Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies
ObjectiveWe present a case of autoimmune cerebellar ataxia (ACA) associated with Homer protein homolog 3 (Homer-3) antibodies. Then, a review of the literature was conducted to summarize its clinical spectrum to improve clinicians' understanding of this rare entity.Case presentationA 25-year-ol...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-07-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2022.951659/full |
| _version_ | 1797859402812751872 |
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| author | Qisi Wu Beibei Gong Anan Jiang Xinyue Qin |
| author_facet | Qisi Wu Beibei Gong Anan Jiang Xinyue Qin |
| author_sort | Qisi Wu |
| collection | DOAJ |
| description | ObjectiveWe present a case of autoimmune cerebellar ataxia (ACA) associated with Homer protein homolog 3 (Homer-3) antibodies. Then, a review of the literature was conducted to summarize its clinical spectrum to improve clinicians' understanding of this rare entity.Case presentationA 25-year-old man suffered from the subacute onset of cerebellar ataxia and psychiatric symptoms with abnormalities in the cerebellum on initial brain MRI and Homer-3 antibodies titers of 1:100 in the serum. His neurological symptoms did not improve after intravenous methylprednisolone but significantly improved following plasma exchange with a modified Rankin Scale (mRS) score of 1. However, 5 months later, he experienced relapse during oral prednisone tapering with enhanced cerebellar lesions and obvious cerebellar atrophy on repeated MRI. Various immunomodulatory approaches, including corticosteroids and plasma exchange, were utilized with no improvement. Then rituximab was given for the first time to treat Homer-3 autoimmunity with partial improvement of symptoms. However, the patient remained profoundly disabled with an mRS score of 4.ConclusionACA associated with Homer-3 antibodies may have a suboptimal response to corticosteroid therapy. More intense immunotherapy such as rituximab may contribute to the improvement of cerebellar syndrome. Relapsing courses and presentation of cerebellar atrophy may suggest a poor prognosis in this entity. |
| first_indexed | 2024-04-09T21:29:56Z |
| format | Article |
| id | doaj.art-22906ff74c3140c68111721bbe433ffd |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-09T21:29:56Z |
| publishDate | 2022-07-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-22906ff74c3140c68111721bbe433ffd2023-03-27T09:59:49ZengFrontiers Media S.A.Frontiers in Neurology1664-22952022-07-011310.3389/fneur.2022.951659951659Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodiesQisi Wu0Beibei Gong1Anan Jiang2Xinyue Qin3Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, ChinaDepartment of Radiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, ChinaDepartment of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, ChinaDepartment of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, ChinaObjectiveWe present a case of autoimmune cerebellar ataxia (ACA) associated with Homer protein homolog 3 (Homer-3) antibodies. Then, a review of the literature was conducted to summarize its clinical spectrum to improve clinicians' understanding of this rare entity.Case presentationA 25-year-old man suffered from the subacute onset of cerebellar ataxia and psychiatric symptoms with abnormalities in the cerebellum on initial brain MRI and Homer-3 antibodies titers of 1:100 in the serum. His neurological symptoms did not improve after intravenous methylprednisolone but significantly improved following plasma exchange with a modified Rankin Scale (mRS) score of 1. However, 5 months later, he experienced relapse during oral prednisone tapering with enhanced cerebellar lesions and obvious cerebellar atrophy on repeated MRI. Various immunomodulatory approaches, including corticosteroids and plasma exchange, were utilized with no improvement. Then rituximab was given for the first time to treat Homer-3 autoimmunity with partial improvement of symptoms. However, the patient remained profoundly disabled with an mRS score of 4.ConclusionACA associated with Homer-3 antibodies may have a suboptimal response to corticosteroid therapy. More intense immunotherapy such as rituximab may contribute to the improvement of cerebellar syndrome. Relapsing courses and presentation of cerebellar atrophy may suggest a poor prognosis in this entity.https://www.frontiersin.org/articles/10.3389/fneur.2022.951659/fullautoimmune cerebellar ataxiaHomer-3 antibodybrain MRI enhanced abnormalitiesrelapserituximab |
| spellingShingle | Qisi Wu Beibei Gong Anan Jiang Xinyue Qin Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies Frontiers in Neurology autoimmune cerebellar ataxia Homer-3 antibody brain MRI enhanced abnormalities relapse rituximab |
| title | Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies |
| title_full | Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies |
| title_fullStr | Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies |
| title_full_unstemmed | Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies |
| title_short | Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies |
| title_sort | case report and literature analysis autoimmune cerebellar ataxia associated with homer 3 antibodies |
| topic | autoimmune cerebellar ataxia Homer-3 antibody brain MRI enhanced abnormalities relapse rituximab |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2022.951659/full |
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