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与肾上腺性征异常症相关的柯萨科夫综合征

KORSAKOFF'S SYNDROME ASSOCIATED WITH ADRENAL VIRILISM.

作者信息

COOKSON B A

出版信息

Can Med Assoc J. 1964 Jun 27;90(26):1449-53.

Abstract

Korsakoff's syndrome of obscure etiology was observed in a 34-year-old single woman with an 11-year history of hirsutism and mood swings, and previous hospitalizations for mania three years ago and depression 11 years ago.Recently the virilism had intensified with increased muscularity and coarsening of facial features. The 24-hour urinary 17-ketosteroids ranged between 14.4 mg. and 21.5 mg. and were suppressed by dexamethasone. The 17-hydroxycorticosteroid excretion was normal. These and other findings suggested a diagnosis of adrenal virilism due to adrenocortical hyperplasia. In the absence of other discernible causes it appeared that the adrenal pathology was responsible for the Korsakoff's syndrome. Both conditions responded well to glucocorticoid therapy although low doses were necessary to avoid mania.It is speculated that the encephalopathy was due to an associated adrenal insufficiency. Although hypoadrenalism is accepted as a complication of only the infant form of adrenal virilism, it is noteworthy that this patient had pathological pigmentation of her skin.

摘要

一名34岁单身女性患有病因不明的科萨科夫综合征,她有11年多毛症和情绪波动病史,3年前曾因躁狂症住院,11年前曾因抑郁症住院。最近,男性化症状加重,肌肉更加发达,面部特征变粗。24小时尿17-酮类固醇含量在14.4毫克至21.5毫克之间,且地塞米松可抑制其分泌。17-羟皮质类固醇排泄正常。这些及其他检查结果提示诊断为肾上腺皮质增生所致的肾上腺性征异常症。在没有其他明显病因的情况下,肾上腺病变似乎是导致科萨科夫综合征的原因。两种病症对糖皮质激素治疗反应良好,不过需要低剂量用药以避免躁狂发作。据推测,脑病是由相关的肾上腺功能不全所致。虽然肾上腺功能减退仅被认为是婴儿型肾上腺性征异常症的一种并发症,但值得注意的是,该患者皮肤有病理色素沉着。

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