三联术治疗痉挛性斜颈

采用三联手术方法治疗旋转型（ｎ２６）、侧屈型（ｎ６）痉挛性斜颈共３２例，其中男性１８例，女性１４例，平均年龄３６．５岁。适应证：１．药物治疗一年无效的旋转型、侧屈型患者。２．颈肌严重痉挛的扭转痉挛患者。结果，旋转型２６例，优１８例（６９．２％）；良７例（２７．０％）；进步１例（４％）。侧屈型６例，优４例（６６％）；良２例（３３％）。本研究结果证实三联手术的疗效较二联手术优越。     

Surgical treatment of spasmodic torticollis: effectiveness of microvascular decompression

We report two patients of spasmodic torticollis who underwent different types of surgery according to their pathogenetic mechanisms. The first case was a 42-year-old man who had two years history of right-sided antecollis. EMG of the sternocleidal muscle on the affected side showed pathological findings. Characteristic findings on the vertebral angiography were a low position of the branching of tortuous PICA. At operation, we confirmed sandwich-like compression of left accessory nerve between the PICA and vertebral artery. Decompression of the nerve from these arteries led to a remarkable relief of the symptom. The second case was a 70-year-old man whose neck had been forced to bend backward; retrocollis over two years. EMG study was negative. The vertebral angiography showed no abnormality, either. Because there was no evidence of vascular compression on both accessory nerves at surgery, we chose bilateral C1-3 anterior root section which was effective in consequence. We found so far sixteen cases of spasmodic torticollis in the literature including ours who were benefited by microvascular decompression (MVD). All of them presented antecollis or horizontal type. The characteristic operative finding was the compression of the accessory nerve located at between the dural perforation of the vertebral artery and dural attachment of C1 dentate ligament. We can therefore propose a mechanism that the compression at the junction of the central and peripheral myelin causes such a neuroexcitatory symptom as in cases of facial spasm and trigeminal neuralgia.(ABSTRACT TRUNCATED AT 250 WORDS)     

电生理监测下改良Foerster-Dandy手术治疗痉挛性斜颈初探

目的探讨电生理监测下改良Foerster-Dandy手术治疗痉挛性斜颈的安全性和疗效。方法前瞻性研究2010年11月至2011年7月显微手术治疗的7例痉挛性斜颈病人,全部采用电生理监测下改良Foerster-Dandy手术,手术方法为枕后正中入路硬膜下双侧副神经根、C1脊神经根切断、C2-4脊神经前、后根选择性部分切断术。结果全部病人术后立即感到痉挛状态明显缓解,随访期间缓解率为85.7%(6/7),生活质量提高率在随访期间为85.7%,术后6例发生不同程度转颈无力、耸肩无力,随访期间均有所好转,无吞咽困难和头颈部支撑困难病例,无严重并发症发生。随访期间1例斜颈复发,使用盐酸度洛西汀+苯海索治疗后症状显著改善。结论电生理监测下改良Foerster-Dandy手术治疗痉挛性斜颈安全有效,熟悉局部解剖、掌握显微手术技巧是手术安全的基础;选择合适的病例、合理使用电生理监测技术、个体化制定神经根选择性切断的程度是手术疗效的保证。本研究只是改良Foerster-Dandy手术治疗痉挛性斜颈的初步探索,该术式大宗病例和长期随访资料尚需进一步积累完备。     

An exteroceptive reflex in the sternocleidomastoid muscle produced by electrical stimulation of the supraorbital nerve in normal subjects and patients with spasmodic torticollis

We examined suppression of EMG activity in the contracting sternocleidomastoid muscles, produced by electrical stimulation of the supraorbital nerve in 10 normal subjects and 9 patients with spasmodic torticollis. This exteroceptive reflex in the sternocleidomastoid muscle consisted of 2 or 3 phases: (1) an early, small, and unstable phase of facilitation, followed by (2) a period of suppression beginning 35 msec after the stimulus, lasting for 35 msec with a reduction in EMG activity to approximately 40% of the prestimulus level, and (3) a further phase of facilitation at a latency of 70 msec, with duration 35 msec and an increase in EMG activity to approximately 35% above prestimulus levels. The latency and duration of the suppressive phase of this reflex were similar to the exteroceptive suppression of EMG activity in the masseter muscle after supraorbital nerve stimulation (masseter silent period). In patients with spasmodic torticollis, the depth of this exteroceptive suppression in the sternocleidomastoid muscles was less than that observed in an age-matched cohort of normal subjects, although the latency and duration were normal. In contrast, exteroceptive suppression in the masseter muscle was normal. These findings suggest abnormal function of inhibitory interneuronal networks between the 5th cranial nerve and the motor neurons of the spinal accessory and upper cervical nerves which mediate exteroceptive suppression in the sternocleidomastoid muscle in patients with spasmodic torticollis.     

