Arthroscopic surgical treatment for neglected congenital muscular torticollis in adults.

BACKGROUND: Congenital muscular torticollis (CMT) is the third most common congenital musculoskeletal anomaly. The efficacy of surgical treatment of neglected CMT after skeletal maturity in adults remains controversial. The aim of this retrospective study was to determine the efficacy of arthroscopic release of the sternocleidomastoid (SCM) muscle for the treatment of neglected CMT in adult cases. METHODS: From April 2008 to July 2013, a total of 20 adult patients (9 men and 11 women) with a mean age of 23.0 years were surgically treated for neglected CMT with local anesthesia. The SCM muscle and clavicle were preoperatively marked. Through an artificial lacuna, an arthroscope and a radiofrequency probe were inserted. The sternal and clavicular attachments were dissected and divided via radiofrequency. Clinical evaluation was assessed using a modified Cheng and Tang score. RESULTS: The operation was successfully completed in all the patients. Follow-up examinations from 12 to 40 months in the 20 patients showed complete muscular release and satisfactory cosmetic appearance with no recurrence. There was no evidence of serious complications. The neck scars were no longer visible after several weeks. According to the modified Cheng and Tang scale, 11 patients displayed an excellent result and 9 patients had a good result. CONCLUSIONS: Arthroscopic release of the SCM muscle with local anesthesia is a simple and effective way to surgically manage neglected CMT without any risk for either damage to the neurovascular structures or leaving visible scars.

Selective peripheral denervation for cervical dystonia: long-term follow-up.

OBJECTIVE: 61 procedures with selective peripheral denervation for cervical dystonia were retrospectively analysed concerning surgical results, pain, quality of life (QoL) and recurrences. METHODS: The patients were assessed with the Tsui torticollis scale, Visual Analogue Scale (VAS) for pain and Fugl-Meyer scale for QoL. Evaluations were performed preoperatively, early postoperatively, at 6 months, then at a mean of 42 (13-165) months. All patients underwent electromyogram at baseline, which was repeated in cases who presented with recurrence of symptoms after surgery. RESULTS: Six months of follow-up was available for 55 (90%) of the procedures and late follow-up for 34 (56%). The mean score of the Tsui scale was 10 preoperatively. It improved to 4.5 (p<0.001) at 6 months, and 5.3 (p<0.001) at late follow-up. VAS for pain improved from 6.5 preoperatively to 4.2 (p<0.001) at 6 months and 4 (p<0.01) at late follow-up. The Fugl-Meyer score for QoL improved from 43.3 to 46.6 (p<0.05) at 6 months, and to 51.1 (p<0.05) at late follow-up. Major reinnervation and/or change in the dystonic pattern occurred following 29% of the procedures, and led in 26% of patients to reoperation with either additional denervation or pallidal stimulation. CONCLUSIONS: Selective peripheral denervation remains a surgical option in the treatment of cervical dystonia when conservative measures fail. Although the majority of patients experience a significant relief of symptoms, there is a substantial risk of reinnervation and/or change in the pattern of the cervical dystonia.

[Significance of local synchronization and oscillatory processes of thalamic neurons in goal-directed human behavior].

The time-frequency characteristics and interneuron interaction in the cell ensembles of non-specific (CM-Pf) and motor (Voi) thalamus were analyzed. Neuronal activity was registered by microelectrode technique during 18 stereotactic neurosurgery operations in spasmodic torticollis patients. The presentation of functionally significant verbal stimuli was accompanied by the emergence of short-term (0.5-1.5 s) local synchronization and stabilization of the oscillatory (3-6 Hz) activity in nearby neurons of nonspecific (CM-Pf) thalamus. These focuses of synchronized oscillatory neuronal activity were correlated with the moment of the greatest concentration of selective attention. Similar phenomenon of short-term synchronization was observed in the motor (Voi) and nonspecific (CM-Pf) thalamus of the human brain during the voluntary movements. Synchronization of neuronal activity occurred at the height of the motor act implementation, correlating with the maximum muscle tension, as well as in aftereffect of the voluntary movement. Overall, the findings suggest an important role of the local oscillations (3-6 Hz) and synchronization ofthalamic neurons in the mechanisms of relevant information transmission during goal-directed human behavior.

