Department of Endocrinology, Diabetology and Metabolic Diseases, Avicenne Hospital, Route de Stalingrad, Bobigny Cedex, France.
J Neurol. 2010 Jul;257(7):1129-33. doi: 10.1007/s00415-010-5477-8. Epub 2010 Feb 16.
The diagnosis of adrenal insufficiency is discussed in case of low blood pressure and digestive symptoms. Rare inaugural abdomino-crural muscle contracture can be a misleading symptom. Here we report two new cases. A 50-year-old woman presenting a leaning forward walking attitude and negligence for the past 2 months was referred to the neurologic unit. Abdomino-crural contracture, clinical hypogonadism, and hyponatremia directed towards a panhypopituitarism, which was confirmed by subsequent investigations. Pituitary MRI found an empty sella turcica. The outcome was dramatic after hormone replacement therapy, with drawing up of the trunk and re-establishment of walking after a few days. The second case is a 58-year-old man, hospitalized with altered general condition, with a weight loss of 22 kg, and anorexia in the aftermath of a comminuted fracture of the right lower limb, complicated by pseudoarthrosis. There was amyotrophy on the extremities with intense cruralgia. The patient had an antalgic attitude in the flexion affecting his rehabilitation. During 1 year, the symptoms were mimicking psychiatric disorders, malabsorption, or cancer before the final diagnosis of central hypocorticism with normal MRI was established. The evolution was remarkable after a few days of therapy with hydrocortisone, where the myalgia disappeared, the patient quickly gained weight, and the disappearance of the retractions allowed rehabilitation. These two observations emphasize the delayed diagnosis of adrenal insufficiency in the case of abdomino-crural-related symptoms and the presence of misleading neurological symptoms. The mechanisms of this syndrome remain unknown.
肾上腺皮质功能减退症的诊断应考虑低血压和消化道症状。罕见的首发腹-股肌痉挛可能是一个误导性症状。我们在此报告两例新病例。一名 50 岁女性,2 个月来出现前倾向行走姿势和对周围事物漠不关心,被转诊至神经科。腹-股肌痉挛、临床性腺功能减退症和低钠血症提示全垂体功能减退症,进一步检查证实了这一点。垂体 MRI 发现空蝶鞍。激素替代治疗后病情显著改善,几天后躯干挺直,行走恢复。第二例是一名 58 岁男性,因全身状况改变住院,体重减轻 22 公斤,右下肢粉碎性骨折后食欲不振,伴有假关节形成。四肢有肌萎缩,股部剧痛。患者有屈肌疼痛导致的止痛性姿势,影响康复。在最终诊断为中枢性皮质功能减退症(MRI 正常)之前,1 年内症状表现为精神障碍、吸收不良或癌症,类似的情况持续存在。接受氢化可的松治疗数天后,病情显著改善,肌痛消失,患者体重迅速增加,痉挛消失,康复得以进行。这两例观察强调了在出现腹-股肌相关症状时,肾上腺皮质功能减退症的诊断可能会被延误,并且可能存在误导性的神经症状。该综合征的发病机制尚不清楚。