Cases reported "Dementia"

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1/41. Dementia. Part 2: Person-centred assessment.

    A positive attitude towards people with dementia will enable the nurse to ascertain their strengths and abilities. Person-centred assessment can enhance the patient's experience and lead to more accurate information. nurses should also consider the needs and role of the patient's carer.
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2/41. Is a picture worth a thousand words? Evidence from concept definitions by patients with semantic dementia.

    Nine patients with semantic dementia (the temporal lobe variant of frontotemporal dementia) were asked to define concrete concepts either from presentation of a picture of the object or from its spoken name. As expected, the patients with the most severe semantic impairment produced the least detailed definitions, and the quality of the definitions overall was significantly related to concept familiarity. Further analyses of the definitions were designed to assess two key theoretical aspects of semantic organization. (i) Do objects and their corresponding names activate conceptual information in two neuroanatomically separable (modality-specific) semantic systems? If so, then-apart from any expected commonality in performance attributable to factors such as concept familiarity-one would not predict striking item-specific similarities in a patient's picture- and word-elicited definitions. (ii) Do sensory/perceptual features and more associative/functional attributes of conceptual knowledge form two neuroanatomically separable subsystems? If so, then one would predict significant dissociations in the prominence of these two types of information in the patients' definitions. The results lead us to favor a model of the semantic system that is divided by attribute type but not by modality.
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3/41. Semantic knowledge and episodic memory for faces in semantic dementia.

    Previous studies have documented poor recognition memory for faces in patients with semantic dementia. Preserved face recognition memory was found in this study, however, so long as atrophy was confined predominantly to the left temporal lobe. patients with structural damage to the right temporal lobe were typically impaired, with the status of the hippocampus and parahippocampal gyrus (including the perirhinal cortex) on the right being critical. Two single-case studies of patients with predominantly left temporal lobe pathology confirmed good recognition memory for famous faces, even if semantic knowledge about the celebrities depicted was severely degraded. An effect of semantic knowledge on recognition memory became apparent only when perceptually different photographs of the famous people were used at study and test. These results support the view that new episodic learning typically draws on information from both perceptual and semantic systems.
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4/41. Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity.

    Subacute diencephalic angioencephalopathy (SDAE) is a rare and fatal disease of unknown etiology that involves the thalami bilaterally. To date, there have been four cases reported, in which the diagnosis was established only after post mortem examination of the brain. We report two male patients, ages 69 and 41 years, who presented with progressive dementia and somnolence. Radiological evaluation revealed enhancing lesions involving both thalami. The differential diagnosis included a number of neoplastic, inflammatory and vascular processes. In both cases, pathological evaluation of biopsy specimens suggested the diagnosis of SDAE. Despite supportive care, the disease progressed rapidly and both patients died within weeks after initial presentation. The diagnosis was confirmed at autopsy in both cases. SDAE is a rare cause of bithalamic disease that can be mistaken for a neoplasm as well as a number of conditions that necessitate different treatment choices. The histopathological findings can establish the diagnosis when combined with radiological and clinical information. This report emphasizes the utility of stereotactic biopsy in early diagnosis of SDAE.
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5/41. Episodic memory: insights from semantic dementia.

    Semantic dementia, also known as the temporal lobe variant of fronto-temporal dementia, results in a progressive yet relatively pure loss of semantic knowledge about words, objects and people, and is associated with asymmetric, focal atrophy of the antero-lateral temporal lobes. Semantic dementia provides a unique opportunity to study the organization of long-term memory particularly since initial observations suggested sparing of episodic memory. Recent studies reveal, however, a more complex but theoretically revealing pattern. On tests of autobiographical memory, patients with semantic dementia show a 'reverse step function' with sparing of recall of events from the most recent 2 to 5 years but impairment on more distant life periods. Anterograde recognition memory for visual materials is extremely well preserved, except in the most deteriorated cases, although performance is heavily reliant upon perceptual information about the studied stimuli, particularly for items that are no longer known by the subjects. On tests of verbal anterograde memory such as word learning, performance is typically poor even for words which are 'known' to the patients. A source discrimination experiment, designed to evaluate familiarity and recollection-based anterograde memory processes, found that patients with semantic dementia showed good item detection, although recollection of source was sometimes impaired. Semantic knowledge about studied items and measures of item detection and source discrimination were largely independent. The implications of these findings for models of long-term memory are discussed. The results support the concept that episodic memory, or at least the recall of temporally specific autobiographical experiences, draws upon a number of separable memory processes, some of which can function independently of semantic knowledge.
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6/41. Action naming in dementia.

    Recent studies of action naming in dementia report contradictory results. Some studies have shown that naming of pictured actions is impaired and indeed worse than naming of pictured objects, whereas other studies report the opposite result, i.e. action naming is better preserved than object naming. One reason for these conflicting results may be that actions vary in their relationships with object knowledge. Instrumental actions, e.g. hammering, require access to knowledge about a specific object (a tool), whereas non-instrumental actions can be named correctly without access to knowledge about a specific object, e.g. running. Moreover, many instrumental action names share a name relationship with the instrument used to perform the action (homophony), whereas other action names do not, e.g. digging. In this case report, we describe an anomic patient RS with dementia affecting his access to knowledge about objects from visual, verbal and tactile input. By contrast, RS displays relatively well-preserved knowledge and naming of actions. We found an effect of instrumentality on pictured action naming, i.e. actions that depict an actor using a tool are named less accurately than actions that depict an actor performing an action without a tool. We argue that the instrumentality effect is independent of the name relationship between the action and the object and also the visual complexity of the action. We consider several explanations of the instrumentality effect and conclude that an impairment to the areas underpinning sensory feature and sensorimotor information (specific to manipulation) can account for an effect of instrumentality on action naming in dementia.
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7/41. Case study approach to removing physical restraint.

