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Background: Pain is a common non-motor symptom in patients with cervical dystonia (CD), severely impacting their quality of life. The pathophysiology of CD is incompletely understood but it involves altered processing of proprioceptive and pain signals., Objectives: The purpose of this proof-of-concept study was to determine if vibro-tactile stimulation (VTS)-a non-invasive form of neuromodulation targeting the somatosensory system-can modulate neck pain in people with CD., Methods: In a multi-center study, 44 CD patients received VTS to sternocleidomastoid and/or trapezius muscles for up to 45 min under 9 different stimulation conditions that either targeted a single or a pair of muscles. The primary outcome measure was a perceived pain score (PPS) rated by participants on a 100-point analogue scale., Results: During VTS, 29/44 (66%) of participants experienced a reduction in PPS of at least 10% with 17/44 (39%) reporting a reduction in pain of 50% or higher. After VTS cessation, 57% of participants still reported a 10% or higher reduction in PPS. Effects were significant at the group level and persisted for up to 20 min post-treatment. No distinct optimal stimulation profiles were identified for specific CD phenotypes. Clinical markers of disease severity or duration did not predict the degree of VTS-induced pain reduction., Conclusion: This proof-of-concept study demonstrates the potential of VTS as a new non-invasive therapeutic option for treating neck pain associated with CD. Further research needs to delineate optimal dosage and long-term effects., (© 2024. The Author(s).)

The study examined how neck muscle imbalance and plagiocephaly affect the lying and rolling activities in 118 infants aged 4 to 6 months with postural torticollis. Outcome measures included age, sex, cervical movement, and plagiocephaly severity. Neck muscle function was assessed with the Muscle Function Scale (MFS), and infant motor abilities in lying and rolling were evaluated through the corresponding dimensions of the Gross Motor Function Measure (GMFM). Multiple regression analysis showed that a better MFS score of the affected neck was significantly associated with improved lying and rolling activities in the GMFM ( p  < .01), and importantly, the interaction between the plagiocephaly and the MFS scores of the affected neck muscle in these activities was found to be significant ( p  < .05). These results highlight the need for early intervention in infants with torticollis to address muscle imbalance and plagiocephaly, crucial for early motor development (KCT0008367).

Introduction: Cervical dystonia is defined by excessive contraction of muscles that produce abnormal postures and movements of the head, neck, and sometimes the shoulders. Many affected individuals also have pain, local muscle hypertrophy, and/or abnormally increased EMG activity. However, abnormal movements are considered the defining feature., Cases: Three cases are described suggesting that some features of cervical dystonia may occur without abnormal movements. In these cases, the only clinical features are pain, local muscle hypertrophy, or abnormal EMG activity. These features may occur years before abnormal movements emerge, or they may occur coincidentally with dystonia affecting regions other than the neck. In some cases, some features associated with cervical dystonia may occur without any obvious abnormal movements., Conclusions: Some symptoms of cervical dystonia may occur without abnormal movements of the head or neck. The purpose of this report is not to question current diagnostic criteria for cervical dystonia, but to call attention to a phenomenon that deserves further attention. Such cases may be considered to have a pro-dromal form of cervical dystonia or a formes fruste of cervical dystonia. Whatever diagnostic label is applied, the phenomenon is important to recognize, because symptoms may be readily alleviated with botulinum toxin., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: Tremor is an important phenotypic feature of dystonia. Using the new concept (Col-Cap) of classification we examined the frequency of tremor in cervical dystonia (CD) patients, their main subtypes and muscles injected., Methods: In this large study conducted at multiple movement disorder centres in Europe and India, between January and June 2019, we examined 293 patients with idiopathic CD who were all treated with botulinum toxin (BTX)., Results: The dystonic head tremor (DHT+) was present in 57.6 % of CD patients and they had a significantly longer duration of symptoms than patients without head tremor (DHT-). In DHT+ patients torticaput was the most common subtype and the majority (63.3%) had one or two subtypes only. There was no significant difference between the number of unilateral injections for any of the muscles in the DHT+ and DHT- groups, while the number of patients receiving bilateral injections in splenius capitis (78 vs 25; p = 0.00001), sternocleidomastoid (31 vs 6; p = 0.0005), trapezius (28 vs 9; p = 0.01), and obliquus capitis inferior (15 vs 2; p = 0.008) were significantly more in the DHT+ group. The mean doses of all three types of BTX/A were not significantly different between the two groups., Conclusions: The frequency of head tremor was 57.6% in our CD patients and torticaput was the most common dystonic subtype associated with tremor. Simple forms of CD seemed more likely associated with head tremor, than complex forms of CD. Most of the DHT+ patients received bilateral injections. The use of 'Col-Cap' classification was helpful in the identification of muscles likely to be involved in tremor in CD patients., Competing Interests: Dr. Sanjay Pandey and Dr. Christian Altmann have no conflict of interest. All other authors were speakers and consultants for Allergan, Merz/Desitin and Ipsen, but none relevant to this study (and had no financial or other support)., (Copyright: © 2020 The Author(s).)

Unilateral agenesis of the sternocleidomastoid (SCM) muscle is a rare phenomenon known to cause torticollis. There have been around 12 reported instances of SCM agenesis in the literature; in almost every case, torticollis was easily resolved nonsurgically with stretching and physical therapy. We report the case of a 6-year-old boy with severe torticollis due to unilateral SCM absence who underwent the surgical release of the contralateral SCM. To our knowledge, this is the first time a surgical release of the SCM was performed to correct torticollis associated with agenesis of the SCM.

The aim of this study was to systematically review the measurement properties of instruments which assess cervical spine function in infants with torticollis. Electronic searches were performed in MEDLINE, CINAHL, Embase, Web of Science and the Cochrane Library, combining three constructs ('torticollis', 'cervical spine assessment' and 'measurement properties'). Two reviewers independently rated the methodological quality and the quality of measurement properties of identified articles, using both the COSMIN (COnsensus-based Standards for the selection of health status Measurement INstruments) checklist and quality criteria for measurement properties. Five studies, using six instruments, met the inclusion criteria and were analysed. Included instruments were the goniometer, electronic pendular goniometer, protractor, still photography, Muscle Function Scale and a range of motion limitation scale. All studies assessed reliability, and one study also assessed content validity and hypothesis testing. The methodological quality of the studies varied from poor to excellent according to the COSMIN checklist. Two instruments were found to have good measurement properties from high-quality studies: still photography for the assessment of habitual head tilt in supine and the Muscle Function Scale for the assessment of side-flexor muscle function in lateral head righting.Conclusion: This systematic review identified two reliable tools for the assessment of cervical spine function in infants with torticollis. Further research is required to assess the measurement properties of tools already described in the literature and to develop further tools for use in this population. What is known? • A thorough assessment of the infant presenting with torticollis is essential, in order to correctly diagnose, rule out 'red flags' and manage appropriately • Assessment tools need to have robust measurement properties in order to be of value for clinical practice and research What is new? • This systematic review identified two valid and reliable tools for the assessment of cervical spine function in infants with torticollis • Further research is required to assess the measurement properties of tools already described in the literature and to develop further tools for use in infants with torticollis.

