Department of Neurology, Mayo Clinic, Rochester, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, USA.
Thorac Cancer. 2019 Apr;10(4):1001-1004. doi: 10.1111/1759-7714.13009. Epub 2019 Feb 27.
Paraneoplastic neurological autoimmunity is often associated with small-cell lung cancer (SCLC), a highly malignant neuroendocrine tumor. Paraneoplastic autoimmunity often correlates with longer survival. We describe the paraneoplastic neurological manifestations of patients with SCLC with and without SCLC-predictive autoantibodies and the correlation between autoimmunity and survival. We reviewed the records of 116 patients (51% male) from the Mayo Clinic with histopathologically confirmed SCLC for whom stored serum was available for neural autoantibody testing. Cancer was limited stage in 41%; the median age at diagnosis was 64 years. Paraneoplastic neurological manifestations were recorded in 61% (decreasing frequency: peripheral neuropathy, dysautonomia, cognitive decline, cerebellar ataxia, neuromuscular junction disorder, seizures, cranial neuropathy, movement disorder, brainstem disorder, or myelopathy). Neural autoantibodies, some with pathogenic potential, were detected in the sera of SCLC patients with and without neurological autoimmunity. The most frequent among patients with neurological manifestations were: anti-neuronal nuclear antibody-type 1, voltage-gated calcium channel (VGCC)-N-type, VGCC-P/Q-type, glutamic acid decarboxylase 65 (GAD65), SOX1, and muscle acetylcholine receptor (AChR); while the most common in patients without neurological manifestations were: GAD65, muscle-AChR, and VGCC-P/Q-type. Neither cancer stage at diagnosis nor survival correlated with neurological manifestations or autoantibody-positivity, except for shorter survival in patients with myelopathy. The only predictor of longer survival was limited-stage disease at diagnosis.
副肿瘤性神经自身免疫通常与小细胞肺癌（SCLC）有关，SCLC 是一种高度恶性的神经内分泌肿瘤。副肿瘤自身免疫通常与更长的生存时间相关。我们描述了伴有和不伴有 SCLC 预测性自身抗体的 SCLC 患者的副肿瘤性神经表现，以及自身免疫与生存之间的相关性。我们回顾了梅奥诊所 116 例组织病理学证实为 SCLC 患者的记录，这些患者的储存血清可用于神经自身抗体检测。41%的患者癌症为局限期；诊断时的中位年龄为 64 岁。记录到 61%的患者存在副肿瘤性神经表现（递减频率：周围神经病、自主神经功能障碍、认知能力下降、小脑共济失调、神经肌肉接头疾病、癫痫发作、颅神经病、运动障碍、脑干障碍或脊髓病）。在伴有和不伴有神经自身免疫的 SCLC 患者血清中检测到了神经自身抗体，其中一些具有潜在致病性。在有神经表现的患者中最常见的是：抗神经元核抗体 1 型、电压门控钙通道（VGCC）-N 型、VGCC-P/Q 型、谷氨酸脱羧酶 65（GAD65）、SOX1 和肌肉乙酰胆碱受体（AChR）；而在无神经表现的患者中最常见的是：GAD65、肌肉-AChR 和 VGCC-P/Q 型。诊断时的癌症分期和生存均与神经表现或自身抗体阳性无关，除了脊髓病患者的生存时间更短。唯一预测生存时间较长的因素是诊断时为局限期疾病。
