Kato H, Shirahama M, Ohmori K, Sunaga T
Department of Internal Medicine, Saga Medical School, Japan.
Angiology. 1995 Feb;46(2):169-73. doi: 10.1177/000331979504600212.
Protein C deficiency is a cause of thromboembolic disease. Venous thrombosis is the most common clinical manifestation. Arterial thrombosis is unusual and involvement of the intracranial arteries is especially rare. Herein the authors describe a case of cerebral [correction of cerebellar] infarction associated with protein C deficiency and review the relevant medical literature. A thirty-year-old man was hospitalized because of dysarthria, right limb ataxia, and a gait disturbance. Cranial computed tomography disclosed an infarction in the right cerebellar hemisphere and brachium pontis. Three months earlier the patient had had a transient ischemic attack with truncal ataxia and gait disturbances. On admission, the protein C antigen was 57% and protein C activity was 45%. Investigation of family members revealed protein C deficiency in an uncle. Literature review of stroke cases associated with protein C deficiency revealed that most had had a previous vascular event and/or a positive family history or had used oral contraceptives chronically. Protein C deficiency should be considered in young stroke patients with a positive family history of vaso-occlusive disease, previous ischemic events, or chronic oral contraceptive use.
蛋白C缺乏是血栓栓塞性疾病的一个病因。静脉血栓形成是最常见的临床表现。动脉血栓形成较为罕见，颅内动脉受累尤其罕见。在此，作者描述了一例与蛋白C缺乏相关的脑梗死病例，并回顾了相关医学文献。一名30岁男性因构音障碍、右肢共济失调和步态障碍入院。头颅计算机断层扫描显示右侧小脑半球和脑桥臂梗死。3个月前，该患者曾有过一次短暂性脑缺血发作，伴有躯干共济失调和步态障碍。入院时，蛋白C抗原为57%，蛋白C活性为45%。对家庭成员的调查发现一名叔叔存在蛋白C缺乏。对与蛋白C缺乏相关的中风病例的文献回顾显示，大多数患者既往有血管事件和/或家族史阳性，或长期使用口服避孕药。对于有血管闭塞性疾病家族史阳性、既往有缺血事件或长期使用口服避孕药的年轻中风患者，应考虑蛋白C缺乏。
