Takasugi Junji, Shimamura Munehisa, Koda Toru, Kishikawa Toshihiro, Hanamoto Atsushi, Inohara Hidenori, Sato Kazuaki, Morii Eiichi, Motomura Masakatsu, Sakaguchi Manabu, Nakatsuji Yuji, Mochizuki Hideki
Department of Neurology, Osaka University Graduate School of Medicine, Japan.
Department of Otorhinolaryngology-Head and Neck Surgery, Osaka University Graduate School of Medicine, Japan.
Intern Med. 2018 Feb 15;57(4):587-590. doi: 10.2169/internalmedicine.9333-17. Epub 2017 Nov 1.
Paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS) are usually associated with small-cell lung carcinoma (SCLC). PCD-LEMS with extrapulmonary non-SCLC tumors; however, has not been previously reported. A 78-year-old man presented with dysarthria, dysphagia, staggering gait, and lower extremity muscle fatigue. He was diagnosed with PCD-LEMS associated with neuroendocrine carcinoma of the oropharynx, based on the histological findings of the biopsy, the existence of antibodies against P/Q-type voltage-gated calcium channels, and an incremental response of the compound muscle action potentials during repetitive nerve stimulation tests. Thus, PCD-LEMS should be included in the differential diagnosis of neurological dysfunction, even in extrapulmonary non-SCLC patients.
副肿瘤性小脑变性和兰伯特-伊顿肌无力综合征(PCD-LEMS)通常与小细胞肺癌(SCLC)相关。然而,此前尚未报道过伴有肺外非小细胞肺癌肿瘤的PCD-LEMS。一名78岁男性出现构音障碍、吞咽困难、蹒跚步态和下肢肌肉疲劳。根据活检的组织学结果、抗P/Q型电压门控钙通道抗体的存在以及重复神经刺激试验中复合肌肉动作电位的递增反应,他被诊断为与口咽神经内分泌癌相关的PCD-LEMS。因此,即使在肺外非小细胞肺癌患者中,PCD-LEMS也应纳入神经功能障碍的鉴别诊断中。