Mizuno Yuri, Yamaguchi Hiroo, Uehara Taira, Yamashita Kenichiro, Yamasaki Ryo, Kira Jun-Ichi
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
Rinsho Shinkeigaku. 2017 Jun 28;57(6):298-302. doi: 10.5692/clinicalneurol.cn-001008. Epub 2017 May 26.
We report a case of flexion contractures in a patient's legs secondary to postpartum hypopituitarism. A 56-year-old woman presented with a 3-year history of worsening flexion contractures of the hips and knees. On admission, her hips and knees could not be extended, and she had muscle stiffness and tenderness to palpation of the lower extremities. We first suspected stiff-person syndrome or Isaacs' syndrome because of her muscle stiffness. However, multiple hormones did not respond to stimulation tests, and an MRI of the brain showed atrophy of the pituitary gland with an empty sella. A subsequent interview revealed that she had suffered a severe hemorrhage while delivering her third child. She was diagnosed with panhypopituitarism and started on cortisol replacement therapy. After 1 week of treatment with hydrocortisone (10 mg/day), her symptoms quickly improved. We then added 75 μg/day of thyroid hormone. During the course of her treatment, autoantibodies against VGKC complex were found to be weakly positive. However, we considered the antibodies to be unrelated to her disease, because her symptoms improved markedly with low-dose steroid treatment. There are a few reports describing flexion contractures of the legs in patients with primary and secondary adrenal insufficiency. As these symptoms are similar to those seen in stiff-person syndrome, adrenal and pituitary insufficiency should be taken into account to achieve the correct diagnosis and treatment in patients with flexion contractures and muscle stiffness.
我们报告一例产后垂体功能减退继发患者腿部屈曲挛缩的病例。一名56岁女性,有3年髋部和膝部屈曲挛缩逐渐加重的病史。入院时,她的髋部和膝部无法伸直,下肢有肌肉僵硬和触痛。由于她的肌肉僵硬,我们最初怀疑是僵人综合征或艾萨克斯综合征。然而,多项激素对刺激试验无反应,脑部MRI显示垂体萎缩伴空蝶鞍。随后的询问发现她在分娩第三个孩子时发生了严重出血。她被诊断为全垂体功能减退,并开始接受皮质醇替代治疗。用氢化可的松(10毫克/天)治疗1周后,她的症状迅速改善。然后我们添加了75微克/天的甲状腺激素。在她的治疗过程中,发现抗VGKC复合物自身抗体弱阳性。然而,我们认为这些抗体与她的疾病无关,因为她的症状在低剂量类固醇治疗后明显改善。有一些报告描述了原发性和继发性肾上腺功能不全患者的腿部屈曲挛缩。由于这些症状与僵人综合征所见症状相似,对于有屈曲挛缩和肌肉僵硬的患者,应考虑肾上腺和垂体功能不全,以实现正确的诊断和治疗。