Aim: To compare clinical findings for patients with congenital muscular torticollis (CMT) between those with and without a sternocleidomastoid (SCM) lesion., Methods: Medical records of 182 patients with CMT were retrospectively reviewed and the patients were divided into SCM lesion and nonlesion groups by ultrasonographic results. Intrauterine position, age, duration of therapy, rotation/tilting side, and the passive range of motion and angle of the neck were compared., Results: There were 74 SCM lesion and 108 nonlesion cases. The mean age at the first visit was 55.3 days in the SCM lesion group and 146.6 days in the nonlesion group. The mean therapy time in the nonlesion group was 66.5 days, significantly shorter than for the SCM lesion group (117.5 d). Tilting and rotation of the head in the same direction was observed only in the nonlesion group (n=9, 8.3%). Rotational limitation of the affected muscle side was 22.6 degree in the SCM lesion and 3.6 degree in the nonlesion group, and the tilting limitation was 19.2 degree in the SCM lesion and 10.4 degree in the nonlesion group., Conclusions: The nonlesion group had a better prognosis with shorter treatment duration. This group was more limited in head tilting than in head rotation, and the pattern of head rotation/tilting in the same direction was observed only in this group. These findings suggest that pathophysiological mechanisms and clinical characteristics may differ between CMT patients with and without SCM lesions., Level of Evidence: Level II-prognostic studies, retrospective study.

Background: An abnormal sternocleidomastoid muscle in congenital muscular torticollis can be classified into one of the four types via sonography. However, this categorization lacks quantitative measurements. The purpose of the study was to determine quantitative measurements of the sonograms via image analysis., Methods: Infants younger than 12 months of age suspected of having congenital muscular torticollis were included. Intraclass correlation coefficient estimates for interobserver reliability and a simple regression analysis for criterion validity were calculated. Spearman correlation analysis was then performed. The analyzed parameters included cervical passive range of motion for lateral flexion and rotation, area, brightness, max/min Feret's diameters, and muscular width/thickness., Results: Of the 29 (4.0 ± 2.6 months) screened infants, 13 (1.9 ± 1.7 months) were included. Nine were male, and 4 were female. Seven infants with mass were ultrasonographically classified into type I, and the other six infants were classified into type II. The affected/unaffected side ratios of cervical passive range of motion for lateral flexion and rotation were 0.92 ± 0.13 and 0.88 ± 0.16, respectively. The parameters measured on the sonograms were reliable, and the max/min Feret's diameters were valid measurements. The affected/unaffected side ratio of cervical passive range of motion for rotation significantly correlated with the affected/unaffected side ratios of the sternocleidomastoid muscle sonogram on area (r = -0.62, p = 0.03) and min Feret's diameter (r = -0.69, p = 0.01)., Conclusions: The area and min Feret's diameter were efficacious parameters for image analysis on sternocleidomastoid sonograms, and the min Feret's diameter would be more suitable than thickness for measuring the thickening SCM in transverse view. A healthy control group, more data and follow-up would be needed to confirm the changes on the SCM sonograms for clinical decision., (Copyright © 2018 Chang Gung University. Published by Elsevier B.V. All rights reserved.)

Purpose: To systematically review the recent evidence on physical therapy (PT) diagnosis, prognosis, and intervention of congenital muscular torticollis to inform the update to the PT management of congenital muscular torticollis evidence-based clinical practice guideline., Methods: From 2012 to 2017, 7 databases were searched for studies that informed PT diagnosis, prognosis, or intervention of infants and children with congenital muscular torticollis. Studies were appraised for risk of bias and quality., Results: Twenty studies were included. No studies informed PT diagnosis. Fourteen studies informed prognosis, including factors associated with presence of a sternocleidomastoid lesion, extent of symptom resolution, treatment duration, adherence to intervention, cervical spine outcomes, and motor outcome. Six studies informed intervention including stretching frequency, microcurrent, kinesiology tape, group therapy, and postoperative PT., Conclusions: New evidence supports that low birth weight, breech presentation, and motor asymmetry are prognostic factors associated with longer treatment duration. Higher-level evidence is emerging for microcurrent intervention.

Purpose: To establish inter- and intrarater reliability for determining severity grades of the congenital muscular torticollis severity classification system (CMT-SCS)., Methods: A prospective reliability study with 145 physical therapists recorded severity ratings on 24 randomly-ordered patient cases including age of infant, cervical range of motion, and presence or absence of sternocleidomastoid mass. To compute intrarater reliability, cases were randomly reordered and graded by 82 of the original raters., Results: For the CMT-SCS, overall reliability was good with an interrater reliability intraclass correlation coefficient (ICC) (2,1) of 0.83 (95% confidence interval [CI], 0.74-0.91) and an intrarater reliability ICC (3,1) of 0.81 (95% CI, 0.66-0.91)., Conclusions: The CMT-SCS has good reliability for infants up to 12 months of age. Physical therapists can use the scale for initial assessment of infants suspected to have CMT. The CMT-SCS should be standard documentation for infants with CMT.

Congenital muscular torticollis (CMT) is characterized by shortening or excessive contraction of the sternocleidomastoid muscle (SCM). The main purpose of this study was to evaluate the feasibility of quantifying SCM stiffness using acoustic radiation force impulse (ARFI) sonoelastography in infants with CMT. Twenty infants with an SCM thickness greater than 10 mm with or without involvement of the entire SCM length (limitation of neck rotation passive range of motion [PROM]: group 1S >30°, group 1M = 15°-30°) and 12 infants with an SCM thickness smaller than 10 mm with or without involvement of any part of SCM (group 2) were included. The SCM thickness was measured using real time B-mode ultrasound, and the local SCM shear wave velocity (SWV) and subcutaneous fat layer using ARFI sonoelastography. The neck rotation PROM was significantly greater in group 1S (36.5° ± 5.3°) than in group 1M (18.8° ± 4.9°; P < .01); the SWV of the SCM in the affected side (2.96 ± 0.99 m/s) was significantly higher than that in the unaffected side (1.50 ± 0.30 m/s; P < .01) in group 1. The SWV of the SCM was significantly higher in group 1S than in group 1M. There was significant correlation between the degree of PROM deficit of neck rotation and the SWV of the affected SCM (r = .75; P < .01) in all infants. This study revealed a difference in the SWV of the affected SCM in relationship to the limitation of neck rotation PROM in infants with CMT, if there was no difference in SCM thickness among infants.

