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Objective@#To search for biomarkers for human familial prion disease.@*Methods@#Two-dimensional differential gel electrophoresis (2D-DIGE) proteomic analysis has been performed in frontal lobe tissues of 3 patients suffering from human familial prion disease (PrP) and 3 age-and sex-matched patients suffering from sudden death due to heart failure without neurological disease.@*Results@#The maps revealed 14 polypeptide chains differentially modulated in the PrP samples, among those, 7 could be identified upon digestion and MALDI-TOF/MS analysis, of which 6 appeared to be up-regulated, 1 being down-regulated.@*Conclusions@#We highlight Galectin-1(Gal-1), ryanodine receptor 2 (RyR2), ubiquitin, Rab-interacting lysosomes protein-like protein 1 (RILPL-1) profillin 2 (PFN2), in the differential map. These proteins are related to neurogenesis, the clearance of misfolded proteins, stasis of calium channel, myoclonus and so on. These proteins are potential biomarkers or targets for treatment of prion disease.
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Objective To investigate the clinical presentation,diagnosis,and surgical management of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis.Methods One case of anti-NMDA receptor encephalitis in a previously healthy 17-year-old female related to the development of NMDA receptor autoantibodies triggered by an ovarian teratoma was reported.The related literature was reviewed and the clinical feature was summarized.Results Removal of the ovarian teratoma combined with intravenous immuneglobulin and corticosteroid proved curative with eventual resolution of the paraneoplastic disease process and associated psychiatric symptoms.Conclusion Increasingly,reports of anti-NMDA receptor encephalitis associated with ovarian teratomas,as well as a novel assay to measure these antibodies suggest an etiology for this disease process that may be amenable to prompt surgical excision.
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Objective To explore the clinical and imaging features of reversible posterior leukoencephalopathy syndrome(RPLES).Methods The data of clinic five cases with RPLES were analyzed retrospectively.Results The primary diseases in this group were hypertension for one case,uraemia for three cases and lupus nephropathy for another.Clinical features of five patients include acute onset of hypertension,headache with vomiting.Neurological symptoms were conscious disturbance,seizures(in 5 cases)and visual disorder(in 3 cases).The movement of limbs were normal.The abnormal laboratery data was renal inadequacy in 5 cases.The findings of imaging of all cases included bilateral white matter of parietal and occipital lobe abnormalities,which appeared as large areas of low density on CT scan,hypo-intense in T1 and hyper-intense in T2 on MRI scan.Four cases presented clinical symptoms improvement in a short time and radiological recovery obviously after anti-hypertensive and anhydration therapy.One patient died caused on brain herniation.Conclusions Clinical features of RPLES is brain damage associated with malignant hypertension or the use of immunosuppressive agents.The most important pathogenesis is considered to be failure of the self regulation of cerebral vascular circulation.The imaging findings are bilateral symmetry cortical and subcortical edema in the posterior areas of the brain.Most patients can be recovery by timely therapy,otherwise the irreversible neuronal injury even death can be also found in very few serious cases.
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Objective To confirm the presence of human cytomegalovirus (HCMV) antigen and DNA in intracranial arterial walls and the association of the virus with the development of atherosclerosis in patients with atherosclerotic cerebral infarction Methods The subjects of this study were divided into two groups, the atherosclerosis group and the control group All patients were autopsied within 12 hours after death During autopsy, samples from intracranial arteries were formalin fixed and paraffin embedded All sections were routinely stained with hematoxylin and eosin (HE) and observed on the presence of atherosclerotic plaques The atherosclerosis group was subdivided into grade Ⅰ～Ⅱ group and grade Ⅲ～Ⅳ group according to the degrees of arterial lumen stenosis Only those vessel walls without signs of atheroma formation were included in the control group HCMV antigens and DNA were detected by immunohistochemistry, in situ hybridization and polymerase chain reaction (PCR) respectively The monoclonal antibody was against HCMV early (E) antigen Probes and primers were derived from major immediate early (MIE) genomic regions of cytomegalovirus strain AD169 As a positive control for the reactions, tissues from human small intestine membrane known to be positive for HCMV by H&E staining were used Results There were 18 of 35 atherosclerotic arteries defined as grade Ⅰ～Ⅱ atherosclerosis, and the rest were grade Ⅲ～Ⅳ The DNA of HCMV was found in 14/35 and 2/20 of arterial walls with atherosclerosis and negative control group by in situ hybridization, in 21/35 and 6/20 by PCR, and HCMV antigen in 12/35 and 2/20 by immunohistochemical studies respectively, and significant difference was be found among them ( P =0 018, P =0 032, P =0 047) There was also significant difference between grade Ⅲ～Ⅳ and grade Ⅰ～Ⅱ atherosclerosis by all the above three types of studies ( P =0 027, P =0 009, P =0 003) Conclusion Our results revealed that HCMV could be found in the atherosclerotic arterial walls, and it is suggested that the arterial walls might be the potential sites of existence of the virus We also postulate that there might be an association between the HCMV DNA and antigens in intracranial arterial walls and atherosclerotic plaques