Pathological evidence of vacuolar myelinopathy in a child following vigabatrin administration.
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.
Horton
Myles
M
Section of Neurology, Department of Medicine, University of Manitoba, Winnipeg, Canada.
Rafay
Mubeen
M
Del Bigio
Marc R
MR
D000927:Anticonvulsants / Q000009:adverse effects*; D001921:Brain / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D001925:Brain Damage, Chronic / Q000139:chemically induced* / Q000473:pathology / Q000503:physiopathology; D015984:Causality; D002547:Cerebral Palsy / Q000150:complications / Q000473:pathology / Q000503:physiopathology; D003711:Demyelinating Diseases / Q000139:chemically induced* / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D020925:Hypoxia-Ischemia, Brain / Q000150:complications / Q000503:physiopathology; D007049:Iatrogenic Disease; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009413:Nerve Fibers, Myelinated / Q000187:drug effects / Q000473:pathology; D047928:Premature Birth; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000209:etiology; D014617:Vacuoles / Q000187:drug effects / Q000473:pathology; D020888:Vigabatrin / Q000009:adverse effects*
D000927:Anticonvulsants; D020888:Vigabatrin