title: Pachymeningeal involvement in POEMS syndrome: dramatic cerebral MRI improvement after lenalidomide therapy.
abstract: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystemic disease associated with plasma cell dyscrasia and increased serum or plasma vascular endothelial growth factor (VEGF) levels, the latter likely responsible for several POEMS syndrome manifestations. Whereas peripheral neuropathy is the main neurological feature and a mandatory diagnostic criterium, central nervous system involvement is less common except for papilledema and stroke. We recently reported the frequent occurrence at brain MRI of cranial pachymeningeal involvement ina series of POEMS syndrome patients. Meningeal histopathology revealed hyperplasia of meningothelial cells, neovascularization, and obstructive vessel remodeling without inflammatory signs pointing to a role of VEGF in the meningeal manifestations. Here, we report the dramatic pachymeningeal improvement in patients undergoing lenalidomide therapy. These findings support the therapeutic role of lenalidomide and might shed further light on the pathophysiology of the disease
authors.affiliation: Department of Neurosciences, University of Padova, Padova, Italy. chiara.briani@unipd.it
authors.forename: Chiara
authors.initials: C
authors.lastname: Briani
authors.forename: Renzo
authors.initials: R
authors.lastname: Manara
authors.forename: Federica
authors.initials: F
authors.lastname: Lessi
authors.forename: Valentina
authors.initials: V
authors.lastname: Citton
authors.forename: Renato
authors.initials: R
authors.lastname: Zambello
authors.forename: Fausto
authors.initials: F
authors.lastname: Adami
mesh_terms: D000368:Aged; D005871:Castleman Disease / Q000150:complications / Q000188:drug therapy; D002986:Clinical Trials as Topic; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006801:Humans; D000077269:Lenalidomide; D008279:Magnetic Resonance Imaging; D008297:Male; D008578:Meninges / Q000473:pathology*; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D009101:Multiple Myeloma / Q000150:complications / Q000188:drug therapy; D010014:Osteolysis / Q000188:drug therapy / Q000209:etiology / Q000532:radiotherapy; D016878:POEMS Syndrome / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D011446:Prospective Studies; D013792:Thalidomide / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D042461:Vascular Endothelial Growth Factor A / Q000097:blood
chemical_list: C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D013792:Thalidomide; D003907:Dexamethasone; D000077269:Lenalidomide