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Marked cerebrospinal fluid collection associated with hepatic failure
T Enomoto1, M Hashikawa, T Nose
1Department of Neurosurgery, University of Tsukuba, Ibaraki, Japan.
Brain & Development
|January 1, 1988
Summary
Two patients with abnormal cerebrospinal fluid (CSF) accumulation faced poor outcomes. Underlying conditions like ornithine transcarbamylase deficiency and hyperammonemia contributed to severe encephalopathy, despite CSF management.
Area of Science:
- Neurology
- Metabolic Disorders
- Pediatrics
Background:
- Cerebrospinal fluid (CSF) accumulation can occur due to various pathologies.
- Effective management of intracranial pressure is crucial in neurological emergencies.
Observation:
- Case 1: Marked ventricular dilatation treated with a ventriculoperitoneal shunt, which proved ineffective.
- Case 2: Subdural effusion following hepatic failure and septicemia, managed with drainage and Ommaya reservoirs.
Findings:
- Case 1 diagnosed with ornithine transcarbamylase deficiency, a urea cycle disorder.
- Both cases experienced severe encephalopathy, likely due to hyperammonemia, leading to poor outcomes.
- Computed tomography (CT) scans demonstrated serial changes in CSF accumulation.
Implications:
- Highlights the importance of considering metabolic disorders in unexplained neurological conditions.
- Suggests hyperammonemia as a significant factor in neurological deterioration, even with CSF control.
- Emphasizes the need for comprehensive diagnostic approaches beyond CSF management.