E-Pearl of the Week: Differentiating POEMS from CIDP

August 13, 2012

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Brought to you by the Resident and Fellow Section of Neurology®.

August 7, 2012

Differentiating POEMS from CIDP

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome, is a rare cause of demyelinating neuropathy. The polyneuropathy is often an initial manifestation and the disorder can be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). Some features might help the clinician differentiate between those two. For example, patients with POEMS neuropathy more frequently have severe leg pain, muscle atrophy and distal dominant muscle weakness. Electrophysiologically, POEMS patients have greater reduction of motor amplitudes, greater slowing of motor and sensory conduction velocities, less prolonged motor distal latencies, less frequent temporal dispersion and conduction block, no sural sparing, greater number of fibrillation potentials in a length dependent pattern, and higher terminal latency indices. Levels of vascular endothelial growth factor and interleukin 12 are increased in POEMS syndrome and may be useful for diagnosis and as a measure of disease activity.

Reference

1. Nasu S, Misawa S, Sekiguchi Y, et al. Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2012; 83: 476–479.
2. Mauermann ML, Sorenson EJ, Dispenzieri A, et al. . Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. J Neurol Neurosurg Psychiatry 2012; 83: 480–486.
3. Kanai K, Sawai, S, Sogawa K. et al. Markedly upregulated serum interleukin–12 as a novel biomarker in POEMS syndrome. Neurology; 79: 575–582.

Submitted by: Chafic Karam, MD

Disclosures: Dr. Karam serves on the editorial team for the Neurology® Resident and Fellow Section.

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