遺伝性出血性毛細血管拡張症に脳静脈血栓症を合併した1例

A case of hereditary hemorrhagic telangiectasia complicated by cerebral venous thrombosis

遺伝性出血性毛細血管拡張症（hereditary hemorrhagic telangiectasia：HHT）は，全身に多発血管奇形を生じる常染色体優性遺伝疾患である。皮膚・粘膜に特徴的な毛細血管拡張がみられるほか，肺・脳・肝・消化管・脊髄などに動静脈奇形が多発し，出血を伴うことがある^1-4）。脳においては肺動静脈奇形に起因する梗塞や膿瘍の合併が有名であるが，脳静脈血栓症の合併は非常にまれである。今回HHTに脳静脈血栓症を合併し，興味深い画像所見を呈した症例を経験したため，文献的検討を加えて報告する。

The patient was a female in her 50 s. She had been diagnosed as hereditary hemorrhagic telangiectasia（HHT）. She was admitted to our hospital because she started to have disturbance of consciousness in the morning. Based on CT and MRI findings, straight sinus thrombus and associated venous infarction were suspected. Angiography with catheter showed thrombus. We report this case because cerebral venous sinus thrombosis associated with HHT are extremely rare.