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Probable torticollis revealed in decapitated skull.

The skeletal features of a moderately decomposed decapitated head recovered in 1993 are consistent with torticollis (wryneck) and inconsistent with other possible syndromes. Asymmetries of the face, cranial vault, mandible, and cervical vertebrae closely resemble published cases of paleopathological and recent torticollis. The laterally directed left occipital condyle and articulation of the basicranium and cervical vertebrae indicate that the head was tipped toward the left shoulder. Right-left asymmetries of areas of muscular attachments are compatible with a leftward head deviation. Mild arthritis of the atlantal-occipital and intervertebral joints, clinoid bridging, and thickening of the inner table of the frontal squamosa may not be related to the possible torticollis. The postural deviations of the head and neck may aid in the identification of this homicide victim, as did skeletal evidence to torticollis in an earlier case from Britain.

Female↗

Torticollis: an unusual presentation of spontaneous pneumomediastinum.

Torticollis is a common clinical sign encountered by pediatricians in a wide variety of childhood illnesses including those from traumatic, neurologic, infectious, inflammatory, and psychogenic causes. We describe a case of spontaneous pneumomediastinum (SPM) manifested as acute torticollis. To the best of our knowledge, torticollis as a result of spontaneous pneumomediastinum has not yet been described. Pneumomediastinum should be included in the differential diagnosis of torticollis.

Acute Disease↗

Spasmodic torticollis: a case report and review of therapies.

BACKGROUND: Spasmodic torticollis is a movement disorder of the nuchal muscles, characterized by tremor or by tonic posturing of the head in a rotated, twisted, or abnormally flexed or extended position or some combination of these positions. The abnormal posturing of the head allows this disorder to be clinically diagnosed. Psychiatric symptoms frequently accompany or precede the diagnosis of the movement disorder. METHODS: Using the key words "torticollis," "spasmodic torticollis," "therapy," "behavior therapy," "botulinum toxin," MEDLINE was searched from 1989 to 1996 for information on the cause and treatment of spasmodic torticollis. RESULTS AND CONCLUSIONS: Therapies include behavior modification, such as biofeedback, hypnosis, or simply training the patient to consciously readjust the position of the head; pharmacotherapy, using a variety of agents, the most commonly prescribed being anticholinergic medications or the botulinum toxin type A; and surgery, which entails selectively denervating the muscles responsible for the abnormal movement or posture of the head. The most effective treatments include surgery and botulinum, with sustained success rates ranging from approximately 60 to 90 percent.

Adult↗

Computer-aided measurement and grading of cranial asymmetry in children with and without torticollis.

Our aim was to develop a simple, non-invasive method that could be used to objectively record cranial symmetry over time. We utilized this new method to investigate the relationship between torticollis and progressive plagiocephaly. From 1995 to 1999, the head shapes of 419 torticollis patients and 1 211 normal children were evaluated. We used Ezeform strip, a splint material, to make a permanent ring of the head circumference. Each head ring was recorded, scanned, and analyzed. An asymmetric index (AI) was designed. Double-blind comparisons of clinical assessment with AI values demonstrated a good agreement. Asymmetry of the cranium was found with similar frequency in torticollis and normal babies up to 12 months old. After 1 year of age, the deformity persists in patients with torticollis into their adolescence, while the normal group shows increased symmetry. This new recording system offers an objective and efficacious methods for following the natural history of cranial asymmetry.

Journal Article↗

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.

Electromyography↗

Perceived stigma in Spasmodic Torticollis.

Little is known about the "stigmatizing" effects of Spasmodic Torticollis--a condition that produces physical disfigurement. This is important in understanding the social dimensions of this disorder. This study examined the presence, the dimensions, and the degree of perceived stigma in patients with Spasmodic Torticollis. The study was completed in two stages. In the first stage, ten patients were interviewed to identify the effects of their condition on their social interactions. In the second stage, a self-rating measure of stigma and questions about the impact of the condition on the patients' lives were devised. Perceived stigma was defined as avoidance of others, avoidance by others, self-consciousness, feeling unattractive, feeling apologetic, and feeling different from others. The questionnaires were sent to one hundred patients. The majority of the patients perceived "some" or "severe" stigma. Stigma was found to affect the patients' social, private, and working lives. It is suggested that stigma in Spasmodic Torticollis needs to be considered as a parameter relevant to the clinical management of these patients.

