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症例は33歳女性,全身性エリテマトーデス(SLE)に対しプレドニゾロン内服にて通院加療中であった。経過中,頭痛をきたし,MRIにて大脳鎌,小脳テントの硬膜肥厚および上矢状洞にempty delta signを認めたため,上矢状洞の血栓症が疑われ入院となった。入院時の血液検査では軽度の炎症反応は認めたが,その他明らかな異常所見を認めず,抗カルジオリピン抗体(aCL),ループスアンチコアグラント(LA)は陰性であった。脳血管撮影では上矢状静脈洞後半部が閉塞していたが,十分な側副血行路が形成されていたため頭蓋内圧亢進所見を認めず,保存的治療の後,自宅退院となった。画像上,肥厚性硬膜炎を伴った慢性的な炎症により静脈洞血栓症をきたし,上矢状洞が閉塞したものと思われた。
A 33-year-old female who had been on a steroid treatment for the past 14 years due to systemic lupus erythematosus (SLE) visited our hospital complaining of mild headache. No neurological deficit and no positive serologic tests for lupus anticoagulants (LAC) and anticardiolipin antibodies (aCL) were noted. Only a mild inflammatory change was observed on routine hematological examination.
On neuroradiological examination, MRI revealed thickened falx cerebri and tentorium cerebelli, and an empty delta sign. These findings were suggestive of sinus thrombosis of superior sagittal sinus (SSS). Angiograms clearly demonstrated occlusion of the posterior part of superior sagittal sinus and transeverse sinus (TS). Conservative treatment was chosen because of no evidence of intracranial hypertension. There was no deterioration in her general and neurological status during her hospital stay and she was discharged. Longstanding vasculitis and pachymeningitis related to lupus erythematosus might be the probable cause of the sinus thrombosis in this case.
(Received : November 1, 2004)
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