Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies
BackgroundImmune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Although cerebellar ataxias (CAs) associated with anti-NMDAR and anti-CASPR2 have been reported in a few cases, they...
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| Format: | Article |
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Frontiers Media S.A.
2022-02-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.813926/full |
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| author | Yu Jia Mingyu Li Dawei Li Mengyao Zhang Huifang Wang Lidong Jiao Zhaoyang Huang Zhaoyang Huang Zhaoyang Huang Zhaoyang Huang Jing Ye Jing Ye Jing Ye Aihua Liu Aihua Liu Aihua Liu Yuping Wang Yuping Wang Yuping Wang Yuping Wang |
| author_facet | Yu Jia Mingyu Li Dawei Li Mengyao Zhang Huifang Wang Lidong Jiao Zhaoyang Huang Zhaoyang Huang Zhaoyang Huang Zhaoyang Huang Jing Ye Jing Ye Jing Ye Aihua Liu Aihua Liu Aihua Liu Yuping Wang Yuping Wang Yuping Wang Yuping Wang |
| author_sort | Yu Jia |
| collection | DOAJ |
| description | BackgroundImmune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Although cerebellar ataxias (CAs) associated with anti-NMDAR and anti-CASPR2 have been reported in a few cases, they have never been studied systematically. This study aimed to analyze the characteristics of anti-NSAbs-associated CAs.MethodsA retrospective investigation was conducted to identify patients using the keywords IMCAs and NSAbs. We collected the clinical data of 14 patients diagnosed with anti-NSAbs-associated CAs.ResultsThe median age was 33 years (16-66), and the male-to-female ratio was 4:3. Nine were positive for NMDAR-Ab, two for LGI1-Ab, two for CASPR2-Ab, and one for AMPA2R-Ab. CAs were initial symptoms in three patients and presented during the first two months of the disease course (10 days on average) among the rest of the patients. After the immunotherapy, two cases were free from symptoms, and eight cases recovered satisfactorily (10/14, 71.4%). Compared with other causes of IMCAs, anti-NSAbs were more frequently associated with additional extra-cerebellar symptoms (85.7%), mostly seizures (78.6%) and mental abnormalities (64.3%). In the CSF analysis, pleocytosis was detected in ten patients (71.4%) and oligoclonal bands (OB) were observed in nine patients (64.3%). Moreover, compared with PCD and anti-GAD65-Ab-associated CAs, anti-NSAbs-associated CAs showed a better response to immunotherapy.ConclusionIMCAs are rare and atypical in autoimmune encephalitis with neuronal surface antibodies. Compared with other forms of IMCAs, more symptoms of encephalopathy, a higher rate of pleocytosis and positive OB in CSF, and positive therapeutic effect were the key features of anti-NSAbs-associated CAs. |
| first_indexed | 2024-12-17T11:59:27Z |
| format | Article |
| id | doaj.art-8a28133476324e98b0bc7cff76876831 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-17T11:59:27Z |
| publishDate | 2022-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-8a28133476324e98b0bc7cff768768312022-12-21T21:49:55ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-02-011310.3389/fimmu.2022.813926813926Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface AntibodiesYu Jia0Mingyu Li1Dawei Li2Mengyao Zhang3Huifang Wang4Lidong Jiao5Zhaoyang Huang6Zhaoyang Huang7Zhaoyang Huang8Zhaoyang Huang9Jing Ye10Jing Ye11Jing Ye12Aihua Liu13Aihua Liu14Aihua Liu15Yuping Wang16Yuping Wang17Yuping Wang18Yuping Wang19Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaBeijing Key Laboratory of Neuromodulation, Capital Medical University, Beijing, ChinaCenter of Epilepsy, Beijing Institute for Brain Disorders, Capital Medical University, Ministry of Science and Technology, Beijing, ChinaInstitute of Sleep and Consciousness Disorders, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaBeijing Key Laboratory of Neuromodulation, Capital Medical University, Beijing, ChinaCenter of Epilepsy, Beijing Institute for Brain Disorders, Capital Medical University, Ministry of Science and Technology, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaBeijing Key Laboratory of Neuromodulation, Capital Medical University, Beijing, ChinaCenter of Epilepsy, Beijing Institute for Brain Disorders, Capital Medical University, Ministry of Science and Technology, Beijing, ChinaDepartment of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, ChinaBeijing Key Laboratory of Neuromodulation, Capital Medical University, Beijing, ChinaCenter of Epilepsy, Beijing Institute for Brain Disorders, Capital Medical University, Ministry of Science and Technology, Beijing, ChinaInstitute of Sleep and Consciousness Disorders, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, ChinaBackgroundImmune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Although cerebellar ataxias (CAs) associated with anti-NMDAR and anti-CASPR2 have been reported in a few cases, they have never been studied systematically. This study aimed to analyze the characteristics of anti-NSAbs-associated CAs.MethodsA retrospective investigation was conducted to identify patients using the keywords IMCAs and NSAbs. We collected the clinical data of 14 patients diagnosed with anti-NSAbs-associated CAs.ResultsThe median age was 33 years (16-66), and the male-to-female ratio was 4:3. Nine were positive for NMDAR-Ab, two for LGI1-Ab, two for CASPR2-Ab, and one for AMPA2R-Ab. CAs were initial symptoms in three patients and presented during the first two months of the disease course (10 days on average) among the rest of the patients. After the immunotherapy, two cases were free from symptoms, and eight cases recovered satisfactorily (10/14, 71.4%). Compared with other causes of IMCAs, anti-NSAbs were more frequently associated with additional extra-cerebellar symptoms (85.7%), mostly seizures (78.6%) and mental abnormalities (64.3%). In the CSF analysis, pleocytosis was detected in ten patients (71.4%) and oligoclonal bands (OB) were observed in nine patients (64.3%). Moreover, compared with PCD and anti-GAD65-Ab-associated CAs, anti-NSAbs-associated CAs showed a better response to immunotherapy.ConclusionIMCAs are rare and atypical in autoimmune encephalitis with neuronal surface antibodies. Compared with other forms of IMCAs, more symptoms of encephalopathy, a higher rate of pleocytosis and positive OB in CSF, and positive therapeutic effect were the key features of anti-NSAbs-associated CAs.https://www.frontiersin.org/articles/10.3389/fimmu.2022.813926/fullimmune-mediated cerebellar ataxianeuronal surface antibodiesCSF analysisoligoclonal bandsimmunotherapy |
| spellingShingle | Yu Jia Mingyu Li Dawei Li Mengyao Zhang Huifang Wang Lidong Jiao Zhaoyang Huang Zhaoyang Huang Zhaoyang Huang Zhaoyang Huang Jing Ye Jing Ye Jing Ye Aihua Liu Aihua Liu Aihua Liu Yuping Wang Yuping Wang Yuping Wang Yuping Wang Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies Frontiers in Immunology immune-mediated cerebellar ataxia neuronal surface antibodies CSF analysis oligoclonal bands immunotherapy |
| title | Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies |
| title_full | Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies |
| title_fullStr | Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies |
| title_full_unstemmed | Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies |
| title_short | Immune-Mediated Cerebellar Ataxia Associated With Neuronal Surface Antibodies |
| title_sort | immune mediated cerebellar ataxia associated with neuronal surface antibodies |
| topic | immune-mediated cerebellar ataxia neuronal surface antibodies CSF analysis oligoclonal bands immunotherapy |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.813926/full |
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