Introduction: Head tremor is a common feature in cervical dystonia (CD) and often less responsive to botulinum neurotoxin (BoNT) treatment than dystonic posturing. Ultrasound allows accurate targeting of deeper neck muscles., Methods: In 35 CD patients with dystonic head tremor the depth and thickness of the splenius capitis (SPL), semispinalis capitis and obliquus capitis inferior muscles (OCI) were assessed using ultrasound. Ultrasound guided EMG recordings were performed from the SPL and OCI., Results: Burst-like tremor activity was present in both OCI in 25 and in one in 10 patients. In 18 patients, tremor activity was present in one SPL and in 2 in both SPL. Depth and thickness of OCI, SPL and semispinalis capitis muscles were very variable., Conclusion: Muscular activity underlying tremulous CD is most commonly present in OCI. Due to the variability of muscle thickness, we suggest ultrasound guided BoNT injections into OCI., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Unilateral congenital sternocleidomastoid muscle (SCM) contracture causing torticollis is well known. Although the unilateral muscular torticollis is quite often recognized, a bilateral contracture of SCM muscle is very rare. A review of the literature showed only three cases of bilateral congenital torticollis reported over the last two decades. We present a case report of a boy with congenital bilateral torticollis with 25 years of follow-up. Bilateral tenotomies of the right SCM were performed and the child was immobilized in Schanz's cervical orthosis. Three months after operative procedure, the physical examination indicated an increasing contracture of the left SCM with time. A similar operative procedure was applied to the left SCM. The follow-up examinations showed good wound healing and a positive outcome. Bilateral congenital muscular torticollis is a very rare form of muscle skeletal disorder. We describe a surgical treatment of such deformation that ended with a satisfactory result confirmed through a 25-year follow-up.

Objective: To identify effects of a deviant motor drive in the autospectral power of dystonic muscles during voluntary contraction in cervical dystonia patients., Methods: Submaximal (20%) isometric head-neck tasks were performed with the head fixed, measuring surface EMG of the sternocleidomastoid, splenius capitis and semispinalis capitis in CD patients and controls. Autospectral power of muscle activity, and head forces was analyzed using cumulative distribution functions (CDF). A downward shift between the theta/low alpha-band (3-10Hz) and the high alpha/beta-band (10-30Hz) was detected using the CDF 10 , defined as the cumulative power from 3 to 10Hz relative to power from 3 to 30Hz., Results: CDF 10 was increased in dystonic muscles compared to controls and patient muscles unaffected by dystonia, due to a 3-10Hz power increase and a 10-30Hz decrease. CDF 10 also increased in patient head forces., Conclusions: Submaximal isometric contractions with the head fixed provided a well-defined test condition minimizing effects of reflexive feedback and tremor. We associate shifts in autospectral power with prokinetic sensorimotor control., Significance: Analysis of autospectral power in isometric tasks with the head fixed is a promising approach in research and diagnostics of cervical dystonia., (Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Botulinum toxin (BoNT) injections in the dystonic muscles is the preferred treatment for Cervical Dystonia (CD), but the proper identification of the dystonic muscles remains a challenge. Previous studies showed decreased 8-14 Hz autospectral power in the electromyography (EMG) of splenius muscles in CD patients. Cumulative distribution functions (CDF's) of dystonic muscles showed increased CDF 10 values, representing increased autospectral powers between 3 and 10 Hz, relative to power between 3 and 32 Hz. In this study, we evaluated both methods and investigated the effects of botulinum toxin. Intramuscular EMG recordings were obtained from the splenius, semispinalis, and sternocleidomastoid muscles during standardized isometric tasks in 4 BoNT-naïve CD patients, 12 BoNT-treated patients, and 8 healthy controls. BoNT-treated patients were measured 4-7 weeks after their last BoNT injections and again after 11-15 weeks. We found significantly decreased 8-14 Hz autospectral power in splenius muscles, but not in the semispinalis and sternocleidomastoid muscles of CD patients when compared to healthy controls. CDF 10 analysis was superior in demonstrating subtle autospectral changes, and showed increased CDF 10 values in all studied muscles of CD patients. These results did not change significantly after BoNT injections. Further studies are needed to investigate the origin of these autospectral changes in dystonia patients, and to assess their potential in muscle selection for BoNT treatment., Competing Interests: M.T: Funds: Nuts-Ohra, Princes Beatrix Muscle fund, Gossweiler foundation, Science Foundation Dystonia Society, Funds Mental Health, Phelps Foundation, Beatrix Children Hospital Fund, Healthy Aging Fund UMCG. M.T. received unrestricted grants from Actelion, Merz, Ipsen, Allergan Pharmaceutics & Medtronic and a Honorarium from the Merz expert meeting in Paris, January 2016. These sponsors had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, and in the decision to publish the results. J.K.: Funds: Princes Beatrix Muscle fund. The AMC movement disorder group received unrestricted educational and research grants from Ipsen and Allergan. These sponsors had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, and in the decision to publish the results.

Objective: To investigate the effects of kinesiology taping and different types of application techniques of kinesiology taping in addition to therapeutic exercises in the treatment of congenital muscular torticollis., Design: Prospective, single blind, randomized controlled trial., Setting: An outpatient rehabilitation clinic in a tertiary university hospital., Subjects: Infants with congenital muscular torticollis aged 3-12 months., Interventions: Group 1 included 11 infants who only received exercises, Group 2 included 12 infants who received kinesiology taping applied on the affected side by using inhibition technique in addition to exercises. Group 3 included 10 infants who additionally received kinesiology taping applied on the unaffected side by using facilitation technique and on the affected side by using inhibition technique., Main Measures: Range of motion in lateral flexion and rotation of the neck, muscle function and degree of craniofacial changes were assessed at pretreatment, post treatment and, 1 month and 3 months' post treatment., Results: Friedman analysis of within-group changes over time revealed significant differences for all of the outcome variables in all groups except cervical rotation in Group 3 ( P<0.05). No significant differences were found between groups at any of the follow-up time points for any of the outcome variables ( P>0.05)., Conclusions: There is no any additive effect of kinesiology taping to exercises for the treatment of congenital muscular torticollis. Also different techniques of applying kinesiology taping resulted in similar clinical outcomes.

Background: Focal dystonia has been associated with deficient processing of sense of effort cues. However, corresponding studies are lacking in cervical dystonia (CD). We hypothesized that dystonic muscle activity would perturb neck force control based on sense of effort cues., Methods: Neck extension force control was investigated in 18 CD patients with different clinical features (7 with and 11 without retrocollis) and in 19 control subjects. Subjects performed force-matching and force-maintaining tasks at 5% and 20% of maximum voluntary contraction (MVC). Three task conditions were tested: i) with visual force feedback, ii) without visual feedback (requiring use of sense of effort), iii) without visual feedback, but with neck extensor muscle vibration (modifying muscle afferent cues). Trapezius muscle activity was recorded using electromyography (EMG)., Results: CD patients did not differ in task performance from healthy subjects when using visual feedback (ANOVA, p>0.7). In contrast, when relying on sense of effort cues (without visual feedback, 5% MVC), force control was impaired in patients without retrocollis (p = 0.006), but not in patients with retrocollis (p>0.2). Compared to controls, muscle vibration without visual feedback significantly affected performance in patients with retrocollis (p<0.001), but not in patients without retrocollis. Extensor EMG during rest, included as covariate in ANOVA, explained these group differences., Conclusion: This study shows that muscle afferent feedback biases sense of effort cues when controlling neck forces in patients with CD. The bias acts on peripheral or central sense of effort cues depending on whether the task involves dystonic muscles. This may explain why patients with retrocollis more accurately matched isometric neck extension forces. This highlights the need to consider clinical features (pattern of dystonic muscles) when evaluating sensorimotor integration in CD., Competing Interests: LC reports grants from Université Pierre et Marie Curie, Paris VI, outside the submitted work. SS reports personal fees from Allergan, personal fees from Ipsen, personal fees from Merz-Pharma, outside the submitted work. VG has nothing to disclose. SM reports grants from Fondation Paul Bennetot, outside the submitted work. MV reports grants from INSERM, grants from APHP, grants from APTES, grants from France Parkinson, grants from AMADYS, personal fees from Movement Disorders Society, outside the submitted work; and Member of the advisory board for Merz and Medtronic. PGL reports grants from Gloria and Jacques Gosweiler Foundation, outside the submitted work and owns shares in Aggero MedTech AB, a company commercializing a measurement instrument for spasticity. In addition, PGL and MAM have patented a method for measurement of manual dexterity (EP2659835A1). JPB reports personal fees from MERZ-PHARMA, personal fees from IPSEN, grants from AMADYS, grants from AFREK, outside the submitted work. This does not alter the authors’ adherence to PLOS ONE policies on sharing data and materials.

