Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study

Chiara Briani, Marny Fedrigo, Renzo Manara, Chiara Castellani, Renato Zambello, Valentina Citton, Marta Campagnolo, Chiara Dalla Torre, Marta Lucchetta, Enrico Orvieto, Antonino Rotilio, Sabrina Marangoni, Stefania Magi, Davide Pareyson, Igor Florio, Elena Pegoraro, Gaetano Thiene, Leontino Battistin, Fausto Adami, Annalisa Angelini

Research output: Contribution to journalArticle

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare plasma cell disease. Vascular endothelial growth factor (VEGF) seems to play a pathogenic role. Peripheral neuropathy is the main neurological feature. Cranial pachymeningitis has occasionally been reported, but no histopathological studies have been performed. The authors extensively evaluated the central nervous system MRI in 11 patients (seven men, four women; mean age at diagnosis 54.45 years) with POEMS syndrome. In two patients, meningeal histopathology with staining for VEGF and VEGF receptor was performed, and pachymeningeal involvement characterised at histopathological, immunohistochemical and confocal microscopy levels. Nine patients presented with cranial pachymeningitis. One patient suffered from migraine, and none complained of cranial nerve palsies or visual loss. None showed any MRI signs of spinal pachymeningitis. No correlation was found with disease duration and VEGF serum level. Histopathology showed hyperplasia of meningothelial cells, neovascularisation and obstructive vessel remodelling, without inflammation. VEGF and VEGF receptor were strongly coexpressed on endothelium, smooth-muscle cells of arterioles and meningothelial cells. In conclusion, POEMS patients present a high prevalence of meningeal involvement. The histological changes, different from those present in chronic pachymeningitis of other aetiology, suggest a possible VEGF role in the pathogenesis of the meningeal remodelling.

Original languageEnglish
Pages (from-to)33-37
Number of pages5
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume83
Issue number1
DOIs
Publication statusPublished - Jan 2012

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Surgery
  • Arts and Humanities (miscellaneous)

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    Briani, C., Fedrigo, M., Manara, R., Castellani, C., Zambello, R., Citton, V., Campagnolo, M., Dalla Torre, C., Lucchetta, M., Orvieto, E., Rotilio, A., Marangoni, S., Magi, S., Pareyson, D., Florio, I., Pegoraro, E., Thiene, G., Battistin, L., Adami, F., & Angelini, A. (2012). Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study. Journal of Neurology, Neurosurgery and Psychiatry, 83(1), 33-37. https://doi.org/10.1136/jnnp-2011-300047