1/38. Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study.We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. magnetic resonance imaging showed a diffuse brain atrophy. A fluorodeoxyglucose positron emission tomography in one patient and a single photon emission computed tomography in another demonstrated a glucose hypometabolism or a hypoperfusion in the medial frontal and thalamic regions. Primary frontal involvement or frontal dysfunction secondary to thalamic lesions may contribute to the nature of dementia in these patients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/38. Missense and silent tau gene mutations cause frontotemporal dementia with parkinsonism-chromosome 17 type, by affecting multiple alternative rna splicing regulatory elements.frontotemporal dementia with parkinsonism, chromosome 17 type (FTDP-17) is caused by mutations in the tau gene, and the signature lesions of FTDP-17 are filamentous tau inclusions. Tau mutations may be pathogenic either by altering protein function or gene regulation. Here we show that missense, silent, and intronic tau mutations can increase or decrease splicing of tau exon 10 (E10) by acting on 3 different cis-acting regulatory elements. These elements include an exon splicing enhancer that can either be strengthened (mutation N279(K)) or destroyed (mutation Delta280(K)), resulting in either constitutive E10 inclusion or the exclusion of E10 from tau transcripts. E10 contains a second regulatory element that is an exon splicing silencer, the function of which is abolished by a silent FTDP-17 mutation (L284(L)), resulting in excess E10 inclusion. A third element inhibiting E10 splicing is contained in the intronic sequences directly flanking the 5' splice site of E10 and intronic FTDP-17 mutations in this element enhance E10 inclusion. Thus, tau mutations cause FTDP-17 by multiple pathological mechanisms, which may explain the phenotypic heterogeneity observed in FTDP-17, as exemplified by an unusual family described here with tau pathology as well as amyloid and neuritic plaques.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/38. Decision-making within the social course of dementia: accounts by Chinese-American caregivers.The goal of this retrospective pilot study was to provide a naturalistic description of the decision-making process regarding dementia, a chronic illness. The hypothesis was that in-depth descriptions by caregivers in a community setting would provide a more comprehensive and realistic representation of decision-making in the case of chronic illness than has been provided by most models. Data were gathered from in-depth, qualitative interviews with seven Chinese-American families living in the boston area and caring for an elderly family member with dementia. These were supplemented with interviews with medical professionals and ethnography done in the community regarding the care of elders with dementia. Based on the findings, this paper proposes a dynamic, more comprehensive model for the social process of decision-making which is particularly applicable to clinical and life situations of decision-making in the case of chronic illness. The model describes: 1) the decision-maker constellation, including multiple family members, professionals and service systems; 2) relationships between decision-makers as "allies" or "competitors"; 3) variations in the nature of the decision-making process; and 4) the interaction of decision-making with larger social, economic and cultural forces. Decision-making in the case of dementia is placed within the conceptual framework of the "social course" of chronic illness.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/38. Gliomatosis cerebri: disconnection of the cortical grey matter, demonstrated on PET scan.18-FDG and 11C methionine PET scans were performed on two patients with gliomatosis cerebri. The cortical grey matter was hypometabolic when compared with normal. The findings support the concept that the cerebral cortex becomes functionally disconnected in this disease owing to the infiltrative nature of the underlying tumour. This may account for the high incidence of dementia in the course of this disease. In one of the cases described here, there was clear evidence of progression from a discrete tumour mass of glioma to gliomatosis cerebri and this progression argues against the WHO classification of this disorder separately from glioma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/38. Deficits in irregular past-tense verb morphology associated with degraded semantic knowledge.Two distinct mechanisms are often considered necessary to account for generation of the past-tense of English verbs: a lexical associative process for irregular forms like speak-->spoke, and a rule-governed process ("add -ed") for regular and novel forms like talk-->talked and wug-->wugged. An alternative account based on a parallel-distributed processing approach proposes that one complex procedure processes all past-tense types. In this alternative view, neuropsychological dissociations are explained by reduced input from word meaning that plays a greater role in successful generation of the past-tense for lower frequency irregular verbs, and by phonological deficits that disproportionately affect regular and novel forms. Only limited evidence has been available concerning the relationship between knowledge of word meaning and verb-tense processing. The study reported here evaluated the past-tense verb abilities of 11 patients with semantic dementia, a neurodegenerative condition characterised by degraded semantic knowledge. We predicted and confirmed that the patients would have essentially normal ability to generate and recognise regular (and novel) past-tense forms, but a marked and frequency-modulated deficit on irregular verbs. Across the set of 11 patients, the degree of impairment for the irregular past-tense was significantly correlated with the degree of comprehension impairment as measured by verb synonym judgements. These results, plus other features of the data such as the nature of the errors to irregular verbs, are discussed in relation to currently developing theories of the language system.