Evoli Amelia, Lancaster Eric
*Department of Neurology, Catholic University, Roma, Italy; and †Department of Neurology, The University of Pennsylvania, Philadelphia, PA.
J Thorac Oncol. 2014 Sep;9(9 Suppl 2):S143-7. doi: 10.1097/JTO.0000000000000300.
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis (MG), acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor for MG and to contactin-associated protein-like 2, protein of the voltage-gated potassium channel complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis. Patients who manifest these disorders should be screened for thymoma at diagnosis, and worsening of these PNDs may be associated with recurrent thymoma. These disorders can cause profound disability but usually respond to immunotherapy, and often improve with thymoma treatment. Close cooperation among a team of specialists is required to take proper care of these patients.
胸腺恶性肿瘤常与副肿瘤性神经系统疾病(PNDs)相关,对于治疗这些患者的医生而言,认识这些疾病很重要。最常见的与胸腺瘤相关的PNDs是重症肌无力(MG)、获得性神经性肌强直(艾萨克斯综合征)、脑炎、莫旺综合征和肌炎。这些疾病的诊断很复杂,但通常通过检测特定自身抗体来辅助诊断,包括针对MG的乙酰胆碱受体以及针对获得性神经性肌强直、莫旺综合征或脑炎患者的接触蛋白相关蛋白样2(电压门控钾通道复合物蛋白)的自身抗体。出现这些疾病的患者在诊断时应筛查胸腺瘤,这些PNDs的恶化可能与胸腺瘤复发有关。这些疾病可导致严重残疾,但通常对免疫治疗有反应,且常随胸腺瘤治疗而改善。需要一组专家密切合作以妥善照料这些患者。