神经外科手术主要包括(1)电生理监测下选择性脊神经后根切断术(SPR或者SDR):手术在脊髓腰段或者颈段进行,分别可以缓解下肢及上肢广泛肌群痉挛。术中通过对脊神经后根也就是感觉根小束进行电刺激,观察肌肉反应分辨出与痉挛形成有关的也就是责任后根小束,切断这些异常小束即可减轻痉挛,同时能够减少对感觉及运动的干扰等并发症的出现。因为是选择性切断感觉神经,因此一般不影响运动功能。注意:4-12岁治疗最佳,该时期神经功能恢复好,治疗后对骨骼肌肉发育影响小!(2)选择性周围神经切断术(SPN):当患者痉挛症状比较局限,保守治疗无效,无固定挛缩畸形时,可采用SPN术。如肌皮神经SPN手术可治疗肘部痉挛,尺神经、正中神经SPN手术可治疗腕部和手指痉挛,胫神经SPN手术可治疗踝痉挛。该方法有一定并发症不建议在低龄小孩使用(3)立体定向神经外科手术:对伴随震颤和扭转痉挛症状效果较好。采用影像立体定向技术和微电极导向神经电生理技术分别对脑内核团进行解剖定位和功能定位,然后用射频电极加热毁损特定核团,定位准确。脑性瘫痪不随意运动型是继发性肌张力障碍应该慎重(4)鞘内巴氯芬泵:鞘内巴氯芬泵即持续鞘内巴氯芬灌注系统。它采用外科手术将程控泵和导管埋在人体内,通过程控泵和导管将巴氯芬注射剂按照设计剂量直接持续注入脑脊液中,对CP引起的肌肉痉挛有显著效果,对不自主运动也有改善。目前国内没有引进该要,风险较大。(5)矫形手术:当患儿没有得到及时有效的治疗,肢体已经出现明显的固定挛缩畸形时,矫形外科手术将成为前述手术的必要补充。总之,脑瘫需要多学科的长期性综合治疗,需要患儿家长给予持之以恒的付出和接受科学的治疗方案。如果患儿能够接受科学、有效和及时的治疗,患儿是能够获得基本的生活和运动能力,获得融入社会的基本条件和机会。目前,社会上出现了很多打着快速有效治疗脑瘫幌子的不正规医疗机构,其危害是巨大的。其不但消耗了患儿家庭有限的经济资源,同时也延误了患儿的治疗时机。我们希望广大脑瘫患儿家属能够选择正规的医疗机构,选择科学的治疗方案。切勿仅仅相信纸面上的关于“神奇治疗”和“微创治疗”的宣传。华山医院神经外科功能组开展脑性瘫痪治疗多年,经验丰富,欢迎大家咨询。
脊神经后根高选择性切断术用于痉挛性瘫痪。通过对脊髓神经的处理,全面调整患者的肌肉张力,使痉挛肌肉的肌张力尽量接近正常状态。 ①单纯痉挛不伴其他运动障碍,肌张力在2级以上者,并有多肌群的肌张力增高; ②无明显固定挛缩畸形或仅有轻度畸形; ③有一定的运动能力; ④智力正常或接近正常,能配合术后的康复训练; 矫形手术在脑瘫病人治疗的主要原则是矫正畸形、平衡和调整肌力、稳定关节、恢复肢体力线。应遵循由近到元,由粗到细,在6岁前骨关节不稳定情况下,尽量减少干预。可以通过矫形器,康复锻炼完成。
卡马西平和奥卡西平是治疗三叉神经痛的一线药物,Stefan最近报道,卡马西平的有效平均剂量为600mg,有效率为98%,而奥卡西平的有效平均剂量为1200mg,有效率为94%。27%的卡马西平治疗患者因药物的毒副作用而减量,因而复发。18%的奥卡西平治疗患者因药物的毒副作用而减量复发。只有少数患者在治疗中出现药物耐受,3%的患者药物治疗后恶化。评价:研究中发现只有少数患者在治疗中出现药物耐受,颠覆了多数患者出现药物耐受的传统概念,我们在临床中常常发现患者早期有效,随着治疗时间延长,效果下降,药量增加,最后患者不能耐受而手术。但我们的患者很多不正规服药,可能影响了我们的效果。
Hemifacial spasm (HFS,also called tic convulsif) is an involuntary twitching of the facial muscles on one side of the face. The facial muscles are controlled by the facial nerve (seventh cranial nerve), which originates at the brainstem and exits the skull below the ear . The facial nerve is primarily a motor nerve, meaning it controls muscles that move the eyebrows, close the eyes, and move the mouth and lips. Hemifacial spasm is rare, affecting only 8 people in 100,000 in the US. The average age of onset is 45 years and occurs slightly more in women,male/female is 2/3.面肌痉挛,又称面肌抽搐。为一种单侧面部不自主抽搐的病症。面部肌肉是由面神经控制的,该神经由脑干发出,经内听道下方出颅.面神经主要为运动型神经,控制眼睑、口腔和嘴唇的活动。面肌痉挛比较少见,在美国,每100000中有8例患者,女性多见,男、女比例约2:3。平均年龄为45岁。SymptomsIn 92% of cases, the spasm starts near the eye and progresses down the face over time. The early symptom is usually an intermittent tic of the eyelid muscle, which may eventually lead to complete closure of the eye. In the other 8% it starts near the chin and progresses upward. The twitching is usually not painful, but it can be embarrassing and interfere with normal expression and vision. The spasm may then spread and eventually involve all of the muscles in the affected side of the face and the spasms may be limited to the upper or lower half only, and excess tearing may occur. Symptoms may be present during sleep.