Cases reported "Dementia"

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1/6. Atypical amyotrophic lateral sclerosis with dementia mimicking frontal Pick's disease: a report of an autopsy case with a clinical course of 15 years.

    This report concerns an autopsy case of atypical amyotrophic lateral sclerosis (ALS) with dementia mimicking frontal Pick's disease. The patient was a Japanese woman without hereditary burden who was 45 years old at the time of death. She developed abnormal behavior and amnesia at age 30, followed by disinhibition, aspontaneity, urinary incontinence, abulia, and rectal incontinence. Neurological signs compatible with ALS developed about 14 years after the disease onset. No respirator was used throughout the clinical course. Macroscopically, neuropathological examination showed atrophy of the frontotemporal lobes with accentuation in the convexities of the frontal lobes. Histologically, there was neuronal loss in the cerebral cortex, parahippocampal gyrus, amygdala, caudate nucleus, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to marked degeneration of the pyramidal tracts. ubiquitin-immunoreactive neuronal inclusions were present in the frontotemporal cortical layer II neurons and motor neurons in the brain stem and spinal cord. In the hippocampal dentate granular cells, many ubiquitin-immunoreactive neurites were present without ubiquitin-immunoreactive intraneuronal inclusions. Based on these clinicopathological findings and a review of the literature, we concluded that our case was atypical ALS with dementia of long disease duration. We also note the possibility that motor neuron disease-inclusion dementia with a long clinical course may develop into ALS in the final stage of the illness.
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2/6. Hyperphosphorylated tau deposition parallels prion protein burden in a case of Gerstmann-Straussler-Scheinker syndrome P102L mutation complicated with dementia.

    Hyperphosphorylated tau (p-tau) deposition has been documented in a limited population of patients with Gerstmann-Straussler-Scheinker syndrome (GSS) with particular point mutations of the prion protein (PrP) gene. Although its pathogenesis is only poorly understood, p-tau in GSS is known to be identical to that in Alzheimer's disease (AD). We conducted immunohistochemical and quantitative image studies on the brain from a 44-year-old man with a 7-year history of dementia, diagnosed as having GSS with a point mutation of the PrP gene at codon 102 (GSS102), the commonest mutation in GSS. Severe spongiform degeneration and numerous PrP plaques were disclosed in the cerebral cortices and hippocampus, consistent with the diagnosis. However, rarely described in GSS102, prominent p-tau deposits as pretangles, neurofibrillary tangles and degenerating neurites were demonstrated adjacent to or around PrP plaques. beta-amyloid protein (Abeta) plaques were generally sparse and appeared invariably to be of a diffuse type. Double-labeling immunohistochemistry yielded co-localization of p-tau with PrP but not with Abeta. Most PrP plaques did not contain Abeta. These results excluded a diagnosis of concomitant AD. Quantitative analysis on a fractional area density of immunoreactive pixels demonstrated that burdens of PrP and p-tau but not Abeta were significantly correlated. These results suggest that p-tau deposition in this GSS102 is secondarily induced by PrP but not by Abeta (secondary tauopathy). Our study also suggests that p-tau deposition might be a more common phenomenon in long-standing GSS.
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3/6. The needs of people with young-onset dementia and their carers.

    Young people who develop dementia and their carers have all the distressing symptoms of older people who have this condition but with the added problems of having a career, financial commitments and often a young family. Getting a diagnosis can be difficult and carers may feel very burdened and isolated. This paper explores the condition and the help that is available.
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4/6. Self-care in elders with dementia: a concept analysis.

    PURPOSE: The purpose of this study was to analyze the concept of self-care in elders with dementia through a review of nursing literature and to provide more understanding of the definition and perspectives of the concept of self-care notion in elders with dementia. methods: The technique developed by Walker and Avant was used as a guide in analyzing the concept of self-care. RESULTS: Attributes of self-care in dementia may include a single or group of actions needed for sustaining life, a personal effort to maintain functional independence while minimizing other's assistance, an outcome behavior from the person's interaction with inter-personal and/or contextual environment, and a functional ability that may decline in parallel to cognitive impairment. Antecedents of self-care in dementia may include at least presence of a certain degree of cognitive appraisal for the self-care needs, self-willingness for the self-care action, spatial and visual orientation, cultural pre-conception of the self-care behavior, presence of environmental context/equipment available for self-care, and sufficient time available. The consequences may include sustaining of life, feel of satisfaction, achieving independence, extended life expectancy, increased self-confidence, decreased caregiver distress and/or burden, savings in health care costs. DISCUSSION: Defining attributes and antecedents and consequences of self-care in dementia identified in this study provided empirical ground of a middle-range theory of self-care for a clinical population with dementia and generated possible hypotheses to be tested in future studies.
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5/6. Encephalopathy in chronic renal failure responsive to deferoxamine therapy. Another manifestation of aluminum neurotoxicity.

    We describe a patient undergoing chronic hemodialysis who developed a neurologic syndrome consisting of seizures, progressive myoclonus, and mild dementia and who responded to chelation therapy with deferoxamine mesylate. Neither her serum nor bone aluminum concentrations indicated aluminum toxicity. However, the presence of a positive deferoxamine-infusion test was suggestive of an elevated body burden of aluminum. Treatment with deferoxamine resulted in marked clinical improvement in her neurologic status within two months. The utility of using the deferoxamine-infusion test rather than serum aluminum levels in evaluating aluminum toxicity in chronic renal failure is suggested.
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6/6. Successful treatment of dialysis osteomalacia and dementia, using desferrioxamine infusions and oral 1-alpha hydroxycholecalciferol.

    A 54-year-old patient with fracturing dialysis osteomalacia and dementia demonstrated rapid deterioration following parathyroidectomy which was performed for sustained hypercalcemia. Reduction of the total body aluminum burden was attempted using desferrioxamine (DFO) as a chelating agent. After 6 months, DFO infusion resulted in sustained clinical remission of both neurological and skeletal symptoms, associated with an improvement in the EEG and improved mineralization of bone. A reduction in total body aluminum burden was reflected by reduced skeletal aluminum content, quantitated histochemically in iliac crest bone biopsies before and after DFO therapy. Dramatic increases in serum aluminum levels were documented in the initial weeks of DFO therapy leading to increased removal of aluminum during dialysis; in vitro studies indicated that the ultrafiltrable fraction of serum aluminum increased from 17 to more than 60% after initiating DFO treatment. However, after 6 months of therapy, serum aluminum levels remained unchanged after DFO infusion. These findings suggest that the serum aluminum response to DFO infusion might be a useful reflection of the total-body aluminum burden and also a reflection of the adequacy of a chelation program designed to reduce whole-body aluminum content.
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