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題 名 | Simultaneous Bilateral Intracerebral Hemorrhage in a Case of Wegener's Granulomatosis: A Case Report and Literature Review=韋格納肉芽腫病人併雙側同時顱內出血:個案報告及文獻回顧 |
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作 者 | 陳渝仁; 黃浩輝; 陳芝琪; 陳嘉玲; 鍾佳英; | 書刊名 | 臺灣復健醫學雜誌 |
卷 期 | 43:4 2015.12[民104.12] |
頁 次 | 頁263-271 |
分類號 | 415.92 |
關鍵詞 | 韋格納肉芽腫; 多發性顱內出血; 血管炎; 高血壓; Granulomatosis with polyangiitis; Wegener's granulomatosis; Multiple intracerebral hemorrhages; Vasculitis; Hypertension; |
語 文 | 英文(English) |
中文摘要 | 同時顱內多處出血在臨床上罕見且造成的病理機制未明,可能原因包括高血壓、血管炎、腦澱粉樣血管病、靜脈竇血栓、凝血功能異常及顱內血管畸形。韋格納肉芽腫為主要影響呼吸道及腎臟的中小型血管的壞死性肉芽腫性血管炎,文獻指出韋格納肉芽腫會影響到中樞神經系統的發生率為0%到16.4%。本個案報告病患為34歲男性,本身有高血壓但未規律服藥控制。病患的初始表現為突然意識喪失及右側肢體無力,腦部電腦斷層顯示左側基底核出血及右側基底核梗塞併出血性轉化,腦部電腦斷層血管攝影顯示右側中大腦動脈M1分支阻塞及左側中大腦動脈血管型態異常,由於多處顱內血管異常,加上鼻竇炎、顯微性血尿及抗嗜中性白血球細胞質抗體(c-ANCA)陽性,因此病人診斷被懷疑是韋格納肉芽腫。我們推論顱內血管異常源自於韋格納肉芽腫,突發的高血壓造成左側基底核出血及右側基底核梗塞併出血性轉化,血管攝影顯示右側中大腦動脈M1分支阻塞但已經建立側支循環,因此臨床上病人的左側肢體在發病後保有完整的肌力。自發性顱內出血是韋格納肉芽腫少見但嚴重的併發症,造成顱內多發性出血的明確生理機轉未明,由於中樞神經的表現有可能是韋格納肉芽腫症的第一個表現症狀,因此在同時顱內多處出血的情況下,韋格納肉芽腫須列入為臨床上可能的鑑別診斷。 |
英文摘要 | Background: Simultaneous multiple intracerebral hemorrhages (ICHs) are rare and their underlying mechanisms are unknown. Reported etiologies include hypertension, vasculitis, cerebral amyloid angiopathy, venous sinus thrombosis, coagulopathy, and intracranial vascular anomalies. Granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is a necrotizing granulomatous vasculitis affecting small- and medium-sized vessels of the respiratory tract and kidneys. The reported frequency of central nervous system (CNS) involvement in GPA is 0%-16.4%. Case presentation: A 34-year-old man with hypertension presented with a sudden loss of consciousness and weakness in the right limbs. Brain computed tomography (CT) showed a left basal ganglion hemorrhage and right basal ganglion infarction with hemorrhagic transformation. Brain CT angiography revealed stenosis of the M1 segment of the right middle cerebral artery (MCA) and irregularity of the left MCA branches. GPA was suspected due to persistent sinusitis, microscopic hematuria, detection of cytoplasmic antineutrophil cytoplasmic antibodies, and multiple cerebral vessel irregularities. We assume that the underlying vessel abnormalities resulted from GPA. Uncontrolled hypertension induced the left basal ganglion hemorrhage and right basal ganglion infarction with hemorrhagic transformation. It is known that right M1 territory collateral circulation had been established before this cerebrovascular accident because cerebral angiography revealed complete occlusion of the right M1 segment with profound collateral circulation. Therefore, our patient had intact left side muscle strength despite right basal ganglion infarction with hemorrhagic transformation. Conclusion: Spontaneous ICH is a rare but fatal complication of GPA. The mechanism underlying simultaneous multiple ICH remains unknown. Considering CNS manifestations can be the first presenting feature of GPA, GPA should be considered in the differential diagnosis of patients with simultaneous multiple ICHs. |
本系統中英文摘要資訊取自各篇刊載內容。