Neurological symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review.
Neurological symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review.
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DOI：
10.1186/s12902-015-0082-6
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发表时间：
2016-01-12
影响因子：
2.7
通讯作者：
Mineura K
中科院分区：
文献类型：
--
作者：
Goto Y;Tatsuzawa K;Aita K;Furuno Y;Kawabe T;Ohwada K;Sasajima H;Mineura K
孤立性促肾上腺皮质激素（ACTH）缺乏症是一种仅以ACTH分泌减少为特征的垂体疾病。虽然潜在的机制仍有待阐明，但这种实体的病例数量一直在增加。我们经历了一个病例表现为步态障碍，需要从特发性正常压力脑积水（iNPH）鉴别诊断。一名69岁女性，主诉行走困难，在以前的医院怀疑患有iNPH，被转介到我科。在此之前的三年里，她患有渐进式步态障碍。磁共振成像（MRI）显示全室扩张。iNPH病例的典型步态特征都是可识别的。神经心理痴呆量表测试显示病情恶化。然而，在MRI上不成比例地扩大蛛网膜下腔的主要特征并不明显。病人在住院期间出现逐渐加重的疲劳。她的症状与下丘脑-垂体肿瘤患者相似。血清ACTH和皮质醇水平低。促肾上腺皮质激素释放激素压力测试无反应，其他使用促甲状腺激素释放激素、促黄体生成素释放激素和生长激素释放激素的压力测试反应正常，提示孤立性ACTH缺乏症的诊断。我们开始皮质类固醇治疗，她的步态障碍迅速改善。单独的ACTH缺乏可能对iNPH的鉴别诊断有重要意义。预计尽早考虑这一实体将有助于作出早期诊断。
Isolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH). A 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly. Isolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.