Quiz Form

Question Date: 04/07/2023

Question: Which of these statements is true regarding POEMS (Polyneuropathy, organomegaly, endocrinopathy, Monoclonal plasma cell disorder and Skin changes) syndrome?

Options:

• It is associated with IgM anti MAG antibody
• POEMS syndrome should be considered in a poorly responsive CIDP
• Demyelination with motor conduction blocks are seen on electrophysiology

Correct Answer: POEMS syndrome should be considered in a poorly responsive CIDP

Explaination: POEMS syndrome or osteosclerotic myeloma is associated with Ig G or Ig A lambda paraprotein in 90% of patients. Anti MAG antibody is associated with distal acquired demyelinating symmetric (DADS) neuropathy. POEMS syndrome presents with a disabling demyelinating neuropathy mimicking CIDP and should be considered in a poorly responsive CIDP. The electrophysiology shows uniform conduction slowing without conduction blocks. It is associated with sclerotic bone lesions (sclerotic myeloma) or Castleman?s disease. Waldenstrom?s macroglobulinemia evolves from IgM MGUS in elderly males and presents with neuropathy and organomegaly.

Reference: Rison, R.A, Beydoun, S.R. Paraproteinemic neuropathy: a practical review. BMC Neurol 16, 13 (2016)