全身性進行性硬化症に合併した左後頭葉出血の1例—脳血管撮影，病理学的検討

A Case of Left Occipital Lobe Hemorrhage in a Patient with Progressive Systemic Sclerosis : Evaluation of Cerebral Angiography and Histology

I．はじめに

　全身性進行性硬化症（progressive systemic scle-ross，以下PSS）に中枢神経系の疾患を合併することは稀である．その理由は，脳実質にはPSSの病変の原因となるコラーゲンが身体の他の部分に比べて少ないためとされる^4）．今回，われわれはPSSに合併した左後頭葉出血の症例に対し，CT，MRI，脳血管撮影，組織学的検討を行い考察した．若干の文献的考察を加えて報告する．

Involvement of the central nervous system is uncommon in progressive systemic sclerosis, with only 2 reported cases associated with intracerebral hemorrhage detected by neuroimaging.

A 55-year-old woman with a 10-year history of scleroderma presented with left occipital lobe hemor-rhage manifesting as headache and vomiting. She had no signs of hypertension, diabetes mellitus and hyperlipidemia. CT and MRI, on admission, showed left occipital lobe hemorrhage with ventricular rupture and acute left subdural hematoma. Serial cerebral angiography was performed on day 0, day 7 and day 14, and found no evidence of aneurysm, arteriovenous multiformation or tumor stain in the left occipital lobe. However, the bilateral anterior cerebral arteries showed increasing segmental narrrowing suggestive of vasculitis. Histological examination of a section from the brain cortex adjacent to the hemorrhage revealed no evidence of vasculitis, fibrinoid degeneration or amyloid deposition. Focal vasculitis may have occured secondary to the homorrhagic lesion.