Magda Delgado-Landa, Selma Scheffler-Mendoza, Eduardo López-Corella, Ana Luisa Rodríguez-Lozano, Francisco Rivas-Larrauri, Melissa Espinosa-Navarro, Hiromi Onuma-Zamayoa, Marco Antonio Yamazaki-Nakashimada
{"title":"Castleman病变异型POEMS综合征表现为儿童多神经根神经病变:1例报告。","authors":"Magda Delgado-Landa, Selma Scheffler-Mendoza, Eduardo López-Corella, Ana Luisa Rodríguez-Lozano, Francisco Rivas-Larrauri, Melissa Espinosa-Navarro, Hiromi Onuma-Zamayoa, Marco Antonio Yamazaki-Nakashimada","doi":"10.1177/08830738251369737","DOIUrl":null,"url":null,"abstract":"<p><p>POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.</p>","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":" ","pages":"8830738251369737"},"PeriodicalIF":1.6000,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.\",\"authors\":\"Magda Delgado-Landa, Selma Scheffler-Mendoza, Eduardo López-Corella, Ana Luisa Rodríguez-Lozano, Francisco Rivas-Larrauri, Melissa Espinosa-Navarro, Hiromi Onuma-Zamayoa, Marco Antonio Yamazaki-Nakashimada\",\"doi\":\"10.1177/08830738251369737\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.</p>\",\"PeriodicalId\":15319,\"journal\":{\"name\":\"Journal of Child Neurology\",\"volume\":\" \",\"pages\":\"8830738251369737\"},\"PeriodicalIF\":1.6000,\"publicationDate\":\"2025-09-26\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Child Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1177/08830738251369737\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Child Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1177/08830738251369737","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.
期刊介绍:
The Journal of Child Neurology (JCN) embraces peer-reviewed clinical and investigative studies from a wide-variety of neuroscience disciplines. Focusing on the needs of neurologic patients from birth to age 18 years, JCN covers topics ranging from assessment of new and changing therapies and procedures; diagnosis, evaluation, and management of neurologic, neuropsychiatric, and neurodevelopmental disorders; and pathophysiology of central nervous system diseases.