title: POEMS Syndrome in a Juvenile Initially Diagnosed as Treatment Resistant Chronic Inflammatory Demyelinating Polyneuropathy.
abstract: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a disorder that mainly affects adults. We report a pediatric patient, initially considered to have Guillain-Barré syndrome, who continued to have progression of neuropathic disease leading to the diagnosis of chronic inflammatory demyelinating polyneuropathy. Diagnosis of POEMS was established by an abnormal bone marrow biopsy, prompted by laboratory and imaging findings, which became abnormal later in the course of the disease. POEMS syndrome is extremely rare in children, and neuropathic features in this age group have not been previously described. This case illustrates that "Guillain-Barré syndrome-like" initial presentation for POEMS, which has not been previously reported. It also emphasizes that in children with progressive acquired neuropathies that are treatment unresponsive, POEMS syndrome should be considered.
authors.affiliation: Departments of *Neurology; †Radiology; and ‡Pathology, University of Texas Health Science Center at Houston, Houston, TX.
authors.forename: Sonia N
authors.initials: SN
authors.lastname: Krish
authors.forename: Thy
authors.initials: T
authors.lastname: Nguyen
authors.forename: Suur
authors.initials: S
authors.lastname: Biliciler
authors.forename: Manickam
authors.initials: M
authors.lastname: Kumaravel
authors.forename: Amer
authors.initials: A
authors.lastname: Wahed
authors.forename: Semyon
authors.initials: S
authors.lastname: Risin
authors.forename: Kazim A
authors.initials: KA
authors.lastname: Sheikh
mesh_terms: D000293:Adolescent; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D016878:POEMS Syndrome / Q000175:diagnosis*; D010525:Peripheral Nerves / Q000473:pathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000503:physiopathology*; D049268:Positron-Emission Tomography