The effectiveness of benzoate in the management of seizures in nonketotic hyperglycinemia
J. A. Wolff, S. Kulovich, A. L. Yu, C. N. Qiao and W. L. Nyhan
Patients with nonketotic hyperglycinemia generally have intractable
seizures that are poorly responsive to anticonvulsant medication. No
effective treatment has been consistently reported. In three patients with
nonketotic hyperglycinemia, the oral administration of sodium benzoate in
dosages designed to lower the cerebrospinal fluid concentration of glycine
was followed by an abrupt change from frequent major seizures before
treatment to no seizures, or only occasional minor ones, after treatment.
This attenuation of seizures was associated with a decrease in the
concentrations of glycine in both plasma and cerebrospinal fluid. There was
no evident change in psychomotor development.