Successful treatment of cervical dystonia induced by basal ganglion venous angioma with gamma knife thalamotomy.

Stereotatic radiosurgery is typically not the first line of treatment for cervical dystonia. We present a patient with a rare cervical dystonia induced by a venous angioma in the right basal ganglion. The patient was successfully treated with a gamma knife thalamotomy after failed treatments of botulinum toxin injections and peripheral denervation.

The role of the parafascicular complex (CM-Pf) of the human thalamus in the neuronal mechanisms of selective attention.

The reactions of 93 neurons in the parafascicular complex (CM-Pf) of the human thalamus were studied by microelectrode recording during stereotaxic neurosurgical operations in patients with spastic torticollis. High reactivity was demonstrated for two previously classified types of neurons with identical irregular (type A) and bursting Ca2+ -dependent (type B) activities in response to presentation of relevant verbal stimuli evoking selective attention in humans. Concordant changes in the network activity of A and B neurons were observed, in the form of linked activatory-inhibitory patterns of responses and the appearance, at the moment of presentation of an imperative morpheme of the command stimulus, of rapidly occurring intercellular interactions consisting of local synchronization with simultaneously developing rhythmic oscillatory (3-4 Hz) activity. Data are presented on the existence of a direct connection between these neuronal rearrangements and activation of selective attention, providing evidence for the involvement of the thalamic parafascicular complex (CM-Pf) in the mechanisms of selective attention and processing of relevant verbal information during the preparative period of voluntary actions.

[Role of the human thalamic parafascicular (CM-Pf) complex in neuronal mechanisms of selective attention].

Responses of 93 single units of the human thalamic CM-Pf complex to relevant and irrelevant verbal (or sensory) stimuli were studied using microelectrode technique in alert diskinetic patients suffering from the tonic forms of spasmodic torticollis during 11 stereotaxic operations. The response patterns of two types units with irregular unitary (A-type) and low-threshold bursting Ca(2+)-dependent (B-type) spike activity were studied. Three main conclusions emerge from the studies: 1) high reactivity of both A- and B-units to presentation of relevant verbal stimuli with differences of their response patterns as determined by the type of constituent elements; 2) close functional connectivity of these neuronal changes with the level of selective attention; 3) at the moment of attention activation, the appearance of transient interneuronal interactions between adjacent A and B cells characterized by the local synchronization and stabilization of rhythmic oscillations. These data point to considerable contribution of the thalamic CM-Pf complex and its neuronal mechanisms into organization of the human selective attention and triggering verbal-related processing during performance of purposive speech-provoked voluntary acts.

Pallidal deep brain stimulation in cervical dystonia: clinical outcome in four cases.

OBJECTIVE: Report on the clinical results following bilateral globus pallidus interna deep brain stimulation in four patients (one female and three males) with severe cervical dystonia, mean age 48 years (range 37-67). METHODS: All four patients had failed extensive medical and botulinum toxin treatment. The mean duration of the disease was nine years (range 4-15 years). Patients were assessed pre and postoperatively using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Pre-operatively, the mean TWSTRS total score was 43.2 (range 28-60.5). Posteroventral pallidal deep brain stimulators were inserted using MRI and microelectrode recording guidance. Last follow-up was 15 months for the four patients. RESULTS: Mean reduction in the TWSTRS total scores at last follow- up was 73% (range 61- 85%). Improvement in pain occurred soon after deep brain stimulation surgery. Motor improvement was delayed and prolonged over several months. Frequent adjustment in the stimulation parameters was necessary in the first three months. CONCLUSION: Bilateral pallidal stimulation is effective in management of selected cases of intractable cervical dystonia.

The Canadian multicenter trial of pallidal deep brain stimulation for cervical dystonia: preliminary results in three patients.

