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Increased intracranial pressure in adrenoleukodystrophy.
Archives of Neurology
|March 1, 1984
Summary
Adrenoleukodystrophy can cause increased intracranial pressure due to brain edema. This rare complication was observed in a 5-year-old boy with elevated CSF protein and significant white matter changes.
Area of Science:
- Neurology
- Pediatrics
- Biochemistry
Background:
- Adrenoleukodystrophy (ALD) is a rare genetic disorder affecting the adrenal glands and white matter of the brain.
- Increased intracranial pressure (ICP) is a serious complication that can occur in ALD, particularly in childhood forms.
- Understanding the mechanisms of ICP in ALD is crucial for patient management.
Observation:
- A 5-year-old boy with ALD presented with increased intracranial pressure as a terminal event.
- Cerebrospinal fluid (CSF) analysis revealed elevated protein concentration (420 mg/dL).
- Computed tomography (CT) demonstrated extensive white matter demyelination in the posterior brain regions.
Findings:
- Autopsy revealed a 20% increase in brain weight and significantly elevated white matter water content (87.6% vs. expected 72.5%).
- These findings are consistent with severe cerebral edema.
- The case highlights the potential role of brain edema in precipitating increased ICP in ALD.
Implications:
- Brain edema should be considered as a potential contributor to increased intracranial pressure in pediatric adrenoleukodystrophy.
- Early recognition and management of cerebral edema may be critical in ALD cases.
- Further research into the pathophysiology of edema in ALD is warranted.