被殻出血を合併したTurner症候群―高アルドステロン血症を呈した1例―

Putaminal Hemorrhage in a Case of Turner's Syndrome with Hyperaldosteronemia

Ⅰ．はじめに

　Turner症候群は性染色体異常を有し，低身長，二次性徴欠如，無月経，翼状頸などの症状を特徴とする疾患である．性染色体異常の基本型は45，Xであるが46，XXとのモザイク型が多いとされる⁶⁾．全身症状のうち血管系では大動脈狭窄症の合併頻度が高く，脳血管障害の報告は稀である．今回われわれは異常な高血圧を伴い被殻出血を合併した若年者Turner症候群の1例を経験したので文献的考察を加え報告する．

A case of Turner's syndrome complicated with intracerebral hemorrhage in a 21-year-old female is presented. The patient experienced acute onset of unconsciousness and the left-sided hemiparesis due to the right-sided putaminal hemorrhage. Severe hypertension was seen at the hospitalization. Cerebral angiography revealed no vascular lesion other than the displacement of lenticulostriate arteries. Emergent surgical evacuation of the hematoma was successfully performed with her favorable outcome. Postoperatively,several hormonal tests indicated hyperaldosteronemia secondary to the increased levels of ACTH and renin as a result of hyper-response to the environmental stress, leading to critical hypertension. Other examinations including CT scan, MRI, MRA, and laboratory data showed no abnormalities suggestive of renal lesions. It is supposed that hypertension, as a frequent presenting feature, should be strictly controlled in Turner's syndrome from the first time of diagnosis.