Aged↗

Torticollis and blepharospasm in systemic lupus erythematosus.

A case of torticollis and blepharospasm as the major manifestation of neurological involvement in systemic lupus erythematosis (SLE) is presented. A 56-year-old woman presented with blepharospasm in February 1985 and with torticollis in April 1985. She was diagnosed as having SLE in 1966, on the basis of positive LE cells, when she presented with fever and joint pains. There was no evidence that she had ever been on major tranquilizers. An attempt has been made to correlate the level of antibody titres and exacerbation of these movements with response to steroids. The possibility of an association between blepharospasm, torticollis, and autoimmune disorders has recently been considered.

Blepharospasm↗

Treatment of idiopathic spasmodic torticollis with botulinum toxin A: a double-blind study on twenty-three patients.

In a double-blind, placebo-controlled study, 23 patients suffering from intractable spasmodic torticollis (ST) were given successively either botulinum toxin A (BTA) or normal saline by intramuscular injections in the affected muscles. Evaluation was carried out by three blinded observers, using a clinical and video assessment of the severity of torticollis, employing a scoring system described by Tsui (1). Patients were also asked to subjectively comment on changes in the amount of pain and on changes in the activities of daily living (ADL). BTA was proven to be superior on all forms of assessment to placebo, and these results were statistically significant. Side effects mainly consisted of pain at the injection site. Tiredness occurred at equal frequency with BTA and placebo. No serious or systemic side effects were noted. Botulinum toxin is a safe, effective and relatively simple treatment for spasmodic torticollis.

Adolescent↗

Spasmodic torticollis: severe compression neuropathy in rami dorsales of cervical nerves C1-6.

In 28 patients with spasmodic torticollis dorsal branches of the cervical nerves C1-6, and in 25 of these patients fascicles of the contralateral accessory nerve were investigated by light and electron microscopy. Significant changes were noted in 15 patients. The alterations were not seen or were less prominent in the 5 control cases studied for comparison. Semiquantitative evaluation of light microscopic findings revealed in 12 cases prominent and numerous Renaut bodies; in 9 cases evidence of regeneration (in 3 of these postoperatively); and in 11 cases disproportionately thin myelin sheaths in relation to axon calibers. In conjunction with endoneurial edema and thickening of the perineurium, these changes were suggestive of compression neuropathy. Whether these changes were the cause, or a side effect of the abnormal muscle contractions in spasmodic torticollis could not be elucidated. Peripheral nerve compression, however, may trigger abnormal activity in the peripheral part of the involved interneuronal circuits and may, thus, be considered as one of the many possible causes of spasmodic torticollis.

Adult↗

Hypoplasia of the internal carotid artery associated with spasmodic torticollis: the possible role of altered vertebrobasilar haemodynamics.

We describe an unusual case of hypoplasia of the left internal carotid artery associated with an anomalous origin of the ipsilateral middle cerebral artery in a patient with spasmodic torticollis. Collateral circulation to the territory of the middle cerebral artery was through the vertebrobasilar circulation, and the left vertebral artery was dilated and compressing the ventrolateral aspect of the cervicomedullary junction, as evidenced by MRI. We believe this to be the first report of unilateral hypoplasia of the internal carotid artery associated with spasmodic torticollis. Compression of the left accesory nerve by the dilated vertebral artery as a result of altered haemodynamics was thought to cause spasmodic torticollis.

Accessory Nerve↗

Treatment of spasmodic torticollis with local injections of botulinum toxin. One-year follow-up in 37 patients.

Thirty-seven patients with spasmodic torticollis (cervical dystonia) who received repeated local injections of botulinum toxin have been followed up for a mean period of 12.3 (10-29) months, during which time 138 treatment sessions were performed. Mean doses per muscle averaged 320 mouse units (mu; range 160-1000 mu botulinum toxin A prepared by CAMR, Porton Down, UK). Eighty-six per cent of patients experienced significant improvement of posture and 84% of those with pain had relief following the first injection. Muscular patterns of recurrent torticollis were relatively constant and in most patients efficacy was maintained with subsequent injections, while 15% of all follow-up sessions failed. Only 2 of 37 patients were consistent nonresponders; 22% and 10% of all sessions were complicated by transient dysphagia and weakness of neck muscles, respectively. It is concluded that local botulinum toxin injections can be a safe and efficaceous long-term treatment of spasmodic torticollis and that optimal doses should be between 200 and 400 mu/muscle.

