Fast track — ArticlesBilateral pallidal deep brain stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study
Introduction
Cerebral palsy (CP) is characterised by motor impairment and abnormal movements and postures that result from injury to the developing brain.1 CP, a clinically heterogeneous disorder, is the most common cause of disability in children, affecting 2–3 per 1000 live births;2, 3 about 10–15% of patients present with severe disabling movement disorders (dystonia-choreoathetosis) and little or no impairment of cognitive function. The dystonia-choreoathetosis forms of CP, with basal ganglia dysfunction, are mainly due to neonatal hypoxic ischaemic encephalopathy in term or near-term infants.1, 2 Medical treatments are ineffective, and because the life expectancy of these patients in high-income countries is similar to that of the general population,2 during early adulthood they will have to deal with motor disabilities, limited autonomy, and social difficulties.
A substantial and sustained benefit of bilateral pallidal deep brain stimulation (BP-DBS) has been consistently reported in patients with primary generalised dystonia.4, 5, 6 BP-DBS has therefore raised great expectations among patients with dystonia-choreoathetosis CP because the disease shares most of the features of primary dystonia (eg, involuntary sustained muscle contractions that lead to abnormal movements and postures). However, dystonia-choreoathetosis CP is considered among the causes of secondary dystonia, which are reputed to respond less well to neurostimulation than primary dystonia.4, 7, 8
The few previous reports of neurostimulation in patients with dystonia-choreoathetosis CP have involved small, heterogeneous series and unmasked designs.7, 9, 10, 11, 12, 13 We did a prospective, multicentre, pilot study with standardised and masked assessments to assess the effects of bilateral pallidal stimulation on motor impairment, functional disability, and quality of life in patients with dystonia-choreoathetosis CP.
Section snippets
Patients
13 patients with dystonia-choreoathetosis CP were operated on between September 2003 and March 2006. The inclusion criteria were: disabling dystonia, defined as involuntary sustained muscle contractions that led to abnormal movements and postures, which could be multifocal or generalised, with a combination of segmental crural dystonia (one leg and the trunk) and involvement of any other segment (face, neck, or upper or lower limbs);14 neonatal hypoxic or ischaemic encephalopathy2 and delayed
Results
13 patients (nine women and four men; median age 33 years [range 20–44]; table 1) were included in the study. Table 2 summarises the severity of abnormal movements and motor disability, as assessed with the movement and disability subscales of the Burke–Fahn–Marsden dystonia rating scale, before surgery and after 1 year of continuous stimulation. The mean Burke–Fahn–Marsden dystonia rating scale movement score was significantly lower at 1 year than it was at baseline (p=0·01), with an overall
Discussion
In these adults with CP secondary to neonatal hypoxic or ischaemic encephalopathy, who had severe movement disorders (dystonia-choreoathetosis), little or no cognitive impairment, and no more than slight abnormalities seen on preoperative T1-weighted MRI, BP-DBS resulted in most cases in a sustained improvement in motor symptoms in most segments of the body (neck, trunk, and limbs) over 1 year compared with their preoperative status. Some patients showed an improvement that was similar to that
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French SPIDY-2 Study group listed at the end of the paper.