Neurolymphomatosis: A report of 2 cases representing opposite ends of the clinical spectrum

Muscle Nerve. 2015 Sep;52(3):449-54. doi: 10.1002/mus.24646. Epub 2015 May 14.

Abstract

Introduction: Neurolymphomatosis (NL) is a rare disorder characterized by invasion of cranial or peripheral nerves, nerve roots, or plexi, usually by aggressive subtypes of non-Hodgkin lymphoma (NHL). The most common clinical presentation is that of a painful polyneuropathy or polyradiculopathy, followed by cranial neuropathy and, less frequently, by painless polyneuropathy.

Methods: Clinical and pathologic findings are reported for 2 NL cases.

Results: The following 2 patients with NL, with disparate clinical presentations, are presented: a patient with subacute onset, painful, multifocal, mixed axonal and demyelinating radiculoplexus neuropathy due to a large B-cell NHL, who required 2 targeted fascicular nerve biopsies to demonstrate NL; and a patient with a slowly progressive, length-dependent axonal polyneuropathy due to a low-grade B-cell lymphoproliferative disorder, as shown on a diagnostic sural nerve biopsy.

Conclusions: The cases described illustrate the wide clinical spectrum of NL.

Keywords: lymphoproliferative disorder; neurolymphomatosis; neuropathy; non-Hodgkin lymphoma; radiculoneuropathy.

Publication types

  • Case Reports

MeSH terms

  • Disease Progression
  • Humans
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Lymphoma, Large B-Cell, Diffuse / physiopathology
  • Male
  • Middle Aged
  • Neoplasm Grading
  • Peripheral Nervous System Neoplasms / pathology
  • Peripheral Nervous System Neoplasms / physiopathology
  • Sciatic Nerve / pathology