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Severe cranial nerve involvement in longstanding demyelinating polyneuropathy: a clinicopathologic correlation
E L McCann1, T W Smith, D A Chad
1Department of Pathology, University of Massachusetts Medical Center, Worcester 01655, USA.
Acta Neuropathologica
|January 1, 1996
Summary
This study details a rare case of acquired demyelinating polyneuropathy with onion bulb formations affecting cranial nerves. The findings highlight severe neurogenic atrophy and hypertrophic neuropathy in a patient with progressive neurological decline.
Area of Science:
- Neuropathology
- Clinical Neurology
Background:
- Onion bulb formations are an uncommon finding in neuropathology.
- Acquired demyelinating polyneuropathies can present with diverse clinical and histological features.
Observation:
- A 69-year-old man presented with progressive muscle weakness, sensory ataxia, and multiple cranial nerve abnormalities.
- Electrodiagnostic studies indicated an acquired demyelinating polyneuropathy.
- Despite treatment with corticosteroids and plasmapheresis, the patient's neurological status did not improve.
Findings:
- Postmortem examination revealed widespread onion bulb formations in cranial nerves (III, IV, V, VI, X, XI, XII), spinal nerve roots, dorsal root ganglia, and peripheral nerves.
- Histological analysis showed foci of epineurial perivascular inflammation and severe neurogenic atrophy in muscle sections.
- The cranial nerves exhibited repetitive demyelination and remyelination cycles.
Implications:
- This case illustrates that longstanding acquired demyelinating neuropathy can lead to cranial nerve involvement with hypertrophic changes.
- The findings contribute to understanding the pathological spectrum of demyelinating neuropathies.
- Repetitive demyelination/remyelination cycles in cranial nerves can result in significant bulbar muscle weakness.