Odagaki Takao, Noguchi Yoshinori, Fukui Tsuguya
Department of General Medicine, Kyoto University Hospital, Kyoto.
Intern Med. 2003 Aug;42(8):710-3. doi: 10.2169/internalmedicine.42.710.
A 51-year-old woman presented with flexion contractures of the legs. Physical examination showed decreased passive movements of the bilateral hip and knee joints without muscle spasms or neurological abnormalities. Laboratory evaluation showed no response of ACTH or plasma cortisol to stimulation with CRH or insulin. Diagnosis of isolated adrenocorticotropic hormone deficiency was made. The patient was started on prednisolone 5 mg daily, and flexion contractures of the legs rapidly disappeared. Although the musculoskeletal manifestation of this patient is similar to that of stiff-person syndrome, flexion contracture of the legs associated with adrenocortical insufficiency seems to be a separate disease entity from stiff-person syndrome.
一名51岁女性因腿部屈曲挛缩就诊。体格检查显示双侧髋关节和膝关节被动活动度降低，无肌肉痉挛或神经功能异常。实验室检查显示促肾上腺皮质激素（ACTH）或血浆皮质醇对促肾上腺皮质激素释放激素（CRH）或胰岛素刺激无反应。诊断为孤立性促肾上腺皮质激素缺乏症。患者开始每日服用5毫克泼尼松龙，腿部屈曲挛缩迅速消失。尽管该患者的肌肉骨骼表现与僵人综合征相似，但与肾上腺皮质功能不全相关的腿部屈曲挛缩似乎是一种与僵人综合征不同的疾病实体。
