BACKGROUND Although coronavirus disease 2019(COVID-19)vaccines have been effective in controlling the COVID-19 pandemic,a variety of post-vaccination neurological complications have been reported worldwide.Amyloidβ-r...BACKGROUND Although coronavirus disease 2019(COVID-19)vaccines have been effective in controlling the COVID-19 pandemic,a variety of post-vaccination neurological complications have been reported worldwide.Amyloidβ-related angiitis(ABRA)is a rare neurological disease.The underlying cause of ABRA is unknown,but several studies suggest that it is caused by an excessive immune response to amyloid-βdeposited in blood vessels.In addition,limited attention has been paid to potential triggers of ABRA,such as infection or vaccination.CASE SUMMARY We report a case of ABRA that developed 2 wk after COVID-19 vaccination.A 75-year-old woman developed a frontal headache after receiving a second dose of COVID-19 BNT162b2 vaccine(Pfizer-BioNTech).Diffusion-weighted magnetic resonance imaging(DW-MRI)of the head showed abnormal hyperintensity,suggesting cerebral infarctions in the left parietal and occipital lobes.We diagnosed her condition as ABRA based on a brain biopsy.We administered steroid pulse therapy and the patient’s symptoms and DW-MRI abnormalities improved.This case had a good outcome due to prompt diagnosis and treatment.CONCLUSION We report a case of ABRA that may have been triggered by COVID-19 vaccination.展开更多
Objective:To summarize the clinical features,imaging manifestations,therapeutic options,and prognosis of the primary angiitis of the central nervous system(PACNS)and to explore the role of high-resolution magnetic res...Objective:To summarize the clinical features,imaging manifestations,therapeutic options,and prognosis of the primary angiitis of the central nervous system(PACNS)and to explore the role of high-resolution magnetic resonance imaging(HR-MRI)in the PACNS diagnosis and treatment.Methods:One patient with PACNS treated by HR-MRI was retrospectively analyzed and summarized by combining relevant literature.Results:The patient was a young female who was hospitalized with progressive cerebral infarction and multiple intracranial arterial stenosis.HR-MRI indicated vasculitic changes.After excluding other diseases,hormone shock combined with immunosuppression was given,followed by long-term rehabilitation treatment.The patient’s condition tended to stabilize,and the prognosis was satisfactory.Conclusion PACNS is challenging to diagnose and is characterized by poor prognosis and easy recurrence.HR-MRI plays an important role in the clinical diagnosis and treatment adjustment for PACNS.展开更多
Frosted branch angiitis(FBA)is a rare retinal vascular disease that usually affects healthy,young adolescents and tends to be bilateral.Cxirrently,there are limited reports on FBA worldwide.In this paper,we will prese...Frosted branch angiitis(FBA)is a rare retinal vascular disease that usually affects healthy,young adolescents and tends to be bilateral.Cxirrently,there are limited reports on FBA worldwide.In this paper,we will present a case of FBA after fever.展开更多
Background:Cutaneous leukocytoclastic angiitis(CLA)is a clinically rele-vant condition,with previous studies suggesting an association with herpes virus infections.However,the causality of this association remains unc...Background:Cutaneous leukocytoclastic angiitis(CLA)is a clinically rele-vant condition,with previous studies suggesting an association with herpes virus infections.However,the causality of this association remains unclear.This study aimed to investigate the causal relationship between herpes viruses and CLA.Methods:Genetic variants linked to the herpes virus were retrieved from the Integrative Epidemiology Unit at the University of Bristol open genome-wide association studies project and FinnGen database.Data on CLA,involving 262 CLA cases and 207,482 healthy controls,were obtained from the FinnGen consortium R7.Mendelian randomization(MR)analysis,including the inverse variance weighted(IVW),MR-Egger,and weighted median methods,was performed.Sensitivity analyzes were conducted to ensure the accuracy of the results.Results:Of the 15 herpes viruses investigated,only human herpes virus 6(HHV-6)demonstrated a causal association with CLA(odds ratio:1.886,95%confidence interval:1.053–3.378,p=0.033),indicating that HHV-6 infection significantly increases the risk of CLA.Furthermore,IVW and MR-Egger tests for heterogeneity confirmed homogeneous MR analysis results without evi-dence of horizontal pleiotropy(p>0.05).No significant causal relationship was observed for other herpes viruses,such as herpes simplex virus,varicella-zoster virus,cytomegalovirus,and Epstein-Barr virus.Conclusion:Our MR analyzes strongly support a causal relationship between HHV-6 and CLA,elucidating the etiology of this condition and highlighting the potential of HHV-6-targeted therapeutic interventions in CLA treatment.However,further research is necessary to expound the underlying mechanisms and explore potential therapeutic interventions targeting HHV-6-associated CLA.展开更多
Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-...Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-year-old male had a fluctuating disease course for more than 3 years. He presented visual disorders, seizure, cognitive impairment, hypersomnia, unsteady gait, dysphasia, dysphagia, and incontinence. Magnetic resonance imaging showed multiple, supratentorial and infratentorial abnormal signals, while cerebrospinal fluid and cerebral angiography were normal. Magnetic resonance spectrum showed a decrease of N-acetyl-aspartate. Brain biopsy revealed nongranulomatous lymphatic vasculitis with reactive gliosis, cicatrization, demyelination and focal hemorrhages.展开更多
Introduction:Primary angiitis of the central nervous system(PACNS)is a vasculitis confined to the CNS.A small proportion of the lesions may present as a tumor-like mass,which is rarely seen in children.Case presentati...Introduction:Primary angiitis of the central nervous system(PACNS)is a vasculitis confined to the CNS.A small proportion of the lesions may present as a tumor-like mass,which is rarely seen in children.Case presentation:A 5-year-old girl was admitted to our hospital because of an intermittent headache.Brain imaging suggested a space-occupying lesion in the right cerebral hemisphere.The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy.Her condition improved after immunotherapy.Conclusion:Pediatricians should consider the possibility of PACNS when encountering intracranial tumor-like lesions.Early diagnosis of tumor-like PACNS and prompt immunotherapy could improve the long-term prognosis and avoid surgery.展开更多
Objective To investigate the etiology, pathology, and mechanism of pathogenesis of Moyamoya disease. Methods A total of 15 human autopsies were analyzed. In addition, in order to create an animal model of the disease,...Objective To investigate the etiology, pathology, and mechanism of pathogenesis of Moyamoya disease. Methods A total of 15 human autopsies were analyzed. In addition, in order to create an animal model of the disease, 21 Japanese rabbits were divided randomly into two groups and subjected to injections of horse serum either intravenously or locally in the area of the sympathetic ganglia. Pathological and immunohistochemical characteristics were observed. Results The pathological features of the autopsies and the animal models both involved intima hyperplasia and stenosis or even occlusion of the lumen in the terminal ends of the internal carotid artery and the anterior and middle cerebral arteries. Disconnections or even breakages of the inner layer of the lumen were also observed, without an obvious inflammatory response. Hyperplasic smooth muscle cells of the medial membrane had extended inward through broken portions of the internal elastic lamina, with intima cell hyperplasia resulting in lumen stenosis. The hyperplastic vascular walls were positive for IgG and IgM.Conclusions The etiology of Moyamoya disease may involve allergic angiitis. A possible mechanism is that proximal portions of the circle of Willis first develop chronic stenosis or occlusion, leading to compensatory small vessel proliferation, which perforates into the cerebral parenchyma.展开更多
文摘BACKGROUND Although coronavirus disease 2019(COVID-19)vaccines have been effective in controlling the COVID-19 pandemic,a variety of post-vaccination neurological complications have been reported worldwide.Amyloidβ-related angiitis(ABRA)is a rare neurological disease.The underlying cause of ABRA is unknown,but several studies suggest that it is caused by an excessive immune response to amyloid-βdeposited in blood vessels.In addition,limited attention has been paid to potential triggers of ABRA,such as infection or vaccination.CASE SUMMARY We report a case of ABRA that developed 2 wk after COVID-19 vaccination.A 75-year-old woman developed a frontal headache after receiving a second dose of COVID-19 BNT162b2 vaccine(Pfizer-BioNTech).Diffusion-weighted magnetic resonance imaging(DW-MRI)of the head showed abnormal hyperintensity,suggesting cerebral infarctions in the left parietal and occipital lobes.We diagnosed her condition as ABRA based on a brain biopsy.We administered steroid pulse therapy and the patient’s symptoms and DW-MRI abnormalities improved.This case had a good outcome due to prompt diagnosis and treatment.CONCLUSION We report a case of ABRA that may have been triggered by COVID-19 vaccination.
基金Basic and Applied Basic Research Fund Project of Guangdong Province(2022A1515220161).
文摘Objective:To summarize the clinical features,imaging manifestations,therapeutic options,and prognosis of the primary angiitis of the central nervous system(PACNS)and to explore the role of high-resolution magnetic resonance imaging(HR-MRI)in the PACNS diagnosis and treatment.Methods:One patient with PACNS treated by HR-MRI was retrospectively analyzed and summarized by combining relevant literature.Results:The patient was a young female who was hospitalized with progressive cerebral infarction and multiple intracranial arterial stenosis.HR-MRI indicated vasculitic changes.After excluding other diseases,hormone shock combined with immunosuppression was given,followed by long-term rehabilitation treatment.The patient’s condition tended to stabilize,and the prognosis was satisfactory.Conclusion PACNS is challenging to diagnose and is characterized by poor prognosis and easy recurrence.HR-MRI plays an important role in the clinical diagnosis and treatment adjustment for PACNS.