内镜辅助下选择性颈神经根切断术治疗痉挛性斜颈

目的 探讨内镜辅助下选择性颈神经根切断术治疗痉挛性斜颈的疗效.方法 回顾性分析2008年1月至2009年6月显微手术治疗的57例痉挛性斜颈患者的临床资料.该显微手术,术中不咬除枕骨鳞部及枕大孔,在内镜辅助下行硬膜下双侧副神经根及颈1脊神经根切断.结果 全部患者平均随访7个月.100%病人术后立即感痉挛状态明显缓解;随访...     

Posttraumatic torticollis

We report six cases of torticollis precipitated by neck trauma. The dystonia began 1 to 4 days after the trauma and differed clinically from idiopathic torticollis by marked limitation of range of motion, lack of improvement after sleep ("honeymoon period"), and absence of geste antagonistique. Worsening with action was not present; nor was there improvement with support as seen with idiopathic torticollis. Onset of pain immediately after the trauma and marked spasms of the paracervical muscles were other predominant features. Anticholinergic therapy was without benefit; however, some improvement occurred with botulinum toxin injection. It is concluded that torticollis can be caused by peripheral trauma and that it has unique clinical characteristics.     

Idiopathic torticollis: Sternocleidomastoid myopathy and accessory neuropathy

Biopsies of the two heads of the sternocleidomastoid muscle were studied in 9 children with idiopathic torticollis, from 8 months to 17 years of age, who were undergoing surgical release of the sternal and clavicular attachments on the side of the contracture. Extensive fibrosis involved mainly the sternal head. Nonspecific myopathic changes, also mainly in the sternal head, included cytoarchitectural alterations of muscle fibers, necrosis, and focal inflammation. Histochemical type grouping and grouped atrophy were extensive in some cases and present in all except one, but the clavicular head was predominantly involved. Denervation and reinnervation are common chronic features in idiopathic torticollis, probably secondary to entrapment neuropathy: the accessory nerve reaches the clavicular head by passing through the sternal head. Separate arterial supplies predispose to ischemia in the sternal head, resulting in focal myopathy and fibrosis.     

Spasmodic torticollis following unilateral VIII nerve lesions: neck EMG modulation in response to vestibular stimuli.

Three patients with spasmodic torticollis following VIII nerve lesions (VIII-ST) underwent quantitative assessment of their sternomastoid EMG during vestibular (otolith and semicircular canal) stimulation. The results were compared with a normal control group and with six patients with idiopathic spasmodic torticollis (ST). Backwards tilt of the VIII-ST patients resulted in a marked increase in the EMG, especially in the more affected sternomastoid, whereas this manoeuvre did not have a significant effect in normal subjects, or had a variable effect in the ST group. These results suggest that those with torticollis following VIII nerve lesions are a distinct group. Since there was no relationship between the side of the VIII nerve lesion and the direction of the torticollis a direct aetiological link between the two is, however, unlikely. The unusual EMG/tilt responses are explained on the basis of peripheral imbalance of utricular signals (maximal in the supine position) in the presence of central deranged processing of information concerning head posture.     

Ekbom's sydrome and spasmodic torticollis: case report

The Ekbom's syndrome, also known as delirium of parasitic infestation, acarophobia, delusional parasitosis, psychogenic parasitosis, is a disease of rare occurrence. Generally it is characterized by the firm conviction of the patients to be infected by worms that come out of the skin, usually from the scalp or even from the mouth, from the eyes or from the genital region. Most of the patients are elderly and female, and with frequent social isolation. Some cases are associated with organic diseases as hyperthyroidism, diabetes, cortical lesion, intoxication by medicines. To our knowledge the association between delusional parasitosis with spasmodic torticollis was not described in literature. We report the case of a 72-year-old-woman with delusional parasitosis associated with spasmodic torticollis.     

Reciprocal inhibition of forearm flexor muscles in spasmodic torticollis.

Reciprocal inhibition between forearm extensor and flexor muscles was tested by means of an H-reflex technique in patients with spasmodic torticollis and normal controls. In both, patients and controls three different phases of reciprocal inhibition could be demonstrated with maximal inhibition at conditioning test intervals of 0 ms, 15 ms and 100 ms, respectively. However, the quantitative amount of this inhibition was different for the patients and the controls. Significantly less inhibition was found for the second and the third phase of reciprocal inhibition in the patient group. Discriminant analysis showed a clear separation between normal subjects and patients if the amount of reciprocal inhibition of the second and third phase were taken into account. We were not able to detect any side differences neither for the patients nor for the controls. The findings demonstrate a functional disturbance of motor control mechanisms of a clinically unaffected extremity in spasmodic torticollis. This is believed to reflect a bilateral disturbance most likely within the basal ganglia or their outflow. Therefore, our data support the idea, that spasmodic torticollis is associated with or even due to a generalized rather than a focal disturbance of motor control mechanisms.     