At first glance, cervical dystonia might be an illustration of the well-known proposition "function follows form". Nevertheless, cervical dystonia is a highly non-physiological condition, which cannot be reproduced by healthy subjects and does not respond to the usual physiological rules. "Dysfunction follows form" might be the most accurate aphorism to define cervical dystonia. Taking into account this situation and recent insights, the anatomic approach needs to be adapted to allow a better understanding of semiology and to improve botulinum toxin therapy. In this review dealing with a new approach to cervical dystonia, we develop some practical anatomical concepts concerning the head and neck complex. Knowledge of cervical spine and muscular dysfunctions in cervical dystonia is an essential stage in treating cervical dystonia patients with botulinum toxin.

Introduction: The centrum medianum- parafascicular complex of the human thalamus has a critical influence on cortical activity and significantly influences somatosensory function, arousal, and attention. In addition to its cortical connections, this region of the intralaminar thalamic nuclei is also connected to motor areas of the basal ganglia and the brain stem., Objective: The goal of this study was to identify movement-related neurons in the centrum medianum-parafascicular complex and analyze the changes in their activity during voluntary movements in patients with cervical dystonia., Methods: Single-unit activity was recorded during the micro-electrode-guided surgical ablation procedures in patients with cervical dystonia. The neural responses and synchronous electromyographic signals of the neck and finger flexor muscles were simultaneously recorded., Results: We found the following 3 types of movement-sensitive neurons in the centrum medianum-parafascicular complex: neurons that responded selectively to voluntary hand movement (hand-only neurons), neurons that selectively responded to neck movements (neck-only neurons), neurons responding to both hand and neck movements (combined neurons). We discovered the following 3 patterns of movement-related changes in neural activity: an increase in the firing rate, a reduction in the bursting activity, and short-term oscillations and synchronization with neighboring neurons. The most pronounced and prolonged responses were observed during movements involving neck muscles as well as during involuntary dystonic movements., Conclusion: The centrum medianum-parafascicular complex of the thalamus is a component of the subcortical network that participates in motor behavior and may be involved in the pathophysiology of cervical dystonia. © 2016 International Parkinson and Movement Disorder Society., (© 2016 International Parkinson and Movement Disorder Society.)

Background: A number of studies have shown that facial asymmetry improves in congenital muscular torticollis (CMT) patients after surgical release. This study confirmed the improvement in facial asymmetry, and analyzed factors that affect the change of facial asymmetry in CMT patients after surgical release by using objective and quantitative methods., Methods: Facial asymmetry was analyzed in 60 CMT patients who underwent surgical release before 10 years of age. Horizontal and lower facial asymmetry angles (HFAA and LFAA) in the clinical photograph were used to measure facial asymmetry. Postoperative improvements in HFAA and LFAA were evaluated in each age group, after grouping the patients by age. Patients were divided into 2 groups according to the postoperative head tilt and functional deficit. Postoperative improvements in HFAA and LFAA were compared between 2 groups. The relationships between postoperative improvements in HFAA and LFAA and independent variables (age, follow-up period, preoperative HFAA or LFAA, postoperative head tilt, and postoperative functional deficit) were analyzed., Results: Mean age at operation was 34.8 months (range, 6-120 mo). Horizontal facial asymmetry angle was improved significantly postoperatively in groups <5 years of age. Lower facial asymmetry angle was improved significantly postoperatively in all age groups. No significant difference was found in the postoperative improvements in HFAA and LFAA between 2 groups according to the postoperative head tilt and functional deficit. In the correlation analysis, postoperative improvements in HFAA and LFAA were proportional to the follow-up period (r = 0.256, P = 0.048) and preoperative HFAA or LFAA (r = 0.600, P < 0.001)., Conclusions: Facial asymmetry in CMT patients can be improved in part if surgical release is performed before 10 years of age and the possibility of improvement may be different according to the area of the face. After surgical release, facial asymmetry will improve over a long period of time, and patients with more severe facial asymmetry have a better remodeling potential to achieve facial symmetry.

