Long term survival and outcomes in patients with paraneoplastic neurologic syndromes
ObjectiveIt is unknown whether delay in diagnosis affects morbidity reportedly in paraneoplastic syndromes (PNS). We aimed to explore various aspects of PNS, including prevalence, clinical characteristics, diagnostic criteria, and treatment outcomes.MethodsWe studied n-PNS diagnosis between 2016 to...
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Frontiers Media S.A.
2024-11-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2024.1466704/full |
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| author | Sapir Bar Mucha Ayal Rozenberg Ayal Rozenberg Lilach Gutter Kapon Alon Gorenshtein Esther Ganelin-Cohen Esther Ganelin-Cohen Rachel Ben Hayun Nataliya Yarovinsky Shahar Shelly Shahar Shelly |
| author_facet | Sapir Bar Mucha Ayal Rozenberg Ayal Rozenberg Lilach Gutter Kapon Alon Gorenshtein Esther Ganelin-Cohen Esther Ganelin-Cohen Rachel Ben Hayun Nataliya Yarovinsky Shahar Shelly Shahar Shelly |
| author_sort | Sapir Bar Mucha |
| collection | DOAJ |
| description | ObjectiveIt is unknown whether delay in diagnosis affects morbidity reportedly in paraneoplastic syndromes (PNS). We aimed to explore various aspects of PNS, including prevalence, clinical characteristics, diagnostic criteria, and treatment outcomes.MethodsWe studied n-PNS diagnosis between 2016 to 2023, and included only patients with positive onconeural antibodies, who developed cancer, and exhibited a recognizable PNS phenotype.ResultsWe identified 12 patients with positive Abs and co-occurring cancer, most prevalent PNS antibodies included anti-GAD65, anti-Recoverin and anti-Yo. The most common phenotypes were limbic encephalitis (n=5, 42%) and encephalomyelitis (n=4,33%). Cancer preceded neurological presentation in 6 cases. Among the 6 patients who initially presented with n-PNS, median time from neurological presentation to oncologic diagnosis was 73 days, as five of them (83%) were diagnosed with cancer during oncological evaluation prompted by the PNS diagnosis or suspicion. Lymphoma was the most frequent cancer (n=3, 25%), followed by lung cancer (n=2, 17%), and ovarian cancer (n=2, 17%). Among patients who received immunotherapy as n-PNS treatment (n=9, 75%), steroids were a part of the management at 78% (n=7). Another immunotherapy used included plasmapheresis (n=5, 55%) and steroid sparing immunosuppressant (n=2, 29%). Four (33%) patients had short term therapeutic benefit with improvement or stabilization at mRS ≤ 4. Median Disability-adjusted life years (DALYs), as disease burden value, was 13 years. Death occurred in 9 of the 12 patients, with most cases deaths attributed to cancer progression. Compering to the expected median survival by type and stage of tumor, from 9 deceased patients, 56% (n=5) died younger than expected. Median survival was 410 days (range 29-2738 days), and 152 days since the appearance of n-PNS (range 8-1434 days). There were no differences in survival between patients who initially presented with n-PNS versus cancer (p=0.39).ConclusionIn up to 8 years of follow up, there was no difference in mortality among patients who presented initially n-PNS. There was a significant decline in the quality of life, most face substantial disability and functional impairment long term. |
| format | Article |
| id | doaj-art-870d6fc528d443da8d212b37841b0b04 |
| institution | Kabale University |
| issn | 1664-3224 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Immunology |
| spelling | doaj-art-870d6fc528d443da8d212b37841b0b042024-11-18T06:10:32ZengFrontiers Media S.A.Frontiers in Immunology1664-32242024-11-011510.3389/fimmu.2024.14667041466704Long term survival and outcomes in patients with paraneoplastic neurologic syndromesSapir Bar Mucha0Ayal Rozenberg1Ayal Rozenberg2Lilach Gutter Kapon3Alon Gorenshtein4Esther Ganelin-Cohen5Esther Ganelin-Cohen6Rachel Ben Hayun7Nataliya Yarovinsky8Shahar Shelly9Shahar Shelly10Department of Neurology, Rambam HealthCare Campus, Haifa, IsraelDepartment of Neurology, Rambam HealthCare Campus, Haifa, IsraelNeuroimmunology Laboratory, Ruth and Bruce Rapaport Faculty of Medicine, Technion – Israel Institute of Technology, Haifa, IsraelNeuroimmunology Laboratory, Rambam HealthCare Campus, Haifa, IsraelAzrieli