副神经血管减压治疗痉挛性斜颈(附2例报告及文献复习)

目的探讨痉挛性斜颈的发病原因,评价副神经血管减压治疗痉挛性斜颈的效果。方法对2例痉挛性斜颈经过其他方法治疗无效且不愿意选择肌肉和神经切断的患者行副神经血管减压。术中发现椎动脉严重压迫副神经,用柔软的涤纶绵将神经与血管隔开而达到充分减压。结果例1在术后早期症状有所改善,术后3个月完全恢复正常。例2手术后3周头、颈位置已恢复正常,术后3个月症状进一步改善。结论血管压迫副神经是引起痉挛性斜颈的病因之一,副神经血管减压对某些痉挛性斜颈确有疗效,但应该严格筛选和控制手术适应证,术后应建立客观评价疗效的标准。     

Cerebrospinal fluid protein patterns in spasmodic torticollis.

In order to investigate the value of CSF-protein analyses in spasmodic torticollis CSF from six patients with probable organic and two patients with probable psychogenic torticollis was examined by isoelectric focusing and electrophoresis. In all the patients with organic torticollis two pathological CSF-protein fractions were found in the alkaline region on electrofocusing and in four cases aberrant fractions occurred also in the acidic pH range. An increasing number of abnormal fractions were noted during at least the first year after onset of symptoms. Lithium treatment of three patients resulted in a striking decrease of torticollis as well as of the number of abnormal CSF-protein fractions. During placebo treatment of two cases, torticollis and the pathological CSF-proteins recurred. Some observations, including a few previous autopsy findings, might indicate that an encephalitogenic agent is involved in the pathogenesis of organic torticollis. In the patients with psychogenic torticollis the CSF-protein pattern was normal. This investigation supports a recent suggestion that organic and psychogenic torticollis might be distinguished by electrofocusing of the CSF-protein.     

Nitrous oxide ameliorates spasmodic torticollis

In a case of therapy-resistant spasmodic torticollis administration of nitrous oxide (N2O) resulted in a dramatic transient amelioration of the dystonic movements. We suggest that manipulations of the opioid system may be useful in the therapy of this condition. Furthermore, our results with N2O may provide insight into the pathogenesis of the disease.     

Familial spasmodic torticollis

Presented are four cases of familial spasmodic torticollis, comprising members of three families. The age of onset tended to be family-specific, and no afflicted family member had evidence of more widespread disease (dystonia musculorum deformans). Familial incidence supports the conclusion that spasmodic torticollis is organic in origin. Familial spasmodic torticollis occurs more often than has been generally recognized.     

旋转型痉挛性斜颈的手术治疗

目的 研究旋转型痉挛性斜颈（rotational spasmodic torticollis，RoST）的临床分型和手术治疗。方法对RoST按痉挛肌肉范围，姿态，肌肉痉挛的方式，病情程度分型。手术治疗：采用下列术式或二至三种术式组合。术式一：面部旋向侧颈后痉挛肌肉选择性切除术。术式二：同侧颈神经1-6（C1-8）后支选择性切断术。术式三：对侧副神经切断术（或加胸锁乳突肌切除术）。结果术后6个月评定疗效，全组423例，斜颈症状消失292例（69％），显效78例（18．4％），进步42例（10％），无效11例（2．6％）。无死亡，无并发症。结论采用颈部痉挛肌肉和神经选择性切断术治疗RoST创伤小、操作简便，安全无残疾。     

Therapeutic outcome of spasmodic torticollis]

