The treatment of severe dystonia in children and adults.

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Abstract
Twenty-three children (aged < 18 yr) and 17 adults with severe widespread dystonia were treated with high doses of benzhexol (up to 130 mg daily introduced slowly over many weeks). Children tolerated higher doses (median 30 mg/day) than adults (median 20 mg/day). Fifty-two percent of the children gained useful benefit, many (43%) without unwanted side effects. Such an approach was less successful in adults; 41% gained benefit, but only 35% had no side effects. Twelve adults with severe axial dystonia and 2 children with life-threatening generalized dystonia were treated with a combination of a low constant dose of tetrabenazine to which were added pimozide and benzhexol as necessary. The dose of tetrabenazine was aimed at 75 mg daily; pimozide was increased (6 to 25 mg/day) until the dystonia was relieved or Parkinsonism and other side-effects prevented further increments; if necessary benzhexol (6 to 30 mg/day) then was added to control side-effects and to provide additional benefit. Seventy-five percent of the adults with severe axial dystonia, and 1 of the 2 children with life threatening generalized dystonia gained useful benefit from this regime. High dose benzhexol is the present first treatment of choice for children with severe dystonia and is worth a try in adults but with less expectation of success. When benzhexol treatment alone fails in adults with severe disabling axial dystonia, or in children with life-threatening generalized dystonia, combined therapy with tetrabenazine, pimozide and benzhexol may give valuable symptomatic relief.