Inflammatory pseudotumor of nerve: Clinicopathological characteristics and a potential therapy

Michelle L. Mauermann, Bernd W. Scheithauer, Robert J. Spinner, Kimberly K. Amrami, Christopher S. Nance, David G. Kline, Mary I. O'Connor, Peter J. Dyck, Janean Engelstad, P. James B. Dyck

Research output: Contribution to journalArticle

5 Scopus citations

Abstract

We sought to determine the clinical, electrophysiological, neuroimaging, and pathological features of inflammatory pseudotumor of nerve. Five patients were identified. All cases presented with a gradually progressive mononeuropathy with symptoms of weakness, sensory loss, and prominent neuropathic pain. The median duration of symptoms was 7 months (range 3-36 months). Electrophysiological results were in keeping with chronic axonal mononeuropathies with variable findings of active denervation and reinnervation. MRI demonstrated irregular, large masses involving and surrounding nerve with heterogenous signal characteristics on T1-and T2-weighted and post-contrast sequences. Histopathological features of the nerve slightly varied but shared commonalities including chronic inflammatory infiltrates, increased collagen, and increased numbers of microvessels. Axonal degeneration and decreased density of myelinated fibers were also noted. Three patients were treated with weekly courses of intravenous steroids for 3 months. All reported improvement in pain and weakness. Inflammatory pseudotumor of nerve is not a neoplasm and has reactive features of inflammation, increased vascularity, and marked fibrosis. It presents as a progressive axonal mononeuropathy with weakness, sensory loss, and pain that may be episodic. The primary pathophysiology is unknown but the inflammation and response to treatment suggests that there may be an immune component.

Original languageEnglish (US)
Pages (from-to)216-226
Number of pages11
JournalJournal of the Peripheral Nervous System
Volume15
Issue number3
DOIs
StatePublished - Sep 1 2010

Keywords

  • fibrosis
  • inflammation
  • mononeuropathy
  • peripheral neuropathy
  • pseudotumor

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Inflammatory pseudotumor of nerve: Clinicopathological characteristics and a potential therapy'. Together they form a unique fingerprint.

  • Cite this