Publication: POEMS syndrome with venous sinus thrombosis and visual failure: A case report
Issued Date
2005-05-01
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ISSN
01252208
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2-s2.0-23044500237
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Mahidol University
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SCOPUS
Bibliographic Citation
Journal of the Medical Association of Thailand. Vol.88, No.5 (2005), 690-694
Suggested Citation
Rawiphan Witoonpanich, Sriphan Phankhian, Saengsuree Jootar, Anuchit Poonyathalang, Surapon Worapongpaiboon, Suchart Phudhichareonrat, Niramol Chanplakorn POEMS syndrome with venous sinus thrombosis and visual failure: A case report. Journal of the Medical Association of Thailand. Vol.88, No.5 (2005), 690-694. Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/16978
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Title
POEMS syndrome with venous sinus thrombosis and visual failure: A case report
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Abstract
POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome is a multisystem disorder associated with plasma cell dyscrasia. Other clinical signs include clubbing of the fingers, edema, papilledema etc. Although papilledema and increased intracranial pressure are common features, their causes or pathophysiology have been uncertain. The authors report here a 16-year-old Thai patient with these features who also suffered from venous sinus thrombosis and visual failure which have never been reported before. The former is considered to be one of the possible causes of the intracranial hypertension and visual failure. MRI of the brain and optic nerve revealed enhancement and swelling of the optic nerve sheaths and optic discs. MRV findings were compatible with chronic veno-occlusive disease. Bone marrow aspiration and biopsy demonstrated an increase of aggregates of intermediate and mature plasma cells. The CSF pressure was markedly elevated. His clinical condition continued to deteriorate and he expired 3 years and 5 months from the onset of his illness. Although, overproduction of vascular endothelial growth factor has been reported and is being considered to be the possible cause of vascular hyperpermeability, the chronic venous sinus thrombosis may play an important role in the pathogenesis of intracranial hypertension and visual failure.