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Case 12 - Polyneuropathy, Organomegaly, Endocrine Manifestations, Monoclonal Protein, and Skin Changes (POEMS) Syndrome

from Peripheral Neuropathies

Published online by Cambridge University Press:  aN Invalid Date NaN

Jessica E. Hoogendijk
Affiliation:
University Medical Center Utrecht
Marianne de Visser
Affiliation:
Amsterdam University Medical Center
Pieter A. van Doorn
Affiliation:
Erasmus MC, University Medical Center, Rotterdam
Erik H. Niks
Affiliation:
Leiden University Medical Center
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Summary

A 49-year-old man noticed a diminished sensation in his feet. Two months later, climbing stairs became difficult due to proximal muscle weakness of his legs. Over the next three months, sensory disturbances and both proximal and distal weakness progressed in arms and legs requiring the use of a walker or a wheelchair. Besides COPD, he was previously in a healthy condition. Based upon ancillary investigations, he was initially diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), had received a course of intravenous Ig (IVIg), and was subsequently treated with high-dose corticosteroids (60 mg/day). He did not smoke or use drugs such as nitric oxide.

Type
Chapter
Information
Neuromuscular Disease
A Case-Based Approach
, pp. 104 - 106
Publisher: Cambridge University Press
Print publication year: 2024

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References

Suggested Reading

Bou Zerdan, M, George, TI, Bunting, ST, Chaulagain, CP. Recent advances in the treatment and supportive care of POEMS syndrome. J Clin Med 2022;11(23):7011. doi: 10.3390/jcm11237011. PMID: 36498588; PMCID: PMC9741379.CrossRefGoogle ScholarPubMed
Dispenzieri, A. POEMS syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol 2019;94(7):812827. doi: 10.1002/ajh.25495. Epub 2019 May 23. PMID: 31012139.CrossRefGoogle ScholarPubMed
D’Sa, S, Khwaja, J, Keddie, S, et al. Comprehensive diagnosis and management of POEMS syndrome. Hemasphere 2022;6(11):e796. doi: 10.1097/HS9.0000000000000796. PMID: 36340912; PMCID: PMC9624442.CrossRefGoogle ScholarPubMed
Khouri, J, Nakashima, M, Wong, S. Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome: a review. JAMA Oncol 2021;7(9):13831391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097.CrossRefGoogle ScholarPubMed
Kim, YR. Update on the POEMS syndrome. Blood Res 2022;57(S1):2731. doi: 10.5045/br.2022.2022001. PMID: 35483922; PMCID: PMC9057663.CrossRefGoogle ScholarPubMed

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