The treatment of severe dystonia in children and adults.

Abstract

SUMMARY Twenty-three children (aged less than 18 years) 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). 52% 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 two children with life-threatening generalised 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.

Status dystonicus: study of five cases

“…Transient improvement was achieved with tetrabenazine and baclofen, and eventually bilateral thalamotomy had to be performed. Marsden et al 10 published a report of 23 children and 17 adults with dystonia treated with high dose of anticholinergics. From the whole group, 2 children were described as presenting "life-threatening generalized dystonia" treated with combined use of tetrabenazine and pimozide, successful in one of them.…”

“…Treatment of SD is mainly empirical, variable and collected from anecdotal reports as described above 4,[5][6][7]10 . Also of note are cases requiring use of continuous infusion of intrathecal baclofen and neurosurgery, including thalamotomy, pallidotomy or placement of DBS electrodes 4,9,11 .…”

Deep Brain Stimulation for Status Dystonicus: A Case Series and Review of the Literature

“…Frequently reported sequelae of SD include airway compromise, rhabdomyolysis, hyperpyrexia, renal failure, pain, and dehydration. To date, there have been fewer than 70 cases of SD reported in the literature [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29]. These cases are characterized by widely varying etiologies, clinical courses, treatments, and outcomes.…”

Distonias: aspectos terapêuticos

“…Marsden at al. 36 sugeriram que a combinação de um bloqueador pós-sináptico (haloperidol ou pimozide), um depletor pré-sináptico (tetrabenazina) e de um anticolinérgico (tri-hexifendil) poderia proporcionar maior benefício do que qualquer uma das drogas utilizadas isoladamente. Esse esquema surgiu da necessidade de se manter níveis reduzidos de tetrabenazina devido à significativa tendência dessa droga em causar depressão.…”