プロテインS欠損症による上矢状洞—静脈洞交会部巨大血栓症の1例

Cerebral sinus thrombosis in a patient with protein S deficiency:a case report

I．はじめに

　一般に静脈洞血栓症の原因としては感染，脱水，妊娠，腫瘍，外傷，経口避妊薬服用等が考えられる^1）．血液凝固系の異常であるプロテインS欠損症による静脈洞血栓症は稀で，1987年にEngesserら^8）により報告されて以来，現在までに17例^{5,7-9,11-13,15-21）}の報告が認められる．われわれの渉猟し得る限りでは，本邦での詳述報告例は未だ認められない．今回われわれは，急激な頭痛で発症した上矢状静脈洞，静脈洞交会部（torcular herophili），横静脈洞，直静脈洞にまたがる巨大血栓症を経験し，その原因として血液凝固抑制機能の異常を呈するプロテインS欠損症と診断したので，文献的考察を加えて報告する．

Cerebral sinus thrombosis associated with protein S deficiency is rare to the best of our knowledge. We re-port here a 22-year-old female who presented sudden onset of headache, vomiting and disturbance of con-sciousness. Neuroradiological studies including com-puted tomography scan, magnetic resonance imaging and cerebral angiography disclosed a huge cerebral sinus thrombosis in the territory of the superior sagittal sinus, torcular herophili, lateral sinus and straight sinus. Hematological studies confirmed the diagnosis of pro-tein S deficiency. We summarize the reported cases of cerebral sinus thrombosis associated with protein S de-ficiency. For young patients presenting occlusive cere-brovascular disease, we stressed the importance of doing extensive hematological investigation to detect possible etiological factors, such as protein S deficiency, protein C deficiency, antithrombin Ⅲ deficiency. Once we discover the etiology of a disease, we may be able to designate the precise treatment or regimen for each patient.