- 期刊
Congenital Afibrinogenemia with Cerebellar and Spinal Cord Infarction Caused by Vertebral Artery Dissection: A Case Report
先天性纖維蛋白原缺乏症合併脊椎動脈剝離導致小腦與脊髓中風：一病例報告
摘要
Background: Congenital afibrinogenemia is a rare fibrinogen disorder. The typical manifestation is bleeding tendency, including mucosal, musculoskeletal, and even intracranial bleeding. Nevertheless, thromboembolic events could still occur. Here we report a case of congenital afibrinogenemia, who presented with concomitant cerebellar and spinal cord infarction related to vertebral artery dissection. Case Report: A 35-year-old woman with congenital afibrinogenemia experienced acute-onset vertigo, left shoulder pain and subsequent left hemiparesis within one week. Initial brain imaging showed left vertebral artery dissection and infarction of left cerebellum and cervical cord. Aspirin was administered initially but was later changed to low-molecular-weight heparin for 3 days because of clinical deterioration. Spinal cord magnetic resonance imaging (MRI) after one week showed hemorrhagic transformation within the infarct area so all antithrombotic agents were withdrawn. Upon discharge, she was able to carry out most of the activities of daily living. Conclusion: This case highlights the importance of recognizing potential thromboembolic events in patients with afibrinogenemia, and vertebral artery dissection related spinal cord infarction may be a unique feature.
並列摘要
背景：先天性纖維蛋白原缺乏症乃一罕見疾病，由於缺乏凝血蛋白而會導致容易有黏膜、腸胃道甚至腦出血的易流血體質。然而，特殊情況下易可能發生栓塞現象。病例報告：一位已知患有先天性纖維蛋白原缺乏症的35歲女性，產生突發性之暈眩及左肩疼痛，並在一週內出現左側肢體無力。腦部核磁共振顯示有左側小腦以及高位頸脊髓梗塞，且合併有左側脊椎動脈剝離。病患剛入院時被投予阿斯匹靈，但由於症狀持續惡化而改用皮下注射之抗凝血劑。一週後的脊髓核磁共振造影顯示頸脊髓梗塞處合併有出血性變化，因此所有抗血栓藥物均停止使用。病患經復健後恢復良好，於出院時已可自行料理日常生活。結論：此病例報告強調先天性纖維蛋白原缺乏症這種易出血體質仍可能會發生罕見的栓塞事件，經文獻回顧後發現，脊髓動脈剝離可能是此疾病產生栓塞之特別現象。