OBJECT: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is beneficial for generalized dystonia and has been proposed as a treatment for cervical dystonia. The Canadian Stereotactic/Functional and Movement Disorders Groups designed a pilot project to investigate the following hypothesis: that bilateral DBS of the GPi will reduce the severity of cervical dystonia at 1 year of follow up, as scored in a blinded fashion by two neurologists using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Secondary outcome measures included pain and disability subscores of the TWSTRS, Short Form-36 quality of life index, and the Beck Depression Inventory. METHODS: Three patients have undergone surgery in Calgary with a follow-up duration of 7.4 +/- 5.9 months (mean +/- standard deviation). One patient underwent inadvertent ineffective stimulation for the first 3 months and did not experience a benefit until DBS programming was corrected. All three patients had rapid response to stimulation, with the muscles relaxing immediately and abnormal movements improving within days. Total TWSTRS scores improved by 79%, and severity subscores improved significantly, from 15.7 +/- 2.1 to 7.7 +/- 2.9 (paired t-test, p = 0.02). Pain and disability subscores improved from 25.5 +/- 4.1 to 3.3 +/- 3.1 (paired t-test, p = 0.002) and from 13.3 +/- 4.9 to 3.3 +/- 4.2 (paired t-test, p = 0.06), respectively. CONCLUSIONS: Although it is too early to reach broad conclusions, this report of preliminary results confirms the efficacy of DBS of the GPi for cervical dystonia.

Post-operative progress of dystonia patients following globus pallidus internus deep brain stimulation.

In the current era of functional surgery for movement disorders, deep brain stimulation (DBS) of the globus pallidus internus (GPi) is emerging as the favoured intervention for patients with dystonia. Here we report our results in 20 patients with medically intractable dystonia treated with GPi stimulation. The series comprised 14 patients with generalized dystonia and six with spasmodic torticollis. Although comparisons were limited by differences in their respective neurological rating scales, chronic DBS clearly benefited both patient groups. Data conveying the rate of change in neurological function following intervention are also presented, demonstrating the gradual but progressive and sustained nature of improvement following stimulation of the GPi in dystonic patients.

Bilateral pallidal stimulation for cervical dystonia. An optimal paradigm from our experiences.

We sought to identify optimal paradigms of bilateral globus pallidus internus (GPi) stimulation in 3 subsequent patients with severe cervical dystonia. At low frequency stimulation (50-60 Hz) with wide pulse width (500 micros) and high amplitude (4.5-8.0 V), we observed immediate and consistent improvement of dystonia and dystonia-associated pain. Stimulation of the posteroventral portion of the GPi led to pronounced alleviation of dystonia; stimulation of the anterodorsal portion or at the dorsal border of the GPi resulted in significant worsening of symptoms. The therapeutic benefit obtained by using the optimal stimulation parameters continues and has lasted for at least 1 year in each patient.

Bilateral internal globus pallidus stimulation for the treatment of spasmodic torticollis.

Three patients with spasmodic torticollis (ST) obtained substantial benefit from bilateral globus pallidus internus (GPi) stimulation. Progressive improvement in ST occurred over several months but residual cervical dystonia remained. These results corroborate those obtained by Krauss et al. on three patients with ST.

Treatment of spasmodic torticollis with intradural selective rhizotomies.

To determine the effects of ventral cervical and selective spinal accessory nerve rhizotomy on spasmodic torticollis, 58 patients who had undergone surgery between 1979 and 1987 were reviewed retrospectively. At the time of surgery, each nerve rootlet was electrically stimulated to determine its effect on the nuchal musculature prior to sectioning. Forty-nine patients (85%) had a marked improvement in their condition, with 33 (57%) attaining an excellent result and 16 (28%) noting significant improvement. Patients complained of abnormal head posture, nuchal muscle spasms, and pain prior to surgery. Muscle spasms were completely relieved in 42 patients (72%) and markedly reduced in 10 (17%). Of the 47 patients with preoperative pain, 30 (64%) were free of their pain and eight (17%) noted that the pain was reduced in intensity and frequency. Thirty-four patients (59%) reported that their resting head posture was restored to a neutral position. The likelihood that a patient's head posture returned to normal was inversely proportional to the preoperative duration of the spasmodic torticollis. Twenty-six patients (45%) suffered mild transient difficulty with swallowing solid foods in the immediate postoperative period. In most cases these minor difficulties abated in the months following surgery.

Management of spasmodic torticollis.

