The successful use of intrathecal baclofen, a structural analogue of g
amma-aminobutyric acid, is described in the treatment of a 9-year-old
boy with intractable torsion dystonia, not responding to conservative
treatment. To our knowledge, this is the first reported case of contin
uous intrathecal baclofen for hereditary torsion dystonia. This case s
uggests that a continuous intrathecal infusion of baclofen may facilit
ate remission of intractable torsion dystonia, and provides a basis fo
r further investigation of the treatment of intractable childhood dyst
onia.