2016
DOI: 10.1007/s00347-016-0287-8
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POEMS-Syndrom als seltene Ursache eines bilateralen Papillenödems

Abstract: The POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare paraneoplastic syndrome based on a clonal plasma cell disorder. Optic disc edema (ODE) is a frequent ocular sign in POEMS syndrome. The cause of the ODE has not yet been entirely clarified. This article reports the case of a 62-year-old male suffering from POEMS syndrome with a bilateral ODE.

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“…Additional case reports have been published to substantiate the relationship between papilledema as an initial presenting symptom and POEMS Syndrome for patients between 16 and 62 years. [134][135][136][137][138][139][140] The same studies have also outlined the presence of cystic macular edema (CME). [138][139][140][141] The etiology of ODE is thought to be elevated intracranial pressure and CSF protein, increased vascular permeability due to cytokines, inflammation (vasculitis), and infiltration.…”
Section: Systemic Amyloidosismentioning
confidence: 99%
“…Additional case reports have been published to substantiate the relationship between papilledema as an initial presenting symptom and POEMS Syndrome for patients between 16 and 62 years. [134][135][136][137][138][139][140] The same studies have also outlined the presence of cystic macular edema (CME). [138][139][140][141] The etiology of ODE is thought to be elevated intracranial pressure and CSF protein, increased vascular permeability due to cytokines, inflammation (vasculitis), and infiltration.…”
Section: Systemic Amyloidosismentioning
confidence: 99%
“…Its definitive diagnosis requires the presence of polyneuropathy and proof of clonal plasma cell proliferation that produces a monoclonal paraprotein, usually of the lambda type. POEMS syndrome is a rare entity, with an estimated prevalence of 0.3 cases per 100,000 inhabitants per year; the acronym, created by Barwick in 1980, refers to several of the syndrome´s distinctive features (polyneuropathy, organomegaly, endocrinopathy, measurable monoclonal component and cutaneous alterations); additionally, patients exhibit other manifestations such as: Papilledema (usually bilateral) [1]; extravascular volume overload revealed by edemas, pleural and pericardial effusion and ascites; sclerotic bone lesions and hematological alterations such as thrombocytosis or erythrocytosis [2].…”
Section: Introductionmentioning
confidence: 99%