title: POEMS syndrome with prominent acute axonal lesions.
abstract: Polyneuropathy is a common presenting component of POEMS syndrome whose symptoms are attributed to an overproduction of vascular endothelial growth factor (VEGF). We report two female patients with POEMS syndrome presenting as a severe predominantly axonal neuropathy. A nerve biopsy was performed for these patients; pathological data confirmed unusual numerous acute axonal lesions associated with other classical signs of POEMS syndrome. POEMS syndrome is usually associated with demyelinating neuropathy (and secondary axonal loss); however, prominent axonal neuropathy (with acute axonal lesions on nerve biopsy) can also be observed in this disease. These observations illustrate the heterogeneity of peripheral nervous system involvement in POEMS syndrome.
authors: Department of Neurology, CHU Poitiers, University of Poitiers, 2 rue de la Milétrie, 86021, Poitiers Cedex, France. s.mathis@chu-poitiers.fr
authors: S
authors: S
authors: Mathis
authors: L
authors: L
authors: Magy
authors: R
authors: R
authors: Kaboré
authors: F
authors: F
authors: Faugeras
authors: L
authors: L
authors: Richard
authors: J-M
authors: JM
authors: Vallat
mesh_terms: D000208:Acute Disease; D000328:Adult; D001369:Axons / Q000473:pathology* / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology