Hansen Niels, Lange Claudia, Maass Fabian, Hassoun Lina, Bouter Caroline, Stöcker Winfried, Schott Björn Hendrik, Wiltfang Jens, Fitzner Dirk
Department of Psychiatry and Psychotherapy, University of Goettingen, Von-Siebold-Str. 5, 37075 Goettingen, Germany.
Department of Neurology, University Medical Center of Goettingen, Robert-Koch Str. 40, 37075 Goettingen, Germany.
Brain Sci. 2021 May 21;11(6):673. doi: 10.3390/brainsci11060673.
(1) Background: autoimmune encephalitis associated with neurexin-3α antibodies is a seldom reported disease entity often accompanied by a severe clinical neuropsychiatric syndrome. (2) Method: we report on the case of a 58-year-old man diagnosed with neurexin-3α-associated autoimmune encephalitis revealing cognitive decline and depression before the proof of neurexin-3α antibodies. He underwent neuropsychological testing, peripheral blood and cerebrospinal fluid analysis, neuroimaging and electroencephalography. (3) Results: our patient's main clinical feature was amnestic cognitive decline in combination with depressive symptoms. CSF analysis showed elevated phosphorylated tau protein 181 and positive proof of serum neurexin-3α antibodies in a cell-based assay. An 18F-FDG-PET/CT of the brain initially showed bilateral cerebral hypometabolism prefrontal and parietal, which was absent in follow up. The brain MRI was unremarkable. EEG recordings showed frontotemporal slowing in the theta and delta range. (4) Conclusions: taken together, we assumed autoimmune encephalitis associated with serum neurexin-3α antibodies. To the best of our knowledge, we are the first to report on a predominantly mild clinical manifestation entailing amnestic mild cognitive impairment in addition to depression, thus broadening the clinical spectrum associated with neurexin-3α antibodies.
(1) 背景：与神经纤毛蛋白-3α抗体相关的自身免疫性脑炎是一种报道较少的疾病实体，常伴有严重的临床神经精神综合征。(2) 方法：我们报告一例58岁男性病例，该患者在检测到神经纤毛蛋白-3α抗体之前被诊断为与神经纤毛蛋白-3α相关的自身免疫性脑炎，表现出认知功能下降和抑郁。他接受了神经心理学测试、外周血和脑脊液分析、神经影像学检查和脑电图检查。(3) 结果：我们患者的主要临床特征是遗忘性认知功能下降并伴有抑郁症状。脑脊液分析显示磷酸化tau蛋白181升高，基于细胞检测的血清神经纤毛蛋白-3α抗体检测呈阳性。脑部18F-FDG-PET/CT最初显示双侧额叶和顶叶大脑代谢减低，随访时消失。脑部MRI无明显异常。脑电图记录显示额颞叶θ和δ频段慢波。(4) 结论：综合来看，我们推测为与血清神经纤毛蛋白-3α抗体相关的自身免疫性脑炎。据我们所知，我们是首个报道除抑郁外主要表现为遗忘性轻度认知障碍的轻度临床表现病例，从而拓宽了与神经纤毛蛋白-3α抗体相关的临床谱。
