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Background: Gilles de la Tourette's syndrome (GTS) is a complex neuropsychiatric disorder characterized by disabling motor and vocal tics. The pathophysiology of GTS remains poorly understood. Conventional treatment consists in pharmacological and behavioral treatment. For patients suffering severe adverse effects or not responding to pharmacological treatment, deep brain stimulation (DBS) presents an alternative treatment. However, the optimal target choice in DBS for GTS remains a divisive issue. Methods: A PubMed search from 1999 to 2012 was conducted. Thirty-three research articles reporting on DBS in patients with GTS were selected and analyzed. Results: Eighty-eight patients with Tourette's syndrome were treated since 1999 with DBS. The majority of patients received thalamic stimulation. Significantly fewer patients were treated with globus pallidus internus stimulation. Occasionally, the anterior limb of the internal capsule and the nucleus accumbens were implanted. The subthalamic nucleus was selected once. All targets were reported with positive results, but of variable extent. Only 14 patients exhibited level 1 evidence. Conclusion: In light of the wide spectrum of associated behavioral co-morbidities in GTS, multiple networks modulation may result in the most efficacious treatment strategy. The optimal locations for DBS within the cortico-basal gangliathalamocortical circuits remain to be established. However, at the current stage, comparison between targets should be done with great caution. Significant disparity between number of patients treated per target, methodological variability, and quality of reporting renders a meaningful comparison between targets difficult. Randomized controlled trials with larger cohorts and standardization of procedures are urgently needed. [ABSTRACT FROM AUTHOR]

Long-term results show that benefits from chronic deep brain stimulation in dystonia are maintained for many years. Despite this, the neurophysiological long-term consequences of treatment and their relationship to clinical effects are not well understood. Previous studies have shown that transcranial magnetic stimulation measures of abnormal long-term potentiation-like plasticity (paired associative stimulation) and GABAa-ergic inhibition (short-interval intracortical inhibition), which are seen in dystonia, normalize after several months of deep brain stimulation. In the present study, we examine the same measures in a homogenous group of 10 DYT1 gene-positive patients after long-term deep brain stimulation treatment for at least 4.5 years. Recordings were made ‘on’ deep brain stimulation and after stopping deep brain stimulation for 2 days. The results show that: (i) on average, prior to discontinuing deep brain stimulation, the paired associative stimulation response was almost absent and short-interval intracortical inhibition was reduced compared with normal. This pattern differs from that in both healthy volunteers and from the typical pattern of enhanced plasticity and reduced inhibition seen in deep brain stimulation-naïve dystonia. It is similar to that seen in untreated Parkinson’s disease and may relate to thus far unexplained clinical phenomena like parkinsonian symptoms that have sometimes been observed in patients treated with deep brain stimulation. (ii) Overall, there was no change in average physiological or clinical status when deep brain stimulation was turned off for 2 days, suggesting that deep brain stimulation had produced long-term neural reorganization in the motor system. (iii) However, there was considerable variation between patients. Those who had higher levels of plasticity when deep brain stimulation was ‘on’, had the best retention of clinical benefit when deep brain stimulation was stopped and vice versa. This may indicate that better plasticity is required for longer term retention of normal movement when deep brain stimulation is off. (iv) Patients with the highest plasticity ‘on’ deep brain stimulation were those who had been receiving stimulation with the least current drain. This suggests that it might be possible to ‘shape’ deep brain stimulation of an individual patient to maximize beneficial neurophysiological patterns that have an impact on clinical status. The results are relevant for understanding long-term consequences and management of deep brain stimulation in dystonia. [ABSTRACT FROM AUTHOR]

Cerebral palsy (CP) is a heterogeneous group of non-progressive syndromes with lots of clinical variations due to the extent of brain damages and etiologies. CP is majorly defined by dystonia and spasticity. The treatment of acquired dystonia in CP is very difficult. Many pharmacological treatments have been tried and surgical treatment consists of deep brain stimulation (continuous electrical neuromodulation) of internal globus pallidus (GPi). A peculiar cause of CP is neonatal encephalopathy due to an anoxic event in the perinatal period. Many studies showed an improvement of dystonia in CP patients with bilateral GPi DBS. However, it remains a variability in the range of 1% to 50%. Published case-series concerned mainly small population with a majority of adult patients. Selection of patients according to the clinical pattern, to the brain lesions observed on classical imaging and to DTI is the key of a high success rate of DBS in children with perinatal hypoxemic encephalopathy. Only a large retrospective study with a high number of patients in a homogeneous pediatric population with a long-term follow-up or a prospective multicenter trial investigation could answer with a high degree of certitude of the real interest of this therapeutic in children with hypoxemic perinatal encephalopathy. [ABSTRACT FROM AUTHOR]

