From the Division of Neuropathology and Neurochemistry (G.R., A.F., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (T.Z., S.M., P.A., P.R., T. Berger), Medical University of Vienna, Austria; Department of Neurology (J.T., J.W.), Klinikum Klagenfurt, Austria; Institute of Clinical Chemistry (K.K.F., K.-P.W., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Institute of Pathology (A.K.), Klinikum Klagenfurt, Austria; Clinical Department of Laboratory Medicine (A.F., G.M.), Proteomics Core Facility, Medical University Vienna, Austria; Center of Physiology and Pharmacology (H.K.), Department of Neurophysiology and Neuropharmacology, Medical University of Vienna, Austria; and Department of Neurology (T. Bartsch, F.L.), University Hospital Schleswig-Holstein, Kiel, Germany.
Neurol Neuroimmunol Neuroinflamm. 2021 May 20;8(4). doi: 10.1212/NXI.0000000000001019. Print 2021 Jul.
To report an unusual clinical phenotype of alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis and describe associated neuropathologic findings.
We retrospectively investigated 3 AMPAR encephalitis patients with autoimmune global hippocampal amnesia using comprehensive cognitive and neuropsychologic assessment, antibody testing by in-house tissue-based and cell-based assays, and neuropathologic analysis of brain autopsy tissue including histology and immunohistochemistry.
Three patients presented with acute-to-subacute global amnesia without affection of cognitive performance, attention, concentration, or verbal function. None of the patients had epileptic seizures, change of behavior, personality changes, or psychiatric symptoms. The MRI was normal in 1 patient and showed increased fluid-attenuated inversion recovery/T2 signal in the hippocampus in the other 2 patients. Two patients showed complete remission after immunotherapy. The one patient who did not improve had an underlying adenocarcinoma of the lung and died 3.5 months after disease onset because of tumor progression. Neuropathologic analysis of the brain autopsy revealed unilateral hippocampal sclerosis accompanied by mild inflammatory infiltrates, predominantly composed of T lymphocytes, and decrease of AMPAR immunoreactivity.
AMPAR antibodies usually associate with limbic encephalitis but may also present with immune responsive, acute-to-subacute, isolated hippocampal dysfunction without overt inflammatory CSF or MRI changes.
报告一种α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体（AMPAR）脑炎的不常见临床表型，并描述相关的神经病理学发现。
我们通过综合认知和神经心理学评估、基于组织的和基于细胞的内部检测抗体测试，以及对包括组织学和免疫组织化学在内的脑尸检组织的神经病理学分析，回顾性研究了 3 名患有自身免疫性全海马性遗忘症的 AMPAR 脑炎患者。
3 名患者表现为急性至亚急性全记忆障碍，而认知表现、注意力、集中力或言语功能不受影响。没有患者出现癫痫发作、行为改变、人格改变或精神症状。1 名患者的 MRI 正常，另外 2 名患者的 MRI 显示海马区的液体衰减反转恢复/T2 信号增加。2 名患者在免疫治疗后完全缓解。1 名未改善的患者患有肺癌，并因肿瘤进展在发病后 3.5 个月死亡。脑尸检的神经病理学分析显示单侧海马硬化，伴有轻度炎症浸润，主要由 T 淋巴细胞组成，以及 AMPAR 免疫反应性降低。
AMPAR 抗体通常与边缘性脑炎相关，但也可能表现为免疫反应性、急性至亚急性、孤立性海马功能障碍，无明显炎症性 CSF 或 MRI 改变。
