Résumé
Objective:To analyze the clinical characteristics of domestic sporadic Creutzfeldt-Jakob disease (sCJD).Methods:One patient diagnosed with probable sCJD in our hospital in October 2019 was firstly reported; the clinical data of this patient were retrospectively analyzed combining with those of 82 patients publicly reported in domestic core journals on China Biology Medicine disc, CNKI and WANFANG databases from 2009 to 2019 and met with MRI-CJD consortium criteria for sCJD.Results:Of the total 83 patients, 78 were diagnosed as clinically probable sCJD and other 5 as definite sCJD. There were 45 males and 38 females; the median age was 63 (58, 68) years. The course of disease was recorded in 56 patients, and the time from onset to death was 4.3 (2.9, 7.0) months. The onset and main symptoms were diverse and non-specific, however, progressive cognitive impairments, myoclonus, ataxia, visual dysfunction, akinetic mutism frequently occurred during the whole disease course. As compared with those in patients less than or equal to 65 years old, cerebellar symptoms and visual impairment occurred more frequently in patients over 65 years old ( P< 0.05). High diffusion weighted imaging (DWI) signals were detected on the cortex and/or the basal ganglia and/or the thalamus of 92.4% patients (73/79). Meanwhile, akinetic mutism was more likely to appear in patients with basal ganglia involvements than the unaffected ones (62.5% vs. 25.8%, P<0.05). Abnormal cortical DWI high signals in one patient were detected 7 months before the appearance of first symptom, and four patients who were clinically confirmed by dynamic follow-up DWI presented clinical symptoms highly corresponding to changes in DWI signals in brain regions. Conclusions:The sCJD tends to occur in the middle-aged and elderly patients, with high heterogeneity in clinical symptoms, durations and abnormal DWI signals, and symptoms caused by damage in posterior cranial fossa occur more frequently in patients over 65 years old; the DWI lesions in basal segment might be seen as a sign for deterioration of disease. Dynamic DWI follow-up may be helpful for early detection and objective reflection of sCJD intracerebral lesions.
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Almost all active immunotherapy attempts which targeted at clearing or reducing β-amyloid (Aβ) plaques in brains of patients with Alzheimer'disease (AD) were fallen into unprecedented difficulties,because of unsatisfactory curative effects.Recently,more and more evidences support that low density lipoprotein receptor related protein 1 (LRP1) is involved in Aβ production and clearance through multiple non-immune pathways,which has showed the potential as a whole-new interference target different with classical Aβ immunotherapies.So,we try to summarize the research developments of roles of LRP1 in Aβ metabolic process in physiological and AD conditions,and look forward to its possible applications in the prevention and treatment of AD.
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Objective To study the effects of embryonic neural stem cells transplantion on trauma of red nucleus neu-rons of the rats with spinal cord injury.Methods NSCs in logarithmic phage were labeled with BrdU,a Sprague Dawley rat mode of spinal cord injury (SCI) was developed with electrocircuit control spinal cord injuring device.Thirty SD rats were randomly divided into three groups: sham group,SCI group and NSC group.The NSCs were trans-planted into injured site three days after SCI.Then NSCs labeled with Brdu were detected by immunohistochemisty,rubrospinal tract (RST) neurons were labeled by retrograde transport of the horseradish peroxidase (HRP) from the lesion site,which were taken by damaged axons and remained in the neurons,then the labeled red nucleus (RN) neurons were counted.Hind limb function of experimental rats was evaluated by a blinder observer using BBB open field locomotion rating score.Results BrdU positive NSCs were detected in the spinal cord after transplantation,the number of RST neurons labeled by HRP in NSC group was more than that in SCI group (P <0.01),the BBB score of NSC group was higher than SCI group (P <0.01).Conclusion The transplanted NSCs can survive in the injured site of spinal cord and protect RN,then promote more remarkably functional recovery after SCI.