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Primary systemic amyloidosis presenting with demyelinating features.

A Gutierrez1, L J Turkewitz, H Correa

  • 1Louisiana State University Health Sciences Center, Department of Neurology and Neuropathology, New Orleans, LA 70112 , USA. agutie@lsuhsc.edu

Neurology, Neurophysiology, and Neuroscience
|January 30, 2007
PubMed
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Primary systemic amyloidosis can cause an atypical polyneuropathy. Consider amyloidosis in patients with demyelinating polyneuropathy for earlier diagnosis and treatment.

Area of Science:

  • Neurology
  • Internal Medicine
  • Pathology

Background:

  • Primary systemic amyloidosis is a rare multisystemic disorder.
  • Small-fiber axonal polyneuropathy is the most common neurologic manifestation.
  • Diagnosis is often delayed when neuropathy is the presenting feature.

Observation:

  • Two cases of primary systemic amyloidosis presenting with multifocal polyneuropathy with demyelinating features are described.
  • Autopsy-proven amyloidosis was confirmed in both cases.
  • The polyneuropathy exhibited demyelinating characteristics.

Findings:

  • Primary systemic amyloidosis can manifest as a demyelinating polyneuropathy, not exclusively axonal.
  • This presentation can mimic other demyelinating neuropathies, complicating diagnosis.

Related Experiment Videos

  • Autopsy confirmed the presence of amyloidosis in patients with these atypical neuropathic features.

    • Implications:

    • Amyloidosis should be included in the differential diagnosis for patients presenting with demyelinating polyneuropathy.
    • Recognizing this atypical presentation can lead to earlier diagnosis of systemic amyloidosis.
    • Prompt diagnosis of amyloidosis is crucial for timely management and improved patient outcomes.