POEMS syndrome: a unique presentation and a diagnostic challenge

BMJ Case Rep. 2019 Dec 3;12(12):e230284. doi: 10.1136/bcr-2019-230284.

Abstract

We present the case of a previously well, 70-year-old lady who presented with gait deterioration and a clinicoradiological picture of myositis with T2-enhancement on MRI of the distal lower limb muscles. A muscle biopsy pointed towards neurogenic amyotrophy and a mixed demyelinating and axonal sensorimotor polyneuropathy was confirmed on nerve conduction studies. This was initially thought to be secondary to a myeloproliferative disorder but a positron emission tomography scan showed uptake in the left hip which corresponded to what was previously reported as a subchondral cyst on CT. A biopsy showed this to be a plasmacytoma in the context of a normal serum protein electrophoresis and a polyclonal increase in light chains on serum-free light chain estimation. The patient was started on treatment 6 months after the presentation for a diagnosis of POEMS syndrome, by which time, the patient had become bed-bound and fully dependent.

Keywords: Haematology (incl blood transfusion); Muscle disease; Musculoskeletal and joint disorders; Peripheral nerve disease; Radiology.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Diagnosis, Differential
  • Female
  • Gait Disorders, Neurologic / etiology*
  • Humans
  • Magnetic Resonance Imaging
  • Myositis / etiology
  • POEMS Syndrome / complications
  • POEMS Syndrome / diagnosis*
  • Plasmacytoma / complications
  • Plasmacytoma / diagnosis