Yuebing Li1, Jason Valent2, Payam Soltanzadeh3, Nimish Thakore3, Bashar Katirji4. 1. Department of Neurology, Cleveland Clinic, Cleveland, OH 44195, USA. Electronic address: liy@ccf.org. 2. Department of Hematology and Oncology, Cleveland Clinic, Cleveland, OH 44195, USA. 3. Department of Neurology, Cleveland Clinic, Cleveland, OH 44195, USA. 4. Department of Neurology, University Hospitals Cleveland Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA.
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.
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 CIDPpatients not responding to standard treatment. A polyradiculoneuropathy rather than a pure polyneuropathy seems to exist in POEMS.