Hanağası Haşmet, Çakar Arman, Hanağası Figen, Samancı Bedia, Tüfekçioğlu Zeynep, Bilgiç Başar, Emre Murat
Istanbul University, Istanbul Faculty of Medicine, Department of Neurology, Istanbul, Türkiye.
Gayrettepe Florence Nightingale Hospital, Department of Neurology, Istanbul, Türkiye.
Noro Psikiyatr Ars. 2025 Feb 15;62(1):94-96. doi: 10.29399/npa.28517. eCollection 2025.
Oromandibular dystonia and laryngospasm are defined as paraneoplastic syndromes of anti-Ri antibody. Herein, we report a 50-year-old woman admitted to the outpatient clinic with cervical contraction, speech and gait disturbance, and mental deterioration persisting for one year. She was diagnosed with undifferentiated nasopharyngeal carcinoma during further examination for two years of tinnitus and underwent radiotherapy. Her neurological symptoms started six months after radiotherapy. During this period, she underwent a tracheostomy due to a sudden laryngospasm. Anti-Ri antibody was positive in the paraneoplastic antibody screening. Her cervical dystonia and mental deterioration partially improved with intravenous pulse steroid and immunoglobulin therapies. However, the patient deceased due to aspiration pneumonia after six months. This rare clinical presentation, characterized by cervical dystonia, laryngospasm, spastic quadriparesis, and mental deterioration, should be considered anti-Ri antibody-associated paraneoplastic syndrome for patients with nasopharyngeal carcinoma.
口下颌肌张力障碍和喉痉挛被定义为抗Ri抗体相关的副肿瘤综合征。在此，我们报告一名50岁女性，因颈部收缩、言语和步态障碍以及精神衰退持续一年而入住门诊。她在因耳鸣两年接受进一步检查期间被诊断为未分化鼻咽癌，并接受了放疗。她的神经症状在放疗后六个月开始出现。在此期间，她因突然发生喉痉挛而接受了气管切开术。副肿瘤抗体筛查中抗Ri抗体呈阳性。静脉注射脉冲类固醇和免疫球蛋白治疗后，她的颈部肌张力障碍和精神衰退部分得到改善。然而，患者在六个月后因吸入性肺炎死亡。这种以颈部肌张力障碍、喉痉挛、痉挛性四肢瘫和精神衰退为特征的罕见临床表现，对于鼻咽癌患者应考虑为抗Ri抗体相关的副肿瘤综合征。
