This is a 76 year-old female with no significant past medical history who presented to her primary care physician for evaluation of changes in visual acuity and color perception. Two months after initial presentation she was referred to see an ophthalmologist who noted bilateral papilledema. An MRA, MRV, and MRI were obtained without intracranial abnormities. A lumbar puncture was performed on three separate occasions, all revealing a normal opening pressure, increased protein, and normal cell counts. During this time, the patient noted a “pins and needles” sensation in her bilateral hands and feet, which over time progressed proximally. She noted worsening weakness manifested by falls at home, difficulty walking up stairs, and difficulty holding cups and began needing a walker to ambulate. Over the next few months the patient developed increasing fatigue, chronic diarrhea, weight gain, lower extremity edema, and abdominal swelling. She was diagnosed with hypothyroidism. Approximately one year after initial presentation, the patient was admitted to our hospital for expedited work-up of possible POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) syndrome. On exam she was noted to have 2+ lower extremity edema, grade 2 ascites, and decreased sensation to light touch bilateral feet and hands. Serum protein electrophoresis revealed a lambda M-component and a bone marrow biopsy revealed a small plasma cell population with lambda light chain predominance. A skeletal survey to evaluate for bone lesions was normal, but a CT scan obtained given a high index of suspicion revealed mixed lytic and sclerotic bony lesions. VEGF levels returned elevated at 248 pg/mL (9-86pg/mL). Further findings are discussed in Table 1. POEMS syndrome was confirmed.
Discussion:
POEMS syndrome is an elusive diagnosis as patients can develop seemingly unrelated systemic symptoms at irregular intervals. A key feature for our patient was the presence of bilateral papilledema, noted in 26-64% of cases POEMS syndrome. The diagnostic picture began to take shape after work-up of more common causes of papilledema was unrevealing and following the development of additional symptoms. Sclerotic bones lesions are present in approximately 97% of cases and their presence may help guide treatment. CT has better sensitivity for small lesions.
Conclusions:
Patients with bilateral papilledema and systemic disease; particularly peripheral neuropathy, should be worked up for POEMS syndrome. When the pre-test probability of POEMS syndrome is high, a skeletal survey alone cannot confirm the absence of skeletal lesions and a more sensitive study, such as a CT scan, should follow up a negative study in order to rule out bony lesions.