多発性皮質下出血を合併したSLEの1例

A Case of Systemic Lupus Erythematosus Complicated with Multiple Subcortical Hemorrhage

Ⅰ．はじめに

　全身性エリテマトーデス（systemic lupus erythematosus以下，SLE）には多彩な中枢神経障害の合併が報告されている．なかでも脳血管障害は，多数の臨床および病理学的所見の検討結果から，SLEの臨床経過に大きく影響することがわかった^1,9,12)．特に，頭蓋内出血性病変を有するSLE患者においては，外科的治療の予後に果たす役割は多大である．一方，剖検における脳病変の検索では脳内に大小の出血巣が頻繁に認められるものの，生前に脳内出血で発症したとするSLE症例は比較的稀である^1,2,6,7)．最近，われわれは同時期に多発する皮質下出血を合併したSLEの1例に対して手術治療を行い良好な予後を得た．この特異な臨床像を通して，SLEでの脳内出血の発生機序につき，文献的考察を加え報告する．

We described a case of systemic lupus erythematosus (SLE) complicated with multiple intracerebral hemorrhage. A 52-year-old female with no history of hypertension had been treated with steroid hormone therapy for more than 15 years. Four days before admission,she experienced acute onset of headache and nausea. On the day of admission,the patient suffered from headache and nausea again followed by a deteriorated level of consciousness. The computed tomographic scan revealed two subcortical hematomas in the right frontal and left temporo-occipital area with atypical findings compared to those of the usual intracerebral hemorrhage. Cerebral angiography demonstrated that cortical arteries around the hematomas were markedly stretched and displaced. Multiple segmental stenosis and irregular lumen of the internal carotid artery and bilateral cortical arteries were possibly representative of vasculopathy. Transit time of blood flow was severely delayed with no apparent obstruction of the cerebral arteries,veins and dural sinuses. These findings suggested the existence of severely increased intracranial pressure. Emergent surgical evacuation of the hematomas was successfully performed with a favorable outcome for the patient. Intraoperatively,the brain was congested even after the evacuation of the hematomas. Surgical manipulation was able easily to cause bleeding from the cavity of the hematomas,which was hard to control. It is supposed that vasculopathy including fibrinoid degeneration and vasculitis may have affected the susceptibility of small arteries and veins to rupture in the present case. Subsequent increase of intracranial pressure might cause another hematomas by adding of hemodynamic stress to small vessels apart from those at the site of the first hemorrhage.