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A 71-year-old male with 4 decades of symptoms referable to both central and peripheral nervous system

Dianna Quan1, B K Kleinschmidt-DeMasters

  • 1Department of Neurology, University of Colorado Health Sciences Center, Denver, USA.

Brain Pathology (Zurich, Switzerland)
|January 5, 2006
PubMed

Insights

This case report details a rare disorder involving combined central and peripheral nervous system demyelination, presenting diagnostic challenges and highlighting the importance of autopsy in understanding complex neurological conditions like multiple sclerosis (MS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Area of Science:

  • Neurology
  • Pathology

Background:

  • Combined central and peripheral nervous system demyelination is an uncommon neurological disorder with varied terminology.
  • Accurate diagnosis can be challenging due to overlapping symptoms with other conditions like Guillain-Barrè Syndrome (GBS), Friedreich ataxia, and multiple sclerosis (MS).

Observation:

  • A 71-year-old male presented with a progressive nervous system disorder starting in his 30s.
  • Clinical diagnoses during his lifetime were uncertain, including GBS, Friedreich ataxia, or MS.
  • Autopsy revealed central nervous system (CNS) demyelination consistent with MS and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with onion bulb formation in peripheral nerves.

Findings:

  • Autopsy confirmed co-existing CNS demyelination (MS-like) and peripheral demyelination (CIDP).
  • Microscopic examination showed onion bulb formation in both CNS and peripheral nerves.
  • The case underscores the complexity of diagnosing and classifying combined demyelinating disorders.

Implications:

  • This case contributes to understanding the nosology of combined central and peripheral demyelinating diseases.
  • Highlights the utility of post-mortem examination in clarifying complex neurological diagnoses.
  • Provides insights for differentiating MS and CIDP when they co-occur.

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