Abstract：
BACKGROUND: Cushing\'s syndrome consists of signs and symptoms related to prolonged exposure to high levels of glucocorticoid, and should be considered in individuals with the discriminatory signs and symptoms. Proximal myopathy is an important discriminatory sign.
METHODS: We report the case of a 36-year-old Iranian man who presented with proximal muscle weakness. He visited a rheumatologist in an outpatient clinic, and according to proximal muscle weakness and heliotrope rash (based on the rheumatologist\'s notes) with the impression of dermatomyositis, prednisolone and azathioprine were prescribed for him that did not improve his clinical status and he was gradually wheelchair dependent. He was admitted to the hospital for evaluation of paraneoplastic syndromes. Standard laboratory tests and imaging were unremarkable, other than a brain magnetic resonance imaging that demonstrated a 30 × 12 mm homogeneously enhancing mass in the sellar region with extension to the suprasellar area. He had serum cortisol of 295 ng/mL, and adrenocorticotropic hormone of 222 pg/mL (on 5 mg prednisolone twice daily), with a diagnosis of Cushing\'s disease. He underwent two sessions of trans-sphenoidal surgery 4 months apart. After the first surgery, the proximal muscle weakness improved dramatically and he was walking with the aid of a walker, and after the second surgery he is walking without any aids.
CONCLUSIONS: This case report emphasizes the high diagnostic importance of proximal muscle weakness as the sole presenting manifestation of Cushing\'s syndrome/disease.
METHODS: We report the case of a 36-year-old Iranian man who presented with proximal muscle weakness. He visited a rheumatologist in an outpatient clinic, and according to proximal muscle weakness and heliotrope rash (based on the rheumatologist\'s notes) with the impression of dermatomyositis, prednisolone and azathioprine were prescribed for him that did not improve his clinical status and he was gradually wheelchair dependent. He was admitted to the hospital for evaluation of paraneoplastic syndromes. Standard laboratory tests and imaging were unremarkable, other than a brain magnetic resonance imaging that demonstrated a 30 × 12 mm homogeneously enhancing mass in the sellar region with extension to the suprasellar area. He had serum cortisol of 295 ng/mL, and adrenocorticotropic hormone of 222 pg/mL (on 5 mg prednisolone twice daily), with a diagnosis of Cushing\'s disease. He underwent two sessions of trans-sphenoidal surgery 4 months apart. After the first surgery, the proximal muscle weakness improved dramatically and he was walking with the aid of a walker, and after the second surgery he is walking without any aids.
CONCLUSIONS: This case report emphasizes the high diagnostic importance of proximal muscle weakness as the sole presenting manifestation of Cushing\'s syndrome/disease.
摘要：
背景：库欣综合征包括与长期暴露于高水平糖皮质激素相关的体征和症状，并应在具有歧视性体征和症状的个体中考虑。近端肌病是一个重要的鉴别标志。
方法：我们报告了一名36岁的伊朗男子，他表现为近端肌肉无力。他在门诊部拜访了风湿病专家，并根据近端肌肉无力和天刚性皮疹（根据风湿病学家的注释）与皮肌炎的印象，为他开出的泼尼松龙和硫唑嘌呤没有改善他的临床状态,他逐渐依赖轮椅.他被送进医院评估副肿瘤综合征。标准的实验室检查和影像学检查并不明显，除了脑磁共振成像显示在鞍区有30×12mm均匀增强的质量，并延伸到鞍上区域。他的血清皮质醇为295ng/mL,和222pg/mL的促肾上腺皮质激素（每天两次使用5mg泼尼松龙），诊断为库欣病。他相隔4个月接受了两次蝶骨手术。第一次手术后,近端肌肉无力显著改善，他在助行器的帮助下行走，第二次手术后，他在没有任何辅助设备的情况下行走。
结论：本病例报告强调近端肌无力作为库欣综合征/疾病的唯一表现的高度诊断重要性。
方法：我们报告了一名36岁的伊朗男子，他表现为近端肌肉无力。他在门诊部拜访了风湿病专家，并根据近端肌肉无力和天刚性皮疹（根据风湿病学家的注释）与皮肌炎的印象，为他开出的泼尼松龙和硫唑嘌呤没有改善他的临床状态,他逐渐依赖轮椅.他被送进医院评估副肿瘤综合征。标准的实验室检查和影像学检查并不明显，除了脑磁共振成像显示在鞍区有30×12mm均匀增强的质量，并延伸到鞍上区域。他的血清皮质醇为295ng/mL,和222pg/mL的促肾上腺皮质激素（每天两次使用5mg泼尼松龙），诊断为库欣病。他相隔4个月接受了两次蝶骨手术。第一次手术后,近端肌肉无力显著改善，他在助行器的帮助下行走，第二次手术后，他在没有任何辅助设备的情况下行走。
结论：本病例报告强调近端肌无力作为库欣综合征/疾病的唯一表现的高度诊断重要性。