Botulinum Toxins↗

EMG biofeedback treatment of torticollis: a controlled outcome study.

Successful treatment of torticollis with electromyographic (EMG) biofeedback has been reported in a number of single case and single group studies. The present investigation represents the first controlled outcome study. Twelve torticollis patients were randomly assigned to EMG biofeedback or relaxation training and graded neck exercises (RGP). The procedure involved three sessions of baseline assessment, 15 sessions of EMG BF or RGP, 6 sessions of EMG BF or RGP plus home-management, 6 sessions of home-management alone, and follow-up 3 months after the end of treatment. A variety of outcome measures were used including physiological (EMG from the two sternocleidomastoid muscles, skin conductance level), behavioral (angle of head deviation, range of movement of the head), and self-report (depression, functional disability, body concept), therapist and "significant other" reports and independent observer assessment of videos. In both groups, neck muscle activity was reduced from pre- to posttreatment. This reduction was greater in the EMG biofeedback group. There was evidence of feedback-specific neck muscle relaxation in the EMG biofeedback group. Therefore, the outcome was not due to nonspecific factors and could be attributed to feedback-specific effects. Changes in skin conductance level showed that neck muscle relaxation was not simply mediated by a general reduction of "arousal." Significant improvements of extent of head deviation, and range of movement of the head, as well as reductions of depression were present, which were not different in the two groups. At the end of treatment, no patient was asymptomatic. Any therapeutic benefit was generally maintained at follow-up. The results and the procedural simplicity of RGP make the issue of cost-efficacy of EMG biofeedback a pertinent one. Further controlled outcome studies of EMG biofeedback treatment of torticollis with larger samples are required.

Adult↗

Bilateral microsurgical lysis of the spinal accessory nerve roots for treatment of spasmodic torticollis. Follow up of 33 cases.

In 1981 we reported about a new surgical procedure for the treatment of spasmodic torticollis (ST). 33 patients, who failed to respond to the available conservative treatment, underwent a bilateral microsurgical lysis (BML) of the spinal accessory nerve roots (SRAN). Anastomoses between SRAN and the dorsal roots of the first and second cervical nerve (DRC 1/DRC 2) were cut. DRC 1 and sometimes DRC 2 were divided bilaterally. Moreover, SRAN was freed of all adhesions and vascular contacts. Up to 60 months after surgery we have excellent results in 5(5), good results in 10(7) and improved symptoms in 12(8) patients. In 3(7) patients symptoms were unchanged, 2(1) patients deteriorated (patients self assessment is given in brackets). One patient died during hospitalisation. Comparing torticollis symptoms and the post-operative outcome it can be shown that patients with horizontal ST have the best results (21 out of 22). Bad results were obtained in patients with combined torticollis symptoms such as retrocollis, antecollis and the rotatory/horizontal type (5 out of 9). These results support the hypothesis of a peripheral factor in the aetiology of horizontal ST. It is assumed that a unilateral disturbance of proprioceptive afferents for head control, which reach the CNS via anastomosis between DRC 1/DRC 2 and SRAN (in 94% of the cases) could be involved. This hypothesis is discussed with special regard to different anatomical findings in patients with ST and those revealed in a study on human cadavers without this disease.

Accessory Nerve↗

Swallowing in torticollis before and after rhizotomy.

To determine risk factors for dysphagia after ventral rhizotomy, videofluoroscopic barium swallowing examinations were done on 41 spasmodic torticollis patients before and after surgery. Radiologic abnormalities were present in 68.3% of the patients before surgery, but these were only mildly abnormal in the majority. After surgery 95.1% showed radiologic abnormalities which were moderate or severe in one-third of the patients. Swallowing abnormalities correlated significantly with duration of torticollis and subjective complaints of swallowing difficulty both before and after surgery, but not with age, sex, or type of torticollis. The major acute postoperative finding was aggravation of preexisting pharyngeal dysfunction. Follow-up from about half of our original sample showed that gradual improvement occurred from 4 to 24 weeks after surgery by subjective report. We review the innervation of intrinsic and extrinsic pharyngeal musculature, and suggest that C1-3 rhizotomies and selective sectioning of the spinal accessory nerve are responsible for aggravation of pharyngeal swallowing dysfunction in the acute postsurgical period.

