皮質静脈血栓によるくも膜下出血と延髄内側梗塞を合併したSLEの１例

Systemic Lupus Erythematosus Manifesting as Subarachnoid Hemorrhage Induced by Cortical Venous Thrombosis and Followed by Medial Medullary Infarction

要旨　皮質静脈血栓によるくも膜下出血と，その急性期加療中に延髄内側梗塞を合併した全身性エリテマトーデス（SLE）症例を報告した。患者は43歳女性でくも膜下出血にて発症し，脳血管撮影では動脈瘤や動静脈奇形は認めず，出血源として皮質静脈血栓が強く疑われた。保存的療法を開始したが，発症6日目に延髄内側梗塞に伴う左片麻痺を併発した。当初は，出血発症例のため積極的な抗血小板療法や抗凝固療法は躊躇したが，経過中に延髄梗塞を合併したため，まず抗血小板療法から開始し，その後，段階的に抗凝固療法へ切り換えた。計測した抗カルジオリピン-b2 glycoprotein I抗体，lupus anticoagulantが陰性のため，抗リン脂質抗体症候群（APS）の確定診断は得られなかった。しかし，APSでは多数の抗リン脂質抗体の関与が示唆されており，病態的にはAPSに伴う脳血管障害であった可能性が強く示唆された。

Abstract

A 43-year-old female, who had been treated for systemic lupus erythematosus（SLE）, presented with a subarachnoid hemorrhage（SAH）induced by rupture of cortical venous thrombosis, and be followed by medial medullary infarction during the acute stage of the SAH. The patient initially manifested a SAH. Angiography demonstrated no evidence of any aneurysms or arteriovenous malformations, but revealed cortical venous thrombosis. She suddenly developed left hemiplegia caused by medial medullary infarction on the 6th day. An active anticoagulant therapy was thought to be inappropriate because of initial symptoms as a hemorrhage. Since she had been accompanied by the medullary infarction, then, initially started by antiplatelet therapy. After the confirmation of no saccular or dissecting aneurysms with 2nd angiography, her treatment could be changed to anticoagulant therapy. Because of the sustained negative reactions of anti-cardiolipin b2 glycoprotein I antibody and lupus anticoagulant during the course of SLE, the definite diagnosis of antiphospholipid syndrome（APS）could not be made. However, this case is pathogenically thought to be cerebrovascular disease based upon APS, considering that this syndrome may be related to various antigen/antibody systems.