The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome
Susac’s syndrome is a rare and debilitating disease characterized by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. All manifestations may not be clinically apparent at presentation resulting in delayed diagnosis. Early recognition of the syndrome may...
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| Format: | Article |
| Language: | English |
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Wiley
2020-01-01
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| Series: | Case Reports in Neurological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2020/9317232 |
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| author | Suran L. Fernando Therese Boyle Annika Smith John D.E. Parratt |
| author_facet | Suran L. Fernando Therese Boyle Annika Smith John D.E. Parratt |
| author_sort | Suran L. Fernando |
| collection | DOAJ |
| description | Susac’s syndrome is a rare and debilitating disease characterized by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. All manifestations may not be clinically apparent at presentation resulting in delayed diagnosis. Early recognition of the syndrome may prevent disease sequelae such as permanent cognitive, visual, and hearing loss. We present such a case of Susac’s syndrome that was also refractory to conventionally prescribed combination of immunosuppressive treatments including high-dose potent corticosteroids, intravenous cyclophosphamide, methotrexate, plasma exchange, rituximab, and mycophenolate. His disease was stabilized with infliximab in combination with a tapering course of low-dose prednisone. After 2 years of remission with TNF treatment, consideration is being given to ceasing therapy. He has the sequelae of bilateral sensorineural hearing loss but no visual impairment or cognitive deficits on follow-up with neuropsychometric testing. This is the first case report to our knowledge of the successful use of infliximab for a patient with Susac’s syndrome that was necessary following treatment with cyclophosphamide and then rituximab. |
| format | Article |
| id | doaj-art-62920fa9e9094870a25a049e08bcc326 |
| institution | Kabale University |
| issn | 2090-6668 2090-6676 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Neurological Medicine |
| spelling | doaj-art-62920fa9e9094870a25a049e08bcc3262025-02-03T01:28:10ZengWileyCase Reports in Neurological Medicine2090-66682090-66762020-01-01202010.1155/2020/93172329317232The Successful Use of Infliximab in a Relapsing Case of Susac’s SyndromeSuran L. Fernando0Therese Boyle1Annika Smith2John D.E. Parratt3Immunology Laboratory, New South Wales Health Pathology, Royal North Hospital, Sydney, AustraliaImmunology Laboratory, New South Wales Health Pathology, Royal North Hospital, Sydney, AustraliaDepartment of Clinical Immunology and Allergy, Royal North Hospital, Sydney, AustraliaSydney Medical School, University of Sydney, Sydney, AustraliaSusac’s syndrome is a rare and debilitating disease characterized by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. All manifestations may not be clinically apparent at presentation resulting in delayed diagnosis. Early recognition of the syndrome may prevent disease sequelae such as permanent cognitive, visual, and hearing loss. We present such a case of Susac’s syndrome that was also refractory to conventionally prescribed combination of immunosuppressive treatments including high-dose potent corticosteroids, intravenous cyclophosphamide, methotrexate, plasma exchange, rituximab, and mycophenolate. His disease was stabilized with infliximab in combination with a tapering course of low-dose prednisone. After 2 years of remission with TNF treatment, consideration is being given to ceasing therapy. He has the sequelae of bilateral sensorineural hearing loss but no visual impairment or cognitive deficits on follow-up with neuropsychometric testing. This is the first case report to our knowledge of the successful use of infliximab for a patient with Susac’s syndrome that was necessary following treatment with cyclophosphamide and then rituximab.http://dx.doi.org/10.1155/2020/9317232 |
| spellingShingle | Suran L. Fernando Therese Boyle Annika Smith John D.E. Parratt The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome Case Reports in Neurological Medicine |
| title | The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome |
| title_full | The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome |
| title_fullStr | The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome |
| title_full_unstemmed | The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome |
| title_short | The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome |
| title_sort | successful use of infliximab in a relapsing case of susac s syndrome |
| url | http://dx.doi.org/10.1155/2020/9317232 |
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