Anti-amphiphysin encephalitis: Expanding the clinical spectrum

ObjectiveAn analysis of the clinical features of autoimmune encephalitis accompanied by anti-amphiphysin antibodies.MethodsThe data of encephalitis patients with anti-amphiphysin antibodies were retrospectively evaluated, including demographics, neurological and laboratory findings, imaging, treatme...

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Main Authors: Yueqian Sun, Xiaoxiao Qin, Danxia Huang, Ziqi Zhou, Yudi Zhang, Qun Wang
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-04-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2023.1084883/full
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author Yueqian Sun
Xiaoxiao Qin
Danxia Huang
Ziqi Zhou
Yudi Zhang
Qun Wang
Qun Wang
Qun Wang
author_facet Yueqian Sun
Xiaoxiao Qin
Danxia Huang
Ziqi Zhou
Yudi Zhang
Qun Wang
Qun Wang
Qun Wang
author_sort Yueqian Sun
collection DOAJ
description ObjectiveAn analysis of the clinical features of autoimmune encephalitis accompanied by anti-amphiphysin antibodies.MethodsThe data of encephalitis patients with anti-amphiphysin antibodies were retrospectively evaluated, including demographics, neurological and laboratory findings, imaging, treatment, and prognostic predictions.ResultsTen patients aged between 29 and 78 years (median age 52 years) were included. The male: female ratio was 4:6. Limbic encephalitis was found in nine patients while epileptic seizures were present in seven patients. All patients showed anti-amphiphysin antibody positivity in sera while one ninth was positive for CSF antibody. The EEG findings were abnormal, including reductions in background activity, and the presence of diffuse slow waves, sharp waves, and spikes and waves. Five patients showed signs of increased T2 signals in the medial temporal lobe on MRI while PET showed either hyper- or hypo-metabolic changes in several brain regions, including the temporal lobe, hippocampus, basal ganglia, frontal and parietal cortices. Nine of ten patients were treated with immunotherapy, with improvements of varying degrees. There was a significant reduction in seizure frequency, and all patients were seizure-free at last follow-up.ConclusionAutoimmune encephalitis with anti-amphiphysin antibodies has a variety of clinical manifestations. The most common symptom is limbic encephalitis. Although relief from seizures can be achieved relatively easily, many patients suffer psychiatric, cognitive, and sleep sequelae. The disease was found to be associated with a lower incidence of cancer than has been previously reported for paraneoplastic neurologic syndromes.
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spelling doaj.art-a3df59b285dd4413a18bb3b0dbe9b4922023-04-05T05:11:55ZengFrontiers Media S.A.Frontiers in Immunology1664-32242023-04-011410.3389/fimmu.2023.10848831084883Anti-amphiphysin encephalitis: Expanding the clinical spectrumYueqian Sun0Xiaoxiao Qin1Danxia Huang2Ziqi Zhou3Yudi Zhang4Qun Wang5Qun Wang6Qun Wang7Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Fujian Medical University Affiliated First Quanzhou hospital, Quanzhou, ChinaDepartment of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, ChinaNational Center for Clinical Medicine of Neurological Diseases, Beijing, ChinaBeijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, ChinaObjectiveAn analysis of the clinical features of autoimmune encephalitis accompanied by anti-amphiphysin antibodies.MethodsThe data of encephalitis patients with anti-amphiphysin antibodies were retrospectively evaluated, including demographics, neurological and laboratory findings, imaging, treatment, and prognostic predictions.ResultsTen patients aged between 29 and 78 years (median age 52 years) were included. The male: female ratio was 4:6. Limbic encephalitis was found in nine patients while epileptic seizures were present in seven patients. All patients showed anti-amphiphysin antibody positivity in sera while one ninth was positive for CSF antibody. The EEG findings were abnormal, including reductions in background activity, and the presence of diffuse slow waves, sharp waves, and spikes and waves. Five patients showed signs of increased T2 signals in the medial temporal lobe on MRI while PET showed either hyper- or hypo-metabolic changes in several brain regions, including the temporal lobe, hippocampus, basal ganglia, frontal and parietal cortices. Nine of ten patients were treated with immunotherapy, with improvements of varying degrees. There was a significant reduction in seizure frequency, and all patients were seizure-free at last follow-up.ConclusionAutoimmune encephalitis with anti-amphiphysin antibodies has a variety of clinical manifestations. The most common symptom is limbic encephalitis. Although relief from seizures can be achieved relatively easily, many patients suffer psychiatric, cognitive, and sleep sequelae. The disease was found to be associated with a lower incidence of cancer than has been previously reported for paraneoplastic neurologic syndromes.https://www.frontiersin.org/articles/10.3389/fimmu.2023.1084883/fullamphiphysinautoimmune encephalitisclinical featurestreatmentprognosis
spellingShingle Yueqian Sun
Xiaoxiao Qin
Danxia Huang
Ziqi Zhou
Yudi Zhang
Qun Wang
Qun Wang
Qun Wang
Anti-amphiphysin encephalitis: Expanding the clinical spectrum
Frontiers in Immunology
amphiphysin
autoimmune encephalitis
clinical features
treatment
prognosis
title Anti-amphiphysin encephalitis: Expanding the clinical spectrum
title_full Anti-amphiphysin encephalitis: Expanding the clinical spectrum
title_fullStr Anti-amphiphysin encephalitis: Expanding the clinical spectrum
title_full_unstemmed Anti-amphiphysin encephalitis: Expanding the clinical spectrum
title_short Anti-amphiphysin encephalitis: Expanding the clinical spectrum
title_sort anti amphiphysin encephalitis expanding the clinical spectrum
topic amphiphysin
autoimmune encephalitis
clinical features
treatment
prognosis
url https://www.frontiersin.org/articles/10.3389/fimmu.2023.1084883/full
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