症状90%的患者起病从眼轮匝肌开始抽搐,然后向下逐渐涉及整个面。早期症状眼睑肌肉的间断抽搐,可导致眼睛关闭。10%的患者从下巴开始,向上进展。常不伴疼痛,但导致患者窘迫和影响患者的正常表情和形象。痉挛可扩散到一侧脸部,也可能局限在上部或下部。可能出现流泪现象。睡着时症状也可出现。DiagnosisMedical history and a neurological exam usually can lead a doctor to diagnose HFS. An MRI scan may rule out other conditions such as a brain tumor, aneurysm, or AVM that may be causing facial nerve compression. An electromyogram (EMG) study of the face is often done along with a nerve conduction velocity (NCV) study to measure facial muscle and nerve electrical activity.诊断病史和常规的神经病学检查可以诊断面肌痉挛,MRI可以排除因肿瘤、动脉瘤或动静脉畸形引起的面神经压迫。肌电图和神经传导速度的检查可以测判面肌和神经的电活动。TreatmentsThere are three treatments for hemifacial spasm: medication, surgery, and botulinum toxin injections. Till now,the only effective treatment for HFS if MVD.Medication: Your doctor may prescribe anti-convulsant drugs such as carbamazepine (Tegretol) or phenytoin (Dilantin) to block firing of the nerve. Muscle relaxants such as baclofen (Lioresal), diazepam (Valium), and clonazepam (Klonopin) may also be prescribed. These drugs can be successful in treating mild cases but cause side effects (e.g., drowsiness, unsteadiness, nausea, skin rash, dependence). Therefore, patients are monitored routinely and undergo blood tests to ensure that drug levels remain safe and that the patient doesn't develop blood disorders.Botox injections: Botulinum toxin, or Botox, is a protein produced by the C. botulinum bacteria that cause muscle paralysis by blocking the electrical messages that “tell” the muscle to move. Messages are carried by a neurotransmitter called acetycholine. Botox blocks the release of acetycholine; as a result, the muscle doesn’t receive the message to contract. A very fine needle is used to deliver 1 to 3 injections into facial muscles invoiled. Botox usually works within three days and usually lasts for three months. Botox injections can be repeated indefinitely, however the effectiveness diminishes over the years due to the buildup of antibodies. Side effects include temporary facial weakness, drooping eyelid, eye irritation and sensitivity.Surgery: Medications and injections sometimes fail to control spasms or cause side effects. A procedure, called microvascular decompression(MVD), can relieve the nerve compression.For effective,and safe,more and more doctors approve MVD treatment. SO,although MVD is the only effective treatmrnt for HFS,it is never the first choice for treating HFS. A neurosurgeon makes a hole in the bone (craniotomy)of the head behind the