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/38. Power in the nursing home: the case of a special care unit.This article examines the effects and nature of power in a special care unit of a nursing home devoted to elders with dementia and/or disturbed behaviors. Drawing from two case studies and contemporary theories on power, I illustrate how the hierarchical structure of the clinic, together with the diffuseness and pervasiveness of disciplinary power, serves to shape the lives of--and to constrain the resistance opportunities open to--elders within the clinic. I also discuss the dilemma facing ethnographers of the clinic who may witness the sometimes disastrous effects of power but feel incapable, in their positions as researchers, of challenging the actions of clinical staff. At the same time, I observe how the contradictions of disciplinary power are often experienced by clinical staff who themselves struggle between taking actions they feel they must and those they would prefer. Far from acceding to the impotency that clinical anthropologists too often feel within a research setting, I argue that they can help to incite in their clinical colleagues the urgency of carrying out more productive alternatives to conventional "disciplinary" practices.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/38. Combined paravertebral lumbar plexus and parasacral sciatic nerve block for reduction of hip fracture in a patient with severe aortic stenosis.PURPOSE: To report the use of a combined paravertebral lumbar plexus and parasacral sciatic nerve block for reduction of hip fracture in an elderly patient with severe aortic stenosis. Clinical features: In an 87-yr-old lady with severe aortic stenosis and fracture of the right trochanter due to a fall, a combined right-sided paravertebral lumbar plexus and parasacral sciatic nerve block was used successfully for operative reduction of the fracture. A moderate amount of phenylephrine was required to maintain adequate systemic blood pressure despite the largely unilateral nature of the blocks. CONCLUSION: Combined paravertebral lumbar plexus and parasacral sciatic nerve block can be a viable alternative to general anesthesia and epidural or spinal block for hip surgery in patients with severe aortic stenosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/38. Nothing to say, something to sing: primary progressive dynamic aphasia.We describe a 76-year-old man (ADY) with dynamic aphasia in the setting of a degenerative frontal lobe dementia: primary progressive dynamic aphasia. He displayed a striking paucity of propositional speech despite intact speech production, and preserved singing and prosody. Vocal expression in the verbal and musical domains was investigated in a series of neuropsychological experiments based on novel language and musical tasks that were designed to establish the nature and specificity of the verbal output deficit. The features of the language disorder indicated that the speech output pathway was disrupted at the early stage of generation of a new pre-verbal message. In contrast, tests of musical output demonstrated that the generation of new musical ideas was unimpaired. The domain-specificity of dynamic aphasia may result from the disruption of specific cognitive processes necessary for the creation of verbal messages, as well as selective damage of brain regions involved in language production.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/38. The effects of motor neurone disease on language: further evidence.It might sound surprising that Motor Neurone disease (MND), regarded still by many as the very example of a neurodegenerative disease affecting selectively the motor system and sparing the sensory functions as well as cognition, can have a significant influence on language. In this article we hope to demonstrate that language dysfunction is not only a pronounced and well documented symptom in some MND patients but also that the study of language in MND can address interesting theoretical questions about the representation of language and conceptual knowledge in the brain. After a brief introduction delineating clinical and pathological features of the disease we discuss the evidence available in the literature for language dysfunction in MND. We then present linguistic data from our own study of seven patients with MND/dementia/aphasia syndrome focusing on the dissociation between noun and verb processing. To illustrate the clinical, neuropsychological and linguistic aspects of MND we describe in more detail the patient E.N., a pathologically confirmed case of MND/dementia. Finally, we attempt to characterise the nature of the linguistic impairment in MND in the light of current debates about the mechanisms underlying noun/verb dissociation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/38. Treatment of sexual disinhibition in dementia: case reports and review of the literature.Behavioral problems are common in dementia patients. They are complex in nature and are often the presenting symptoms of dementia. Its management poses continuous challenge. Gabapentin is an antiepileptic agent that has a role in the management of behavioral problems in demented patients such as agitation and aggression. Its role in treating sexual disinhibition has never been addressed. We describe 3 nursing home residents in 2 dementia units whose sexual disinhibition was effectively treated with gabapentin.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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