1/14. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population.Acquired cerebellar lesions in adults have been shown to produce impairments in higher function as exemplified by the cerebellar cognitive affective syndrome. It is not yet known whether similar findings occur in children with acquired cerebellar lesions, and whether developmental factors influence their presentation. In studies to date, survivors of childhood cerebellar tumours who demonstrate long-term deficits in cognitive functions have undergone surgery as well as cranial irradiation or methotrexate treatment. Investigation of the effects of the cerebellar lesion independent of the known deleterious effects of these agents is important for understanding the role of the cerebellum in cognitive and affective development and for informing treatment and rehabilitation strategies. If the cerebellar contribution to cognition and affect is significant, then damage in childhood may influence a wide range of psychological processes, both as an immediate consequence and as these processes fail to develop normally later on. In this study we evaluated neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither cranial irradiation nor methotrexate chemotherapy. Impairments were noted in executive function, including planning and sequencing, and in visual-spatial function, expressive language, verbal memory and modulation of affect. These deficits were common and in some cases could be dissociated from motor deficits. Lesions of the vermis in particular were associated with dysregulation of affect. Behavioural deficits were more apparent in older than younger children. These results reveal that clinically relevant neuropsychological changes may occur following cerebellar tumour resection in children. Age at the time of surgery and the site of the cerebellar lesion influence the neurobehavioural outcome. The results of the present study indicate that the cerebellar cognitive affective syndrome is evident in children as well as in adults, and they provide further clinical evidence that the cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/14. Monozygotic boys with fragile x syndrome.Monozygotic twin boys with fragile x syndrome underwent thorough genetic, psychiatric, neurological, and language evaluations at 10 years of age. They both demonstrated physical features, speech and language difficulties, social problems, and attentional deficits that characterize the behavioural phenotype of fragile x syndrome. Despite identical genetic constitutions, there were important developmental and behavioural heterogeneities. Twin A showed less social interaction and symbolic play and more speech and language dysfunction than twin B. Twin A also had significantly larger caudate volumes. It is suggested that the Xq27.3 anomaly may not be sufficient to account for all the behavioural phenotypic and neuroanatomical features of fragile x syndrome.- - - - - - - - - - ranking = 1.5keywords = behaviour (Clic here for more details about this article) |
3/14. Clinical diagnostic and intervention studies of children with semantic-pragmatic language disorder.The diagnosis of semantic-pragmatic language disorder (SPLD) has been the subject of a number of research studies over the last two decades. classification and diagnostic debates, while illuminating, have done little to develop tools to improve services to these children. In this paper, two children whose communication difficulties are suggestive of an SPLD diagnosis but who have differing profiles are studied. Using existing models of psycholinguistics and pragmatics to guide assessment and intervention, the diversity of language and social communicative behaviours that are covered by the label SPLD are exemplified. Consideration is given to whether the term SPLD is appropriate for both children or whether Bishop's revision of the diagnosis to 'pragmatic language impairment' might be more useful. methods of intervention and evaluation for semantic and pragmatic deficits in these two cases are described. It is argued that existing tools can enable accurate explanation and modelling of the communication of children with SPLD and that there is a role for intervention studies in helping to refine those tools, to improve therapies and to understand the nature of the condition more fully.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
4/14. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy.We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and prominent impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the kluver-bucy syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
5/14. Pure post-stroke cerebellar cognitive affective syndrome: a case report.Cerebellar pathology commonly shows important motor signs and less evident cognitive dysfunction. The 'cerebellar cognitive affective syndrome' is characterised by impairment on executive function, spatial cognition, language and behaviour. We report the case of a man with acute onset of transitory motor features and severe mental disorders. Cranial CT and brain MRI revealed extended cerebellar lesions. Neuropsychological assessment disclosed deficits of attention, executive function and memory. Auditory event-related potentials showed abnormal P300. These data suggest a pure "cerebellar cognitive affective syndrome"and strengthen the hypothesis of cerebellar cognitive function modulation.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
6/14. Topic repetitiveness after traumatic brain injury: an emergent, jointly managed behaviour.Topic repetitiveness is a common component of pragmatic impairment and a powerful contributor to social exclusion. Despite this, description, characterization and intervention remain underdeveloped. This article explores the nature of repetitiveness in traumatic brain injury (TBI). A case study of one individual after TBI provides the basis for a conceptualization of topic repetitiveness in terms of two major strands of thinking in pragmatics. Firstly, it is viewed as an emergent response to underlying deficits in non-linguistic abilities. Secondly, it is described in terms of joint management of topic development between the person with TBI and his interlocutor.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
