Highlights
- •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.
Abstract
Objective
To analyze the clinical characteristics of patients with POEMS presenting with polyneuropathy
and discuss associated diagnostic challenges.
Method
Retrospective analysis of 6 patients from 2 tertiary-care institutions.
Results
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.
Conclusion
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.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- POEMS syndrome: update on diagnosis, risk-stratification, and management.Am. J. Hematol. 2015; 90: 951-962
- Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy.J. Neurol. Neurosurg. Psychiatry. 2012; 83: 476-479
- Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis.Brain. 2005; 128: 1911-1920
- Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy.Muscle Nerve. 2015; 52: 658-659
- 18F-FDG PET/CT in the evaluation of POEMS syndrome.Eur. J. Radiol. 2010; 76: 180-182
- Usefulness of (18)F-FDG PET/CT for detecting lesions in patients with POEMS syndrome.Rinsho Ketsueki. 2012; 53: 2013-2017
- POEMS syndrome diagnosed 10 years after disabling peripheral neuropathy.Case Rep. Med. 2011; 2011: 126209
- POEMS syndrome - a case report revealing a complex evolving diagnosis.Clin. Case Rep. 2015; 3: 60-63
- The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome.Blood. 2011; 118: 4663-4665
- Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP.J. Neurol. Neurosurg. Psychiatry. 2012; 83: 480-486
- Patterns of nerve conduction abnormalities in POEMS syndrome.Muscle Nerve. 2002; 26: 189-193
- CIDP diagnostic pitfalls and perception of treatment benefit.Neurology. 2015; 85: 498-504
- Spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy.Brain. 1999; 122: 1383-1390
- A prospective phase II trial of lenalidomide and dexamethasone (LEN-DEX) in POEMS syndrome.Blood. 2014; 124: 36
- Polyneuropathy improvement following autologous stem cell transplantation for POEMS syndrome.Neurology. 2015; 84: 1981-1987
Article info
Publication history
Published online: May 11, 2017
Accepted:
May 10,
2017
Received in revised form:
April 26,
2017
Received:
March 18,
2017
Identification
Copyright
© 2017 Elsevier B.V. All rights reserved.
