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November 2021; 8 (6) ArticleOpen Access

Neurological Autoimmunity Associated With Homer-3 Antibody

A Case Series From China

Mange Liu, Haitao Ren, Siyuan Fan, Weihe Zhang, Yao Xu, Weili Zhao, Hongzhi Guan, and the Encephalitis Collaborative Group
First published September 27, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001077
Mange Liu
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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  • For correspondence: 15635158759@163.com
Haitao Ren
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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  • For correspondence: rht20080808@163.com
Siyuan Fan
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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  • For correspondence: siyuanfan@outlook.com
Weihe Zhang
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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  • For correspondence: z_weihe@hotmail.com
Yao Xu
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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  • For correspondence: yzxyao@aliyun.com
Weili Zhao
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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  • For correspondence: wlzhao1968@163.com
Hongzhi Guan
From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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From the Department of Neurology (M.L., H.R., S.F., H.G.), Peking Union Medical College Hospital, Beijing; Peking Union Medical College and Chinese Academy of Medical Sciences (M.L., H.R., S.F., H.G.), Beijing; Department of Neurology (Weihe Zhang), China-Japan Friendship Hospital, Beijing; Department of Neurology (Y.X.), Medical Collage of Yangzhou University, Jiangsu; and Department of Neurology (Weili Zhao), Affiliated Hospital of Chifeng University, Inner Mongolia, China.
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Citation
Neurological Autoimmunity Associated With Homer-3 Antibody
A Case Series From China
Mange Liu, Haitao Ren, Siyuan Fan, Weihe Zhang, Yao Xu, Weili Zhao, Hongzhi Guan, and the Encephalitis Collaborative Group
Neurol Neuroimmunol Neuroinflamm Nov 2021, 8 (6) e1077; DOI: 10.1212/NXI.0000000000001077

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Abstract

Background and Objective To present 6 new cases with Homer-3 antibodies that expand their clinical spectra and to evaluate the effect of immunotherapy.

Methods Patients with suspected autoimmune cerebellar disorder were tested for rare autoimmune cerebellar ataxia (ACA) antibodies (anti-Tr(DNER)/Zic4/ITPR1/Homer-3/NCDN/PKCγ/PCA-2/AP3B2/mGluR1/ATP1A3 antibodies) using both cell-based and tissue-based assays. Patients with positive serum or CSF results who were diagnosed with ACA were registered and followed up. This study reports and analyzes cases with Homer-3 antibodies.

Results Of the serum and CSF samples of 750 patients tested, 6 were positive for Homer-3 antibodies. All manifested subacute or insidious-onset cerebellar ataxia. Furthermore, 2 patients each exhibited encephalopathy, myeloradiculopathy, REM sleep behavior disorder, and autonomic dysfunction. Brain magnetic resonance images were normal (n = 1) or revealed cerebellar atrophy (n = 1), cerebellum and pons atrophy with the hot cross bun sign (n = 2), and bilateral cerebral abnormalities (n = 2). Definite leukocytosis was identified in the CSF of 2 patients, protein concentration elevation was observed in the CSF of 1 patient, and oligoclonal bands were present in 2 patients. All patients received immunotherapy, including corticosteroid, IV immunoglobulin, plasma exchange, and mycophenolate mofetil, after which the residual disability was still severe (modified Rankin Scale score ≥3 at the last follow-up in 4 patients and final Scale for the Assessment and Rating of Ataxia scores of 12–29), although 4 patients partially improved and 1 patient stabilized. The remaining 1 patient continued to deteriorate after repeated immunotherapy. Two patients relapsed.

Discussion Disorders associated with Homer-3 antibody can mimic multiple system atrophy with cerebellar features in both clinical and radiologic aspects. Accurate identification of autoimmune-mediated cases is critical. Timely, comprehensive immunotherapy is warranted, given the possibility of long-term clinical benefit.

Glossary

ACA=
autoimmune cerebellar ataxia;
RBD=
REM sleep behavior disorder;
MSA-C=
multiple system atrophy with cerebellar features;
IVIg=
IV immunoglobulin

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • ↵* These authors contributed equally to this work.

  • The Article Processing Charge was funded by the authors.

  • Received April 19, 2021.
  • Accepted in final form July 6, 2021.
  • Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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