Hansen Niels, Sagebiel Anne, Rentzsch Kristin, Hirschel Sina, Wiltfang Jens, Schott Björn H, Claudia Bartels
Department of Psychiatry and Psychotherapy, University Medical Center Göttingen, Göttingen, Germany.
Department of Psychiatry and Psychotherapy, Translational Psychoneuroscience, University Medical Center Göttingen, Göttingen, Germany.
Front Psychiatry. 2023 Jan 12;13:1054461. doi: 10.3389/fpsyt.2022.1054461. eCollection 2022.
Neurofascin 186 autoantibodies are known to occur with a diseased peripheral nervous system. Recently, also additional central nervous system (CNS) involvement has been reported in conjunction with neurofascin 186 autoantibodies. Our case enlarges the spectrum of neurofascin 186 antibody-related disease to include mild cognitive impairment (MCI).
We report here a case after having examined the patient files retrospectively, including diagnostics such as blood and cerebrospinal fluid (CSF) analysis involving the determination of neural autoantibodies, brain magnetic resonance imaging (MRI), brain fluorodesoxyglucose positron emission tomography (FDG-PET), and extensive neuropsychological testing.
We report on two patients with MCI. Brain MRI showed cerebral microangiopathy in both patients, but brain FDG-PET demonstrated pathology in the right prefrontal cortex, in the right inferior parietal cortex, and in both lateral occipital cortices in one patient. Neurofascin 186 antibodies were detected in serum in both patients, and neurofascin 186 autoantibodies were also detected in the CSF of one of these patients. At follow-up six month later, neurofascin 186 autoantibodies disappeared in one patient while persisting in the other.
We report on two individuals presenting MCI associated with neurofascin 186 antibodies, thus expanding the potential spectrum of neurofascin 186-associated disease. This report supports the recommendation to consider also neurofascin 186 autoantibodies in not just peripheral nerve disease, but also in disorders involving CNS autoimmunity. More studies are needed to clarify the lack of association between neurofascin 186 autoantibodies and cognitive decline.
已知神经束蛋白186自身抗体与患病的外周神经系统有关。最近，也有报道称神经束蛋白186自身抗体与中枢神经系统（CNS）受累有关。我们的病例扩大了神经束蛋白186抗体相关疾病的范围，包括轻度认知障碍（MCI）。
我们在此报告一例经过回顾性检查患者病历后的病例，包括血液和脑脊液（CSF）分析等诊断，其中涉及神经自身抗体的测定、脑磁共振成像（MRI）、脑氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）以及广泛的神经心理学测试。
我们报告了两名患有MCI的患者。两名患者的脑MRI均显示脑微血管病变，但一名患者的脑FDG-PET显示右侧前额叶皮质、右侧顶下皮质和双侧枕叶皮质有病变。两名患者血清中均检测到神经束蛋白186抗体，其中一名患者的脑脊液中也检测到神经束蛋白186自身抗体。在六个月后的随访中，一名患者的神经束蛋白186自身抗体消失，而另一名患者的该抗体持续存在。
我们报告了两名出现与神经束蛋白186抗体相关的MCI患者，从而扩大了神经束蛋白186相关疾病的潜在范围。本报告支持不仅在周围神经疾病中，而且在涉及中枢神经系统自身免疫的疾病中也考虑神经束蛋白186自身抗体的建议。需要更多研究来阐明神经束蛋白186自身抗体与认知衰退之间缺乏关联的情况。
