1. A case of Neuropsychiatric systemic lupus erythematosus with varying stenotic lesions in several cerebral major arteries and a vertebral artery aneurysm
- Author
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SUGIYAMA, Seiji, KITAMURA, Akihiro, KANEKO, Shunya, YAMAKAWA, Isamu, SANADA, Mitsuru, and URUSHITANI, Makoto
- Subjects
multiple vasospasm ,cerebral aneurysm ,脳動脈瘤 ,NPSLE ,多発脳血管攣縮 ,RCVS - Abstract
症例は42 歳女性,約20 年前に全身性エリテマトーデス(systemic lupus erythematosus,以下SLE と略記)と診断された.ステロイド誘発性精神障害のためステロイド漸減中に錯乱状態を主とする神経精神SLE(neuropsychiatric SLE,以下NPSLE と略記)を発症し,MRI では右側頭葉皮質を中心に急性期梗塞像,複数の脳主幹動脈で亜急性に変動する狭窄と拡張,右椎骨動脈瘤形成を認めた.シクロホスファミド静注療法等を早期から導入し動脈瘤は拡大なく血管狭窄は改善した.NPSLE における血管攣縮と拡張,動脈瘤形成は稀であるが,疾患活動性の指標となり,強固な免疫治療を検討する必要性があると考え報告した., A 42 years old female suffered from systemic lupus erythematosus (SLE) about 20 years ago. While steroid was tapered for a steroid-induced psychiatric disorder, she presented with an acute confusional state and was diagnosed with neuropsychiatric SLE (NPSLE). MRI showed acute infarction mainly in the cortex of the right temporal lobe and MRA demonstrated dynamic subacute morphological changes such as stenosis and dilation in several major intracrainal arteries. The right vertebral artery diffusely dilated and subsequently formed an aneurysm in a week. Contrast-enhanced MRI vessel-wall imaging showed a remarkable enhancement of the aneurysm wall, which might indicate an unstable unruptured aneurysm. The prompt introduction of intravenous cyclophosphamide improved both clinical and radiological signs. Our case indicates that intensive immunosuppressive treatments should be considered in NPSLE patients with varying vasospasm and aneurysm, indicating exacerbated disease activity.
- Published
- 2023