TOUCH MEDICAL MEDIA 94 Case Report Neuromuscular Diseases Print Publication Date: November 6, 2018 The Challenge of Distinguishing POEMS from Chronic Infammatory Demyelinating Polyneuropathy—Importance of Early Recognition and Diagnosis of POEMS Frances Chow, 1 Leila Darki 2 and Said R Beydoun 2 1. Department of Neurology, Keck School of Medicine, University of Southern California, Los Angeles, CA, US; 2. Neuromuscular Division, Department of Neurology, Keck School of Medicine, University of Southern California, Los Angeles, CA, US. P OEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome. Keywords POEMS syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), demyelinating neuropathy, vascular endothelial growth factor (VEGF), monoclonal gammopathy Disclosures: Frances Chow, Leila Darki and Said R Beydoun have no relevant conficts to declare. Review Process: Double-blind peer review. Compliance with Ethics: Procedures were followed in accordance with the responsible committee on human experimentation and with the Helsinki Declaration of 1975 and subsequent revisions. Informed consent was received from the patient for publication of this case report. Authorship: All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship of this manuscript, take responsibility for the integrity of the work as a whole, and have given fnal approval to the version to be published. Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, adaptation, and reproduction provided the original authors and source are given appropriate credit. © The Authors 2018. Received: March 18, 2018 Accepted: May 14, 2018 Citation: US Neurology. 2018;14(2):94–7 Corresponding Author: Frances Chow, Department of Neurology, Keck School of Medicine, University of Southern California, 1520 San Pablo St, Suite 3000, Los Angeles, CA 90033, US. E: Frances.Chow@med.usc.edu Support: No funding was received in the publication of this article. POEMS syndrome is a rare paraneoplastic syndrome characterized by a complex constellation of systemic findings in the setting of a proliferative cell disorder. Named for peripheral neuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes, these findings are predominant among several other associated features of the syndrome. We describe a case in which the diagnosis of POEMS was suspected in the setting of neuropathy with skin changes, and was confirmed by subsequent hematological evaluation. Special emphasis in this case report is placed on the neurological clinical suspicion and workup in identifying a life-threatening condition, particularly the clinical and diagnostic challenge of distinguishing POEMS from chronic inflammatory demyelinating polyneuropathy (CIDP). Our experience with this patient reinforces the importance of early recognition and diagnosis of POEMS. Case report A 44-year-old previously healthy male presented to his primary care provider with 2 weeks of dull bilateral foot pain localized at the great toes and aggravated by movement. He denied any injury to the toe or erythema of the joint. Examination at that time was notable for absent sensation to light touch and monofilament over the distal parts of the feet. Radiographic imaging demonstrated mild bilateral hallux valgus deformities with small bunions, without any fracture, dislocation, or suspicious osseous lesion. At a subsequent visit, 3 months after the onset of symptoms, an electrodiagnostic study was requested. It revealed a sensory-motor neuropathy with length-dependent characteristics (Table 1, Table 2). Pathophysiology was consistent with a demyelinating process with secondary axonal loss. Diagnosis of CIDP was considered by the electromyographer. The patient was subsequently lost to follow-up and presented 8 months later at the Neurology Clinic with progressive weakness and worsening distal sensory dysesthesias. Basic neuropathy workup for treatable causes was unremarkable. Cerebrospinal fluid (CSF) showed albuminocytologic dissociation with a protein of 98 mg/dL. The patient was then referred to our Neuromuscular Clinic with a clinical diagnosis of CIDP and recommendation for intravenous immunoglobulin therapy (IVIG). Additional history obtained indicated an unintentional weight loss of 50 pounds over 8 months and intermittent headache with blurry vision over 3 months. Motor examination (based on the Medical Research Council Motor Examination scale DOI: https://doi.org/10.17925/USN.2018.14.2.94