两耐素(AMPH)自身免疫与多种神经系统并发症有关,包括脑炎,周围神经病变,脊髓病,和小脑综合征.其诊断基于临床神经功能缺损和血清抗AMPH抗体的存在。主动免疫疗法,比如静脉注射免疫球蛋白,类固醇,和其他免疫抑制疗法,据报道对大多数患者有效。然而,恢复的程度因情况而异。在这里,我们报道了一名75岁女性半快速进行性系统性震颤的病例,视觉幻觉,和烦躁。住院后,她出现了轻度发烧和认知障碍。脑磁共振成像(MRI)显示半快速进行性弥漫性脑萎缩(DCA)超过3个月,而没有观察到明显的异常强度。神经传导研究显示四肢感觉和运动神经病。固定的基于组织的测定(TBA)未能检测到抗神经元抗体;然而,基于商业免疫印迹,怀疑存在抗AMPH抗体.因此,进行血清免疫沉淀,这证实了抗AMPH抗体的存在。患者还患有胃腺癌。大剂量甲基强的松龙,并给予静脉注射免疫球蛋白并进行肿瘤切除术,导致认知障碍的解决和治疗后MRI上DCA的改善。免疫治疗和肿瘤切除后,使用免疫沉淀法分析患者的血清,显示抗AMPH抗体水平降低。这种情况值得注意,因为DCA在免疫治疗和肿瘤切除后显示出改善。此外,该病例表明,TBA阴性和商业免疫印迹阳性并不一定表示假阳性结果.
Amphiphysin (AMPH) autoimmunity is associated with a variety of neurological complications, including encephalitis, peripheral neuropathy, myelopathy, and cerebellar syndrome. Its diagnosis is based on clinical neurological deficits and the presence of serum anti-AMPH antibodies. Active immunotherapy, such as intravenous immunoglobulins, steroids, and other immunosuppressive therapies, has been reported to be effective in most patients. However, the extent of recovery varies depending on the
case. Herein, we report the
case of a 75-year-old woman with semi-rapidly progressive systemic tremors, visual hallucinations, and irritability. Upon hospitalization, she developed a mild fever and cognitive impairment. Brain magnetic resonance imaging (MRI) showed semi-rapidly progressive diffuse cerebral atrophy (DCA) over 3 months, while no clear abnormal intensities were observed. The nerve conduction study revealed sensory and motor neuropathy in the limbs. The fixed tissue-based assay (TBA) failed to detect antineuronal antibodies; however, based on commercial immunoblots, the presence of anti-AMPH antibodies was suspected. Therefore, serum
immunoprecipitation was performed, which confirmed the presence of anti-AMPH antibodies. The patient also had gastric adenocarcinoma. High-dose methylprednisolone, and intravenous immunoglobulin were administered and tumor resection was performed, resulting in resolution of the cognitive impairment and improvement in the DCA on the post-treatment MRI. After immunotherapy and tumor resection, the patient\'s serum was analyzed using
immunoprecipitation, which showed a decrease in the level of anti-AMPH antibodies. This
case is noteworthy because the DCA showed improvement after immunotherapy and tumor resection. Additionally, this case demonstrates that negative TBA with positive commercial immunoblots do not necessarily indicate false positive results.