    The pathway to discarding the use of restraints on older people with a dementing illness is cluttered with misinformation. While exploring the reasons restraints are used as an intervention on an older person with a dementing illness, we find duty of care is an important aspect. This paper presents the process that occurred when an aged care facility used an education and consultation approach in an attempt to remove the need for physical and chemical restraint. This case involved the family of an older person with a dementing illness and staff of the aged care facility.
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8/41. frontotemporal dementia: clinical, neuroimaging, and molecular biological findings in 6 patients.

    Establishing the diagnosis in patients with clinical signs and symptoms suggesting primary degenerative disease with marked frontal lobe involvement is difficult. neuroimaging methods, in particular positron emission tomography (PET) with the tracer 18fluoro-2-deoxyglucose (FDG) and cerebrospinal fluid (CSF) examination of beta-amyloid and tau-protein levels may give additional information. We report five patients with clinical and radiological features of degenerative dementia with predominantly frontal involvement and one patient with primary progressive aphasia Diagnostic work-up included computed tomography (CT), magnetic resonance imaging (MRI), PET and tau-protein and beta-amyloid level determination in CSF. While neuropsychological performance varied among patients, CT and MRI demonstrated persistently frontal lobe involvement. PET revealed corresponding changes with frontal hypometabolism, but in addition, four patients (among them two with no corresponding temporal changes in CT or MRI) showed a decreased glucose uptake in the temporal cortices. CSF samples from five patients revealed elevated beta-amyloid 1-42 and tau levels in three and two patients, respectively. Reduced beta-amyloid 1-40 was found in two patients. We conclude that occurrence of clinical symptoms of frontotemporal dementia is accompanied by frontal hypometabolism regardless of additional clinical findings. The value of determination of beta-amyloid and tau protein levels remains to be determined.
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9/41. The neurological masquerade of intravascular lymphomatosis.

    BACKGROUND: Intravascular lymphomatosis (IVL) is an uncommon systemic disease characterized by occlusion of small vessels by malignant lymphomatous cells. central nervous system involvement usually presents as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events. OBJECTIVE: To increase awareness about IVL, an uncommon cause of neurological disease. DESIGN: This is a retrospective case series of 8 pathologically proved cases of IVL with neurological disease. patients were part of a pathological series collected between April 1962 and October 1998 at indiana University School of medicine and the Armed Forces Institute of pathology, washington, DC. SETTING: Neurological and neuropathological examinations were performed at tertiary referral hospitals. patients: Eleven patients were diagnosed pathologically as having IVL, but 3 were not included in this evaluation because of a lack of appropriate clinical information. Of the final sample (n = 8), there were 4 men and 4 women (mean /- SD age, 62.9 /- 9.9 years). RESULTS: All 8 patients had focal neurological deficits, 7 had encephalopathy or dementia, 5 had epileptic seizures, and 2 had myelopathy. death occurred at a mean of 7.7 months (range, 1-24 months) after the onset of symptoms. All patients had elevated cerebrospinal fluid protein levels, 4 had pleocytosis, and 2 had an elevated IgG level in their cerebrospinal fluid. Of the 4 patients who underwent a brain biopsy, 1 was diagnosed as having IVL before death. CONCLUSIONS: Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. Diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.
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10/41. Relearning of verbal labels in semantic dementia.

    Semantic dementia is a degenerative disorder of temporal neocortex characterised by loss of word and object concepts. There is limited evidence that temporary relearning of lost vocabulary may be possible, attributed to sparing of hippocampal structures. However, learning is variable across patients and factors underlying learning success are poorly understood. The study investigated relearning of object names in two severely anomic semantic dementia patients. Following memory models that assume that hippocampal memories require some neocortical representation to underpin them it was predicted that relearning would be influenced by patients' residual semantic information about stimuli. Experiment 1 confirmed that residual knowledge influenced learning success. On the assumption that neocortical knowledge encompasses concepts of space and time, as well as words and objects, it was predicted that learning would be affected by the availability of contextual (temporo-spatial) information. Experiment 2 demonstrated effective learning of object names, attributed to the patient's use of temporal order and spatial position knowledge. Retention of object names over months was linked to the patient's capacity for autobiographical experiential (temporo-spatial contextual) association. The findings indicate that relearning of lost vocabulary is possible in semantic dementia, indicating a role of the medial temporal lobes in the acquisition of semantic information. Effective learning does not imply reinstatement of lost concepts, but, it is argued, does involve some reacquisition of meaning. The findings challenge the traditional semantic-episodic memory dichotomy and are consistent with a "levels of meaning" account of semantic memory.
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