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Subacute necrotizing encephalopathy (Leigh's disease): two unusual cases
Southern Medical Journal
|October 1, 1976
Summary
Subacute necrotizing encephalopathy, a rare neurological disorder, presents with unusual symptoms like hirsutism and agenesis of the corpus callosum. Electron microscopy reveals axonal swelling and myelin sheath abnormalities, offering insights into its pathology.
Area of Science:
- Neurology
- Pathology
- Electron Microscopy
Background:
- Subacute necrotizing encephalopathy (SNE) is a rare, progressive neurological disorder.
- Understanding the underlying pathology is crucial for diagnosis and potential treatments.
Observation:
- Two unusual cases of SNE are presented.
- One case featured significant hirsutism, suggesting potential endocrine involvement.
- The second case exhibited agenesis of the corpus callosum, a rare association with SNE.
Findings:
- Electron microscopy of Leigh's disease (a form of SNE) revealed marked axonal swelling.
- Abnormalities in the myelin sheath, including lamellar splitting, were observed, explaining the spongy appearance under light microscopy.
- Striated muscle tissue appeared histologically normal in the examined case.
Implications:
- These findings highlight the diverse clinical presentations of SNE.
- Ultrastructural analysis provides critical insights into the pathomechanisms of SNE.
- The exclusion of postmortem artifact strengthens the validity of the observed cellular changes.