Adult↗

A difficult airway is not more prevalent in patients suffering from spasmodic torticollis: a case series.

PURPOSE: We designed this retrospective study to assess the frequency of difficult airway and difficult intubation in patients with spasmodic torticollis and compare it to that of the general population. METHODS: After Institutional Review Board approval, data were collected from the charts of all the patients with spasmodic torticollis who underwent selective peripheral denervation at our institution between 1988 and 2001. The intubation grade was determined using the Cormack and Lehane laryngoscopic classification. The best laryngeal view was recorded. RESULTS: Data from 342 patients were available for analysis. Fourteen patients had a difficult airway. In two patients, intubation was difficult with three attempts at laryngoscopy in one patient and use of fibreoptic bronchoscopy in the other. Twelve (3.5%) patients presented with laryngoscopic grades of III or IV. The combined prevalence of laryngoscopic view grade III and IV and difficult intubation was 4.4%. CONCLUSIONS: This study assesses the frequency of difficult intubation in patients suffering from spasmodic torticollis. When compared to the general population, these patients do not appear to have a higher frequency of difficult airway or difficult intubation.

Adult↗

Rotary atlanto-axial subluxation with torticollis following central-venous catheter insertion.

Atlanto-axial subluxation with torticollis is an uncommon condition that occurs in children usually as a result of pharyngeal infection, minor trauma, or neck surgery. Passive motion of the head and neck during general anesthesia is probably another etiologic factor. Torticollis is the most common presenting physical finding. Pain may or may not be present, but is commonly present with passive neck motion. Neurologic sequelae are uncommon. Our case illustrates this condition as a complication of central venous catheter (CVC) insertion in a child under general anesthesia. The surgeon should suspect this pathology when a child presents with torticollis following CVC placement. Precautions should be taken in the operating room to avoid aggressive rotation and extension of the child's neck while under general anesthesia whether or not cervical inflammation is present. Special attention to head and neck positioning should be taken in patients with Down's syndrome since they are at increased risk for atlanto-axial subluxation. The prognosis is excellent when diagnosed early. A delay in diagnosis can result in the need for surgical intervention.

Anesthesia, General↗

Surgical treatment of muscular torticollis for patients above 6 years of age.

Eighteen patients with congenital muscular torticollis, aged 6-22 (average 11) years, underwent surgical releases of the contractive bands. There were 8 boys and 10 girls. Preoperative open mouth radiograph of the odontoid process in 16 patients showed asymmetry of articular facets of the axis and tilt of the odontoid process to the side of the torticollis. Sixteen patients received bipolar releases, the others received distal releases. After the operation, a neck collar was applied and an exercise program was started. At an average follow-up of 5 years and 7 months, there were excellent or good results in 10 patients. All of the patients except 2 showed improvement of the facial asymmetry and all except 1 had a satisfactory range of motion of the neck. One patient underwent a second operation due to recurrence. The follow-up radiographs showed improvement of the tilt of the odontoid process, but the asymmetry of the articular facets of the axis persisted. We suggest that bipolar release is the treatment of choice for congenital muscular torticollis in patients of more than 6 years of age. To determine the influence of a bipolar release on the functions of the cervical spine, longer follow-up intervals are needed.

Adolescent↗

A study of multimodality evoked responses in idiopathic spasmodic torticollis.

A wide variety of anatomical sites have been implicated in the pathogenesis of torticollis, based primarily on animal studies. These include lesions in the mesencephalic tegmentum, third ventricle, diencephalon, pre-tectal area, prestitial nucleus of the mid-brain, cervical cord, atlanto-axial junction and motor cortex, amongst others. This study describes the use of multimodality evoked potentials including visual, auditory, bilateral median and peroneal somatosensory evoked responses, undertaken in patients with idiopathic spasmodic torticollis. An attempt was made to non-invasively identify any physiological disruption along these major pathways which could help locate a causative lesion. Normal evoked responses were obtained both ipsilateral and contralateral to the affected side in all patients. It is concluded that the lesion in idiopathic spasmodic torticollis does not discernibly involve the visual, auditory or motor somatosensory pathways.

Adult↗