Andela Cornelie D, van Haalen Femke M, Ragnarsson Oskar, Papakokkinou Eleni, Johannsson Gudmundur, Santos Alicia, Webb Susan M, Biermasz Nienke R, van der Wee Nic J A, Pereira Alberto M
Department of MedicineDivision of Endocrinology and Center for Endocrine Tumors, Leiden University Medical Center, Leiden, The NetherlandsLeiden Institute for Brain and CognitionLeiden, The NetherlandsDepartment of Endocrinology Diabetes and MetabolismSahlgrenska University Hospital and Sahlgrenska Academy, University of Gothenburg, Gothenburg, SwedenEndocrinology/Medicine DepartmentsHospital Sant Pau, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, IIB-Sant Pau, Universitat Autònoma de Barcelona (UAB), Barcelona, SpainDepartment of PsychiatryLeiden University Medical Center, Leiden, The Netherlands Department of MedicineDivision of Endocrinology and Center for Endocrine Tumors, Leiden University Medical Center, Leiden, The NetherlandsLeiden Institute for Brain and CognitionLeiden, The NetherlandsDepartment of Endocrinology Diabetes and MetabolismSahlgrenska University Hospital and Sahlgrenska Academy, University of Gothenburg, Gothenburg, SwedenEndocrinology/Medicine DepartmentsHospital Sant Pau, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, IIB-Sant Pau, Universitat Autònoma de Barcelona (UAB), Barcelona, SpainDepartment of PsychiatryLeiden University Medical Center, Leiden, The Netherlands
Department of MedicineDivision of Endocrinology and Center for Endocrine Tumors, Leiden University Medical Center, Leiden, The NetherlandsLeiden Institute for Brain and CognitionLeiden, The NetherlandsDepartment of Endocrinology Diabetes and MetabolismSahlgrenska University Hospital and Sahlgrenska Academy, University of Gothenburg, Gothenburg, SwedenEndocrinology/Medicine DepartmentsHospital Sant Pau, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, IIB-Sant Pau, Universitat Autònoma de Barcelona (UAB), Barcelona, SpainDepartment of PsychiatryLeiden University Medical Center, Leiden, The Netherlands.
Eur J Endocrinol. 2015 Jul;173(1):R1-14. doi: 10.1530/EJE-14-1101. Epub 2015 Feb 3.
Cushing's syndrome (CS) is characterized by excessive exposure to cortisol, and is associated with both metabolic and behavioral abnormalities. Symptoms improve substantially after biochemical cure, but may persist during long-term remission. The causes for persistent morbidity are probably multi-factorial, including a profound effect of cortisol excess on the brain, a major target area for glucocorticoids.
To review publications evaluating brain characteristics in patients with CS using magnetic resonance imaging (MRI).
Systematic review of literature published in PubMed, Embase, Web of Knowledge, and Cochrane databases.
Nineteen studies using MRI in patients with CS were selected, including studies in patients with active disease, patients in long-term remission, and longitudinal studies, covering a total of 339 unique patients. Patients with active disease showed smaller hippocampal volumes, enlarged ventricles, and cerebral atrophy as well as alterations in neurochemical concentrations and functional activity. After abrogation of cortisol excess, the reversibility of structural and neurochemical alterations was incomplete after long-term remission. MRI findings were related to clinical characteristics (i.e., cortisol levels, duration of exposure to hypercortisolism, current age, age at diagnosis, and triglyceride levels) and behavioral outcome (i.e., cognitive and emotional functioning, mood, and quality of life).
Patients with active CS demonstrate brain abnormalities, which only partly recover after biochemical cure, because these still occur even after long-term remission. CS might be considered as a human model of nature that provides a keyhole perspective of the neurotoxic effects of exogenous glucocorticoids on the brain.
库欣综合征（CS）的特征是皮质醇暴露过多，与代谢和行为异常有关。生化治愈后症状有显著改善，但在长期缓解期可能持续存在。持续发病的原因可能是多因素的，包括皮质醇过多对大脑（糖皮质激素的主要靶器官）产生的深远影响。
综述使用磁共振成像（MRI）评估CS患者脑特征的相关文献。
对发表于PubMed、Embase、Web of Knowledge和Cochrane数据库的文献进行系统综述。
选取了19项对CS患者使用MRI的研究，包括对活动期患者、长期缓解期患者的研究以及纵向研究，共涉及339例不同患者。活动期患者表现为海马体积减小、脑室扩大、脑萎缩以及神经化学浓度和功能活动改变。在皮质醇过多消除后，长期缓解期结构和神经化学改变的可逆性并不完全。MRI检查结果与临床特征（即皮质醇水平、高皮质醇血症暴露持续时间、当前年龄、诊断时年龄和甘油三酯水平）及行为结果（即认知和情绪功能、情绪和生活质量）相关。
活动期CS患者存在脑异常，生化治愈后仅部分恢复，因为即使在长期缓解期这些异常仍会出现。CS可被视为一种自然的人类模型，为外源性糖皮质激素对脑的神经毒性作用提供了一个关键视角。
