Splenic marginal zone lymphoma: An indolent malignancy leading to the development of neurolymphomatosis

Muscle Nerve. 2017 Mar;55(3):440-444. doi: 10.1002/mus.25404. Epub 2016 Sep 29.

Abstract

Introduction: Acute neuropathic pain and weakness with a sensory level in a patient with a history of lymphoma has a broad differential diagnosis. Evaluation of such a presentation often includes MRI, neurophysiologic studies, and cerebrospinal fluid evaluation. We report a patient with splenic marginal zone lymphoma who developed acute weakness, sensory loss, and neuropathic pain due to neurolymphomatosis.

Methods: Clinical evaluation, MRI of the lumbar spine, cerebrospinal fluid evaluation, electrodiagnostic (EDx) studies, and biopsy of a dorsal nerve root were undertaken.

Results: EDx studies were consistent with an acute, acquired demyelinating sensorimotor polyradiculoneuropathy. Treatment with intravenous immunoglobulin and plasma exchange did not lead to clinical improvement. Ultimately, biopsy of a dorsal nerve root was performed and revealed neurolymphomatosis.

Conclusion: This case emphasizes that, when it can be performed safely, biopsy for suspected neurolymphomatosis is imperative for appropriate diagnosis and treatment. Muscle Nerve 55: 440-444, 2017.

Keywords: Guillain-Barré syndrome; electromyography and nerve conduction studies; magnetic resonance imaging; nerve biopsy; neurolymphomatosis; splenic marginal zone lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Animals
  • Cauda Equina / diagnostic imaging*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Lymphoma / cerebrospinal fluid
  • Lymphoma / complications*
  • Lymphoma / diagnostic imaging
  • Lymphoma / therapy
  • Magnetic Resonance Imaging
  • Male
  • Marek Disease / diagnostic imaging
  • Marek Disease / etiology*
  • Middle Aged
  • Neoplasms
  • Neural Conduction
  • Plasma Exchange / methods

Substances

  • Immunoglobulins, Intravenous