Primary amyloidosis presenting as upper limb multiple mononeuropathies

Muscle Nerve. 2010 May;41(5):710-5. doi: 10.1002/mus.21561.

Abstract

Peripheral neuropathy in primary (AL) amyloidosis is usually lower-limb predominant, length-dependent, symmetrical, and affects small (pain and autonomic) fibers, as much or more than large fibers. We report a patient with stepwise progressive, multiple upper limb mononeuropathies that were due to nerve biopsy-proven primary amyloidosis (lambda light chain), with no systemic or autonomic features. Recognition that light chain amyloidosis may be the cause of a multiple mononeuropathy pattern adds to the differential diagnosis of this clinical phenotype.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Aged
  • Amyloid Neuropathies / pathology*
  • Amyloid Neuropathies / physiopathology
  • Amyloidosis / complications
  • Amyloidosis / pathology*
  • Amyloidosis / physiopathology
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • Arm / innervation
  • Arm / physiopathology
  • Biomarkers / analysis
  • Biomarkers / metabolism
  • Biopsy
  • Diagnosis, Differential
  • Disease Progression
  • Electrodiagnosis
  • Female
  • Humans
  • Immunoglobulin Light Chains / analysis
  • Immunoglobulin Light Chains / metabolism
  • Immunoglobulins, Intravenous / therapeutic use
  • Mononeuropathies / genetics
  • Mononeuropathies / pathology*
  • Mononeuropathies / physiopathology
  • Neural Conduction / physiology
  • Peripheral Nerves / metabolism
  • Peripheral Nerves / pathology*
  • Peripheral Nerves / physiopathology
  • Predictive Value of Tests
  • Rituximab
  • Sensitivity and Specificity
  • Treatment Failure

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Biomarkers
  • Immunoglobulin Light Chains
  • Immunoglobulins, Intravenous
  • Rituximab