BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been ...BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported successively. But its imageological detection needs to be further investigated. OBJECTIVE: To analyze the characteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE. DESIGN: Retrospective analysis. SETTING: The General Hospital of Chinese PLA. PARTICIPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives. METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem. MAIN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients. RESULTS: Ten patients with WE were involved in the final analysis. ①Nine patients presented various degrees of mental and conscious disturbance. Six patients initially presented vertigo, nausea, and vomiting. Five patients showed opthalmoplegia. Three patients presented hypotension (BP < 120/60 mm Hg, 1 mm Hg= 0.133 kPa). Two patients showed ataxia. One patient showed severe polyneuropathy. Both lower extremities were more severe. EMG showed axonal degeneration. ②Five patients accepted skull MRI examination. Three patients showed typical high signals in periaqueductal regions and circumference of third and fourth ventriculus in T2 -weighted and flair-weighted images, two of them showed high signals in fornixes in T2 -weighted and flair-weighted images, and one of them showed high signals in optic chiasma, both mammillary bodies, and subcortical white matter. No abnormality was found in the other two patients. Two patients who accepted the supplements of thiamine showed obviously improvements in the second MRI examination. ③Macroscopically, the border between gray and white matters was clear in the coronal section of cerebrum. Congestions, edema and multiple petechial hemorrhages were found in periaqueductal regions, circumference of third and fourth ventriculus, and both mammillary bodies. Microscopically, various degrees of necrosis of parenchymal structures, loss of nerve cells and ischemic changes were found. Myelinated fibers were more affected than neurons. There were focal capillary proliferation and multiple petechial hemorrhages. Prominent astrocytic proliferations were found in gelatinous fiber staining. And demyelinations were found in myelin staining. These pathological findings were all in accord with the diagnostic criteria of WE. ④In the diagnosis before death, 4 of 5 patients who were supplemented with thiamine had obvious improvement 2 weeks later and 1 of them abandoned therapy due to progressive aggravation of jaundice caused by recurrence of stomach cancer. The other 5 patients who were not diagnosed definitely before death and not supplemented with thiamine died. Final diagnosis was performed in autopsy examination. CONCLUSION: The clinical manifestations of this group of WE patients are atypical. MRI and pathological examination results are corresponding, and both have the characteristic manifestations of WE.展开更多
Background Lipoprotein glomerulopathy (LPG), once recognized as a rare glomerular disease, has been reported around the world in recent years. In this study, we reported 8 patients of LPG and aimed to explore the cli...Background Lipoprotein glomerulopathy (LPG), once recognized as a rare glomerular disease, has been reported around the world in recent years. In this study, we reported 8 patients of LPG and aimed to explore the clinical features and pathological characteristics of LPG under light microscope, immunofluorescence staining, and electron microscope.Methods Clinical manifestations were recorded on the day of renal biopsy. Biochemical patterns of lipids and lipoproteins were detected by routine examination. Plasma concentrations of apo B and apo E were determined by radial immunodiffusion assays. Biopsy specimens were then processed for light microscopy, immunohistochemical staining for immunoglobulins and complement components, and electron microscopy. Glomerular deposition of apo A, B, and E were detected using monoclonal antibodies on cryostatic sections.Results All of the eight patients presented with edema, microscopic hematuria, severe proteinuria, anemia, and enlarged kidney size. Biochemical profiles revealed high levels of triglycerides, apo B, and apo E. We noted increments of glomerular size and lipoprotein thrombi occupying capillary lumina in the glomeruli of all patients. Immunofluorescence staining showed that the thrombi were strongly positive for apo A, B, and E. Granules and various sizes of vacuoles were observed in the thrombi under electron microscope.Conclusion Compared with previous reports on LPG in other countries, unique clinical and pathological features were found in this group of Chinese LPG patients.展开更多
Objective To further investigate the association among clinical pathology,complement activation and renal secretory IgA(SIgA)deposition in patients with IgA nephropathy(IgAN).Methods The activation of serum complement...Objective To further investigate the association among clinical pathology,complement activation and renal secretory IgA(SIgA)deposition in patients with IgA nephropathy(IgAN).Methods The activation of serum complements was detected by immunoturbidimetry and ELISA.Renal deposition of SIgA and activation of complements were detected by immunofluorescence.展开更多
