most of the children had additional complex impairments (91% had mild pyramidal tetraplegia, 81% learning difficulties, 60% congenital defects, 41% neuropsychiatric problems and 28% epilepsy). Over half of the children had significant medical problems in the first year, but mean age at diagnosis was 6 years. There were no obvious causes in pregnancy or birth. Six children had a family history of WDS and 32% (12/37) had abnormal neuroimaging including five with bilateral perisylvian polymicrogyria. In our experience, WDS is not uncommon, is relatively easily diagnosed and is crucial not to miss as the management of these children's multiple impairments is complex and requires a careful team approach. WDS falls clearly within the cerebral palsies as a syndrome that includes motor impairment arising from static damage to the brain in early life. The common presence of cognitive, behavioural and seizure impairments strongly supports the cerebral cortical (presumably perisylvian) localization. Its core elements are a suprabulbar paresis, a mild spastic tetraplegia and a significant excess of cognitive and behavioural impairments and epilepsy. The complete overlap in phenotype between WDS and the bilateral perisylvian syndrome leads us to propose that they are the same condition. WDS is startlingly absent from epidemiological studies of the cerebral palsies and rarely diagnosed, presumably because of lack of clinical awareness of the condition and lack of major gross motor impairments. (+info)
Randomised controlled trial of community based speech and language therapy in preschool children.
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OBJECTIVE: To compare routine speech and language therapy in preschool children with delayed speech and language against 12 months of "watchful waiting." DESIGN: Pragmatic randomised controlled trial. SETTING: 16 community clinics in Bristol. PARTICIPANTS: 159 preschool children with appreciable speech or language difficulties who fulfilled criteria for admission to speech and language therapy. MAIN OUTCOME MEASURES: Four quantitative measures of speech and language, assessed at 6 and 12 months; a binary variable indicating improvement, by 12 months, on the trial entry criterion. RESULTS: Improvement in auditory comprehension was significant in favour of therapy (adjusted difference in means 4.1, 95% confidence interval 0.5 to 7.6; P=0.025). No significant differences were observed for expressive language (1.4, -2.1 to 4.8; P=0.44); phonology error rate (-4.4, -12.0 to 3.3; P=0.26); language development (0.1, -0.4 to 0.6; P=0.73); or improvement on entry criterion (odds ratio 1.3, 0.67 to 2.4; P=0.46). At the end of the trial, 70% of all children still had substantial speech and language deficits. CONCLUSIONS: This study provides little evidence for the effectiveness of speech and language therapy compared with watchful waiting over 12 months. Providers of speech and language therapy should reconsider the appropriateness, timing, nature, and intensity of such therapy in preschool children. Continued research into more specific provision to subgroups of children is also needed to identify better treatment methods. The lack of resolution of difficulties for most of the children suggests that further research is needed to identify effective ways of helping this population of children. (+info)
Down syndrome and the phonological loop: the evidence for, and importance of, a specific verbal short-term memory deficit.
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Individuals with Down syndrome are thought to perform poorly on tests of verbal short-term memory, such as measures of word span or digit span. This review critically examines the evidence for a specific deficit in verbal short-term memory in Down syndrome, and outlines a range of possible explanations for such a deficit. The potential implications of a verbal short-term memory impairment for broader aspects of development are outlined, in particular with respect to vocabulary development. Possible intervention strategies, which might improve verbal short-term memory performance in Down syndrome are also considered. However, we argue that further research is needed to fully clarify the nature of a verbal short-term memory deficit in Down syndrome, before the merits of these various intervention approaches can be properly evaluated. (+info)
Brief functional analysis and treatment of bizarre vocalizations in an adult with schizophrenia.
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Variables responsible for the maintenance of bizarre vocalizations emitted by an adult diagnosed with schizophrenia were examined via a brief functional analysis, and results suggested that the behavior was maintained by attention. A treatment consisting of extinction and differential reinforcement of appropriate vocalizations was effective in reducing bizarre vocalizations and increasing appropriate vocalizations. The use of functional analysis methodology to examine variables that maintain problem behavior in this population is discussed. (+info)
Paroxysmal symptoms in multiple sclerosis.
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The clinical features of paroxysmal symptoms occurring in 26 patients with multiple sclerosis are reviewed. The findings are considered to provide further support for the concept of lateral spread of axonal excitation within demyelinated plaques. (+info)
Meningitis in infancy in England and Wales: follow up at age 5 years.
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OBJECTIVE: To describe important sequelae occurring among a cohort of children aged 5 years who had had meningitis during the first year of life and who had been identified by a prospective national study of meningitis in infancy in England and Wales between 1985 and 1987. DESIGN: Follow up questionnaires asking about the children's health and development were sent to general practitioners and parents of the children and to parents of matched controls. The organism that caused the infection and age at infection were also recorded. SETTING: England and Wales. PARTICIPANTS: General practitioners and parents of children who had had meningitis before the age of 1 year and of matched controls. MAIN OUTCOME MEASURES: The prevalence of health and developmental problems and overall disability among children who had had meningitis compared with controls. RESULTS: Altogether, 1584 of 1717 (92.2%) children who had had meningitis and 1391 of 1485 (93.6%) controls were successfully followed up. Among children who survived to age 5 years 247 of 1584 (15.6%) had a disability; there was a 10-fold increase in the risk of severe or moderate disability at 5 years of age among children who had had meningitis (relative risk 10.3, 95% confidence interval 6.7 to 16.0, P<0.001). There was considerable variation in the rates of severe or moderate disability in children infected with different organisms. CONCLUSION: The long term consequences of having meningitis during the first year of life are significant: 32 of 1717 (1.8%) children died within five years. Not only did almost a fifth of children with meningitis have a permanent, severe or moderately severe disability, but subtle deficits were also more prevalent. (+info)
Evaluation and outcome of aphasia in patients with severe closed head trauma.
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In this study long-term observation of 12 patients with aphasia secondary to severe closed head trauma took place. The most frequent symptoms were amnestic aphasia and verbal paraphasia. Only one patient with a constant slow wave EEG focus in the dominant hemisphere had severe receptive symptoms. In all other patients the aphasia recovered rather well, though not totally, but the presence and degree of concomitant neuropsychological disorders were most important for the final outcome. (+info)
Speech disorder in schizophrenia: review of the literature and exploration of its relation to the uniquely human capacity for language.
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The language capacity of modern humans is thought by some to be clearly distinct from that of nonhuman primates (Bickerton 1990). Crow (1997, 1998a) has proposed that a disturbance in the uniquely human aspects of language is central to the genetic etiology of schizophrenia. A review of the literature on language disorder in schizophrenia provides evidence for widespread deficits in comprehension, production, attention, and cerebral lateralization of language. We focused here on those anomalies that are uniquely human aspects of language. Bickerton's five distinctly human language devices were examined in patients with schizophrenia and their families by using a structured scoring format on oral soliloquies. The chronic patients showed reduced use of clausal embedding and used fewer words than first episode patients or well family members. The amount of sentence complexity was found to be familial and to cosegregate with schizophrenia within families. These data are consistent with previous literature and additionally show a familial component to these measures, thus suggesting that deficits in specifically human aspects of language may be related to the genetics of schizophrenia. (+info)