ear to expose the facial nerve at the brainstem. A Teflon sponge is placed between the offending blood vessel and the facial nerve. About 90% of patients return to their regular life style after two months. Like all surgeries, there are risks. More frequent side effects include decreased hearing and facial weakness. In 95% of surgical cases there appears to be a blood vessel compressing the nerve. In general, results of surgery including :· 85% experience immediate relief from spasms· 9% report diminished spasms· 2% report delay in facial spasm in the month following surgery· 7% experience a recurrence of spasms after surgery治疗有药物、肉毒素局部注射和微血管减压三种治疗方法,但到目前为止,只有微血管减压手术是有效治疗。药物治疗:可采用安定类药物如地西泮、肌肉缓解药巴氯芬、抗癫痫药物卡马西平或大仑丁等。这些药物对症状较轻的患者可能有效,但副作用较大,如嗜睡、行走不稳、恶心、皮肤红斑和成瘾等。各种药物还有各自的副作用,患者需定期检查,防止严重的副作用。肉毒素局部注射:肉毒素是由肉毒细菌产生的可阻断神经肌肉电传导引起肌肉麻疲剧毒蛋白质物质。神经肌肉之间传导的神经递质为乙酰胆碱,肉毒素可阻止其释放,肌肉失去收缩信号。经注射器向发生抽搐的面肌注射肉毒素,一般3天后出现效果,可持续3个月。该治疗可反复进行,但效果逐渐减小,因为随时间延长,患者体内产生肉毒素抗体,影响其疗效。副作用包括面部无力、眼睑下垂、眼睛感觉过敏不适等。手术:药物和肉毒素治疗无效或副作用大,患者不能忍受这些副作用的,微血管减压手术可治愈该疾病,由于该手术创伤小,效果显著,越来越多的医师提倡该手术治疗(短暂药物治疗无效,影像学检查明确面神经受压的,为手术指证)。尽管手术治疗是目前面肌痉挛唯一有效治疗,但从来不是第一治疗选择。采用耳后(枕下乙状窦后径路)小切口,做3×2.5cm骨窗,切开脑膜,进入桥小脑角,找出Ⅶ、Ⅷ颅神经,如发现有占位性病变或蛛网膜粘连即进行切除和分解,如有压迫性血管,可在显微镜下利用显微器械给以分离开,如果分不开,可用Silicone或Teflon片隔垫开,亦可用肌肉片填塞在血管与神经之间。超过90%的患者恢复正常工作与生活。手术发现95%以上患者面神经有血管压迫,该手术的主要并发症是短暂单侧面瘫(11%)和耳聋(3%)。治疗效果:85%术后立即消失;10%术后痉挛明显减少;2%患者手术1月后再次出现痉挛;7%患者复发。早期有效率95%,长期有效率90%。
脑海绵状血管瘤局灶性癫痫患者:病理学发现、临床特征和手术治疗原则【据《ActqNeuropothol》2014年6月报道】题:脑海绵状血管瘤局灶性癫痫患者:病理学发现、临床特征和手术治疗原则(作者Je
1975~2011儿童颅咽管瘤的治疗趋势和结果【据《Neruo-Oncol》2013年3月报道】题:1975~2011儿童颅咽管瘤的治疗趋势和结果(作者Cohen M等) 由于其与周围重要组织结构关系
A craniopharyngioma (CP) is an embryonic malformation of the sellar and parasellar region.颅咽管瘤是鞍区 胚胎发育异常.The annual incidence is 0.5-2.0 cases/million/year and approximately 60 % of CP are seen in adulthood. 每年疾病的发病率为0.5-2.0/百万,60%为成年人. Typical initial manifestations at diagnosis in adults are visual disturbances, hypopituitarism and symptoms of elevated intracranial pressure. 典型的表现为视力损害,垂体功能低下和颅内压增高.Therapy of choice is surgery, followed by cranial radiotherapy in about half of the patients. 治疗方法为手术和辅助放疗.The standardised overall mortality rate varies 2.88-9.28 in cohort studies.死亡率为 2.88-9.28%. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. 颅咽管瘤患者心脏血管引起死亡率是正常人的3-19倍.Women with CP have an even higher risk外科治疗包括肿瘤全切除/肿瘤部分切除和囊液引流术.1.肿瘤全切除手术风险和手术后并发症多,需要有非常高超手术技巧的医师,本人对于年龄在25周岁以下的所有患者采用肿瘤全切除,目前,仅有一例复发,再次手术仍全切除,无死亡和致残发生.对于多发囊性颅咽管瘤患者,我也采用全切除,对于成人实质性肿瘤,有视力改变的,需做肿瘤部分切除的,多数我也采用肿瘤全切除.2.次全切除,对于有视力损害/肿瘤体积较大的,患者年龄超过45岁,可考虑次全切除,后放疗或立体定向放疗,在治疗的患者中只有1例.3.对于单个囊状有压迫症状的成人患者,可采用囊液引流术.通过立体定向技术作囊穿刺,并植入引流管,连接ommaya 储囊,以便重复抽囊液.根据本人的治疗经验,放疗对于囊性颅咽管瘤的治疗不理想.
