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Vigabatrin Caused Rapidly Progressive Deterioration in Two Cases With Early Myoclonic Encephalopathy Associated With Nonketotic Hyperglycinemia

Department of Pediatrics Division of Child Neurology, University Medical School

Gul Serdarolu, MD

Department of Pediatrics Division of Child Neurology, University Medical School, gul{at}med.ege.edu.tr

Bulent Karapinar, MD

Department of Pediatrics Intensive Care Unit, University Medicine School

Muzaffer Polat, MD

Department of Pediatrics Division of Child Neurology, University Medical School

Serap Yurtsever, MD

Department of Pediatrics Division of Child Neurology, University Medical School

Aye Tosun, MD

Department of Pediatrics Division of Child Neurology, University Medical School

Mahmut Coker, MD

Department of Endocrinology and Metabolism, University Medical School

Sarenur Gokben, MD

Department of Pediatrics Division of Child Neurology University Medical School Izmir, Turkey

Vigabatrin, a structural analogue of -aminobutyric acid (GABA), is used for the treatment of generalized and partial seizures in infants. The drug inhibits the GABA transaminase and elevates the GABA concentration in the brain. Here we present the vigabatrin experience in two patients with early myoclonic encephalopathy owing to nonketotic hyperglycinemia (glycine encephalopathy). Both patients had early infantile seizures characterized by fragmentary myoclonic jerks associated with burst-suppression pattern on electroencephalography. Nonketotic hyperglycinemia was diagnosed with elevated cerebrospinal fluid and plasma glycine levels. The seizures were initially thought to be infantile spasms, and vigabatrin (50 mg /kg/day) was started for the treatment of seizures. Rapidly progressive deterioration was noticed after a few days. Acute encephalopathy associated with sleepiness and respiratory failure developed. Vigabatrin produced acute encephalopathy, which regressed in a few days after vigabatrin was stopped in the first patient. However, in the second case, despite the discontinuation of vigabatrin, there was no recovery of general conditions. Our observations in two cases indicate the risk of using vigabatrin in patients with nonketotic hyperglycinemia. The elevated GABA concentration in the brain can enhance the encephalopathy, together with the elevated levels of glycine. (J Child Neurol 2006;21:82—84).

Journal of Child Neurology, Vol. 21, No. 1, 82-84 (2006)
DOI: 10.1177/08830738060210011801


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