[Choreo-ballistic status treated by GABA].

A successful GABA replacement therapy for choreo-ballistic status in a 9-year-old girl, who had been previously treated medically or surgically without beneficial results, was reported. Involuntary movements completely ceased after intravenous administration of GABA (the maximal doses: 533 mg/kg/day) of 3 weeks' duration. Chorea and ballism are assumed to be caused by hypofunction of the GABA-neurons in the basal ganglia. Administration of GABA has been considered to be ineffective for these involuntary movements, since GABA has been believed not to cross the blood-brain barrier. The present case suggests that a large amount of intravenous administration of GABA gives a chance to treat involuntary movements caused by deficiency of GABA-neurons, although the essential mechanism of intracerebral penetration of GABA remains uncertain.