最近连续手术治疗年龄超过75岁老人五例,最大为79岁,患者及其家属强烈要求手术治疗,手术后三叉神经痛症状消失,无明显手术并发症,因此,80岁以下无明显脏器功能损害者可耐受手术治疗.
Neurosurgery. 2012 Aug;71(2):E577-8.201Comparative evaluation of percutaneous radiofrequency rhizotomy, stereotactic radiosurgery and microvascular decompression in the management of refractory trigeminal neuralgia.Hitchon PW, Wassef SN, Pennigton E, Noeller J, Johnson V.AbstractINTRODUCTION: : Choosing the optimal treatment modality for refractory trigeminal neuralgia (TN) is multifactorial, and has not been well defined in the literature. In this review we specifically explore the role of three factors in determining the treatment modality: age, recurrence rate, and cost.METHODS: : Retrospective chart review of patients who underwent percutaneous Radiofrequency Rhizotomy (RFR), Stereotactic Radiosurgery (SRS) and Microvascular Decompressions (MVD) for trigeminal neuralgia between the periods of 2003 to 2011.RESULTS: : A total of 95 procedures were identified, including 46 MVDs, 27 RFR, and 22 SRS. The average age of patients who had: (1) MVD was 52 ± 12 (24-75), (2) SRS was 67 ± 15 (25-85), and (3) RFR was 71 ± 11 (51-89). Preliminary results suggest recurrence of symptoms in 3/46 patients treated with MVD, 3/21 treated with RF, and none in the patients initially treated with SRS. The total charges for the 3 procedures were (mean ± SD): 56 ± 2.5 × 1000, 1.3 ± 0.8 × 1000, and 44 ± 1.6 × 1000 US dollars, for MVD, RS, and SRS respectively. Actual payments varied depending on health insurance coverage.CONCLUSION: : Despite their unique advantages, the charges for the three procedures vary considerably. MVD was the most expensive procedure, and was least likely associated with facial numbness. SRS and RFR were recommended for older patients. SRS is expensive, and requires several weeks before symptomatic improvement. RFR is associated with immediate response, performed as an outpatient, and is the cheapest of the three procedures; however it is associated with the highest rate of recurrence
微血管减压(MVD)是三叉神经痛(TN)患者最有效的治疗方法。TN的发生率随年龄的增大而增加,因此老年患者的安全性是手术医师面临的问题。美国学者最近通过国家数据系列,分析患者年龄对MVD手术并发症发生率的影响。 通过回顾1999年到2008年住院患者(NIS),统计患者在住院期间的并发症,包括死亡率、并发症、住院时间及费用等。并发症包括急性疾病。本研究采用2个老年年龄时间点,65岁和75岁。共3273例TN患者作MVD,年龄6~93岁,平均为57岁。超过65岁患者占31.5%,超过75岁患者占10.9%。65岁以下的TN患者为2241例,65岁以上患者为1032例。75岁以下患者为2923例。75岁以上患者为350例。1. 以65岁为分界 65岁以下患者的死亡率为0.13%,65岁以上患者死亡率为0.68%(P=0.0087),后者是前者的5.1倍,并发症的发病率:心脏(0.95%对1.94%,P=0.0087),肺脏(1.09%对2.23%,P〈0.0001),血栓性疾病(0.27%对1.16%,P=0.0097),脑血管病(1.38%对3.29%,P=0.0003)。年龄与手术后颅内感染无明显关系。65岁以下患者平均住院时间3.3天,65岁以上的为4.0天(P〈0.0001)。 2. 以75岁为界 75岁以下患者住院死亡率为0.21%,75岁以上为1.16%(P=0.0021),后者是前者的5.6倍。并发症的发病率:心脏(0.79%对2%,P=0.0244),肺脏(1.13%对4.0%,P〈0.0001)。血栓性疾病(0.39%对2%,P〈0.0001),脑血管病(1.74%对34%,P=0.0044)。年龄与术后感染无关。住院天数老年为4.3天而非老年为3.4天(P〈0.0001)。 微血管减压(MVD)是治疗TN最有效的方法。尽管最近有报道随年龄增长并不增加MVD的风险,本文通过大数据病例分析得随着年龄的增长,MVD的死亡率和并发症明显增加,虽然发生率仍较低,仍应引起临床医师的注意。