Deep brain stimulation for camptocormia in dystonia and Parkinson’s disease

Abstract

Camptocormia, or "bent spine syndrome", may occur in various movement disorders such as primary dystonia or idiopathic Parkinson's disease (PD). Although deep brain stimulation (DBS) is an established treatment in refractory primary dystonia and advanced PD, few data are available on the effect of DBS on camptocormia comparing these two conditions. Seven patients (4 with dystonia, 3 with PD; mean age 60.3 years at surgery, range 39-73 years) with camptocormia were included in the study. Five patients underwent bilateral GPi DBS and two patients underwent bilateral STN DBS guided by CT-stereotactic surgery and microelectrode recording. Pre- and postoperative motor assessment included the BFM in the dystonia patients and the UPDRS in the PD patients. Severity of camptocormia was assessed by the BFM subscore for the trunk at the last available follow-up at a mean of 17.3 months (range 9-36 months). There were no surgical complications. In the four patients with dystonia there was a mean improvement of 53% in the BFM motor score (range 41-79%) and of 63% (range 50-67%) in the BFM subscore for the trunk at the last available follow-up (mean 14.3 months, range 9-18 months). In the three patients with camptocormia in PD who underwent bilateral STN DBS (2 patients) or pallidal DBS (1 patient), the PD symptoms improved markedly (mean improvement in the UPDRS motor subscore stimulation on/medication off 55%, range 49-61%), but there was no or only mild improvement of camptocormia in the two patients who underwent STN DBS, and only moderate improvement in the patient with GPi DBS at the last available follow-up (mean 21 months, range 12-36 months). GPi DBS is an effective treatment for camptocormia in dystonia. The response of camptocormia to chronic STN or GPi DBS in PD is more heterogenous. The latter may be due to a variety of causes and needs further clarification.

Subthalamic nucleus stimulation improves Parkinson's disease-associated camptocormia in parallel to its preoperative levodopa responsiveness

“…Other reported outcomes varied from excellent improvements7 16–19 to only mild or no benefits 1 16 19 20. As similar results were obtained with deep brain stimulation (DBS) of the globus pallidus internus (GPi),20–22 recent consensus may regard DBS as not a specific and separate treatment for camptocormia in patients with PD 2…”

“…Djaldetti et al 3 found that in some patients with PD camptocormia was ameliorated by dopaminergic agents, suggesting a difference in its levodopa responsiveness and the possible existence of factors predictive of the effect of chronic DBS20 on the cardinal motor symptoms of PD 23 24…”

Camptocormia in Parkinson's disease: definition, epidemiology, pathogenesis and treatment modalities

“…857 Third, there have been a few cases showing that camptocormic symptoms could be reversed following levodopa. 6369 Fourth, camptocormic symptoms were also improved by high-frequency subthalamic nucleus 1970–75 or globus pallidus interna deep brain stimulation 7677 (STN or GPi-DBS) together with improved motor symptoms in patients with PD. The argument against this idea is that most cases of camptocormia in PD did not respond to either levodopa 858 or DBS.…”

“…Evidence has shown that anticholinergics, benzodiazepines and baclofen, failed to provide benefit for most patients with camptocormia. 1958 Such mixed evidence about neurotransmitter systems makes it difficult to draw any conclusions, but does suggest that there is not a simple relationship. Investigations of which structures in the central nervous system are involved in the pathogenesis of camptocormia in PD, such as measuring brain metabolism or functional connectivity, are limited because the camptocormic symptoms disappear while lying in the scanner.…”

Treatment of camptocormia with botulinum toxin

“…Deep Brain Stimulation (DBS) surgery is beneficial for motor improvement in PD, whether placed in the subthalamic nucleus (STN) or globus pallidus. The effect on camptocormia is variable, it may significantly improve, even if previously severe, or may not change despite an overall successful surgical outcome (Umemura et al, 2010;Capelle et al, 2011). One study suggested DBS to the STN for PD improved camptocormia only in those who had reported this axial deformity within 18 months prior to the operation (Schulz-Schaeffer et al, 2015).…”