Intrathecal perfusion pump with baclofen in generalized dystonia

Objectives. We show the effectiveness of treatment with continuous intrathecal baclofen infusion in a case of hereditary generalized dystonia refractary to anticholinergics, tetrabenazine, pimozide, L-dopa, benzodiazepines and thalamotomy. Clinical case. A 26 years old female patient, when she was 11 years old began with torsion dystonia in her left feet, that progressively worsened to involve her entire body. She had painful spasms. She had four brothers, three of them with dystonia and one healthy. Her uncle grandfather had similar symptoms. Complementary explorations to reject secondary origin was negatives. She was treated with high and progressive dosages of anticholinergics, pimozide, tetrabenazine, benzodiazepines, L-dopa and thalamotomy without improvement. Underwent intrathecal baclofen test dosing, we used 25, 50, 100 mu g/day, the last one with improvement during 10 hours. A pump was inserted with an initial dose: of 220 mu g/day. After pump insertion, baclofen dosage was gradually inn eased to 450 mu g/day. She had a great improvement in right part of her body and less in her left body. Painful spasms had disappeared Conclusion. We propose continuous baclofen intrathecal infusion pump for patients with severe torsion dystonia that not response to ordinal treatment.