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What is straight ahead to a patient with torticollis?

Vestibular and neck proprioceptive signals are known to be used in judging the locations of objects in space and relative to the body. Given that these signals are asymmetric in patients with spasmodic torticollis, one would expect such patients to have abnormal spatial perception. We tested this idea by measuring patients' perception of visual straight ahead (VSA) under various conditions: with the body in its primary position, i.e. with the head and trunk as closely aligned as possible, and after well defined passive rotations of the head and/or trunk. In the primary body position, patients' VSA direction showed considerable variations which were similar, however, to those of normal subjects; it was independent of torticollis direction, of the head torque it produced, and of the weak spontaneous nystagmus recorded in seven of the 10 patients. After whole-body rotations, i.e. where head and trunk underwent the same motion, the VSA was shifted in both patients and normal subjects, and in both groups the shift was symmetrical after rotations to the right or left. After motions where the trunk rotated under the stationary head (neck proprioceptive stimulation) or the head on the stationary trunk (combined vestibular and neck stimulus), the VSAs of normal subjects coincided rather well with their head midsagittal planes, whereas the VSAs of patients were shifted considerably towards the trunk, again in a symmetrical way. We suggest two mechanisms to explain the findings in patients: (i) a central compensation which restores symmetry of the afferent inflow in the patients (unlike the motor efference); (ii) shifting of the reference for the VSA from the head towards the trunk, because the trunk is a more reliable egocentric reference than the head in the patients. Our findings do not support the assumption that asymmetries in afferent inflow are responsible for the asymmetry of motor output in spasmodic torticollis.

Adult↗

Abnormal interaction between vestibular and voluntary head control in patients with spasmodic torticollis.

The functional status of vestibulo-collic reflexes in the sternocleidomastoid (SCM) muscles was investigated in 24 patients with spasmodic torticollis using small, abrupt 'drops' of the head. None had been treated with botulinum toxin injections during at least 4 months preceding the study. Eight of the patients, four of whom had been studied before surgery, were also studied after selective peripheral denervation of neck muscles. The reflex was of normal latency and duration in the 'passive drop' condition, in which subjects were instructed not to oppose the fall of the head. To study voluntary interaction with the reflex response, subjects were then asked to flex the neck as quickly as possible after onset of the head drop ('active drop'). In this condition, voluntary responses in patients were delayed, smaller and less effective in counteracting the head fall than in normal subjects. The same abnormalities were also found in patients after surgery when the head posture was improved. Somatosensory/auditory voluntary reaction times in SCM were normal, as was the latency of the startle reflex. We conclude that voluntary interaction with the vestibulo-collic reflex is disrupted in patients with spasmodic torticollis, a finding which corroborates the patients' aggravation of their symptoms by head or body perturbations. Lack of effective interaction between two major systems controlling head position may contribute to torticollis.

Adult↗

Adaptive changes of saccadic eye-head coordination resulting from altered head posture in torticollis spasmodicus.

We asked whether and how the abnormal head posture in torticollis patients affects saccadic gaze shifts and impairs the associated head movements. We wanted to learn to what extent observed changes directly result from the disease or reflect compensatory mechanisms, secondary to the altered head posture. We compared the results of patients with those of normal subjects. When patients viewed a centric target, their heads were a priori deviated in the direction of the torticollis, with orbital eye position showing a compensatory offset in the opposite direction. These abnormal eye and head positions were re-established when patients returned from an eccentric gaze position by means of a centripetal gaze shift, independently of its direction and magnitude, unlike in normal subjects who always recentred eyes and head. In normal subjects the share of the head in the total gaze shift amounted to about 70%, whereas in patients it contributed only 30%, necessitating correspondingly larger orbital eye displacements and eccentricities. Moreover, patients' head movements were asymmetric; they were larger when gaze was shifted into, or returned from the hemifield contralateral to the torticollis direction compared with gaze shifts in the ipsilateral hemifield. The eyes displayed a reversed asymmetry. Patients showed a significant increase in gaze latency and head versus eye delay as well as in the number of corrective saccades. However, head velocity was normal in four out of seven patients. Moreover, all patients made normal eye saccades (peak velocity, duration, gaze error), except for the increase in latency, which also occurred when gaze was shifted without head movements. Thus, patients' saccadic eye-head coordination showed abnormalities which mainly concerned the involved head movements. We suggest that the observed changes do not reflect a direct involvement of the disease upon the gaze shift mechanism, but can be interpreted as adaptive changes that compensate for the altered head posture. We formalized this view in the form of a dynamic model.

Adaptation, Physiological↗

The determinants of treatment duration for congenital muscular torticollis.

