下垂体前葉機能低下症および橋本病の合併により初発症状が修飾された大脳皮質基底核症候群の1例

A Case of Corticobasal Syndrome Complicated with Hypopituitarism and Hashimoto's Disease

症例は71歳の女性で，認知機能障害およびパーキンソニズムを認め受診した。これらは同時に発見された下垂体前葉機能低下症および橋本病に起因するものと考えられ，ホルモン補充療法により一部の認知機能障害は改善した。当初は大脳皮質基底核症候群（CBS）を考慮されていなかったが，認知機能障害およびパーキンソニズムが残存し，左優位の大脳皮質症状も明らかとなった。MRI，脳血流シンチグラフィーおよびPET検査を踏まえ，CBS-CBDと診断した。下垂体前葉機能低下症および橋本病といった内分泌疾患が，背景にある神経変性疾患の症候を修飾することがあり，慎重な臨床経過観察の重要性が示唆された。

Abstract

We report the case of an individual with corticobasal syndrome (CBS), hypopituitarism due to a post-traumatic leptomeningeal cyst, and Hashimoto's disease. A 71-year-old woman was admitted to our hospital because of cognitive dysfunction and bradykinesia. Following a primary diagnosis of hypopituitarism and hypothyroidism, she was given hormone replacement therapy, and her clinical symptoms appeared to improve. However, some cognitive impairment and extrapyramidal symptoms remained. The results of careful neurological examinations, as well as magnetic resonance, single-photon emission computed tomography, and positron emission tomography images, suggested a diagnosis of CBS-CBD (corticobasal degeneration). Because parkinsonism and cognitive impairment can be caused by endocrinopathy, it was initially difficult to reach the complete diagnosis that included CBS. Thus, it is important to understand that complicated neurological presentations can be caused by several different disorders.

(Received September 4, 2014; Accepted October 15, 2014; Published June 1, 2015)