POEMS syndrome: a neuromuscular perspective

Area of Science:

• Neurology
• Hematology
• Genetics

Background:

• POEMS syndrome is a rare multisystemic disorder linked to plasma cell dyscrasias.
• It frequently presents with peripheral neuropathy, often misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy.
• The condition's complexity and heterogeneity contribute to diagnostic challenges and delayed management.

Purpose of the Study:

• To highlight key differentiating features of POEMS syndrome.
• To emphasize the role of vascular endothelial growth factor (VEGF) in its pathophysiology.
• To underscore the importance of early diagnosis and multidisciplinary management.

Main Methods:

• Review of clinical, neurophysiological, and imaging characteristics.
• Discussion of diagnostic criteria and laboratory investigations, including VEGF levels and monoclonal protein screening.
• Overview of therapeutic strategies based on disease extent.

Main Results:

• Peripheral nerve involvement in POEMS syndrome is primarily demyelinating with early axonal degeneration.
• Elevated VEGF levels are central to the pathophysiology, disrupting the blood-nerve barrier.
• Specific diagnostic features aid in differentiating POEMS from other demyelinating neuropathies.

Conclusions:

• Accurate diagnosis of POEMS syndrome requires a multidisciplinary approach and advanced testing.
• Early identification and intervention are critical to prevent irreversible nerve damage and optimize patient prognosis.
• Prompt recognition of characteristic findings improves treatment efficacy and long-term outcomes.