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Kennedy Disease Misdiagnosed as Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome: A Case Report

OBJECTIVE: The aim of this paper was to report the first case of Kennedy disease misdiagnosed as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. CLINICAL PRESENTATION AND INTERVENTION: A 58-year-old Chinese man presented with limb numbness, pro...

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Detalles Bibliográficos
Autores principales: Yuan, Min, Chen, Weiping, Zhou, Huangyan, Xiao, Zhilong, Wang, Wei, Wang, Weidong, Yin, Xiaoping, Xu, Lijun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5588369/
https://www.ncbi.nlm.nih.gov/pubmed/26618536
http://dx.doi.org/10.1159/000442822
Descripción
Sumario:OBJECTIVE: The aim of this paper was to report the first case of Kennedy disease misdiagnosed as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. CLINICAL PRESENTATION AND INTERVENTION: A 58-year-old Chinese man presented with limb numbness, progressive limb proximal weakness, lymph node and thyroid enlargement, edema, pigmentation in the lower limb, and obvious gynecomastia, which was initially diagnosed as POEMS syndrome and was treated with dexamethasone and small doses of cyclophosphamide without any improvement after 6 months. Finally, the patient diagnosis was confirmed as Kennedy disease (KD) by gene analysis. CONCLUSION: This case suggests that clinicians should pay more attention to the differential diagnosis between KD and POEMS syndrome. Gene analysis was helpful in detecting this rare confusing disease in this patient.