Erdil Yucesoy Esra, Tunc Handenur, Erdem Sema Nur, Bozkurt Suheyla, Tuncer Nese
Neurosciences, King's College Hospital, London, GBR.
Department of Neurology, Marmara University Pendik Training and Research Hospital, İstanbul, TUR.
Cureus. 2023 Jun 14;15(6):e40399. doi: 10.7759/cureus.40399. eCollection 2023 Jun.
Limbic encephalitis is a well-defined clinical disorder among paraneoplastic neurological syndromes. Although it is not always possible to identify specific autoantibodies in limbic encephalitis, the presence of anti-neuronal nuclear antibody type 1 (ANNA1 or anti-Hu), anti-Ma2, collapsin response mediator protein 5 (CRMP-5-IgG or anti-CV2), anti-GABA receptors and anti-amphiphysin antibodies are often detected. A 66-year-old male patient with complaints of forgetfulness was evaluated in our clinic after having seizures. In the neurological examination, the patient was found to be confused. In cranial MR fluid-attenuated inversion recovery (FLAIR) and T2-weighted images, the right hippocampal and parahippocampal structures showed hyperintense areas complying with limbic encephalitis. He had improvement with a course of 2 g/kg intravenous immunoglobulin (IVIG) followed by high-dose methylprednisolone therapy. Following the high-dose methylprednisolone therapy, anti-PCA1 (Yo) and anti-amphiphysin antibodies were positive and the tissue pathology report confirmed combined small-cell carcinoma and large-cell neuroendocrine carcinoma of the lung. In recent years, paraneoplastic neurological syndromes are better recognized with the identification of specific antibodies and the ubiquitous information on pathogenesis. This is the first known report in the literature that a case with both positive anti-PCA1 (Yo) and anti-amphiphysin antibodies together and underlying small-cell and large-cell neuroendocrine carcinomas.
边缘叶脑炎是副肿瘤性神经系统综合征中一种明确的临床疾病。虽然在边缘叶脑炎中并非总能鉴定出特异性自身抗体,但常检测到1型抗神经元核抗体(ANNA1或抗Hu)、抗Ma2、塌陷反应介导蛋白5(CRMP - 5 - IgG或抗CV2)、抗GABA受体和抗 amphiphysin抗体。一名66岁男性患者,因癫痫发作后在我们诊所接受评估,主诉健忘。神经系统检查发现该患者意识模糊。在头颅磁共振液体衰减反转恢复序列(FLAIR)和T2加权图像上,右侧海马和海马旁结构显示符合边缘叶脑炎的高信号区。他在接受2 g/kg静脉注射免疫球蛋白(IVIG)疗程后,接着进行大剂量甲泼尼龙治疗,病情有所改善。在大剂量甲泼尼龙治疗后,抗PCA1(Yo)和抗amphiphysin抗体呈阳性,组织病理学报告证实为肺小细胞癌合并大细胞神经内分泌癌。近年来,随着特异性抗体的鉴定以及关于发病机制的广泛信息,副肿瘤性神经系统综合征得到了更好的认识。这是文献中首次报道的一例抗PCA1(Yo)和抗amphiphysin抗体均为阳性且伴有小细胞和大细胞神经内分泌癌的病例。