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Published on: July 29, 2015
Pathological evidence of vacuolar myelinopathy in a child following vigabatrin administration
Myles Horton1, Mubeen Rafay, Marc R Del Bigio
1Section of Neurology, Department of Medicine, University of Manitoba, Winnipeg, Canada.
Abstract:
Vigabatrin, a gamma-aminobutyric acid (GABA) aminotransferase- inhibiting drug used for seizure control, has been associated with white matter vacuolation and intramyelinic edema in animal studies. Similar pathological lesions have never been described in the central nervous system of human participants treated with the drug. Described here is a child with quadriparetic cerebral palsy secondary to hypoxic-ischemic brain injury following premature birth, who received vigabatrin for the treatment of infantile spasms at 9 months of age. A severe deterioration of neurologic function immediately followed the initiation of vigabatrin, and the child died 3 weeks later. Neuropathological examination revealed white matter vacuolation and intramyelinic edema. This represents the first reported case of vigabatrin-induced intramyelinic edema in humans. It validates the concerns regarding vigabatrin safety in infants and individuals with preexisting abnormalities of myelin.
Insights
Vigabatrin, an anti-seizure drug, caused white matter vacuolation and edema in a human infant, mirroring animal study findings. This case highlights potential risks in infants, especially those with prior myelin issues.
Area of Science:
- Neuroscience
- Pharmacology
- Pediatric Neurology
Background:
- Vigabatrin is a gamma-aminobutyric acid (GABA) aminotransferase inhibitor used to control seizures.
- Animal studies linked vigabatrin to white matter vacuolation and intramyelinic edema.
- These lesions had not been previously documented in human patients receiving vigabatrin.
Observation:
- A child with cerebral palsy received vigabatrin for infantile spasms at nine months of age.
- Neurologic function rapidly declined after vigabatrin initiation.
- The child died three weeks after starting the medication.
Findings:
- Neuropathological examination revealed white matter vacuolation and intramyelinic edema.
- This is the first documented human case of vigabatrin-induced intramyelinic edema.
- The findings align with observations from animal studies.
Implications:
- This case validates concerns about vigabatrin's safety profile in infants.
- It underscores potential risks for individuals with pre-existing myelin abnormalities.
- Further caution is warranted when prescribing vigabatrin to vulnerable pediatric populations.
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