Based on a review of the literature, the management of spasmodic torticollis may begin with the conservative measures of pharmacotherapy, sensory feedback, or percutaneous dorsal column stimulation. Approximately 50% of patients will benefit from an adequate trial of these modalities. With particularly resistant and disabling torticollis, the ablative procedures of microsurgical cervical rhizotomy or stereotaxic thalamotomy have offered significant relief in about 74% and 56%, respectively, of properly selected cases. Nevertheless, the variability in the duration of response to all forms of therapy has made the treatment of spasmodic torticollis a difficult and often frustrating undertaking.

[Long-term follow-up study of spasmodic torticollis].

Since 1958, 61 patients with spasmodic torticollis, 46 of whom did not develop other kinds of involuntary movements, have been experienced in our neurosurgical clinic. The course of these 46 patients was followed for more than one year. The retrospective analysis revealed 25 patients were treated conservatively, and 21 patients were treated surgically. Surgical treatment consisted of stereotactic ventrolateral thalamotomy in 19 patients, and Olivecrona's operation in the other 2. The long-term outcome of conservative therapy was compared to that of surgical therapy. One patient with severe retrocollis with horizontal components was treated successfully but transiently by implantation of stimulating electrodes in the cervical epidural spinal cord. The peak incidence of the onset of the disease was in the fourth decade; however, the time of onset ranged from 7 to 55 years of age. The period from disease onset to the final evaluation in the follow-up was 9.3 years (mean) in the conservative group, and 8.5 years in the surgically treated group (no significant difference). There were no significant differences between the groups in the interval from the time of disease onset to the initial evaluation in our clinic, stage determined at evaluation, and follow-up period after the initial evaluation. Of the 25 conservatively treated patients, symptoms and signs remained unchanged in 8, deteriorated in one, but improved significantly in most of the remaining patients. Many of the surgically treated patients showed improvement one week after surgery, but the long-term outcome was not significantly better than that of the conservatively treated patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Stereotaxic thalamotomy in 55 cases of dystonia.

The results of stereotaxic thalamotomy in 55 cases of dystonia are presented. The 16 cases with generalized dystonia were of varied pathogenesis, only 7 being typical of the idiopathic form of adolescent onset. Four of the 16 cases benefited considerably, but the others showed little or no lasting improvement. These results are in contrast to those obtained by Cooper (1976). Of the 27 cases with segmental or focal dystonia, 22 had spasmodic torticollis; 16 of these had bilateral thalamotomies, and 62 per cent were much improved. The incidence of operative complications, in particular dysarthria, was high following bilateral lesions. The incidence of hemiparesis, known to have persisted for more than a year, was 15 per cent. This complication was as frequent in those with unilateral as with bilateral thalamotomies. The incidence of dysarthria in those without preoperative bulbar dystonia was much higher in those who had bilateral lesions (56 per cent) as compared with those who had unilateral lesions (11 per cent). The group that has been identified as benefiting greatly from stereotaxic surgery comprises those with hemidystonia following unilateral brain damage. In these patients, symptomatic improvement in abnormal movement is striking and the incidence of operative side effects from unilateral lesions is low.

Surgical possibilities for alleviation of axial dyskinesias - comparison with forced movements of the head and eyes.

107 patients suffering from axial dyskinesias (torticollis and torsion dystonia) were observed at our clinic over a period of 10 years. In 15 patients, stereotactic lesions involving the head-turning bundle at the caudatocapsular level, which mediates forced adversive movements, were performed. In 70% good to excellent results were confirmed. However, differences between forced adversive movements and torticollis were identified. There are striking motor and motivational similarities between torticollis and ritualized fixed action patterns of low-ranking subjects. The aetiology of axial dyskinesias, especially of torticollis, is multifactorial, long-term psychic stress being an important factor. The relationship of axial dyskinesias, especially of torticollis, to compulsive neuroses is discussed, and Mazar's 'adversive' target area is identified as a partial orbitomedial leukotomy.

VOI thalamotomy in spasmodic torticollis.

We performed stereotactic VOI thalamotomy as the only surgical treatment in spasmodic torticollis in 17 patients since 1972. The patients selected for surgery mainly presented horizontal torticollis. All the patients improved after unilateral thalamotomy. In most cases there was a delay in improvement varying from 3 months to 2-3 years postoperatively. The severity of the psychic disturbances present in most patients at the time of onset of their torticollis did not have any relevance to their final outcome after surgery.