Bheemavarapu Bhumika, Singh Arkaja, Ranjini Nived Jayaraj, Abhilash Meda Venkata Sai, Patil Dhrumil
Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
Mahatma Gandhi Medical College and Hospital, Riico Industrial Area, Jaipur, India.
Case Rep Neurol. 2023 Aug 22;15(1):187-191. doi: 10.1159/000532093. eCollection 2023 Jan-Dec.
Stiff person syndrome (SPS) is an extremely rare disease that presents with episodic painful muscle spasms and progressive muscle rigidity. Recent evidence suggests that SPS can rarely manifest with life-threatening respiratory complications. However, the pathophysiology behind respiratory failure in SPS is still not clearly understood. Here, we explored an extremely rare case of a 36-year-old African-American female with SPS presenting with multiple episodes of respiratory failure events for the past 9 years. She had an in-situ tracheostomy and was admitted to the hospital for tracheostomy evaluation and decannulation. 11 years ago she initially presented with gait abnormalities, stiffness, and spastic episodes. She was diagnosed 1 year later with SPS after detecting elevated anti-glutamic acid decarboxylase antibody levels in her blood. Through this report, we were able to follow a very rare case of SPS that presented with multiple episodes of respiratory failure. We pointed out the importance of early start and regular administration of diazepam, baclofen, and IVIg in not only controlling the symptoms and progression of the disease but also in preventing further respiratory failure and possible sudden death.
僵人综合征（SPS）是一种极为罕见的疾病，表现为发作性疼痛性肌肉痉挛和进行性肌肉僵硬。最近的证据表明，SPS很少会出现危及生命的呼吸并发症。然而，SPS呼吸衰竭背后的病理生理学仍未完全清楚。在此，我们探讨了一例极为罕见的病例，一名36岁非裔美国女性患有SPS，在过去9年中出现了多次呼吸衰竭事件。她接受了原位气管造口术，并因气管造口术评估和拔管入院。11年前，她最初表现为步态异常、僵硬和痉挛发作。1年后，在检测到其血液中抗谷氨酸脱羧酶抗体水平升高后，她被诊断为SPS。通过本报告，我们得以追踪一例极为罕见的SPS病例，该病例出现了多次呼吸衰竭。我们指出了早期开始并规律使用地西泮、巴氯芬和静脉注射免疫球蛋白（IVIg）的重要性，这不仅有助于控制疾病的症状和进展，还能预防进一步的呼吸衰竭和可能的猝死。
