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The Annual Scientific Meeting of the Australian Association of Neurologists includes a Grand Rounds Session in which unusual cases are presented. The Chairmen of the 2006 session, Drs Richard Stark and Heather Waddy, lead the audience through a discussion of the history, physical signs, diagnostic investigations and finally the diagnosis at intervals throughout the case presentation. In introducing the cases Dr. Stark emphasized that the cases were selected because they were unusual and the final diagnosis may not be evident at the outset. The approach to the clinical problems and the process of thinking through the differential diagnoses and the method of investigation were as important to the discussion of these cases as the final diagnosis itself. These cases are reported here including an edited summary of the discussion with the same emphasis on the method of clinical analysis and the diagnostic process.  相似文献   
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Glucocorticoids (GCs, cortisol in human) are associated with impairments in declarative memory retrieval. Brain regions hypothesized to mediate these effects are the hippocampus and prefrontal cortex (PFC). Our aim was to use fMRI in localizing the effects of GCs during declarative memory retrieval. Therefore, we tested memory retrieval in 21 young healthy males in a randomized placebo-controlled crossover design. Participants encoded word lists containing neutral and emotional words 1 h prior to ingestion of 20 mg hydrocortisone. Memory retrieval was tested using an old/new recognition paradigm in a rapid event-related design. It was found that hydrocortisone decreased brain activity in both the hippocampus and PFC during successful retrieval of neutral words. These observations are consistent with previous animal and human studies suggesting that glucocorticoids modulate both hippocampal and prefrontal brain regions that are crucially involved in memory processing. Electronic Supplementary Material The online version of this article (doi:) contains supplementary material, which is available to authorized users.  相似文献   
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Coeliac disease has been associated with a variety of neurological conditions, most frequently cerebellar ataxia and peripheral neuropathy. To date, chorea has not been associated with coeliac disease. We present the case histories of 4 individuals with coeliac disease and chorea (4 women, average age of onset of chorea 61 years). Unexpectedly, most of these patients showed a notable improvement in their motor symptoms after the introduction of a gluten-free diet.  相似文献   
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BACKGROUND: Elevated cortisol levels might account for the reduction in central serotonin 1A (5-hydroxytryptamine [5-HT](1A)) receptor binding and function observed in patients with major depression. We tested this hypothesis by studying the effect of acute administration of hydrocortisone on 5-HT(1A) receptor binding potential (BP) in subjects recovered from depression. METHODS: We studied 14 subjects (8 male, 6 female) who had recovered from at least two episodes of major depression and had been euthymic and drug free for at least 6 months. Serotonin 1A receptor BP was measured by [(11)C]WAY-100635 in conjunction with positron emission tomography. Subjects were tested on two occasions in a double-blind, random-order, crossover design after administration of either hydrocortisone (100 mg orally) or placebo 12 hours previously. Positron emission tomography scans were analyzed with a region of interest analysis. RESULTS: Hydrocortisone treatment did not decrease 5-HT(1A) receptor BP either in the hippocampus, which was our a priori hypothesis, or in other cortical 5-HT(1A) regions; however, female subjects had a higher 5-HT(1A) receptor BP in certain brain areas compared with male subjects. CONCLUSIONS: These data are consistent with an earlier study in healthy volunteers and do not support the proposal that decreased 5-HT(1A) receptor BP in patients with acute major depression is a consequence of cortisol hypersecretion.  相似文献   
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Anthracyclines induced cardiotoxicity in children remains a major problem. Classical data are found: cumulative dose, way of injection, anterior cardiopathy, cardiac area irradiation, interaction with other cytotoxic drugs ad age. 2 kinds of disorders are recorded: 1) Acute rhythmic dysfunctions, early and often transitory; 2) congestive heart failure often not reversible and depending on total dose. Prognostic is still poor and intricate with prognostic of the tumor. Over all incidence is 2.7%. Very young children seem to be more sensitive. Continuous infusion is less toxic than IV bolus. Studies with new anthracyclines are yet uncommon but seems to be comparative with adult literature. Monitoring is identical to adults. EKG is of minor interest. Endomyocardial biopsies have been reported. There is no prognostic value of ultrasound tomogram, but exercise echocardiography can be performed. Nuclide fraction ejection is more reliable.  相似文献   
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Background : The present paper addressed the issue of whether pretreatment with intravenous (IV) chemotherapy affects response rate or survival in patients receiving hepatic artery chemotherapy (HAC). Methods : Case note reviews of 164 patients treated in a teaching hospital from June 1990 to July 1996 were carried out. Results : The response rate and carcino-embryonic antigen (CEA) fall in the two groups was almost identical. There was a nonsignificant survival advantage in the non-pretreatment group. Conclusions : Previous administration of IV chemotherapy did not affect the CEA response of patients receiving HAC.  相似文献   
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