Article
Early chronic GPI stimulation in paediatric patients suffering from intractable primary dystonia
Frühzeitige tiefe Hirnstimulation im GPI bei pädiatrischen Patienten mit therapierefraktärer primärer Dystonie
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Published: | April 11, 2007 |
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Objective: GPI (globus pallidus internus) deep brain stimulation (DBS) offers a very effective stereotactic neurosurgical therapy for medically intractable dystonia. Although generalized dystonia is presenting mainly at young age and might be severely disabling, GPI-DBS is still usually not applied in the early course of the disease. We here present the immediate post-operative results and clinical follow for 5 paediatric patients undergoing GPI-DBS.
Methods: All patients (mean age 13 years (range 8-16 years), 3 female/2 male, 2 DYT1-positive) were suffering from intractable generalized dystonia. Electrode-implantation (DBS 3389, Medtronic) was performed under propofol anaesthesia with MRI-guided stereotaxy using a modified Leksel/Lerch system. The lowest possible electrode was chosen for stimulation and intensity was set relatively high from the beginning (mean 80µA). After one week of successful stimulation by an external test stimulation system, permanent implantation of the stimulation system (Soletra/Kinetra, Medtronic) was performed. Electrode position was verified by MRI and clinical outcome was assessed using the Burke-Fahn-Marsden (BFM) motor and disability scores (mean follow-up 30 months, range 12-60 months).
Results: All patients experienced a significant improvement of symptoms already during the first week. After 3 months, the BFM scores had improved in the range from 75% to 82% compared to preoperatively. After 12 months, relief of symptoms was unchanged compared to the 3-month status in 2 patients and further improved in 3 patients. The 2 patients with long-term follow-up (60 months) showed a stable relief of symptoms. Local infection occurred in one patient, making electrode-explantation necessary. Re-implantation was performed three months later without further complications and stable effectiveness. No other therapy-related morbidity was observed.
Conclusions: GPI deep brain stimulation offers a very effective and safe therapy in paediatric patients suffering from primary dystonia. Stable relief of symptoms can be achieved. Early neurosurgical intervention is crucial to prevent irreversible impairment of motor function.