Introduction: We tested whether a change in head/neck position initiates head deviation in drug-naïve patients with cervical dystonia and to identify the electrophysiological and neuroanatomical correlates of dystonic head rotation., Methods: Twenty-five consecutive drug-naïve patients with cervical dystonia and 25 healthy controls underwent the simultaneous surface electromyographic (EMG) recording of sternocleidomastoid (SCM) muscle contractions during head/neck position changes, blink reflex recovery cycle (BRrc), DAT-SPECT, and advanced structural neuroimaging analysis using voxel-based morphometry (VBM)., Results: Surface EMG recordings of SCM muscle activity during changes in head/neck position demonstrated an insignificant asymmetric low amplitude of the SCM muscle contractions in the horizontal position in both patients and controls, but an asymmetric high amplitude in SCM muscle contractions leading to abnormal head movements in vertical positions in patients with cervical dystonia. All controls had a symmetric low increase in amplitude of SCM muscle contractions in response to changes in head/neck position. VBM analysis in 19 patients showed abnormal decreases of gray matter (GM) volume in the bilateral motor (localized in the homunculus of the head) and premotor cortices when compared to controls. In addition, the side of these neuroanatomical changes was asymmetrically related to abnormal head deviations in these patients. All subjects had normal results during BRrc and DAT-SPECT., Conclusions: The passage from inactive horizontal position to active vertical head/neck posture initiates head deviation in drug-naïve patients with cervical dystonia, and the anatomical correlates of this dystonic head rotation is a restricted abnormal pattern of GM changes in the motor cortices., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Objectives: Anterocollis as a rare subtype of cervical dystonia is difficult to treat and thus less appreciated than other subtypes of cervical dystonia. This review aimed at summarising and discussing recent advances in the management of anterocollis., Methods: Literature review., Results: Pure anterocollis is a rare condition but 1-24% of the cases of complex cervical dystonia present with an anterocollis component. Applying the collum-caput concept, anterocollis may be subdivided into conceptual anterocollis, anterocaput, and forward sagittal shift, which is useful to direct selection of dystonic muscles for treatment. Additionally, identification of dystonic muscles in conceptual anterocollis, anterocaput, or forward sagittal shift is achieved by electromyography, computed tomography, magnetic resonance imaging, or FDG-positron emission tomography. Treatment of choice is botulinum toxin A. In case of treatment failure, more rarely affected muscles need to be identified and injected. Deep muscles, as are frequently involved in conceptual anterocollis, anterocaput, and forward sagittal shift, should be injected only under guidance of electromyography, endoscopy, or imaging. The more accurately affected muscles are identified, the better the outcome., Conclusions: Anterocollis as a subtype of cervical dystonia, responds poorly to botulinum toxin but management of this condition can be improved by application of identifying and guiding technologies., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Background: The preferred treatment for cervical dystonia (CD) is injection of botulinum toxin in the dystonic muscles. Unfortunately, in the absence of reliable diagnostic methods it can be difficult to discriminate dystonic muscles from healthy muscles acting in compensation. We investigated if dystonic muscle activation patterns could be identified in cervical dystonia patients during a harmonized isometric contraction task. Furthermore, we investigated whether dystonia worsens at higher levels of voluntary contraction, which might further improve the identification of dystonic muscle activity., Methods: An isometric device was used to investigate muscle activation during voluntary contraction tasks in 10 controls and 10 CD patients. Surface electromyography (EMG) of the sternocleidomastoidus, splenius capitis, and semispinalis capitis muscles was evaluated during a rest task and when performing submaximal (20%) and maximal voluntary contractions for eight head transversal force directions and for head twist. Two measures were developed to identify dystonic activation: 1) Muscle activity in the contraction direction in which the contribution of the muscle was lowest (Minimum EMG), and 2) the average muscle activity over all contraction directions (Total Mean EMG)., Results: Patients showed increased dystonic activity in the rest task and during submaximal contractions relative to controls, but not during maximal contractions. Increases in Minimum EMG indicated an inability of patients to deactivate dystonic muscles counteracting the task. Increases in Total Mean EMG indicated dystonic activity in all task directions. During maximal contractions these effects were absent in dystonic muscles. Dystonia is therefore found not to worsen at higher levels of isometric voluntary contraction. The activity of dystonic muscles modulated with different loading directions similar to controls. Using Minimum EMG 54% of the muscles clinically diagnosed as dystonic and 91% of non-dystonic muscles were predicted correctly., Conclusions: Dystonic muscle activity was found in cervical dystonia patients during submaximal contractions in all task directions using a harmonized isometric task, but no differences were found during maximal contractions. With some adaptation this method may prove useful to identify dystonic muscles., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Objective: To investigate the immediate effect of kinesiology taping (KT) on muscular imbalance in the lateral flexors of the neck., Design: Randomized controlled trial., Participants: Twenty-nine infants with congenital muscular torticollis and muscular imbalance in the lateral flexors of the neck were chosen consecutively. In addition, 5 healthy infants with no signs of muscular imbalance in the neck were tested., Method: The infants were randomly allocated to either an intervention group or a control group. The intervention group had kinesiology taping applied on the affected side using the muscle-relaxing technique. The healthy infants were tested both with and without kinesiology taping. The evaluator was blinded to whether the infants were or were not taped., Results: There was a significant difference in the change of Muscle Function Scale (MFS) scores between the groups (P < .0001). In the intervention group, there were significantly lower scores on the affected side that had been taped (P < .0001) and also significantly higher scores on the unaffected side (P = .01). There were no significant differences in the control group. For the healthy infants, with no imbalance in the lateral flexors of the neck, there were no changes to the MFS scores regardless of whether the kinesiology tape was applied., Conclusions: For infants with congenital muscular torticollis, kinesiology taping applied on the affected side had an immediate effect on the MFS scores for the muscular imbalance in the lateral flexors of the neck., (Copyright © 2015 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.)

Cervical dystonia is characterized by abnormal posturing of the head, often combined with tremor-like oscillatory head movements. The nature and source of these oscillatory head movements is controversial, so they were quantified to delineate their characteristics and develop a hypothetical model for their genesis. A magnetic search coil system was used to measure head movements in 14 subjects with cervical dystonia. Two distinct types of oscillatory head movements were detected for most subjects, even when they were not clinically evident. One type had a relatively large amplitude and jerky irregular pattern, and the other had smaller amplitude with a more regular and sinusoidal pattern. The kinematic properties of these two types of oscillatory head movements were distinct, although both were often combined in the same subject. Both had features suggestive of a defect in a central neural integrator. The combination of different types of oscillatory head movements in cervical dystonia helps to clarify some of the current debates regarding whether they should be considered as manifestations of dystonia or tremor and provides novel insights into their potential pathogenesis., (© 2015 International Parkinson and Movement Disorder Society.)

Objective: Botulinum toxin injections in the dystonic muscles are the preferred treatment for cervical dystonia (CD), but proper selection of the dystonic muscles remains a challenge. We investigated the use of EMG coherence and autospectral analysis as discriminative tools to identify dystonic muscles in CD patients., Methods: We compared the occurrence of 8-14 Hz autospectral peaks and 4-7 Hz intermuscular coherences between 10 CD patients and 10 healthy controls. Secondly, we compared the muscles with significant 4-7 Hz coherences with the muscles that were selected clinically for botulinum toxin treatment., Results: Autospectral peaks between 8 and 14 Hz were significantly more often absent in the splenius capitis (SPL) muscles of CD patients compared to controls (p<0.01). Contrary to previous findings, there was no significant difference in the occurrence of 4-7 Hz intermuscular coherences between patients and controls and the diagnostic accuracy of coherence analysis to identify the clinically dystonic muscles was low., Conclusion: Intermuscular EMG coherence analysis cannot reliably discriminate patients from controls. Autospectral changes in the SPL muscles are a more discriminative feature of CD. In patients, coherence analysis does not seem to be a reliable method to identify dystonic muscles. The clinical relevance and the origin of the autospectral changes need further study., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Objective: To compare the effects of a combination of therapeutic exercise and ultrasound with or without additional microcurrent therapy in infants with congenital muscular torticollis involving the entire sternocleidomastoid muscle., Design: Prospective, randomized, placebo-controlled trial., Setting: An outpatient rehabilitation clinic in a tertiary university hospital., Subjects: Infants (n = 20) with congenital muscular torticollis involving the entire sternocleidomastoid muscle., Interventions: Group 1 comprised 10 infants who received therapeutic exercise with ultrasound alone and Group 2 comprised 10 infants who received the same treatment with microcurrent therapy., Main Measures: Passive cervical rotational range of motion was measured at before treatment and one, two, three, and six months after initial treatment. Thickness, cross-sectional area, and red pixel intensity on colour histograms, which were all assessed before treatment and at three months after initial treatment. Additionally, the duration of treatment was measured., Results: The mean passive cervical rotational range of motion measured at three months posttreatment was significantly greater in Group 2 (101.1°) than that in Group 1 (86.4°), and the thickness, cross-sectional area, and red pixel intensity of the affected sternocleidomastoid muscle were all less in Group 2 (7.8 mm, 100.3 mm(2), and 126.1, respectively) than those in Group 1 (9.6 mm, 121.5 mm2, and 140.5, respectively). The mean duration of treatment was significantly shorter in Group 2 (2.6 months) than in Group 1 (6.3 months)., Conclusions: Microcurrent therapy may increase the efficacy of therapeutic exercise with ultrasound for the treatment of congenital muscular torticollis involving the entire sternocleidomastoid muscle., (© The Author(s) 2013.)

