A Rare Case of Refractory Papilledema as an Initial Presentation of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, Skin Changes (POEMS) Syndrome
Ashish Kumar Shrestha1, Raman Shrestha1, Casey Judge1, Negar Moheb1
1Lehigh Valley Fleming Neuroscience Institute
Objective:
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Background:

POEMS syndrome is a rare paraneoplastic monoclonal plasma cell disorder with manifestations involving multiple organ systems. Ocular manifestations include papilledema and macular edema. The pathophysiology of papilledema in POEMS is not fully understood but may result from increased vascular permeability due to imbalance between pro-inflammatory, angiogenic, and anti-inflammatory cytokines.

Design/Methods:
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Results:

A 29-year-old female presented with four weeks of headache and transient visual obscurations. Ophthalmology exam demonstrated bilateral papilledema. Lumbar puncture revealed an opening pressure of 26 cm H2O, WBCs of 2 /cmm, elevated protein at 138 m/dl. MRI brain and orbits demonstrated segmental T2 hyperintensity along the left optic nerve and indirect signs of intracranial hypertension. MRI spine revealed diffuse sclerotic bone changes and enhancement of cauda equina nerve roots. CT scan revealed splenomegaly and lymphadenopathy. On serum testing, she had elevated levels of kappa and lambda light chains, platelets (406,000/µL) and VEGF (297, ref. 9-86 pg/mL). IgA lambda band monoclonal protein was present on serum protein electrophoresis. Acetazolamide 1500 mg daily was started with improvement of visual symptoms and papilledema. 

One year later, she had a recurrence of transient visual obscurations. Optical Coherence Tomography revealed thickening of peripapillary retinal nerve fiber layer OU with mean 185 micrometers OD and 349 micrometers OS, prompting additional testing. Bone marrow biopsy showed clonal plasma cells with lymphoid aggregates of mixed B and T cells. There was moderate sensorimotor demyelinating predominant polyneuropathy on electrodiagnostic testing. With these new findings, she met the diagnostic criteria for POEMS syndrome. Dexamethasone, lenalidomide, and daratumumab immunotherapy were initiated with consideration to consolidate stem cell transplant. At 1-month follow-up post treatment, her papilledema improved (83 micrometers OD, 141 micrometers OS). 

Conclusions:

This patient’s work-up for refractory papilledema led to the diagnosis of POEMS syndrome. She was then initiated on appropriate immunotherapy with improved papilledema.

10.1212/WNL.0000000000216627
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