An Adult with Polyneuropathy and Hypogonadism Due to Poems Syndrome. | [Journal of College of Physicians and Surgeons--Pakistan : JCPSP • 2017]

Author(s):

1. Saba Zaidi: Department of Neurology, The Aga Khan University Hospital, Karachi, Pakistan

2. Sidra Sattar: Department of Community Health Sciences, Ziauddin University Hospital, Karachi, Pakistan

3. Khealani Bhojo Asumal: Department of Neurology, National Medical Centre, Karachi, Pakistan

Abstract:

POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein myeloma and skin changes), is a rare disease which occurs in the setting of plasma cell dyscrasias. We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. Nerve conduction study showed severe sensorimotor polyneuropathy. Serum immunofixation showed lambda light chain restricted monoclonal gammopathy. Bone marrow biopsy consistent with plasma cell dyscrasia. Hormonal assay showed decreased FSH, LH and estradiol levels which led us to diagnosis of hypogonadotrophic hypogonadism. The patient responded well to combination therapy of thalidomide, melphalan and dexamethasone. Eight months after the therapy, she noted decreased paresthesias and increased strength. She had reduced edema and ascites.

Page(s): 651-653

DOI: DOI not available

Published: Journal: Journal of College of Physicians and Surgeons--Pakistan : JCPSP, Volume: 27, Issue: 10, Year: 2017

Keywords:

Polyneuropathy POEMS syndrome Edema Ascites Hyperpigmentation Hypogonadism

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