1/24. The development of a dementia process within the family context: the case of Alice.Qualitative analysis was used to analyse a diary written over a period of two years by the sister of a dementia patient. The analysis is directed at the question of how a patient and a social network respond to each other during the dementia process. The diary highlights features in the development of the dementia process which receive scant attention in empirical studies: changes in the interaction process between patient and social network; a patient's residual capacities; a caregiver's perceived rewards of caregiving. We designated three phases in the interaction process: the phase of recognition, the stable phase and the phase of destabilization. This diary illustrates how a stable phase in the interaction between patient and primary caregiver can be established. The caregiver derived rewards by noticing and using the patient's residual capacities and by a feeling of being useful. In this case caregiving is not unidirectional. The quality of future support programmes may be enhanced by combining programmes aimed to influence patient's behaviour and to support caregivers.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/24. Clinical and neuroradiological follow-up in mucopolysaccharidosis type III (Sanfilippo syndrome).Mucopolysaccharidosis type III (Sanfilippo syndrome) is an autosomal recessive disorder characterised by progressive nervous system involvement with mental retardation, behavioural problems and seizures. Three patients, of 20 months to 12 years of age, were followed up for 3 years both clinically and by using brain magnetic resonance imaging (MRI). Our results suggest that in MPS III patients MRI findings, including atrophy and abnormal or delayed myelination, may precede the onset of overt neurological symptoms. The increasing neurological morbidity is accompanied by different degrees of progressive atrophic changes, mainly affecting the cerebral cortex and the corpus callosum. However, it appears that, across subjects, the rate of MRI changes is unrelated to the severity of the clinical phenotype. On this basis it could be argued that in MPS III the worsening of the neurological symptoms might not necessarily reflect only the progressive cerebral abnormalities detectable by MRI.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/24. Childhood epilepsy with neuropsychological regression and continuous spike waves during sleep: epilepsy surgery in a young adult.We describe the case of a man with a history of complex partial seizures and severe language, cognitive and behavioural regression during early childhood (3.5 years), who underwent epilepsy surgery at the age of 25 years. His early epilepsy had clinical and electroencephalogram features of the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia (landau-kleffner syndrome), which we considered initially to be of idiopathic origin. seizures recurred at 19 years and presurgical investigations at 25 years showed a lateral frontal epileptic focus with spread to Broca's area and the frontal orbital regions. Histopathology revealed a focal cortical dysplasia, not visible on magnetic resonance imaging. The prolonged but reversible early regression and the residual neuropsychological disorders during adulthood were probably the result of an active left frontal epilepsy, which interfered with language and behaviour during development. Our findings raise the question of the role of focal cortical dysplasia as an aetiology in the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
4/24. Continuous spikes and waves during slow sleep (CSWS): outcome in adulthood.We report a longitudinal, electroencephalographic and neuropsychological analysis of epilepsy with continuous spikes and waves during slow sleep (CSWS) in a 19 year-old boy. The clinical course fluctuated, with temporary worsening or improvement of the paroxysmal abnormalities, epilepsy and cognitive functions. At the end of the follow-up period, seizures persisted. Evaluation of the boy's behaviour, language and cognitive function suggested a dysexecutive syndrome. We discuss the relationship between paroxysmal abnormalities and neuropsychological disorders.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/24. The significance of intratumoural neurones and neuronal differentiation in diffuse gliomas: a case series.We describe four patients, ranging from 26-40 years of age, who presented with seizures and large, poorly circumscribed cerebral tumours on magnetic resonance imaging. The resected tumours demonstrated a histopathology similar to low-grade glioma, but with admixed mature neurones. immunohistochemistry demonstrated expression of putative neuronal antigens in the neuronal component as well as in tumour cells which did not show neuronal morphology. These tumours did not have the usual radiological and pathological features typical of gangliogliomas, but demonstrated an infiltrative pattern of growth and subsequent progressive behaviour. The term ganglioglioma, with its implication of good prognosis, is therefore inappropriate for tumours of this type. The expression of "neuronal" antigens by astrocytomas requires further investigation.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/24. Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome.We report six patients with clinically diagnosed and electrophysiologically confirmed motor neurone disease (MND), in whom communication problems were an early and dominant feature. All patients developed a progressive non-fluent aphasia culminating in some cases in complete mutism. In five cases, formal testing revealed deficits in syntactic comprehension. comprehension and production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall deterioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The behavioural symptoms ranged from mild anosognosia to personality change implicating frontal-lobe dementia. In three cases, post-mortem examination has confirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced pathological changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/24. hiv, stigma and health: integration of theoretical concepts and the lived experiences of individuals.AIM: To explore individual concepts of health and the role of stigma in human immunodeficiency virus (hiv) and hiv disease. The balance of power and knowledge are examined from both macro and micro perspectives, including both concepts of health and health and illness behaviour. METHOD: A literature review which supports the integration of theoretical concepts and research findings demonstrating the lived experiences of individuals. CONCLUSION: Stigma is a social construct which has significant impact on the life experiences of individuals both infected with and affected by hiv. The stigma experienced is unique to each individual and changes dynamically throughout the course of the hiv illness trajectory. Each of the four identified phases poses unique stigmatic qualities.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/24. Cerebellar liponeurocytoma. Case report with considerations on prognosis and management.BACKGROUND: Cerebellar liponeurocytoma has only recently been recognized as a distinct clinicopathological entity. Although the few cases described in literature support the relatively benign nature of this lesion, optimum treatment strategy and long term behaviour still have to be defined. CLINICAL PRESENTATION: This 61-year-old man came under our observation with unspecific signs of intracranial hypertension and a symptomathology revealing a probable posterior fossa lesion. Imaging studies showed a cerebellar mass lesion with prevalent adipose content. INTERVENTION: Gross total tumour removal was performed. The pathological examination revealed a lesion composed of medulloblastoma-like cells with prominent areas of lipidisation. A review of literature has been made in an attempt to investigate on recurrence patterns, importance of the extent of removal and the usefulness of radiotherapy. CONCLUSION: The few cases reported in literature so far seem to confirm the relatively benign nature of cerebellar liponeurocytoma. No radiotherapy was given in our case and the reasons for this decision are reported.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/24. Long-term neuropsychological follow-up and nosological considerations in five patients with continuous spikes and waves during slow sleep.Continuous spikes and waves during slow sleep (CSWSS) is a well-known EEG pattern that can be associated with cognitive and behavioural deterioration. We present the long-term neuropsychological follow-up and nosological considerations of five patients who developed CSWSS during childhood. All five of our patients presented CSWSS, although the duration and severity of this pattern varied. The outcome was of three basic types: acquired frontal dementia, language deficits and normal. Four of our patients were initially diagnosed with landau-kleffner syndrome but have had markedly diverse outcomes in terms of the severity and type of compromise. Our data suggest that the initial diagnosis, according to current nosological categories, has almost no prognostic significance, while the length and the age of onset of CSWSS, the site of epileptiform activity and the individual neuropsychological profile are more useful for identifying the long-term outcome of patients with CSWSS.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/24. Genetically confirmed clinical Huntington's disease with no observable cell loss.Huntington's disease (HD) results from neurodegeneration of the neostriatum. The mutation on chromosome 4 is an expansion in a triplet repeat (CAG)(n) located within the IT15 gene. Only six patients have been reported with clinical features of HD in association with limited neuropathology. Of these, only one has had the diagnosis confirmed by genetic (dna) testing. We describe a patient with the clinical phenotype and genetically confirmed HD but unexpected limited neuropathology. The patient was seen because of aggressive behaviour and memory problems of two years duration. The differential diagnosis included HD although there was no family history. dna testing was positive for the HD mutation. Clinical follow up three months later confirmed classic features of HD. Progression of the disease was rapid with death three years later. Neuropathology revealed a largely intact neostriatum with bilateral ischaemic damage and cell loss in the external globus pallidus. Such pathology alone could explain the clinical features of HD. This is only the second report of genetically confirmed clinically manifest HD with little evidence of HD neuropathology. There are several unusual features which could not have been predicted by the clinical picture, in particular the progressive course of bilateral ischaemic changes restricted to the external globus pallidus. The potential to miss other HD cases at post-mortem examination, and the implications of this for family members, are discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
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