Division of Neurology, Department of Medicine, Jichi Medical University, Yakushiji 3311-1, Shimotsuke, Tochigi, 329-0498, Japan.
Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Yakushiji 3311-1, Shimotsuke, Tochigi, 329-0498, Japan.
J Thromb Thrombolysis. 2020 May;49(4):681-684. doi: 10.1007/s11239-020-02109-4.
Cerebral amyloid angiopathy-related inflammation is a syndrome of reversible encephalopathy with cerebral amyloid angiopathy, however the pathology is not well understood. We clear a part of the pathology through the first case of an 80-year-old man with cerebral amyloid angiopathy-related inflammation induced by relapsing polychondritis (RP) analysis. An 80-year-old man was diagnosed with RP by auricular cartilage biopsy. Almost no abnormality including intracranial microbleeding was detected by cranial magnetic resonance image (MRI) at diagnosis. However, he developed a headache and hallucination after five months. Seven-month cranial MRI showed novel, multiple, intracranial microbleeding, especially in the bilateral but asymmetry posterior, temporal, and parietal lobes. I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography showed increased cerebral blood flow in the bilateral posterior lobes. After treatment, both of his neurological symptoms and increased cerebral blood flow improved to mild. Photon emission computed tomography using Pittsburgh compound B (PiB) for evaluation of brain amyloidosis at 12 months after onset showed an amyloid deposit in the bilateral frontal lobes, but a lack of uptake corresponded to the RP lesions. Our case suggests that inflammation coupled with an amyloid deposit, induced the multiple intracranial bleeding, and resulted in the lack of PiB uptake. Findings from our case show that inflammation including excess blood flow coupled with an amyloid deposit synergistically facilitate intracranial bleeding.
脑淀粉样血管病相关炎症是可逆性脑淀粉样血管病伴发的脑病综合征,但病理机制尚不清楚。我们通过对一例由复发性多软骨炎(RP)引起的脑淀粉样血管病相关炎症的 80 岁男性患者的分析,部分阐明了其病理机制。该 80 岁男性患者经耳廓软骨活检诊断为 RP。在诊断时,头颅磁共振成像(MRI)几乎未发现任何异常,包括颅内微出血。然而,他在 5 个月后出现头痛和幻觉。7 个月时的颅脑 MRI 显示新出现的多发性颅内微出血,特别是在双侧但不对称的后、颞和顶叶。I-N-异丙基-p-碘安非他命单光子发射计算机断层扫描显示双侧后叶脑血流增加。经治疗,他的神经症状和脑血流增加均改善至轻度。发病 12 个月后,使用匹兹堡化合物 B(PiB)进行脑淀粉样蛋白评估的正电子发射断层扫描显示双侧额叶有淀粉样沉积,但与 RP 病变相对应的部位无摄取。我们的病例提示炎症与淀粉样沉积共同导致多发颅内出血,导致 PiB 摄取减少。我们的病例结果表明,包括血流增加在内的炎症与淀粉样沉积共同促进了颅内出血的发生。