Faculty of Medicine, Bar-Ilan University, Safed, IsraelNeuroimmunology Clinic, Institute of Pediatric Neurology, Schneider Children’s Medical Center of Israel, Petah Tikva, IsraelFaculty of Medical and Health Sciences, Tel Aviv University, Tel Aviv, IsraelDepartment of Neurology, Rambam HealthCare Campus, Haifa, IsraelDepartment of Neurology, Rambam HealthCare Campus, Haifa, IsraelDepartment of Neurology, Rambam HealthCare Campus, Haifa, IsraelNeuroimmunology Laboratory, Ruth and Bruce Rapaport Faculty of Medicine, Technion – Israel Institute of Technology, Haifa, IsraelObjectiveIt is unknown whether delay in diagnosis affects morbidity reportedly in paraneoplastic syndromes (PNS). We aimed to explore various aspects of PNS, including prevalence, clinical characteristics, diagnostic criteria, and treatment outcomes.MethodsWe studied n-PNS diagnosis between 2016 to 2023, and included only patients with positive onconeural antibodies, who developed cancer, and exhibited a recognizable PNS phenotype.ResultsWe identified 12 patients with positive Abs and co-occurring cancer, most prevalent PNS antibodies included anti-GAD65, anti-Recoverin and anti-Yo. The most common phenotypes were limbic encephalitis (n=5, 42%) and encephalomyelitis (n=4,33%). Cancer preceded neurological presentation in 6 cases. Among the 6 patients who initially presented with n-PNS, median time from neurological presentation to oncologic diagnosis was 73 days, as five of them (83%) were diagnosed with cancer during oncological evaluation prompted by the PNS diagnosis or suspicion. Lymphoma was the most frequent cancer (n=3, 25%), followed by lung cancer (n=2, 17%), and ovarian cancer (n=2, 17%). Among patients who received immunotherapy as n-PNS treatment (n=9, 75%), steroids were a part of the management at 78% (n=7). Another immunotherapy used included plasmapheresis (n=5, 55%) and steroid sparing immunosuppressant (n=2, 29%). Four (33%) patients had short term therapeutic benefit with improvement or stabilization at mRS ≤ 4. Median Disability-adjusted life years (DALYs), as disease burden value, was 13 years. Death occurred in 9 of the 12 patients, with most cases deaths attributed to cancer progression. Compering to the expected median survival by type and stage of tumor, from 9 deceased patients, 56% (n=5) died younger than expected. Median survival was 410 days (range 29-2738 days), and 152 days since the appearance of n-PNS (range 8-1434 days). There were no differences in survival between patients who initially presented with n-PNS versus cancer (p=0.39).ConclusionIn up to 8 years of follow up, there was no difference in mortality among patients who presented initially n-PNS. There was a significant decline in the quality of life, most face substantial disability and functional impairment long term.https://www.frontiersin.org/articles/10.3389/fimmu.2024.1466704/fullparaneoplastic syndromecancer related neurological symptomsencephalitisdiagnosislong term follow-upprognosis |
| spellingShingle | Sapir Bar Mucha Ayal Rozenberg Ayal Rozenberg Lilach Gutter Kapon Alon Gorenshtein Esther Ganelin-Cohen Esther Ganelin-Cohen Rachel Ben Hayun Nataliya Yarovinsky Shahar Shelly Shahar Shelly Long term survival and outcomes in patients with paraneoplastic neurologic syndromes Frontiers in Immunology paraneoplastic syndrome cancer related neurological symptoms encephalitis diagnosis long term follow-up prognosis |
| title | Long term survival and outcomes in patients with paraneoplastic neurologic syndromes |
| title_full | Long term survival and outcomes in patients with paraneoplastic neurologic syndromes |
| title_fullStr | Long term survival and outcomes in patients with paraneoplastic neurologic syndromes |
| title_full_unstemmed | Long term survival and outcomes in patients with paraneoplastic neurologic syndromes |
| title_short | Long term survival and outcomes in patients with paraneoplastic neurologic syndromes |
| title_sort | long term survival and outcomes in patients with paraneoplastic neurologic syndromes |
| topic | paraneoplastic syndrome cancer related neurological symptoms encephalitis diagnosis long term follow-up prognosis |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2024.1466704/full |
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