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Brain and Nerve No to Shinkei Volume 45, Issue 8 （August 1993）

Brain and Nerve 脳と神経 45巻8号（1993年8月発行）

Japanese English

症例報告

副腎皮質ステロイドに反応する脳浮腫・反復性意識障害を呈しACTH単独欠損症と考えられた1例 A Case of Isolated Adrenocorticotropic Hormone Deficiency with Adrenocorticosteroid-responsive Brain Edema and Repeated Confusions 根来清 ¹ , 森松光紀 ¹ Kiyoshi Negoro ¹ , Mitsunori Morimatsu ¹ ¹山口大学医学部神経内科 ¹Department of Neurology, Yamaguchi University School of Medicine キーワード： adrenocorticosteroid , brain edema , repeated confusional state , isolated adrenocorticotropic hormone（ACTH）deficiency , white matter lesion Keyword: adrenocorticosteroid , brain edema , repeated confusional state , isolated adrenocorticotropic hormone（ACTH）deficiency , white matter lesion pp.727-731

発行日 1993年8月1日 Published Date 1993/8/1

DOI https://doi.org/10.11477/mf.1406900518

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　60歳，男性。著明な脳波異常，脳浮腫，意識障害を呈したが副腎皮質ステロイド投与が劇的に有効であった。副腎皮質ステロイド減量で容易に意識障害が誘発され，再投与で意識障害は速やかに消失した。内分泌学的検査・副腎皮質ステロイドに対する反応性などからACTH単独欠損症と考えられた。脳浮腫は副腎皮質ステロイド投与で消失し，脳浮腫消失後のMRIでは大脳白質病変を認めた。

A 60-year-old man developed confusional statewith severely abnormal electroencephalogram and brain edema on computed tomography (CT) of brain. Adrenocorticosteroid therapy was dramati-cally effective for the confusional state and brain edema. The reduction of adrenocorticosteroid in-duced confusional states, and they disappeared with the increase of adrenocorticosteroid. When brain edema on CT disappeared, T 2-weighted magnetic resonance imagings revealed diffuse high signal inthe white matter. A diagnosis of isolated adrenocor-ticotropic hormone deficiency was made by endo-crinological examinations and clinical features.

We suggest that brain edema in this patient is due to defficiency of glucocorticoid. High intensity area in the white matter may show the degeneration of the white matter following brain edema.