文摘BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported successively. But its imageological detection needs to be further investigated. OBJECTIVE: To analyze the characteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE. DESIGN: Retrospective analysis. SETTING: The General Hospital of Chinese PLA. PARTICIPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives. METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem. MAIN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients. RESULTS: Ten patients with WE were involved in the final analysis. ①Nine patients presented various degrees of mental and conscious disturbance. Six patients initially presented vertigo, nausea, and vomiting. Five patients showed opthalmoplegia. Three patients presented hypotension (BP < 120/60 mm Hg, 1 mm Hg= 0.133 kPa). Two patients showed ataxia. One patient showed severe polyneuropathy. Both lower extremities were more severe. EMG showed axonal degeneration. ②Five patients accepted skull MRI examination. Three patients showed typical high signals in periaqueductal regions and circumference of third and fourth ventriculus in T2 -weighted and flair-weighted images, two of them showed high signals in fornixes in T2 -weighted and flair-weighted images, and one of them showed high signals in optic chiasma, both mammillary bodies, and subcortical white matter. No abnormality was found in the other two patients. Two patients who accepted the supplements of thiamine showed obviously improvements in the second MRI examination. ③Macroscopically, the border between gray and white matters was clear in the coronal section of cerebrum. Congestions, edema and multiple petechial hemorrhages were found in periaqueductal regions, circumference of third and fourth ventriculus, and both mammillary bodies. Microscopically, various degrees of necrosis of parenchymal structures, loss of nerve cells and ischemic changes were found. Myelinated fibers were more affected than neurons. There were focal capillary proliferation and multiple petechial hemorrhages. Prominent astrocytic proliferations were found in gelatinous fiber staining. And demyelinations were found in myelin staining. These pathological findings were all in accord with the diagnostic criteria of WE. ④In the diagnosis before death, 4 of 5 patients who were supplemented with thiamine had obvious improvement 2 weeks later and 1 of them abandoned therapy due to progressive aggravation of jaundice caused by recurrence of stomach cancer. The other 5 patients who were not diagnosed definitely before death and not supplemented with thiamine died. Final diagnosis was performed in autopsy examination. CONCLUSION: The clinical manifestations of this group of WE patients are atypical. MRI and pathological examination results are corresponding, and both have the characteristic manifestations of WE.
文摘Background Lipoprotein glomerulopathy (LPG), once recognized as a rare glomerular disease, has been reported around the world in recent years. In this study, we reported 8 patients of LPG and aimed to explore the clinical features and pathological characteristics of LPG under light microscope, immunofluorescence staining, and electron microscope.Methods Clinical manifestations were recorded on the day of renal biopsy. Biochemical patterns of lipids and lipoproteins were detected by routine examination. Plasma concentrations of apo B and apo E were determined by radial immunodiffusion assays. Biopsy specimens were then processed for light microscopy, immunohistochemical staining for immunoglobulins and complement components, and electron microscopy. Glomerular deposition of apo A, B, and E were detected using monoclonal antibodies on cryostatic sections.Results All of the eight patients presented with edema, microscopic hematuria, severe proteinuria, anemia, and enlarged kidney size. Biochemical profiles revealed high levels of triglycerides, apo B, and apo E. We noted increments of glomerular size and lipoprotein thrombi occupying capillary lumina in the glomeruli of all patients. Immunofluorescence staining showed that the thrombi were strongly positive for apo A, B, and E. Granules and various sizes of vacuoles were observed in the thrombi under electron microscope.Conclusion Compared with previous reports on LPG in other countries, unique clinical and pathological features were found in this group of Chinese LPG patients.
文摘Objective To further investigate the association among clinical pathology,complement activation and renal secretory IgA(SIgA)deposition in patients with IgA nephropathy(IgAN).Methods The activation of serum complements was detected by immunoturbidimetry and ELISA.Renal deposition of SIgA and activation of complements were detected by immunofluorescence.
