Neuromodulation in Childhood Onset Dystonia: Evolving Role of Deep Brain Stimulation

2020 ◽  
Vol 8 (2) ◽  
pp. 37-43
Author(s):  
Warren A. Marks ◽  
Stephanie Acord ◽  
Laurie Bailey ◽  
John Honeycutt
Cephalalgia ◽  
2016 ◽  
Vol 36 (12) ◽  
pp. 1143-1148 ◽  
Author(s):  
Massimo Leone ◽  
Alberto Proietti Cecchini

Background: Deep brain stimulation of the posterior hypothalamic area was first introduced in 2000 to treat drug-refractory chronic cluster headache (CH). Findings: So far, hypothalamic stimulation has been employed in 79 patients suffering from various forms of intractable short-lasting unilateral headache forms, mainly trigeminal autonomic cephalalgias. The majority were (88.6%) chronic CH, including one patient who suffered from symptomatic chronic CH-like attacks; the remaining were short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), one had paroxysmal hemicranias and one symptomatic trigeminal neuralgia. Overall, after a mean follow up of 2.2 years, 69.6% (55) hypothalamic-stimulated patients showed a ≥50% improvement. Conclusions: These observations need confirmation in randomised, controlled trials. A key role of the posterior hypothalamic area in the pathophysiology of unilateral short-lasting headaches, possibly by regulating the duration rather than triggering the attacks, can be hypothesised. Because of its invasiveness, hypothalamic stimulation can be proposed only after other, less-invasive, neurostimulation procedures have been tried.


CNS Spectrums ◽  
2016 ◽  
Vol 22 (3) ◽  
pp. 282-289 ◽  
Author(s):  
Volker A. Coenen ◽  
Thomas E. Schlaepfer ◽  
Peter Goll ◽  
Peter C. Reinacher ◽  
Ulrich Voderholzer ◽  
...  

Deep brain stimulation (DBS) is a promising putative modality for the treatment of refractory psychiatric disorders such as major depression and obsessive-compulsive disorder (OCD). Several targets have been posited; however, a clear consensus on differential efficacy and possible modes of action remain unclear. DBS to the supero-lateral branch of the medial forebrain bundle (slMFB) has recently been introduced for major depression (MD). Due to our experience with slMFB stimulation for MD, and because OCD might be related to similar dysfunctions of the reward system, treatment with slMFB DBS seams meaningful. Here we describe our first 2 cases together with a hypothetical mode of action.We describe diffusion tensor imaging (DTI) fiber tractographically (FT)-assisted implantation of the bilateral DBS systems in 2 male patients. In a selected literature overview, we discuss the possible mode of action. Both patients were successfully implanted and stimulated. The follow-up time was 12 months. One patient showed a significant response (Yale–Brown Obsessive-Compulsive Scale [YBOCS] reduction by 35%); the other patient reached remission criteria 3 months after surgery (YBOCS<14) and showed mild OCD just above the remission criterion at 12 months follow-up.While the hypermetabolism theory for OCD involves the cortico–striato–thalamo–cortical (CSTC) network, we think that there is clinical evidence that the reward system plays a crucial role. Our findings suggest an important role of this network in mechanisms of disease development and recovery. In this uncontrolled case series, continuous bilateral DBS to the slMFB led to clinically significant improvements of ratings of OCD severity. Ongoing research focuses on the role of the reward system in OCD, and its yet-underestimated role in this underlying neurobiology of the disease.


2020 ◽  
pp. 37-40
Author(s):  
Vibhash D. Sharma ◽  
Shilpa Chitnis

Deep brain stimulation therapy is an effective therapy for selected patients with movement disorders. The procedure is relatively safe, but complications related to the surgical procedure or implanted hardware can occur. The common complications include hemorrhage, infarct, infection, and confusion. Noninfectious cyst formation around the DBS lead is a rare but potential complication of this procedure, which can occur several weeks to months after DBS lead implantation. This chapter describes a case of noninfectious cyst formation at the tip of DBS lead in a patient with essential tremor. Clinical presentation, role of imaging, and the management options for this rare complication are discussed. This case also illustrates the importance of post-DBS imaging in suspected cases with new or unexplained symptoms.


2020 ◽  
Vol 29 (4) ◽  
pp. 557-573
Author(s):  
KATRINA A. MUÑOZ ◽  
JENNIFER BLUMENTHAL-BARBY ◽  
ERIC A. STORCH ◽  
LAURA TORGERSON ◽  
GABRIEL LÁZARO-MUÑOZ

AbstractDystonia is a movement disorder that can have a debilitating impact on motor functions and quality of life. There are 250,000 cases in the United States, most with childhood onset. Due to the limited effectiveness and side effects of available treatments, pediatric deep brain stimulation (pDBS) has emerged as an intervention for refractory dystonia. However, there is limited clinical and neuroethics research in this area of clinical practice. This paper examines whether it is ethically justified to offer pDBS to children with refractory dystonia. Given the favorable risk-benefit profile, it is concluded that offering pDBS is ethically justified for certain etiologies of dystonia, but it is less clear for others. In addition, various ethical and policy concerns are discussed, which need to be addressed to optimize the practice of offering pDBS for dystonia. Strategies are proposed to help address these concerns as pDBS continues to expand.


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