E. P. Nuzhnyi, L. R. Zaripova, E. J. Fedotova, R. N. Konovalov, A. S. Filatov, A. N. Moskalenko, S. Illarioshkin
{"title":"脑膜下浅表血肿症","authors":"E. P. Nuzhnyi, L. R. Zaripova, E. J. Fedotova, R. N. Konovalov, A. S. Filatov, A. N. Moskalenko, S. Illarioshkin","doi":"10.30629/2658-7947-2024-29-3-58-65","DOIUrl":null,"url":null,"abstract":" Introduction. Superficial hemosiderosis of the central nervous system is a chronic, progressive disease caused by continuous low-volume bleeding into the subarachnoid space and, as a result, subpial deposition of hemoglobin de-tritus. Infratentorial superfi cial hemosiderosis (ISS) is characterized by hemosiderin accumulation primarily on the surface of the cerebellum, brainstem and spinal cord. Early detection of ISS may prevent severe disability and raise the chance of successful medical treatment. The purpose of this study was to analyze clinical, radiological, instrumental, and laboratory findings, based on a series of ISS cases diagnosed at the Research Center of Neurology. Material and methods. Seven patients diagnosed with ISS were included in this study (4 men, 3 women). Evaluation of the clinical findings, disease history, brain and spinal cord MRI, MR-angiography, brainstem auditory evoked potentials, audiometry, abdominal ultrasound, and laboratory iron metabolism tests was performed. Results. The median age of subjects was 63 years, and the median duration of disease was 3 years. We identified the cause of ISS in four patients. The most common clinical symptoms were cerebellar ataxia and sensorineural hearing loss. All patients had a typical ISS pattern on brain MRI images. Conclusion. With the development of neuroimaging, iSS is becoming a more frequent finding. Clinicians should be aware of its causes and optimal management strategy. Further studies of possible iSS treatments are needed to reduce free iron neurotoxicity and minimize clinical manifestations of the disease.","PeriodicalId":36724,"journal":{"name":"Russian Neurological Journal","volume":"17 2","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Infratentorial superficial hemosiderosis\",\"authors\":\"E. P. Nuzhnyi, L. R. Zaripova, E. J. Fedotova, R. N. Konovalov, A. S. Filatov, A. N. Moskalenko, S. Illarioshkin\",\"doi\":\"10.30629/2658-7947-2024-29-3-58-65\",\"DOIUrl\":null,\"url\":null,\"abstract\":\" Introduction. Superficial hemosiderosis of the central nervous system is a chronic, progressive disease caused by continuous low-volume bleeding into the subarachnoid space and, as a result, subpial deposition of hemoglobin de-tritus. Infratentorial superfi cial hemosiderosis (ISS) is characterized by hemosiderin accumulation primarily on the surface of the cerebellum, brainstem and spinal cord. Early detection of ISS may prevent severe disability and raise the chance of successful medical treatment. The purpose of this study was to analyze clinical, radiological, instrumental, and laboratory findings, based on a series of ISS cases diagnosed at the Research Center of Neurology. Material and methods. Seven patients diagnosed with ISS were included in this study (4 men, 3 women). Evaluation of the clinical findings, disease history, brain and spinal cord MRI, MR-angiography, brainstem auditory evoked potentials, audiometry, abdominal ultrasound, and laboratory iron metabolism tests was performed. Results. The median age of subjects was 63 years, and the median duration of disease was 3 years. We identified the cause of ISS in four patients. The most common clinical symptoms were cerebellar ataxia and sensorineural hearing loss. All patients had a typical ISS pattern on brain MRI images. Conclusion. With the development of neuroimaging, iSS is becoming a more frequent finding. Clinicians should be aware of its causes and optimal management strategy. Further studies of possible iSS treatments are needed to reduce free iron neurotoxicity and minimize clinical manifestations of the disease.\",\"PeriodicalId\":36724,\"journal\":{\"name\":\"Russian Neurological Journal\",\"volume\":\"17 2\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-24\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Russian Neurological Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.30629/2658-7947-2024-29-3-58-65\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Russian Neurological Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.30629/2658-7947-2024-29-3-58-65","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
简介中枢神经系统浅表性血色素沉着病是一种慢性、进行性疾病,是由于蛛网膜下腔持续低量出血,导致血红蛋白脱睾酮在皮下沉积而引起的。脑底超级血红蛋白沉着症(ISS)的特点是血红蛋白主要积聚在小脑、脑干和脊髓表面。早期发现 ISS 可预防严重残疾,并提高成功治疗的几率。 本研究的目的是根据神经病学研究中心诊断出的一系列 ISS 病例,对临床、放射学、仪器和实验室结果进行分析。 材料和方法本研究共纳入 7 名确诊为 ISS 的患者(4 男 3 女)。研究人员对患者的临床表现、病史、脑和脊髓磁共振成像、磁共振血管造影、脑干听觉诱发电位、听力测定、腹部超声波以及实验室铁代谢测试进行了评估。 结果显示受试者的中位年龄为 63 岁,中位病程为 3 年。我们确定了四名患者的 ISS 病因。最常见的临床症状是小脑共济失调和感音神经性听力损失。所有患者的脑部核磁共振成像均显示出典型的 ISS 模式。 结论随着神经影像学的发展,ISS越来越常见。临床医生应了解其病因和最佳治疗策略。需要进一步研究可能的 iSS 治疗方法,以减少游离铁的神经毒性,并尽量减轻该病的临床表现。
Introduction. Superficial hemosiderosis of the central nervous system is a chronic, progressive disease caused by continuous low-volume bleeding into the subarachnoid space and, as a result, subpial deposition of hemoglobin de-tritus. Infratentorial superfi cial hemosiderosis (ISS) is characterized by hemosiderin accumulation primarily on the surface of the cerebellum, brainstem and spinal cord. Early detection of ISS may prevent severe disability and raise the chance of successful medical treatment. The purpose of this study was to analyze clinical, radiological, instrumental, and laboratory findings, based on a series of ISS cases diagnosed at the Research Center of Neurology. Material and methods. Seven patients diagnosed with ISS were included in this study (4 men, 3 women). Evaluation of the clinical findings, disease history, brain and spinal cord MRI, MR-angiography, brainstem auditory evoked potentials, audiometry, abdominal ultrasound, and laboratory iron metabolism tests was performed. Results. The median age of subjects was 63 years, and the median duration of disease was 3 years. We identified the cause of ISS in four patients. The most common clinical symptoms were cerebellar ataxia and sensorineural hearing loss. All patients had a typical ISS pattern on brain MRI images. Conclusion. With the development of neuroimaging, iSS is becoming a more frequent finding. Clinicians should be aware of its causes and optimal management strategy. Further studies of possible iSS treatments are needed to reduce free iron neurotoxicity and minimize clinical manifestations of the disease.