BACKGROUND AND PURPOSE: Although the success of conservative management of congenital muscular torticollis has been well documented, relatively little is known about the determinants of response to treatment, such as treatment duration. The purpose of this study was to determine how factors such as severity of restriction of range of motion, age at initiation of treatment, and presence of a palpable intramuscular fibrotic sternocleidomastoid muscle mass affect treatment duration. SUBJECTS: One hundred one children (mean age = 4 months, SD = 2.87, range = 0.5-15.5) who were diagnosed with congenital muscular torticollis and referred to physical therapy at British Columbia's Children's Hospital (Vancouver, British Columbia, Canada) prior to 2 years of age were included in the study. METHODS: Following a standardized initial assessment, parents were taught the home treatment program, which included passive stretches of the affected sternocleidomastoid muscle and strengthening exercises for the contralateral side, and positioning and handling skills. Evaluation at 2-week intervals included measurement of passive neck rotation and lateral flexion using an adapted standard goniometer. Treatment duration was defined as the time between initiation of treatment and achievement of full passive neck range of motion. RESULTS: Complete recovery (full passive range of motion) was achieved in all but one of the children in this sample. The mean treatment duration was 4.7 months (SD = 5.06, range = 1-36). Correlations were noted between severity of restriction and treatment duration (r = .31) as well as between presence of a mass and treatment duration (r = .26). Multiple regression analysis revealed that severity of restriction was the strongest predictor of treatment duration. CONCLUSION AND DISCUSSION: The results of this study will make it possible for therapists to better predict treatment duration at the time of the initial assessment. By providing parents with more precise information about the length of treatment, parents may be more willing to adhere to the exercise program. [Emery C. The determinants of treatment duration for congenital muscular torticollis.

Age Factors↗

Snapping during manual stretching in congenital muscular torticollis.

Manual stretching frequently is used in the treatment of congenital muscular torticollis in infants. During manipulation, it is not uncommon for the sternocleidomastoid muscle to snap or suddenly give way. The main objective of this study was to evaluate the predisposing causes and clinical significance of such snapping. Four hundred fifty-five patients younger than 1 year of age with congenital muscular torticollis treated with a standardized gentle manual stretching program during a 13-year period were studied. Using prospective standardized assessment parameters, the pretreatment, treatment, and followup results of a group of 41 patients with snapping detected during treatment were compared with the results of a group of 404 patients without snapping during treatment. The group with snapping was associated with a more severe sternomastoid tumor, higher incidence of hip dysplasia, earlier clinical presentation, and shorter duration of treatment. With a mean followup of 3.5 years, the group with snapping was not different from the group that had no snapping in the final assessment score and percentage requiring surgery. From this study, unintentional snapping during the gentle manipulation treatment of congenital muscular torticollis has clinical and ultrasonographic evidence of partial or complete rupture of the sternocleidomastoid muscle. No long-term deleterious effect on the outcome was observed after the snapping.

Female↗

Torticollis secondary to posterior fossa tumors.

Torticollis in childhood may be a sign of many disorders. Five cases, with torticollis as the initial sign of a posterior fossa tumor, are presented. The diagnosis and treatment of the tumor was considerably delayed in all patients because posterior fossa tumor was not considered in the initial differential diagnosis. In two patients, operative procedures on the sternocleidomastoid muscle were performed before discovering the underlying causative tumors. Four of the five patients also had other associated symptoms such as headache, nausea, and vomiting. It is stressed that in acquired torticollis, posterior fossa tumor be considered in the differential diagnosis.

Astrocytoma↗

Surgical correction of muscular torticollis in older children with Peter G. Jones technique.

In the Department of Pediatric Surgery, Uludağ University Medical Faculty in Bursa, during the last 11 years, the Peter G. Jones technique for the surgical correction of muscular torticollis in older children has been introduced. Twenty children between 4 and 13 years of age were treated for muscular torticollis. They were followed up from 3 months to 10 years after surgery. All patients had a middle-third open transection of the sternocleidomastoid muscle. Preoperative and postoperative assessment by a rigid scoring system showed that all patients improved in terms of function as well as cosmesis. Children younger than 10 years showed the most improvement, with 90% excellent and good results. Late middle-third open transection of the sternomastoid in muscular torticollis may give acceptable results.

Adolescent↗

Eosinophilic granuloma of the atlas presenting as torticollis in a child.

