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Amyloid neuropathy simulating lower motor neuron disease

A Quattrini1, R Nemni, B Sferrazza

  • 1Department of Neurology, San Raffaele Scientific Institute, University of Milan, Italy.

Neurology
|August 26, 1998
PubMed
Summary

A man with leg weakness was diagnosed with amyloidosis, a rare disease involving abnormal protein deposits, after a nerve biopsy revealed nerve fiber damage and amyloid. Diagnosis was challenging until the nerve biopsy.

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Area of Science:

  • Neurology
  • Pathology
  • Nephrology

Background:

  • Amyloidosis is a rare condition characterized by the buildup of abnormal proteins (amyloid) in organs and tissues.
  • Light chain amyloidosis, specifically, involves the deposition of immunoglobulin light chains, often associated with plasma cell disorders.
  • Early diagnosis of amyloidosis can be challenging due to its diverse and often subtle initial symptoms.

Observation:

  • A 57-year-old male presented with progressive, symmetric leg weakness and fasciculations.
  • Electromyography indicated denervation in the leg muscles, suggesting a neuromuscular disorder.
  • A motor nerve biopsy showed axonal degeneration, loss of myelinated fibers, and amyloid deposits.

Findings:

  • The nerve biopsy revealed amyloid deposition composed of lambda light chains.

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  • Serum analysis later detected lambda light chains, confirming systemic involvement.
  • The patient subsequently developed sensory and autonomic symptoms, typical of advanced amyloidosis.

    • Implications:

    • This case highlights the diagnostic utility of nerve biopsy in suspected amyloidosis, especially when other tests are inconclusive.
    • Early identification of lambda light chain amyloidosis is crucial for timely management and potentially slowing disease progression.
    • Neuromuscular symptoms can be the initial presentation of systemic amyloidosis, underscoring the need for comprehensive diagnostic workups.