- •Sensorimotor responses are usually absent in the lower extremities in POEMS syndrome.
- •POEMS should be considered in CIDP patients not responding to immunotherapy.
- •Paraprotein, VEGF and skeletal survey testing are essential in diagnosing POEMS.
- •PET-CT scan is helpful in POEMS patients with negative skeletal survey.
- •Lumbosacral root enhancement on MRI can be detected in POEMS syndrome.
To analyze the clinical characteristics of patients with POEMS presenting with polyneuropathy and discuss associated diagnostic challenges.
Retrospective analysis of 6 patients from 2 tertiary-care institutions.
Six patients presented with progressive sensorimotor deficits predominantly or exclusively in the lower extremities and were diagnosed with POEMS syndrome. In 4 patients, a diagnosis of chronic inflammatory demyelinating polyradiculopathy (CIDP) was mistakenly made. Low concentration of serum paraprotein and elevated vascular endothelial growth factor (VEGF) were detected in all 6 patients. Initial serum immunofixation and skeletal survey were normal in 2 patients each. On nerve conduction studies, motor and sensory responses were absent in the lower extremity in all 6 patients. Conduction velocity slowing and F-wave latency prolongation appear slightly more pronounced than distal motor latency prolongation in the upper extremity. Spinal MRI showed diffuse lumbosacral nerve root enhancement in all. In 3 patients, targeted bone marrow biopsy was needed for confirming the POEMS diagnosis. Treatment with corticosteroids, chemotherapy agent, focal radiation and/or autologous stem cell transplantation led to significant improvement in 5 of 6 patients.
Diagnosis of POEMS should be considered in patients with progressive polyneuropathies of mixed demyelinating and axon loss features, including CIDP patients not responding to standard treatment. A polyradiculoneuropathy rather than a pure polyneuropathy seems to exist in POEMS.
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Published online: May 11, 2017
Accepted: May 10, 2017
Received in revised form: April 26, 2017
Received: March 18, 2017
© 2017 Elsevier B.V. All rights reserved.