Background: Neglected congenital muscular torticollis is rare in adults but may be associated with pain and cosmetic problems. The efficacy of surgical correction in these patients has not been well established in the literature., Questions/purposes: We sought to describe (1) the primary goals that influenced adults with neglected muscular torticollis to seek surgical correction, (2) improvements of patients' function (specifically ROM) and radiographic parameters associated with deformities, (3) patient-derived outcomes using a previously published scale that includes function, and cosmesis, and (4) complications associated with sternocleidomastoid release in one surgeon's series of patients., Methods: We retrospectively reviewed the records of all adult patients who had surgical release of the sternocleidomastoid muscle between 2003 and 2011 by one surgeon and who were followed up for more than 1 year after surgery; 42 of these procedures were performed, and complete followup was available for 37 of them (88%). The mean age of the patients was 27 years (range, 18-48 years), and the minimum followup was 1.5 years (mean, 2.4 years; range, 1.5-4.7 years). The primary goal of the operation for each patient was determined to assess if it was met. Functional outcomes were assessed in terms of motion deficit: lateral flexion and rotation. Radiographic outcomes were assessed for cervicomandibular angle, lateral translation of the head and neck, and Cobb's angle of the cervicothoracic spine. The overall clinical results of surgery were assessed with the modified Cheng and Tang scoring system, and complications were tallied from a chart review., Results: The primary goals of surgery were cosmesis in 17 (46%) patients, pain in 11 (30%) patients, and function in nine (24%) patients. Patients for whom pain was the primary goal of surgery were older than the other patients. ROM and radiographic parameters associated with deformity consistently improved in these patients (p < 0.001 in all planes), and the overall results according to the modified Cheng and Tang scoring system indicated that 21 (57%) patients had an excellent result, 12 (32%) had a good result, and four (11%) had a fair result. There were no serious complications and no reoperations in these patients., Conclusions: Sternocleidomastoid release was beneficial for the treatment of neglected congenital muscular torticollis in adults and was not associated with any serious complications. Surgical treatment led to cosmetic and functional improvements and relieved pain originating from the muscle imbalance brought about by the long-standing deformity., Level of Evidence: Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Few studies have described physical therapy approaches to provide functional independence and reduce pain in individuals with dystonia. This report describes the physical therapy treatment of a 46-year-old woman diagnosed with idiopathic segmental axial dystonia. For two years, the patient was treated with kinesiotherapy (active and resisted movements and stretching of neck and trunk muscles), abdominal taping (kinesiotaping techniques), functional training, and sensory tricks. She was assessed with parts I, II and III of Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS-I, TWSTRS-II and TWSTRS-III), Berg Balance Scale (BBS), Six-Minute Walk Test (6-MWT), and the motor domain of Functional Independence Measure (FIM-motor) before and after the two-year treatment and after the one year follow-up. Postural control and symmetry improved (TWSTRS-I: from 30 to 18), functional independence increased (TWSTRS-II: from 27 to 15; BBS: from 36 to 46; 6-MWT: from 0 to 480 meters (m); FIM-motor: from 59 to 81), and the pain diminished (TWSTRS-III: from 12 to 5). The functional improvement was retained after one year (TWSTRS-I: 14/35; TWRTRS-II: 12/30; TWRTRS-III: 5/20; BBS: 48/56; 6-MWT: 450 m; FIM-motor: 81/91). This program showed efficacy on providing a better control of the dystonic muscles and thus the doses of botulinum toxin needed to treat them could be reduced. Outcomes support the therapeutic strategies used to deal with this type of dystonia.

Rationale: Cervical dystonia, also called spasmodic torticollis, is the most common form of (primary) dystonia. Intramuscular injections with botulinum toxin are the first line of treatment for cervical dystonia. To optimise the treatment response to botulinum toxin correct muscles should be selected. Clinical evaluation is important for muscle selection but the value of additional tests to identify dystonic muscles remains unclear., Objective: To evaluate all relevant literature regarding the best approach to select dystonic muscles for treatment with botulinum toxin., Methods: We conducted a systematic review of studies that had investigated methods of selecting muscles for treatment with botulinum toxin. In addition, we compared all prospective botulinum toxin trials using either clinical evaluation or polymyographic electromyography for muscle selection., Results: Forty relevant studies were included and polymyographic electromyography recordings were most often employed. In several studies, polymyographic electromyography revealed a different pattern of muscle involvement compared to that found during clinical evaluation. In one randomized controlled trial polymyographic electromyography significantly improved the outcome of botulinum toxin treatment. A limited number of studies used positron emission tomography -computed tomography imaging or frequency analysis of the electromyography signal to identify dystonic muscles but their effect on the outcome of treatment has never been studied., Conclusion: Polymyographic electromyography may improve the outcome of botulinum toxin treatment in cervical dystonia, but evidence is limited and larger studies are needed., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Converging data on focal dystonias suggest a widespread disorder of somatosensory processing. The aims of our study were, first, to assess somatosensory activation patterns in cervical dystonia (CD) beyond the representation of the affected body parts and, second, to search for task-related activation changes induced by botulinum toxin type-A (BoNT-A) therapy. Functional magnetic resonance imaging (MRI) during electrical median nerve stimulation was employed in seven CD patients and nine controls; the examination was repeated 4 weeks after BoNT-A application to dystonic neck muscles. The pretreatment activation map of patients showed activation in the contralateral primary somatosensory cortex, but missing activation in the secondary somatosensory cortex and insula, in contrast to controls and patients after treatment. Clinically significant effect of BoNT-A therapy was associated with a significant increase of BOLD response in the contralateral secondary somatosensory, insular, and inferior parietal cortices. The posttreatment somatosensory maps of patients did not significantly differ from controls. This study has brought evidence of widespread disruption of somatosensory processing in CD and its modification with BoNT-A therapy.