文摘Frosted branch angiitis(FBA)is a rare retinal vascular disease that usually affects healthy,young adolescents and tends to be bilateral.Cxirrently,there are limited reports on FBA worldwide.In this paper,we will present a case of FBA after fever.
基金The Natural Science Foundation of Shanxi Province,Grant/Award Number:202203021221269The National Natural Science Foundation of China,Grant/Award Number:82001740。
文摘Background:Cutaneous leukocytoclastic angiitis(CLA)is a clinically rele-vant condition,with previous studies suggesting an association with herpes virus infections.However,the causality of this association remains unclear.This study aimed to investigate the causal relationship between herpes viruses and CLA.Methods:Genetic variants linked to the herpes virus were retrieved from the Integrative Epidemiology Unit at the University of Bristol open genome-wide association studies project and FinnGen database.Data on CLA,involving 262 CLA cases and 207,482 healthy controls,were obtained from the FinnGen consortium R7.Mendelian randomization(MR)analysis,including the inverse variance weighted(IVW),MR-Egger,and weighted median methods,was performed.Sensitivity analyzes were conducted to ensure the accuracy of the results.Results:Of the 15 herpes viruses investigated,only human herpes virus 6(HHV-6)demonstrated a causal association with CLA(odds ratio:1.886,95%confidence interval:1.053–3.378,p=0.033),indicating that HHV-6 infection significantly increases the risk of CLA.Furthermore,IVW and MR-Egger tests for heterogeneity confirmed homogeneous MR analysis results without evi-dence of horizontal pleiotropy(p>0.05).No significant causal relationship was observed for other herpes viruses,such as herpes simplex virus,varicella-zoster virus,cytomegalovirus,and Epstein-Barr virus.Conclusion:Our MR analyzes strongly support a causal relationship between HHV-6 and CLA,elucidating the etiology of this condition and highlighting the potential of HHV-6-targeted therapeutic interventions in CLA treatment.However,further research is necessary to expound the underlying mechanisms and explore potential therapeutic interventions targeting HHV-6-associated CLA.
文摘Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-year-old male had a fluctuating disease course for more than 3 years. He presented visual disorders, seizure, cognitive impairment, hypersomnia, unsteady gait, dysphasia, dysphagia, and incontinence. Magnetic resonance imaging showed multiple, supratentorial and infratentorial abnormal signals, while cerebrospinal fluid and cerebral angiography were normal. Magnetic resonance spectrum showed a decrease of N-acetyl-aspartate. Brain biopsy revealed nongranulomatous lymphatic vasculitis with reactive gliosis, cicatrization, demyelination and focal hemorrhages.
文摘Introduction:Primary angiitis of the central nervous system(PACNS)is a vasculitis confined to the CNS.A small proportion of the lesions may present as a tumor-like mass,which is rarely seen in children.Case presentation:A 5-year-old girl was admitted to our hospital because of an intermittent headache.Brain imaging suggested a space-occupying lesion in the right cerebral hemisphere.The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy.Her condition improved after immunotherapy.Conclusion:Pediatricians should consider the possibility of PACNS when encountering intracranial tumor-like lesions.Early diagnosis of tumor-like PACNS and prompt immunotherapy could improve the long-term prognosis and avoid surgery.
基金ThisworkwassupportedbytheJilinProvinceScienceCommitteeFoundation (No 93 3 5 3 2 1)
文摘Objective To investigate the etiology, pathology, and mechanism of pathogenesis of Moyamoya disease. Methods A total of 15 human autopsies were analyzed. In addition, in order to create an animal model of the disease, 21 Japanese rabbits were divided randomly into two groups and subjected to injections of horse serum either intravenously or locally in the area of the sympathetic ganglia. Pathological and immunohistochemical characteristics were observed. Results The pathological features of the autopsies and the animal models both involved intima hyperplasia and stenosis or even occlusion of the lumen in the terminal ends of the internal carotid artery and the anterior and middle cerebral arteries. Disconnections or even breakages of the inner layer of the lumen were also observed, without an obvious inflammatory response. Hyperplasic smooth muscle cells of the medial membrane had extended inward through broken portions of the internal elastic lamina, with intima cell hyperplasia resulting in lumen stenosis. The hyperplastic vascular walls were positive for IgG and IgM.Conclusions The etiology of Moyamoya disease may involve allergic angiitis. A possible mechanism is that proximal portions of the circle of Willis first develop chronic stenosis or occlusion, leading to compensatory small vessel proliferation, which perforates into the cerebral parenchyma.
