Meng Yao, Xiao Yining, Sun Ruohan, Li Lingyu, Dong Yanhong
Department of Neurology, Hebei General Hospital, Shijiazhuang, China.
Department of Neurology, Graduate School of Hebei North University, Zhangjiakou, China.
Medicine (Baltimore). 2024 Dec 20;103(51):e41010. doi: 10.1097/MD.0000000000041010.
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a treatable condition characterized by an acute or subacute onset, with its primary pathological hallmark being the deposition of amyloid, predominantly β-amyloid (Aβ), within intracranial microvessels. Despite its potential for treatment, CAA-ri is a rare disorder that is frequently underrecognized by clinicians in practice. This article provides a comprehensive overview of the clinical manifestations and therapeutic approaches associated with CAA-ri, aiming to enhance awareness among healthcare professionals.
A 67-year-old male patient who suffered from a sudden decline in cognitive functioning, intermittent headache, and dysphoria underwent brain magnetic resonance imaging, susceptibility weighted imaging, and cerebrospinal fluid analysis and was considered probable CAA-ri.
During the course of disease development, the patient suffered from a sudden decline in cognitive functioning, mainly in the form of unresponsiveness, decreased comprehension, and increased repetitive language, accompanied by intermittent headaches and dysphoria. Brain magnetic resonance imaging showed numerous white matter in both hemispheres. Susceptibility weighted imaging showed multiple spots of hypointensity in the bilateral cerebral and cerebellar hemispheres, a hypointensity signal in the left occipital lobe, and extensive zones of hypointensity in bilateral sulci. Cerebrospinal fluid analysis was abnormal with elevated levels of protein and low levels of P-tau, Aβ42, and Aβ1-42/Aβ1-40. The use of glucocorticoids greatly reduced his symptoms. This lends credence to the probable CAA-ri diagnosis. The symptoms can be successfully alleviated by administering methylprednisolone sodium succinate.
During the patient's hospitalization, immunosuppressive therapy, primarily consisting of methylprednisolone sodium succinate and methylprednisolone, was administered, resulting in a significant improvement in symptoms. Post-discharge, the patient was monitored regularly, revealing a gradual enhancement in cognitive function without recurrence. Consequently, immunosuppressive therapy was discontinued 1 year following the patient's discharge.
CAA-ri is a rare clinical condition, and timely diagnosis and early treatment are very critical for patient prognosis.
脑淀粉样血管病相关性炎症（CAA-ri）是一种可治疗的疾病，其特点是急性或亚急性起病，主要病理特征是淀粉样蛋白，主要是β-淀粉样蛋白（Aβ），在颅内微血管内沉积。尽管有治疗的可能性，但CAA-ri是一种罕见疾病，在临床实践中常未被临床医生充分认识。本文全面概述了与CAA-ri相关的临床表现和治疗方法，旨在提高医护人员的认识。
一名67岁男性患者，出现认知功能突然下降、间歇性头痛和烦躁不安，接受了脑磁共振成像、磁敏感加权成像和脑脊液分析，被认为可能患有CAA-ri。
在疾病发展过程中，患者认知功能突然下降，主要表现为反应迟钝、理解能力下降和重复语言增加，伴有间歇性头痛和烦躁不安。脑磁共振成像显示双侧大脑半球有大量白质。磁敏感加权成像显示双侧大脑半球和小脑半球有多个低信号点，左侧枕叶有一个低信号，双侧脑沟有广泛的低信号区。脑脊液分析异常，蛋白质水平升高，P-tau、Aβ42和Aβ1-42/Aβ1-40水平降低。使用糖皮质激素后他的症状大大减轻。这支持了可能的CAA-ri诊断。通过给予琥珀酸甲泼尼龙可成功缓解症状。
患者住院期间，主要使用琥珀酸甲泼尼龙和甲泼尼龙进行免疫抑制治疗，症状有显著改善。出院后，对患者进行定期监测，显示认知功能逐渐增强且无复发。因此，在患者出院1年后停止免疫抑制治疗。
CAA-ri是一种罕见的临床疾病，及时诊断和早期治疗对患者预后非常关键。
