Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report
Anti-GAD ataxia is one of the most common forms of immune-mediated cerebellar ataxias. Many neurological syndromes have been reported in association with anti-GAD. Ophthalmoparesis has been described in stiff person syndrome. We report a case of anti-GAD ataxia presenting initially with isolated oph...
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| Format: | Article |
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MDPI AG
2021-07-01
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| Series: | Brain Sciences |
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| Online Access: | https://www.mdpi.com/2076-3425/11/7/932 |
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| author | Abel Dantas Belém Thaís de Maria Frota Vasconcelos Rafael César dos Anjos de Paula Francisco Bruno Santana da Costa Pedro Gustavo Barros Rodrigues Isabelle de Sousa Pereira Paulo Roberto de Arruda Tavares Gabriela Studart Galdino Daniel Aguiar Dias Carolina de Figueiredo Santos Manoel Alves Sobreira-Neto Pedro Braga-Neto Paulo Ribeiro Nobrega |
| author_facet | Abel Dantas Belém Thaís de Maria Frota Vasconcelos Rafael César dos Anjos de Paula Francisco Bruno Santana da Costa Pedro Gustavo Barros Rodrigues Isabelle de Sousa Pereira Paulo Roberto de Arruda Tavares Gabriela Studart Galdino Daniel Aguiar Dias Carolina de Figueiredo Santos Manoel Alves Sobreira-Neto Pedro Braga-Neto Paulo Ribeiro Nobrega |
| author_sort | Abel Dantas Belém |
| collection | DOAJ |
| description | Anti-GAD ataxia is one of the most common forms of immune-mediated cerebellar ataxias. Many neurological syndromes have been reported in association with anti-GAD. Ophthalmoparesis has been described in stiff person syndrome. We report a case of anti-GAD ataxia presenting initially with isolated ophthalmoplegia and showing complete resolution after immunotherapy. A 26-year-old male patient presented with ophthalmoparesis characterized by tonic upwards deviation of the right eye. In the following month, he developed progressive ataxia with anti-GAD titers of 1972 UI/mL. After treatment with methylprednisolone and immunoglobulin, there was complete resolution of symptoms and anti-GAD titers decreased. This is the first report of isolated ophthalmoparesis due to tonic eye deviation associated with anti-GAD antibodies without stiff-person syndrome. Tonic eye deviation has been reported in SPS, possibly secondary to continuous discharge in gaze holding neurons in the brainstem (similar to what occurs in spinal motor neurons). With growing evidence for ocular abnormalitites in SPS, anti-GAD associated neurological syndromes should be included in the differential diagnosis of isolated ophthalmoplegia. |
| first_indexed | 2024-03-10T09:44:20Z |
| format | Article |
| id | doaj.art-b6b6dd5af4314862bf72627f7ea4f2b5 |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-03-10T09:44:20Z |
| publishDate | 2021-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-b6b6dd5af4314862bf72627f7ea4f2b52023-11-22T03:20:59ZengMDPI AGBrain Sciences2076-34252021-07-0111793210.3390/brainsci11070932Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case ReportAbel Dantas Belém0Thaís de Maria Frota Vasconcelos1Rafael César dos Anjos de Paula2Francisco Bruno Santana da Costa3Pedro Gustavo Barros Rodrigues4Isabelle de Sousa Pereira5Paulo Roberto de Arruda Tavares6Gabriela Studart Galdino7Daniel Aguiar Dias8Carolina de Figueiredo Santos9Manoel Alves Sobreira-Neto10Pedro Braga-Neto11Paulo Ribeiro Nobrega12Division of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilHospital Infantil Albert Sabin, Fortaleza 60410-790, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilDivision of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Fortaleza 60430-140, BrazilAnti-GAD ataxia is one of the most common forms of immune-mediated cerebellar ataxias. Many neurological syndromes have been reported in association with anti-GAD. Ophthalmoparesis has been described in stiff person syndrome. We report a case of anti-GAD ataxia presenting initially with isolated ophthalmoplegia and showing complete resolution after immunotherapy. A 26-year-old male patient presented with ophthalmoparesis characterized by tonic upwards deviation of the right eye. In the following month, he developed progressive ataxia with anti-GAD titers of 1972 UI/mL. After treatment with methylprednisolone and immunoglobulin, there was complete resolution of symptoms and anti-GAD titers decreased. This is the first report of isolated ophthalmoparesis due to tonic eye deviation associated with anti-GAD antibodies without stiff-person syndrome. Tonic eye deviation has been reported in SPS, possibly secondary to continuous discharge in gaze holding neurons in the brainstem (similar to what occurs in spinal motor neurons). With growing evidence for ocular abnormalitites in SPS, anti-GAD associated neurological syndromes should be included in the differential diagnosis of isolated ophthalmoplegia.https://www.mdpi.com/2076-3425/11/7/932anti-GAD ataxiacerebellar ataxiaophthalmoplegiastiff person syndromeautoimmune |
| spellingShingle | Abel Dantas Belém Thaís de Maria Frota Vasconcelos Rafael César dos Anjos de Paula Francisco Bruno Santana da Costa Pedro Gustavo Barros Rodrigues Isabelle de Sousa Pereira Paulo Roberto de Arruda Tavares Gabriela Studart Galdino Daniel Aguiar Dias Carolina de Figueiredo Santos Manoel Alves Sobreira-Neto Pedro Braga-Neto Paulo Ribeiro Nobrega Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report Brain Sciences anti-GAD ataxia cerebellar ataxia ophthalmoplegia stiff person syndrome autoimmune |
| title | Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report |
| title_full | Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report |
| title_fullStr | Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report |
| title_full_unstemmed | Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report |
| title_short | Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report |
| title_sort | stiff eye syndrome anti gad ataxia presenting with isolated ophthalmoplegia a case report |
| topic | anti-GAD ataxia cerebellar ataxia ophthalmoplegia stiff person syndrome autoimmune |
| url | https://www.mdpi.com/2076-3425/11/7/932 |
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