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1/7. Probable variant Creutzfeldt-Jakob disease in a U.S. resident--florida, 2002.

    On April 18, 2002, the florida Department of health and CDC announced the occurrence of a likely case of variant Creutzfeldt-Jakob disease (vCJD) in a florida resident aged 22 years. This report documents the investigation of this case and underscores the importance of physicians increasing their suspicion for vCJD in patients presenting with clinical features described in this report who have spent time in areas in which bovine spongiform encephalopathy (BSE) is endemic.
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2/7. case reports of two biopsy-proven patients with Creutzfeldt-Jakob disease in singapore.

    INTRODUCTION: Creutzfeldt-Jakob disease (CJD) is the most common transmissible human subacute spongiform encephalopathy. There is limited literature on CJD in Southeast asia. We describe the clinical course and diagnostic evaluation of 2 singapore patients with biopsy-proven CJD. CLINICAL PICTURE: Two patients presented with non-specific symptoms such as withdrawal, forgetfulness, asthenia, giddiness and insomnia. Both patients had spontaneous myoclonic jerks and impairment of multiple neurologic systems (visual, pyramidal, cerebellar and neurocognitive systems). magnetic resonance imaging and electroencephalography provided helpful supportive evidence. diagnosis of CJD was established on brain biopsy. Histological features included spongioform degeneration, neuronal cell loss and astrocytosis. TREATMENT/OUTCOME: Treatment remains palliative. Deterioration in their clinical condition was relentless, progressing to a totally dependent state within 10 to 12 months. CONCLUSION: The early features of CJD can be varied and non-specific. It is important for physicians from different specialties to be cognisant of the clinical manifestations of CJD and the appearance of supportive and definitive investigations.
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3/7. Creutzfeldt-Jakob disease: an emergency department presentation of a rare disease.

    Creutzfeldt-Jakob Disease (CJD) is one of a group of neurodegenerative disorders causing spongiform encephalopathies. CJD is the most common human transmissible spongiform encephalopathy, or prion disease, but has an annual incidence of only 0.4-1.8 cases per million population worldwide. The prognosis for this disease is very poor and there is currently no cure. patients typically present with non-specific neurological or psychiatric complaints and often have multiple physician visits before diagnosis, which requires histological examination of brain tissue. This patient had serial presentations to our Emergency Department, with progressive symptoms and multiple laboratory and radiological tests as well as consults, but her diagnosis remained unclear until her disease rapidly progressed and a brain biopsy was performed. With increasing concerns about prion diseases such as bovine spongiform encephalopathy (BSE)-or mad cow disease-and CJD, awareness of the symptoms and diagnostic challenges associated with these diseases will be helpful to emergency physicians.
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4/7. Spongiform encephalopathies: the physician's responsibility.

    The spongiform encephalopathies encompass several diseases affecting humans and animals. In the united states, the most common of these disorders in humans is Creutzfeldt-Jakob disease. The most frequent manifestations include dementia, pyramidal tract signs, and extrapyramidal movement disorder. Several clinically distinct syndromes can be identified. Often the diagnosis is confused with other forms of dementia, and the only definitive method for establishing the diagnosis is autopsy evaluation of brain tissue. Unfortunately, since the recognition of the infectious etiology of Creutzfeldt-Jakob disease, fear has often unreasonably interfered with clinical care and autopsy evaluation of affected patients. In actuality, because of the low and restricted infectivity of the responsible agent, affected individuals present minimal risks to clinical caretakers, and handling of patient specimens is not dangerous if appropriate precautions are taken. These precautions are well established, and physicians and other health care workers should not refuse care of appropriate evaluation (including autopsy) to individuals with suspected Creutzfeldt-Jakob disease.
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5/7. Altered mental status in the elderly.

    Misdiagnosis of treatable dementia in the aged is costly to society and the family, and is unmeasurable in personal tragedy. The 4 Ds of the elderly--dementia, depression, delirium, and delusion--are discussed. family physicians are encouraged to seek out treatable disease.
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6/7. Creutzfeldt-Jakob disease in a physician: a review of the disorder in health care workers.

    We describe a 58-year-old physician who developed Creutzfeldt-Jakob disease (CJD) 30 years after formal training in pathology. To the best of our knowledge, he is the sixth physician and the 24th health care worker reported with CJD. We summarize the available data on these health care workers.
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7/7. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts -- japan, January 1979-May 1996.

    In 1997, a nongovernmental surveillance group for Creutzfeldt-Jakob disease (CJD) in japan reported to the Ministry of health and Welfare its analysis of a 1996 mail questionnaire survey of neurologic, psychiatric, and neuropathologic institutions throughout japan. This analysis identified 829 patients with CJD diagnosed by physicians during January 1979-May 1996, including a large number (43 patients) who had received a cadaveric dura mater graft during a neurosurgical (42) or orthopedic (one) procedure during 1979-1991. This report presents a summary of features of these 43 cases, which indicated that at least 41 of these patients had received dura mater grafts from the same processor, and describes CJD in the most recent recipient of a dura mater graft. The findings indicate that an international outbreak of CJD associated with a single brand of dura mater grafts is larger than previously recognized and that recipients of contaminated grafts may remain at risk for CJD at least 16 years following receipt of grafts.
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