Table of Contents
Washington University Experience | PERIPHERAL NEUROPATHY | 13 POEMS SYNDROME | 2A0 Case 2 History
Case 2 History ---- The patient is a 61-year-old male with IgA lambda monoclonal gammopathy, sclerotic bone lesions, edema, and a recent 30 pound weight loss, who presented with subacute progressive mixed axonal and demyelinating polyneuropathy. Physical exam showed leg edema and weakness, more prominent distally in the arms and legs with severe sensory loss diffusely. He has sclerotic lesions in the skeleton, an IgA lambda monoclonal protein (IgA is 660), bone marrow showed a plasma cell neoplasm and an infiltrative cardiomyopathy. ANA is 1:320. VEGF was normal at 42. Rheumatoid factor was mildly elevated. Serum testing shows neurofilament light level of 424 (normal 5-40 pg/ml). FGFR3 antibodies are 6000 (normal levels are typically below 3000). Electrodiagnostic testing showed a neuropathy with axonal and demyelinating features. Demyelinating features included prolonged distal motor and sensory latencies and uniform slowing of intermediate motor conduction velocities. Gene testing was normal including for TTR. PET scan showed multiple osseous lesions. Clinical question: Rule out amyloid or POEMS. Operative procedure: sural nerve and gastrocnemius biopsies.
