Prolonged episode of dystonia and dyskinesia resembling status epilepticus following acute intrathecal baclofen withdrawal

: Spasticity is a state of sustained pathological increase in the tension of a muscle. Treatment for spasticity has been revolutionized by the introduction of intrathecal baclofen (ITB) continuous infusion. ITB is associated with a 30% rate of complications mostly as a result of catheter problems that lead to acute ITB withdrawal. We describe a 10-year-old girl with spastic quadriplegia caused by cerebral palsy successfully treated with ITB who developed dystonic-dyskinetic status following acute ITB withdrawal because of a catheter kink resolved by external manipulation. The patient presented with a subacute onset of generalized malaise characterized by anorexia, difficulty in speaking and swallowing, insomnia, worsening of hypertonus with a left predominance, and late appearance of dystonic-dyskinetic movements. Soon after hospitalization the child had a generalized tonic-clonic seizure followed by unresponsiveness. One hour later she developed multiple muscle contractions with dystonic posturing and continuous chaotic movements. She also had pyrexia, tachycardia, and hypertension. A video/EEG recording showed the nonepileptic nature of the symptoms and revealed dystonic-dyskinetic status. We report the clinical features and the video recording of the status. The prompt recognition of this life-threatening complication is essential, as rapid treatment may reduce the increased risk of death. Misdiagnosis is possible, and video/EEG monitoring is useful to this end. Although differing among patients, all symptoms are related to overexcitability of the extrapyramidal and autonomic systems.