Seventy-eight patients with confirmed primary cervical dystonia CD were studied. All patients underwent CT of the soft tissues of the neck using slices at cervical vertebrae and MRI images of the cervical spine and of soft tissues. MRI images of 50 patients who did not have CD were used for comparison. This was followed by measuring the largest diameter along with the description of the shape of all observable muscles including the small muscles of the occipital area. In lateral flexion and rotation, 19% of patients showed disorders of muscles acting on head joints (laterocaput/torticaput). Muscles that act on the cervical spine were affected (laterocollis/torticollis) in 20% of patients. Both types of the disorder, but with various degrees of the caput- and collis- involvement, were presented in 61% of patients. Consequently, the ratio for these forms was approximately estimated as 1:1:3. The following conclusions have been made: In lateral flexion, clinical differentiation between laterocollis and laterocaput is possible. Lateral shift is always a result of laterocollis on one side and laterocaput on the opposite side. In rotation, clinical differentiation between torticollis and torticaput is not always possible. CT slices at levels C1 and C2 are advisable in these cases. Comparing the positions of vertebrae on both levels will provide a safe differentiation between torticollis and torticaput. Analysis of forward flexion (differentiation between antecollis and antecaput) can be accomplished by lateral observation of the angles between the cervical spine and the thoracic spine, respectively, and between the cervical spine and the base of skull. The same applies to the analysis of backward flexion (differentiation between retrocollis and retrocaput). Sagittal shift forwards usually does not require further diagnosis: it is almost always caused by bilateral dystonic activities of the Mm. sternocleidomastoidei.

Anterior and posterior sagittal shift of the head are less common postures in cervical dystonia and, as such, have not been comprehensively studied. In this article, we have detailed both our clinical and electromyography (EMG) findings in 11 patients with prominent dystonic sagittal shift of the head. A new technique of injection of the longus colli, based on a laterocervical approach under EMG guidance, is described. We have detailed the clinical phenotypes of dystonic posterior sagittal shift or "double chin" posture (4 patients) and anterior sagittal shift or "goose neck" posture (7 patients) and proposed specific botulinum toxin (BoNT) treatment protocols for these postures. Seven patients with the goose-neck posture responded well (70%-90% benefit) to BoNT injections. Six patients responded to splenius capitii injections alone, and 1 patient needed, in addition, the injection into both sterno-cleido-mastoid muscles. Four patients with the double-chin posture responded well to BoNT injection (50%-80% benefit). Two patients responded to suprahyoid injection alone, and 2 patients needed, in addition, the injection into the sterno-cleido-mastoid and longus colli muscles. Dysphagia was avoided in all of the double-chin patient group by adjusting our injection technique into the suprahyoid and longus colli muscles. The individualised toxin BoNT protocols have resulted in an improved benefit. The new Longus colli injection technique has allowed for a therapeutic effect of botulinum toxin without causing dysphagia., (Copyright © 2011 Movement Disorder Society.)

Objective: The significance of electromyography (EMG) guidance in botulinum toxin (BT) treatment has been much debated. The aim of this study was to evaluate if EMG guidance in the treatment of torticollis in BT-naive patients had a better outcome than treatment after clinical evaluation alone., Methods: Twenty-six patients with torticollis were included and treated for 1 year in this prospective, blinded study. Quantitative EMG was performed simultaneously in the four most frequently affected muscles: the sternocleidomastoid muscles and the posterior neck muscles on both sides. EMGs were analysed for turns per second. Clinical ratings were performed by an experienced neurologist (A). Injections were given by another neurologist (B), who was blinded to the ratings. In group 1, the results of the EMG were available to the treating neurologist B, whereas in group 2, neurologist B was blinded. In group 1, treatment with BT was given when turns per second were higher than 100., Results: In patients treated guided by EMG, clinical outcome, evaluated by objective ratings, was better than in patients treated based on clinical judgement alone (p = 0.05). In group 2, 105 muscles were treated with BT. Of these, 37 did not show dystonic EMG activity., Conclusions: Treatment with BT guided by EMG results in better clinical outcome than treatment without EMG and reduces the amount of BT used., Significance: EMG guidance by interference pattern analysis may optimise BT treatment in torticollis by a more precise injection and may reduce side effects and the risk of development of antibodies to BT., (Copyright © 2011 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

This study focuses on the treatment of the muscle function imbalance and asymmetric head posture for infants with congenital muscular torticollis (CMT). The aim of this study was to compare treatment time for groups with different strategies for muscle function training. The treatment goal was to achieve a symmetric head posture. Thirty-seven infants were randomised to three groups. Group I was treated only with handling strategies. Group II got the same handling strategies but also received specific strength exercises. Group III received the same treatment as group II but was also provided with weekly training by a physiotherapist. Mean age at the start of treatment was 4.5 months of age, range 1-10.5. The possible effect of covariates was also investigated; age at the start of treatment, range of motion (ROM) in rotation of the neck, ROM in lateral flexion of the neck, the muscle function scale (MFS) score, plagiocephaly, and gender were analysed with ANCOVA. Thirty-one treated infants achieved symmetric head posture before the age of 12 months. Mean treatment time (3.5 month) did not differ significantly between the groups. All infants randomised to group I could stay in that group. The MFS score and age at the start of the treatment influenced treatment time (p < 0.05). The treatment time for all groups was similar. Early referral to physical therapy of infants with CMT and muscle function imbalance in lateral righting could shorten treatment time. Studies about natural course and long-term effects of muscular imbalance must be investigated in future research.

Background: The evidence suggests that the origin of primary dystonia is at least partly associated with widespread dysfunction of the basal ganglia and cortico-striato-thalamo-cortical circuits. The aim of the study was to assess the sensorimotor activation pattern outside the circuits controlling the affected body part in cervical dystonia, as well as to determine task-related activation changes induced by botulinum toxin type A (BoNT-A) treatment., Methods: Seven patients suffering from cervical dystonia and nine healthy controls were examined with functional MRI during skilled hand motor task; the examination was repeated 4 weeks after BoNT-A application to dystonic neck muscles., Results: Functional MRI data demonstrated overall reduced extent of hand movement-related cortical activation but greater magnitude of blood oxygenation level dependent signal change in the contralateral secondary somatosensory cortex in patients compared to controls. Effective BoNT-A treatment led to reduced activation of the ipsilateral supplementary motor area and dorsal premotor cortex in patients. The patients' post-treatment sensorimotor maps showed significantly smaller basal ganglia activation compared to controls., Conclusions: These results provide imaging evidence that abnormalities in sensorimotor activation extend beyond circuits controlling the affected body parts in cervical dystonia. The study also supports observations that BoNT-A effect has a correlate at central nervous system level, and such effect may not be limited to cortical and subcortical representations of the treated muscles., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Botulinum toxin injections ameliorate dystonic symptoms by blocking the neuromuscular junction and weakening dystonic contractions. We asked if botulinum toxin injections in dystonia patients might also affect the integrity of sensorimotor cortical plasticity, one of the key pathophysiological features of dystonia. We applied a paired associative stimulation protocol, known to induce long-term potentiation-like changes in the primary motor cortex hand area to 12 patients with cervical dystonia before and 1 and 3 months after botulinum toxin injections to the neck muscles. Primary motor cortex excitability was probed by measuring transcranial magnetic stimulation-evoked motor evoked potentials before and after paired associative stimulation. We also measured the input-output curve, short-interval intracortical inhibition, intracortical facilitation, short afferent inhibition, and long afferent inhibition in hand muscles and the clinical severity of dystonia. Before botulinum toxin injections, paired associative stimulation significantly facilitated motor evoked potentials in hand muscles. One month after injections, this effect was abolished, with partial recovery after 3 months. There were significant positive correlations between the facilitation produced by paired associative stimulation and (1) the time elapsed since botulinum toxin injections and (2) the clinical dystonia score. One effect of botulinum toxin injection treatment is to modulate afferent input from the neck. We propose that subsequent reorganization of the motor cortex representation of hand muscles may explain the effect of botulinum toxin on motor cortical plasticity. © 2011 Movement Disorder Society., (Copyright © 2011 Movement Disorder Society.)

