Early intraventricular baclofen therapy (IVB) for children with dystonic and dysautonomic storm.

INTRODUCTION: Intrathecal baclofen (ITB) is an effective treatment for managing primary and secondary dystonia. Intraventricular baclofen (IVB) was first developed to allow treating patients in which the use of ITB was difficult due to anatomic anomalies. After that, several studies indicate that intraventricular administration of baclofen, is more effective than ITB in refractory dystonia.

CLINICAL MATERIAL: We report three cases of children with acute dystonic and dysautonomic storm, treated with IVB. The clinical outcome was satisfactory. The response to the treatment continued after the pump disconnection, suggesting that in this kind of cerebral dysregulations, short-term IVB is an effective treatment.

CONCLUSION: Early treatment with IVB may be an effective option in patients with post-anoxic dysautonomic and dystonic storm.