7/14. Presymptomatic semantic impairment in a case of fronto-temporal lobar degeneration associated with the 16 mutation in MAPT.We describe a patient who came to neurological attention because of his at-risk status for the 16 exon 10 splice mutation in the tau gene (microtubule associated protein tau, MAPT), which had given rise to progressive behavioural disturbances in two of his siblings. The patient began to exhibit early signs of behavioural disturbance at around the age of symptom onset in both of his siblings. Although he did not spontaneously complain of difficulties in the domain of language, he met clinical, radiological and neuropsychological criteria for semantic dementia. On the assumption that his illness is mediated by the same pathological process as those of his siblings, we propose that this clinical picture represents the earliest changes of a semantic impairment - a phase of the illness that is often retrospectively described by patients and their relations, but has never previously been documented at first hand. Although typical of semantic dementia in many respects, the illness had several interesting and atypical features that emerged on detailed testing: first, he exhibited no insight into his difficulties; secondly, progression over a twelve-month interval was unusually slow; thirdly, he evinced a striking and consistent advantage for nonliving over living concepts; fourthly, a differential impairment of distinctive over shared knowledge did not emerge except when items that he could still name were compared with those for which he was anomic. Finally, the availability of post mortem pathological analysis from the brains of both of his affected siblings allowed us to attribute his illness to a specific pathological process which is considered unusual for patients with this clinical phenotype.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/14. Severe falciparum malaria and acquired childhood language disorder.language disorders have been reported after severe falciparum malaria but the deficits have not been described in detail. We assessed language outcome in three groups of children aged 6 to 9 years (n=487): those previously admitted to Kilifi District Hospital, kenya, with cerebral malaria (CM; n=152; mean age 7y 4 mo [SD 1y 1mo]; 77 males, 75 females); or those with malaria and complicated seizures (M/S; n=156; mean age 7y 4mo [SD 1y 2mo]; 72 males, 84 females); and those unexposed to either condition (n=179; mean age 7y 6mo [SD 1y 1mo]; 93 males, 86 females). Median age at hospital admission was 28 months (interquartile range [IQR] 19 to 44 mo) among children with a history of CM and 23 months (IQR 12 to 35mo) among children with a history of M/S. A battery of eight assessments covering the major facets of speech and language was used to measure language performance. Cognitive performance, neurological/motor skills, behaviour, hearing, and vision were also measured. Eighteen (11.8%) of the CM group, 14 (9%) of the M/S group, and four (2.2%) of the unexposed group were found to have a language impairment. CM (odds ratio 3.68, 95% confidence interval 1.09 to 12.4, p=0.04) was associated with significantly increased odds of an impairment-level score relative to the unexposed group. The results suggest that falciparum malaria is one of the most common causes of acquired language disorders in the tropics.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
9/14. 49,XXXXY syndrome: behavioural and developmental profiles.Behavioural, psychological, and cognitive profiles of three cases, including a 5 year old male with a 49,XXXXY karyotype and a (3;15) translocation, a 9 year old male with a 49,XXXXY karyotype, and a 32 year old male with 48,XXXY/49,XXXXY mosaicism, are presented. Significant behavioural problems were seen in the two older patients. The degree of mental retardation and impairment of language abilities were shown to be more severe in the older subjects as well. These findings are discussed with respect to the effects of the x chromosome on brain development.- - - - - - - - - - ranking = 2.5keywords = behaviour (Clic here for more details about this article) |
10/14. Impaired grammar with normal fluency and phonology. Implications for Broca's aphasia.Extensive testing of grammatical function, including assessment of spontaneous language, inflectional morphology, ability to produce grammatical structures, syntactic comprehension and grammatical judgement, was carried out in 2 patients with large dominant frontal lobe lesions, including but not confined to, the third frontal convolution. Both patients were fluent and had normal articulation and phonological production and neither was agrammatic, suggesting that even very large frontal lesions do not produce Broca's aphasia and that language cortex proper is confined to the postcentral perisylvian region. Both patients were impaired in the use of more complex syntactic structures and one, who in addition had severe generalized impairment in frontal lobe function, also had impaired judgement regarding the use and placement of functors. These data provide further support for the dissociability of syntactic and morphological aspects of grammar in aphasic patients and, together with other studies, link these functions with the frontal lobe and the postcentral perisylvian cortex, respectively. The sparing of grammatical judgement in 1 patient, despite a very extensive lesion, suggests that very large portions of the frontal lobe are involved in grammatical function. The nature of frontal lobe function in syntax appears to be congruent with the role of the frontal lobes in other aspects of behaviour.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
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