We investigated 117 patients with spasmodic torticollis who had visited us to seek for appropriate treatment in these 14 years. They were 71 men and 46 women, aged 44 +/- 14 (mean +/- SD) years, and suffered from this disorder during 4 +/- 5 years, maximum 26 years. Involuntary abnormal head positions, not only torticollis but also laterocollis and antero- or retrocollis, were contained in this study. Most of them were torticollis due to idiopathic focal dystonia. One or more courses of alcoholization therapy was accomplished in 82 patients who wished to be done. This therapy course consisted of about ten times totally of 99% ethanol injection to the motor point of two most hypertonic neck muscles, either side of the sternocleidomastoideus and the opposite side of the splenius in most cases, repeated every 2 or 3 weeks. One patient received as many as 98 times of this injection and resolved completely. Training to reinforce antagonistic muscles was also instructed. Twenty-one patients (26%) were resolved completely after this treatment. Fifty-four patients (66%) were ameliorated and satisfied partially, but 18 of them relapsed in 1 to 4 years after the treatment and were obliged to repeat one more course of this treatment. On the other hand, in five patients their torticollis improved under certain drug therapy alone. Sixteen patients (14%) gave up to continue the treatment within two months, and 14 patients (12%) dropped out before starting the therapy. This alcoholization therapy resulted in amelioration of torticollis in about 90% of the patients with a long effective period. Nevertheless, this alcohol injection is painful, and requires 5 to 6 months to be completed. In 2 patients who had already received many times of this injection, sudden hoarseness occurred one day immediately after the alcohol injection to the sternocleidomastoideus. This complication was presumably brought about by the unexpected infiltration of alcohol to the laryngeal area, located posterior to that muscle. They recovered in two months, but careful attention should be paid to the adverse effects. If botulinum toxin be available also in our country, we will be able to have another choice of therapy and the treatment of this disorder will become easier.     

Benign paroxysmal torticollis of infancy

Benign paroxysmal torticollis is an episodic functional disorder of unknown etiology that occurs in the early months of life in healthy individuals. The child's head tilts to one side for a few hours or days, usually without any associated symptoms. The disorder, which disappears within the first few years of life, is often misinterpreted and the patient pointlessly undergoes numerous tests. We present our series of 22 patients observed at the pediatric neurology outpatients clinic in Padova with a view to refreshing the pediatrician's memory on this frequent, benign pathology.     

经颅磁刺激、电刺激与肉毒素治疗痉挛性斜颈的疗效观察

目的本研究应用磁刺激、电刺激三叉神经和A型肉毒素局部注射来治疗痉挛性斜颈患者,并对三种治疗方法进行疗效对比。方法以扭转型为主的60例痉挛性斜颈患者,随机分为经颅磁刺激组20例,电刺激三叉神经组20例,同期肉毒素局部注射组20例。三组年龄及病程比较无统计学差异。结果经颅磁刺激组和电刺激三叉神经组在治疗前后临床分级评分及临床见效时间评定和临床疗效评定方面均优先于同期肉毒素局部注射组(p<0.01),前两者比较无显著差异(p>0.01)。结论采用经颅磁刺激及电刺激三叉神经眼支的方法治疗痉挛性斜颈疗效显著,且其近期疗效优于A型肉毒素局部注射治疗。     

痉挛性斜颈的手术治疗(附580例报道)

目的 探讨痉挛性斜颈（ST）的手术方法及其疗效。方法 回顾性分析1995~2015手术治疗的580例痉挛性斜颈的临床资料。借助肌电图、CT或MRI等检查判断参与痉挛的肌群,将痉挛肌肉分为原动肌、协同肌、随动肌,对原动肌做去神经术和肌切断术,协同肌做去神经术,随从肌可不予处理,从而形成三种术式:术式一,面部旋向侧颈后痉挛肌肉选择性切除术;术式二,同侧颈神经 1~6后支选择性切断术;术式三,副神经切断术。旋转型ST和侧屈型ST采用二联术或三联术,后仰型ST采用双侧术式一和术式二组合,前屈型 ST采用双侧术式三组合,混合型ST分期采用颈部神经选择性切断术和肌肉选择性切除术。结果 565例术后随访6个月~15年;术后6个月评估疗效:痊愈429例,显效68例,进步52例,无效16例;优良率为88%。无死亡、无严重并发症。结论 选择性周围神经切断术及肌切断术是治疗痉挛性斜颈安全有效的方法。     

微侵袭手术治疗侧屈型痉挛性斜颈81例

目的研究侧屈型痉挛性斜颈(LFST)微侵袭手术治疗效果。方法将LFST相关的痉挛肌肉分成主要责任肌和次要责任肌,并明确每一肌肉的支配神经。对LFST临床分型,按不同分型制定个体手术方案。采用下列术式组合治疗。术式1:屈向侧颈痉挛肌肉选择性切除术。术式2:屈向侧颈神经1～6后支选择性切断术。术式3:屈向侧副神经切断术(或附加胸锁乳突肌、斜角肌切除术)。三种术式组合称"三联术",术式1和术式3组合称"二联术"。结果术后6～12个月评定疗效,全组81例痊愈69例(85.2%),显效8例(9.9%),进步4例(4.9%),无死亡和严重并发症。35例采用二联术治疗的患者中,痊愈27例,显效5例,进步3例,优良率91.4%(32/35);46例采用三联术治疗患者中,痊愈42例,显效3例,进步1例,优良率97.8%(45/46)。结论采用颈部痉挛肌肉选择性切除和相关神经选择性切断术治疗LFST,手术安全创伤小,效果满意。