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Fulminant demyelinating neuropathy mimicking cerebral death
N Bakshi1, R A Maselli, S M Gospe
1Department of Neurology, University of California-Davis, 95616, USA.
Muscle & Nerve
|December 9, 1997
Summary
Guillain-Barré syndrome rarely mimics brain death with paralysis and cranial nerve issues. This case highlights vincristine-induced peripheral neuropathy, confirmed by electrodiagnostics and pathology, presenting as demyelination.
Area of Science:
- Neurology
- Pediatrics
- Toxicology
Background:
- Guillain-Barré syndrome (GBS) is an autoimmune disorder affecting the peripheral nervous system.
- Rarely, GBS can manifest as acute quadriparesis and cranial nerve palsies, mimicking a locked-in state or brain death.
Observation:
- A pediatric case presented with symptoms resembling brain death following vincristine exposure.
- The patient exhibited acute quadriparesis and cranial nerve involvement.
Findings:
- Electrodiagnostic studies were crucial in differentiating the condition from brain death, diagnosing a peripheral neuropathy.
- Pathologic findings and serial electrodiagnostic studies confirmed demyelination as the underlying cause.
Implications:
- This case underscores the importance of electrodiagnostic studies in diagnosing atypical presentations of neurological disorders.
- It highlights vincristine as a potential neurotoxin in pediatric patients, leading to severe peripheral neuropathy.
- Recognizing this rare presentation is vital for appropriate diagnosis and management in critical care settings.