338 - Battle of the Demyelinating Polyradiculoneuropathies: CIDP versus POEMS; A Case Report

Friday, February 23, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

AB

Austin M. Boos, DO

Resident Physician
Shirley Ryan/ Northwestern
CHICAGO, Illinois, United States

Co-Author(s)

PH

Peter Hurh, MD

Attending Physician/ Assistant Professor
Jesse Brown VA Medical Center/ Northwestern University Feinberg School of Medicine
Chicago, Illinois, United States

Case Diagnosis: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes

Case Description: A 91-year-old man with MGUS and hypothyroidism was admitted with one month of progressive asymmetric bilateral leg weakness and numbness in his bilateral hands and feet. He was ultimately diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) after electrodiagnostic evaluation showed a mixed sensory-motor predominantly demyelinating polyneuropathy. After a suboptimal response to multiple rounds of IVIG and high-dose daily prednisone, he underwent additional workup including a sural nerve biopsy which showed axonal degeneration and demyelination; a PET- CT scan which was significant for splenomegaly and serum VEGF level which returned elevated. The new findings in the context of his asymmetric polyneuropathy that was refractory to CIDP treatment, and a history of MGUS, hypothyroidism, and lower extremity edema suggested a diagnosis of POEMS syndrome which prompted a referral to hematology for bone marrow biopsy and further treatment.

Discussions: Although CIDP and POEMS Syndrome often present with similar symptoms of subacute motor and sensory changes and predominantly demyelinating features on electrodiagnostic evaluation, the underlying pathophysiologic mechanisms, prognosis, and treatment are distinct. While CIDP traditionally demonstrates a favorable response to IVIG and corticosteroids, POEMS syndrome which is a rare paraneoplastic syndrome with a less favorable prognosis requires treatment of the underlying plasma cell dyscrasia for optimal response. In patients with subacute neurologic symptoms, providers should be aware of the lesser-known features of POEMS syndrome which include signs and symptoms of endocrinopathies, skin changes, and organomegaly which if identified should prompt further diagnostic workup.

Conclusions: Early recognition of the clinical features of POEMS syndrome which distinguish it from other etiologies of subacute weakness and sensory changes such as CIDP, is crucial as it can improve patient outcomes by reducing delays in accurate diagnosis and treatment.