STUDY DESIGN: This report describes a case of successful surgical treatment of eosinophilic granuloma of the atlas in a 3.5-year-old boy who presented with torticollis. OBJECTIVE: The purpose of this report was to illustrate the rare clinical presentation of eosinophilic granuloma in the atlas. SUMMARY OF BACKGROUND DATA: Eosinophilic granuloma, a benign solitary lesion that commonly affects children, has a variable clinical course. Although eosinophilic granuloma has been reported to occur in the cervical spine, there have been only five reported cases of eosinophilic granuloma affecting the atlas. The management of eosinophilic granuloma ranges from observation and immobilization of the cervical spine to surgical excision. METHODS: The tumor was located on the left lateral mass of the atlas. Biopsy and curettage were performed through an oblique incision through the posterior border of the sternocleidomastoid muscle. A histopathologic evaluation confirmed the diagnosis of eosinophilic granuloma. RESULTS: The patient tolerated the procedure with no complications, and his torticollis has completely resolved. CONCLUSION: Eosinophilic granuloma of the atlas initially presented in this patient as torticollis. Biopsy for confirmation of diagnosis and curettage for treatment provided a successful outcome in this patient. We suggest biopsy of the lesion and histopathologic evaluation to confirm the diagnosis in atypical cases.

Cefazolin↗

Botulinum toxin type a in the treatment of children with congenital muscular torticollis.

This is a retrospective case series describing the use of botulinum toxin type A in the treatment of children with congenital muscular torticollis who fail to progress with conservative management. A total of 27 children with congenital muscular torticollis, 6-18 mos of age, received 30 botulinum toxin type A injections into their sternocleidomastoid or upper trapezius muscle, or both, at a pediatric tertiary care center between 1995 and 2001. Three children received repeat injections. Twenty of 27 children (74%) had improved cervical rotation or head tilt after the injections, and 2 of 27 (7%) experienced transient adverse events, specifically, mild dysphagia and neck weakness. This series suggests that botulinum toxin type A may be a safe and effective treatment option for children with congenital muscular torticollis who are unresponsive to a traditional regimen of physical therapy and a home program. A prospective, randomized controlled trial is necessary to definitively assess the role of botulinum toxin type A in this population.

Botulinum Toxins, Type A↗

Craniofacial deformity in patients with uncorrected congenital muscular torticollis: an assessment from three-dimensional computed tomography imaging.

Congenital muscular torticollis is caused by idiopathic fibrosis of the sternocleidomastoid muscle that restricts movement and pulls the head toward the involved side. Deformation of the craniofacial skeleton will develop if the restriction is not released and result in aesthetic and functional problems. The purpose of this study was to use three-dimensional computed tomography imaging for qualitative and quantitative evaluation of the craniofacial deformity in a series of patients with uncorrected congenital muscular torticollis, and to assess age as a precipitating factor for severity of the deformity. A total of 14 patients from 1 month to 24 years of age were included. The skull images were rotated into standard orientation and reconfigured for evaluation of the cranium, endocranial base, and facial skeletal structures. The midlines of cranial base and facial bone, angle of midline deviation, width of each hemicranium and hemiface, and the orbital index were defined and measured. The results showed that the cranium and cranial base deformation took place as early as in infant stage, with the most prominent change occurring in the posterior cranial fossa. Facial bone asymmetry started to appear after 5 years of age, at which time the mandibular and occlusal abnormalities were observed. The deformity of the orbits and maxilla occurred at an older age, characterized by the deviation and decreased vertical height on the affected side. The severity of the observed deformities increased with age. The angle of midline deviation was 2.48 +/- 1.68 degrees in the cranial base and 3.26 +/- 3.28 degrees on the facial bone. Both of the midline deviations were significantly correlated with age. Compared with the contralateral side, the width of the ipsilateral posterior hemicranium was longer (54.36 +/- 6.72 mm versus 50.81 +/- 6.55 mm), and the width of the ipsilateral lower hemiface was shorter (35.30 +/- 7.27 mm versus 43.49 +/- 11.34 mm). Both differences were statistically significant. Measurement of the orbital index demonstrated a significantly flatter orbit on the ipsilateral side (89.48 +/- 0.11 versus 92.74 +/- 0.08). This study showed that the cranium and cranial base deformity occurred early in patients with uncorrected torticollis, while the facial bone deformity occurred in childhood stage. The cranial and facial deformity became more severe with age. Early release of the muscle restriction is advised to prevent craniofacial deformation.

Adolescent↗

Congenital muscular torticollis: magnetic resonance imaging and ultrasound diagnosis.

The magnetic resonance imaging (MRI) and sonographic appearances of congenital muscular torticollis were studied in a 6-week-old female infant who presented with a firm mass in the right sternocleidomastoid muscle and clinical signs of torticollis. MRI and ultrasound were performed to exclude neoplasm. MRI showed a diffusely enlarged, right sternocleidomastoid muscle that was isointense to normal muscle on T1-weighted conventional spin-echo images. The muscle became progressively brighter and more heterogeneous in signal intensity on proton density- and T2-weighted conventional spin-echo sequences. A thin rim of bright signal intensity on the T2-weighted images surrounded most of the muscle. Ultrasound demonstrated fusi-form enlargement with patchy inhomogeneous areas of increased echogenicity. At 14 weeks there were signs of clinical improvement, with softening of the mass and decreased torticollis.

