The Neuropathology of Autoimmune Ataxias
Autoimmune-mediated ataxia has been associated with paraneoplastic disease, gluten enteropathy, Hashimoto thyroiditis as well as autoimmune disorders without a known associated disease. There have been relatively few reports describing the neuropathology of these conditions. This review is an attemp...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-02-01
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| Series: | Brain Sciences |
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| Online Access: | https://www.mdpi.com/2076-3425/12/2/257 |
| _version_ | 1797482165007548416 |
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| author | H. Brent Clark |
| author_facet | H. Brent Clark |
| author_sort | H. Brent Clark |
| collection | DOAJ |
| description | Autoimmune-mediated ataxia has been associated with paraneoplastic disease, gluten enteropathy, Hashimoto thyroiditis as well as autoimmune disorders without a known associated disease. There have been relatively few reports describing the neuropathology of these conditions. This review is an attempt to consolidate those reports and determine the ways in which autoimmune ataxias can be neuropathologically differentiated from hereditary or other sporadic ataxias. In most instances, particularly in paraneoplastic forms, the presence of inflammatory infiltrates is a strong indicator of autoimmune disease, but it was not a consistent finding in all reported cases. Therefore, clinical and laboratory findings are important for assessing an autoimmune mechanism. Such factors as rapid rate of clinical progression, presence of known autoantibodies or the presence of a malignant neoplasm or other autoimmune disease processes need to be considered, particularly in cases where inflammatory changes are minimal or absent and the pathology is largely confined to the cerebellum and its connections, where the disease can mimic hereditary or other sporadic ataxias. |
| first_indexed | 2024-03-09T22:27:18Z |
| format | Article |
| id | doaj.art-c0e3ca314cd3471695d3ada29e2aebb4 |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-03-09T22:27:18Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-c0e3ca314cd3471695d3ada29e2aebb42023-11-23T19:04:02ZengMDPI AGBrain Sciences2076-34252022-02-0112225710.3390/brainsci12020257The Neuropathology of Autoimmune AtaxiasH. Brent Clark0Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, MMC 76, 420 Delaware St. SE, Minneapolis, MN 55455, USAAutoimmune-mediated ataxia has been associated with paraneoplastic disease, gluten enteropathy, Hashimoto thyroiditis as well as autoimmune disorders without a known associated disease. There have been relatively few reports describing the neuropathology of these conditions. This review is an attempt to consolidate those reports and determine the ways in which autoimmune ataxias can be neuropathologically differentiated from hereditary or other sporadic ataxias. In most instances, particularly in paraneoplastic forms, the presence of inflammatory infiltrates is a strong indicator of autoimmune disease, but it was not a consistent finding in all reported cases. Therefore, clinical and laboratory findings are important for assessing an autoimmune mechanism. Such factors as rapid rate of clinical progression, presence of known autoantibodies or the presence of a malignant neoplasm or other autoimmune disease processes need to be considered, particularly in cases where inflammatory changes are minimal or absent and the pathology is largely confined to the cerebellum and its connections, where the disease can mimic hereditary or other sporadic ataxias.https://www.mdpi.com/2076-3425/12/2/257cerebellar degenerationparaneoplasticglutenHashimoto |
| spellingShingle | H. Brent Clark The Neuropathology of Autoimmune Ataxias Brain Sciences cerebellar degeneration paraneoplastic gluten Hashimoto |
| title | The Neuropathology of Autoimmune Ataxias |
| title_full | The Neuropathology of Autoimmune Ataxias |
| title_fullStr | The Neuropathology of Autoimmune Ataxias |
| title_full_unstemmed | The Neuropathology of Autoimmune Ataxias |
| title_short | The Neuropathology of Autoimmune Ataxias |
| title_sort | neuropathology of autoimmune ataxias |
| topic | cerebellar degeneration paraneoplastic gluten Hashimoto |
| url | https://www.mdpi.com/2076-3425/12/2/257 |
| work_keys_str_mv | AT hbrentclark theneuropathologyofautoimmuneataxias AT hbrentclark neuropathologyofautoimmuneataxias |