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[Papilloedema and POEMS syndrome]
G H Munteanu1, M Munteanu, M Budău
1Clinica de Oftalmologie, Timişoara.
Oftalmologia (Bucharest, Romania : 1990)
|March 28, 2002
Summary
POEMS syndrome, a rare multisystem disorder involving polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes, was diagnosed in a 16-year-old female. The case highlights diagnostic challenges and discusses current understanding of POEMS syndrome pathogenesis and treatment.
Area of Science:
- Internal Medicine
- Neurology
- Hematology
Background:
- POEMS syndrome is a rare multisystem plasma cell dyscrasia.
- It is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
- Often associated with Castleman disease, its pathogenesis involves inflammatory cytokines and VEGF.
Observation:
- A 16-year-old female presented with a long-standing history of complex clinical symptomatology.
- Diagnosis of POEMS syndrome was established through careful systematization of her symptoms.
- Chronic bilateral papilloedema is a frequent clinical feature, observed in 33-84% of cases.
Findings:
- The case underscores the diagnostic complexities of POEMS syndrome, particularly in younger patients.
- Pathogenesis is multifactorial, with contributions from inflammatory cytokines, VEGF, and potentially HHV-8.
- The patient's diagnosis was based on a comprehensive evaluation of clinical manifestations.
Implications:
- This case highlights the importance of recognizing POEMS syndrome in adolescents presenting with diverse neurological and systemic symptoms.
- Understanding the underlying pathogenesis is crucial for developing targeted therapies.
- Further research into early diagnosis and effective treatment strategies for POEMS syndrome is warranted.