Female↗

Vestibulo-ocular abnormalities in spasmodic torticollis before and after botulinum toxin injections.

In order to establish whether vestibular abnormalities often found in spasmodic torticollis are secondary to the abnormal head posture, the vestibulo-ocular reflex (VOR) was studied in eight patients before and after correction of head posture with botulinum toxin. Eye movements were recorded in the dark during sinusoidal and velocity step rotation. Four patients showed a significantly asymmetric response, with the slow phase of the VOR more active ipsilateral to the torticollis (chin). Despite significant improvement of the head posture in all patients for up to 10 weeks following treatment, no correction of the vestibular asymmetry occurred. This suggests that the VOR abnormalities are not caused by the head posture itself. We interpret the findings as evidence of primary involvement of the vestibular system in torticollis and we postulate a widespread derangement of the sensory-motor mechanisms controlling head posture in this disease.

Adult↗

Psychological functioning before and after treatment of torticollis with botulinum toxin.

The impact of botulinum toxin injection on psychological function was assessed in 26 patients with idiopathic torticollis. Eighty five per cent of the patients and 88% of the relatives considered torticollis to be better following the injections. Symptomatic improvement with the injections was associated with significant reduction of depression and disability, but non-significant improvement in body concept, and self-esteem. This concordant pattern of change in symptoms and psychological function with the injections supports the proposal that in torticollis depression and disability are consequences of the postural abnormality of the head.

Adult↗

Analysis of static and kinetic abnormalities typical of torticollis, and postoperative changes.

The clinical pattern of torticollis and surgical results were evaluated. Head posture and range of motion were measured. The authors use a newly designed device consisting of an orthogonal system to which head position is referred. Preliminary data were obtained on 24 patients with torticollis and 21 healthy control subjects. The examination of posture shows that the head usually twists in opposite directions simultaneously around a vertical and a sagittal axis, and the deflection is greater in one direction. Head position affect body posture, with the trunk often compensating for head deviation. Although there are almost always abnormalities on EMG recordings of neck muscles, these do not indicate the degree and pattern of deformity. Because of the disorder of muscle innervation, head movements are affected, with an asymmetrical decrease in the range of motion in comparison with normals (p < 0.05). Movements are greater toward the direction of postural deviation. Eleven patients were studied before and after undergoing a bilateral C1-C3 rhizotomy and selective section of the XIth rootlets, which carry motor fibers to the sternocleidomastoid muscle. Head posture immediately improved (p < 0.05), with better appearance, despite some residual distortion (less than 10%), and trunk alignment also improved . In contrast to posture, head range of motion was worse than before (p < 0.05). The most improved movement was rotation, followed by flexion/extension. Further improvements were observed at later follow-up. Surprisingly, the range of motion gradually increased, surpassing preoperative limits (p < 0.05). Our study documents the usefulness of surgery in correcting torticollis. Posture is immediately affected; motion increases despite denervation, after an initial decline.

Female↗

Long-term outcome of iontophoresis treatment for torticollis.

In 1969, a patient with torticollis was successfully treated by a procedure called iontophoresis. Between 1958 and 1972 223 patients with torticollis were treated with this procedure. 88 of these patients (39%) were located and 56 (25% of the total, 64% of those located) returned a survey regarding their condition. 28 (13% of the total, 32% of those located, 50% of those responding) enjoyed an initial period of improvement. Of these, 16 patients (7% of the total, 18% of those located, 29% of those responding, 51% of those with initial improvement) reported sustained improvement through the time of the survey. However, 5 of these patients had additional treatment with botulinum toxin or selective denervation. We conclude that the long term effectiveness of iontophoresis treatment in the course of torticollis is minimal.

Adult↗

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.

Adult↗

Electromyographic investigations in torticollis.

In 13 patients with torticollis, the electromyographic (EMG) activity in the neck muscles at rest was investigated before, during and after stimulation of the H1 bundle (in horizontal torticollis) and inner part of oral ventral nucleus (in the rotatory form), as well as after coagulation. Three EMG types of torticollis-spasmodic, myoclonic and mixed - could be differentiated. During the 8-, 25- and 50-Hz stimulations, a diminution in the amplitude and frequency of muscle potentials, followed by electric silence, was most often obtained; in general, the electric silence was preceded by a rhythmic activity of grouped potentials. Following coagulation, no spontaneous EMG activity was any longer recorded in the neck muscles, in the majority of the cases.

Diagnosis, Differential↗

Torticollis in children.

Torticollis is a common clinical sign that is found in a variety of disorders. Childhood torticollis differs from the adult form in that congenital types are common and many frequently encountered disorders found in adults are unusual. Pediatric torticollis related to otolaryngologic conditions is reviewed, and three illustrative cases are presented.

Child↗