Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy
Introduction: From its initial report on two female patients in 1979 by J.O. Susac, Susac syndrome (SuS) or SICRET (small infarctions of cochlear, retinal and encephalic tissue) has persisted as an elusive entity. To date the available evidence for its treatment is based on case reports and case ser...
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| Format: | Article |
| Language: | English |
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Elsevier
2018-03-01
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| Series: | eNeurologicalSci |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2405650217300394 |
| _version_ | 1811255369351036928 |
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| author | Enrique Gomez-Figueroa Sofia Garcia-Trejo Raul Anwar Garcia-Santos Gerardo Quiñones-Pesqueira Juan Manuel Calleja-Castillo |
| author_facet | Enrique Gomez-Figueroa Sofia Garcia-Trejo Raul Anwar Garcia-Santos Gerardo Quiñones-Pesqueira Juan Manuel Calleja-Castillo |
| author_sort | Enrique Gomez-Figueroa |
| collection | DOAJ |
| description | Introduction: From its initial report on two female patients in 1979 by J.O. Susac, Susac syndrome (SuS) or SICRET (small infarctions of cochlear, retinal and encephalic tissue) has persisted as an elusive entity. To date the available evidence for its treatment is based on case reports and case series. The largest systematic review described only 304 reported cases since the 1970s. Here we presented the first reported case to our knowledge in Mexican population and the unusual presentation in a pregnant patient. Case presentation: A 34-year-old Hispanic woman was brought to the ER in our hospital for apathy and behavioral changes. Upon arrival at the ER, her husband described a one-month history of behavioral changes with apathy, progressive abulia, visuospatial disorientation, and gait deterioration. The initial lab test shows no significance except by a positive qualitative hCG. An MRI was obtained and showed hyperintense periventricular white matter lesions in T2 and FLAIR sequences also involving bilateral basal ganglia and with predominant affection of the corpus callosum, in addition to infratentorial cerebellar lesions. After treatment with intravenous immunoglobulins a marked and prompt clinical and radiological improvement was observed. Conclusion: SuS is still an elusive disease. To date, no definitive score or clinical feature can predict the outcome of the disease. The presentation during pregnancy is also rare and therefore the optimal treatment and the prognosis is unknown. We hope that this article will serve as a foundation for future research. Keywords: Susac syndrome, Neuroinflammation, Corpus callosum, Demyelinating disease, Vasculitis |
| first_indexed | 2024-04-12T17:23:46Z |
| format | Article |
| id | doaj.art-abc0538bead24a8e984e56e8b1c311c1 |
| institution | Directory Open Access Journal |
| issn | 2405-6502 |
| language | English |
| last_indexed | 2024-04-12T17:23:46Z |
| publishDate | 2018-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | eNeurologicalSci |
| spelling | doaj.art-abc0538bead24a8e984e56e8b1c311c12022-12-22T03:23:24ZengElseviereNeurologicalSci2405-65022018-03-011014Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancyEnrique Gomez-Figueroa0Sofia Garcia-Trejo1Raul Anwar Garcia-Santos2Gerardo Quiñones-Pesqueira3Juan Manuel Calleja-Castillo4Corresponding author at: Instituto Nacional de Neurologia y Neurocirugia Manuel Velasco Suarez, Insurgentes Sur 3877, Tlalpan, Mexico city, Mexico.; Neurology Department, Instituto Nacional de Neurologia y Neurocirugia, Mexico City, MexicoNeurology Department, Instituto Nacional de Neurologia y Neurocirugia, Mexico City, MexicoNeurology Department, Instituto Nacional de Neurologia y Neurocirugia, Mexico City, MexicoNeurology Department, Instituto Nacional de Neurologia y Neurocirugia, Mexico City, MexicoNeurology Department, Instituto Nacional de Neurologia y Neurocirugia, Mexico City, MexicoIntroduction: From its initial report on two female patients in 1979 by J.O. Susac, Susac syndrome (SuS) or SICRET (small infarctions of cochlear, retinal and encephalic tissue) has persisted as an elusive entity. To date the available evidence for its treatment is based on case reports and case series. The largest systematic review described only 304 reported cases since the 1970s. Here we presented the first reported case to our knowledge in Mexican population and the unusual presentation in a pregnant patient. Case presentation: A 34-year-old Hispanic woman was brought to the ER in our hospital for apathy and behavioral changes. Upon arrival at the ER, her husband described a one-month history of behavioral changes with apathy, progressive abulia, visuospatial disorientation, and gait deterioration. The initial lab test shows no significance except by a positive qualitative hCG. An MRI was obtained and showed hyperintense periventricular white matter lesions in T2 and FLAIR sequences also involving bilateral basal ganglia and with predominant affection of the corpus callosum, in addition to infratentorial cerebellar lesions. After treatment with intravenous immunoglobulins a marked and prompt clinical and radiological improvement was observed. Conclusion: SuS is still an elusive disease. To date, no definitive score or clinical feature can predict the outcome of the disease. The presentation during pregnancy is also rare and therefore the optimal treatment and the prognosis is unknown. We hope that this article will serve as a foundation for future research. Keywords: Susac syndrome, Neuroinflammation, Corpus callosum, Demyelinating disease, Vasculitishttp://www.sciencedirect.com/science/article/pii/S2405650217300394 |
| spellingShingle | Enrique Gomez-Figueroa Sofia Garcia-Trejo Raul Anwar Garcia-Santos Gerardo Quiñones-Pesqueira Juan Manuel Calleja-Castillo Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy eNeurologicalSci |
| title | Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy |
| title_full | Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy |
| title_fullStr | Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy |
| title_full_unstemmed | Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy |
| title_short | Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy |
| title_sort | exquisite response to intravenous immunoglobulin in susac syndrome during pregnancy |
| url | http://www.sciencedirect.com/science/article/pii/S2405650217300394 |
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