Pallidal deep brain stimulation is an established treatment in dystonia. Available data on the effect in DYT-THAP1 dystonia (also known as DYT6 dystonia) are scarce and long-term follow-up studies are lacking. In this retrospective, multicenter follow-up case series of medical records of such patients, the clinical outcome of pallidal deep brain stimulation in DYT-THAP1 dystonia, was evaluated. The Burke Fahn Marsden Dystonia Rating Scale served as an outcome measure. Nine females and 5 males were enrolled, with a median follow-up of 4 years and 10 months after implant. All benefited from surgery: dystonia severity was reduced by a median of 58% (IQR 31-62, p = 0.001) at last follow-up, as assessed by the Burke Fahn Marsden movement subscale. In the majority of individuals, there was no improvement of speech or swallowing, and overall, the effect was greater in the trunk and limbs as compared to the cranio-cervical and orolaryngeal regions. No correlation was found between disease duration before surgery, age at surgery, or preoperative disease burden and the outcome of deep brain stimulation. Device- and therapy-related side-effects were few. Accordingly, pallidal deep brain stimulation should be considered in clinically impairing and pharmaco-resistant DYT-THAP1 dystonia. The method is safe and effective, both short- and long-term. [ABSTRACT FROM AUTHOR]

Presents a study on the effectiveness of deep-brain stimulation in the treatment of a severe dystonia musculorum deformans-1 generalized dystonia (DYT1). Association of this technique with substantial improvement of dystonia and functional disability; Description of the efficacy of bilateral, continuous electrical stimulation of the global pallidus internus in DYT1 patients; Details of the study.

A submammary approach to implanting pulse generators is innovative and has yielded good aesthetic results in the current literature. It was our aim to make a comparison of patient device acceptance, tolerance, and complications between submammary and abdominal device locations in deep brain stimulation. Twenty-five and 28 patients were included in the submammary and abdominal groups, respectively. Our primary criterion was patient acceptance that was calculated using total Florida Patient Acceptance Survey (FPAS) scores in each group. Secondarily, tolerance was assessed in the submammary group by means of a specific questionnaire. Total FPAS scores from the submammary group [total FPAS: 77.1 versus 74.7, P = 0.29] revealed no significant difference when compared with the abdominal group. The same similarities were observed regarding the 4 subscales: return to function [16.3 versus 15.8, P = 0.53], device-related distress [22.0 versus 21.3, P = 0.31], body image concerns [9.2 versus 8.6, P = 0.14], and positive appraisal [17.8 versus 17.4, P = 0.58]. Tolerance was reported as good by the majority of the women from the submammary group. There was no evidence of higher infection rates in the submammary implantation (SMI) group. SMI is a satisfactory alternative to other deep brain stimulation locations. SMI is a feasible option for any young woman who is eligible for deep brain stimulation. [ABSTRACT FROM AUTHOR]

Abstract: Deep brain stimulation (DBS) is a powerful treatment option for movement disorders, including severe generalised dystonia. After several years of treatment, cases have been reported in which DBS has been stopped without any deterioration in clinical benefit. This might indicate that DBS can restore function in some cases. The mechanism of DBS induced clinical retention effects has been addressed before. Here, the question we asked was if such clinical stability is reflected at the underlying physiology level or whether there is indication to believe that a stand-still of symptoms might be at risk because of neurophysiological instability. We recorded patients with pre-intervention life-threatening or severe genetic dystonia with long lasting clinical benefit when turned off DBS. Despite clinical stability, our physiological studies revealed large changes in the excitability of excitatory and inhibitory motor circuits in the cortex, which exceed normal fluctuation. This discrepancy between instability in the motor network physiology caused by removal of DBS and clinical stability alerts as it potentially indicates a risk to fail and cause symptoms to return. [Copyright &y& Elsevier]

Abstract: Object: The parameter adjustment process during deep brain stimulation (DBS) for dystonia remains time consuming and based on clinical observation alone. The aim was to correlate the electric field with the GPi anatomy to be able to study the stimulated volume. Methods: We developed a computer-assisted method (model) for visualizing electric field in reference to the stereotactic space. Electric field values were correlated with the GPi anatomy (stereotactic Magnetic Resonance Imaging) in one reference patient. Results: Using this methodology it becomes possible to correlate the electric field distributions for patient specific parameters with the anatomical information. The application to one patient showed that the 0.1V/mm isofieldline fits best with the lateral GPi borders at the level of the stimulated contacts. Conclusions: The electric field is a crucial parameter as it is assumed to be responsible for triggering action potentials. Electric field visualisation allows the calculation of the stimulated volume for a given isoline. Its application to our whole patient population might help in determining a threshold for obtaining a therapeutic effect, to date unknown, and consequently in optimizing the parameter setting in each patient. [Copyright &y& Elsevier]