关于深脑刺激疗法
Boston Children’s Hospital is now offering deep brain stimulation (DBS) for children withprimary dystonia，一种运动障碍，其中大脑信号会导致非自愿性肌肉收缩。这些导致异常，征税运动，通常会重复扭曲或扭曲手臂，腿部，颈部或其他身体部位。与脑损伤引起的继发性肌张力障碍不同，原发性肌张力障碍被认为是遗传的。
肌张力障碍可能仅发生在身体的特定部分，例如眼睑或手，或者可能影响许多身体区域。通常，肌张力障碍在发作后的头五年中最快地进展和传播，然后开始稳定。肌张力障碍可能会静止，但是当孩子执行特定任务或运动（例如写作或步行）时，通常会变得更糟。结果，肌张力障碍可能会变得高度致残。
Primary dystonia is often painful, and the abnormal movements and postures may embarrass the child and make it difficult to perform well in school. Some children become wheelchair-bound, and if dystonia is left untreated for years, it can cause permanent damage to joints and muscles as the limb “freezes” into the dystonic position. Most children with primary dystonia have a normal life expectancy.
什么是深脑刺激，如何帮助？
DBS therapy uses a small device similar to a pacemaker, called a neurostimulator, to send mild electrical pulses to the parts of the brain that are responsible for the involuntary movements. The neurostimulator, manufactured by Medtronic, is implanted just under the skin, usually below the collarbone. It is connected to electrodes (or “leads”) implanted in the brain by a thin, flexible wire. The electrodes are precisely placed in a part of the brain called the basal ganglia, part of the brain's motor circuit.
When the neurostimulator is turned on, it can give children with primary dystonia more control over their body movements. The settings are adjustable with a handheld wireless device, so the doctor can program the stimulator to the child’s individual needs. The family also receives a programming device to monitor and adjust the device as needed. The system can be safely removed at any time.
How DBS works isn’t completely known, but it has proven effective in several neurological disorders (not just dystonia). It is thought that the brain cells of children with primary dystonia signal too much, and that extra signals “spill over” to activate the wrong muscles. The electrical stimulation “dials back” these abnormal signals. DBS has been in use for more than 20 years, and according to the device manufacturer, more than 100,000 people around the world have received DBS therapy.
由于与帕金森氏病（最常见的DBS应用）相比，肌张力障碍相对较少，因此食品药品监督管理局（FDA）已获得人道主义装置的豁免获得批准。该名称用于被认为是安全的治疗方法，但需要在较大的患者组中进一步研究，才能知道如何最好地使用它们。
深脑刺激|深入
深度大脑刺激期间会发生什么
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Click here for关于深脑刺激的常见问题（常见问题解答）.
Frequently Asked Questions about Deep Brain Stimulation (DBS)
我可以期待肌张力障碍有何改善？
并非所有患有原发性肌张力障碍的儿童都对DBS有反应，但是对于最适合DBS的孩子，该疗法可以将症状降低60％至90％。许多儿童将在功能，生活质量和关节畸形方面具有明显的，有时有时的改善。有时，一个坐轮椅的孩子可能会恢复行走的能力。不过，改进不是立即的，通常需要6到12个月才能看到实质性的差异。
It’s important to note that DBS is not a cure, and abnormal movements will return when the system is turned off. This is true for all treatments for dystonia, not just DBS.
Who is most likely to benefit from DBS?
Under FDA criteria, DBS is indicated for children 7 or older. Children whose primary dystonia affects the whole body and is not controlled well by medications are the best candidates for DBS therapy. DBS can be used for generalized dystonia, hemidystonia (affecting one side of the body), segmental dystonia (affecting two adjacent parts of the body) and cervical dystonia (affecting the neck muscles).
与DBS一起使用的孩子往往年轻，并且在症状会造成永久性身体损害之前开始治疗，例如固定在适当的关节。在波士顿儿童，原发性肌张力障碍儿童由运动障碍神经科医生杰夫·沃（Jeff Waugh，MD），博士和神经外科医师塞氏症（M.通过大脑和神经心理学测试的MRI进一步评估每个孩子。然后，我们的团队开会以确认手术是合适的，并讨论其最佳时机。
What other options are there for treating dystonia?
Currently available treatments for dystonia include physical therapy, oral medications, botulinum toxin (Botox) injections to affected muscles and neurosurgery. These treatments alleviate symptoms, but none provide a cure. Botox works best for dystonia that affects a single area or only a few body parts but is insufficient for dystonias that are generalized throughout the body. There are other drugs with a body-wide effect, such as baclofen, a muscle relaxant, and trihexyphenidyl (Artane), which can relieve stiffness, tremors and spasms. However, these drugs do not benefit all children and can have unwanted side effects.
深脑刺激涉及什么？
DBS therapy requires surgery to implant the neurostimulator, the leads and the connecting wires (known as extensions). The surgery typically lasts several hours, while the child is asleep under general anesthesia, and requires a hospital stay.
首先通过头骨中的一个小开口插入铅，到达大脑内的精确靶向位点。通常，在相同的过程中植入并连接了扩展和神经刺激器（在极少数情况下，扩展和神经刺激器被植入并连接到单独的操作中。）
通常直到孩子从手术中愈合，通常是在手术后四个星期，通常才能打开神经刺激剂。设备“实时”后，将需要为每个孩子量身定制设置。没有关于如何编程DB的“食谱”，每个人的理想设置都不同。这需要进行几次随访 - 首先，在手术后的每个月，然后确定最佳设置后每隔几个月。
inc .将需要每一个所取代few years when its battery starts to run low. This is usually a minor outpatient operation. A rechargeable battery can be used in certain cases to lengthen battery life. The final DBS settings determine how much power output is needed and how long the battery will last.
Children with DBS systems need to follow some specific precautions; for example, they cannot have normal MRI scans, and there are situations in which the stimulator will need to be turned off. Your neurologist and neurosurgeon will review all these precautions with you.
As with any brain surgery and medical treatment, there are some risks to consider with DBS therapy. Your care team will discuss possible surgical complications and therapy side effects with you.
深脑刺激|Research
Recent publications from Boston Children’s
石头Alterman RL。深脑刺激肌张力障碍。在：Youmans Jr，Winn HR，编辑。Youmans神经手术-7Thedition. Philadelphia: Saunders/Elsevier; 2015. [in press]
沃JL;Sharma N.临床神经遗传学：从表型到基因型的肌张力障碍（综述）.Neurol Clin2013年11月;31：969-86。doi：10.1016/j.ncl.2013.04.002。
Waugh J; Sharma N. Pediatric movement disorders and ataxia. In: Sims K, Peters JM, Musolino P, Ward MZ, editors. Handbook of Pediatric Neurology. Philadelphia: Lippincott Williams & Wilkins; 2013.
石头et al.Stimulation of entorhinal cortex promotes adult neurogenesis and facilitates spatial memory.J Neurosci2011 Sep 21; 31:13469-84. doi: 10.1523/JNEUROSCI.3100-11.2011.