Objectives: The study objectives were to observe the clinical efficacy of primary massage of twining manipulation with one finger (PMTMOF) versus conventional tuina manipulation for treating muscular torticollis., Methods: A randomized, controlled, single-blind method was employed. Patients received either PMTMOF (experimental group, n = 265) or conventional tuina manipulation (control group, n = 235), once a day, 20 minutes each, for 15 days of treatment. After four treatment courses, sternocleidomastoid muscle morphology and size were detected using two-dimensional ultrasonography; sternocleidomastoid muscle blood flow was monitored by color Doppler ultrasonography; and head-neck deflection and range of motion were used to determine total curative effects., Results: In the treatment group, 55 patients were cured, 120 patients remarkably responded to the treatment, 75 patients effectively responded, and 15 patients were found to have no response. The total effective response rate to the treatment is 94.34%. In the control group, 15 patients were cured, along with very effective results in 60 patients, effective results in 125 patients, and ineffective results in 35 patients, and the total effective rate is 85.11%. A significant difference in total effective rate was found between experimental and control groups (p < 0.05)., Conclusions: PMTMOF produced more obvious curative treatment effects in infantile muscular torticollis than conventional tuina manipulation and could effectively shorten treatment time and avoid sequelae due to delayed healing.

Vestibular dysfunction has been reported in patients with cervical dystonia (CD), but it is still unclear whether the abnormalities occur as part of the CD syndrome or whether they arise from the abnormal posture and movement of the head. We compared vestibular-evoked myogenic potentials (VEMPs) recorded from the affected neck muscles (i.e., cervical VEMPs) with those recorded from muscles unaffected by the dystonia, the extraocular muscles (i.e., ocular VEMPs). We compared 21 patients and age-matched normal controls to investigate whether these short-latency reflexes are altered in CD. We also measured subjective visual horizontal (SVH). Seven patients had not received botulinum toxin treatment (naïve group), 10 were receiving regular injections (treatment group), and four had previously received treatment but had developed antibodies (antibody group). Both cervical and ocular VEMPs were present in the majority of patients and controls. For both reflexes, there were no significant differences between stimulation of the two sides of the head, between the treatment groups, or between the patients and controls. There was also no difference in degree of deviation of SVH between the groups. Our results showed that VEMPs can be reliably recorded from both the neck and extraocular muscles in patients with CD, even after long disease or treatment durations, and provide evidence for intact short-latency vestibular reflexes in CD., (© 2010 Movement Disorder Society.)

Unilateral congenital agenesis of sternocleidomastoid and trapezius muscles is a rare occurrence. To date, there are only two case reports in the literature. We present a case of a 7-year-old Caucasian boy who was born with torticollis and a cosmetic defect in his neck. An MRI scan of the cervical spine and shoulder confirmed the absence of the right sternocleidomastoid and trapezius muscles. No other significant congenital anomalies were detected. The patient has been followed-up for 7 years with no functional disabilities.

Dystonia in the neck region can be safely and effectively reduced with injections of Botulinum neurotoxin-A and B. People with idiopathic cervical dystonia have been studied the most. Benefits following injection include increased range of movement at the neck for head turning, decreased pain, and increased functional capacity (Class I evidence, level A recommendation). The evidence for efficacy and safety in patients with secondary dystonia in the neck is unclear based on the lack of rigorous research conducted in this heterogeneous population (level U recommendation). Psychometrically sound assessments and outcome measures exist to guide decision-making (Class I evidence, level A recommendation). Much less is known about the effectiveness of therapy to augment the effects of the injection (Class IV, level U recommendation). More research is needed to answer questions about safety and efficacy in secondary spastic neck dystonia, effective adjunctive therapy, dosing and favourable injection techniques.

Congenital muscular torticollis (CMT) is caused by shortening of the sternocleidomastoid (SCM) muscle, which may lead to neck movement limitation and craniofacial deformity. The authors retrospectively reviewed clinical experiences of CMT at their hospital from February 2007 to June 2008. During the study period, 20 CMT patients underwent complete tight fibrous band release and resection. Mean patient age was 47.6 months at operation. Eighteen of the 20 patients started a programme of physical therapy preoperatively. All patients received well-controlled postoperative physical therapy and wore a soft neck collar to correct head position for at least 3 months. At 3 months postoperatively, passive ranges of neck motion were determined, and compared with those of uninvolved sides. Eighteen patients showed a full range of motion of neck rotation and lateral flexion, but one patient showed a 10 degrees limitation in lateral flexion, and another showed 10 degrees limitations of neck rotation and lateral flexion. The authors recommended that the described operative technique involving complete fibrous band release and resection, combined with intensive postoperative physical therapy and application of a soft neck collar, provides good functional and cosmetic results., (Copyright 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Aim: The aim of the study was to evaluate the proportion of left and right congenital muscular torticollis (CMT) in both genders and age groups of the patients as well as to evaluate the duration of physical therapy and treatment outcome in observed population., Methods: In our study, 980 children with CMT without hematoma were treated at University children's Hospital of Belgrade (Serbia). They were divided into 2 groups: group with left torticollis and group with right torticollis. Boys and girls were separately evaluated. Patients were classified into 5 age groups: group of children less than one month of life, group above one to 3 months, group above 3 months to 6 months, group above 6 months to 12 months and group of children above 12 months of life. Optimal time for physical therapy was analyzed in every age group., Results: We diagnosed 496 torticollis in boys and 484 torticollis in girls. There were 458 children with left torticollis and 522 children with right torticollis. In group of children less than one month of life median duration of physical therapy was 1.5+/-0.3 months, in group above one to 3 months of life 5.9+/-0.6, in group above 3 to 6 months 7.2+/-0.6, in group above 6 to 12 months 9.8+/-0.6 and in group of children above 12 months of life 10.3+/-0.8 months., Conclusion: Right torticollis is frequent in both genders and age groups. Younger children have lower treatment duration and better treatment outcome. Boys have longer treatment duration and not significantly better treatment outcome.

Purpose: To describe the early effectiveness of microvascular decompression (MVD) for the treatment of spasmodic torticollis (ST)., Methods: Twelve patients with spasmodic torticollis were treated by microvascular decompression of the accessory nerves using a microscopic neurosurgical technique via the retrosigmoid approach. The most common compressing blood vessels were the ipsilateral posterior inferior cerebral artery (PICA) and/or the vertebral artery. The intraoperative monitor was introduced to detect the accessory nerve and to avoid unnecessary damage to the nerve., Results: Ten patients were cured (83%), and the other two (17%) improved with moderate spasms. In most cases, the improvement was noticed 1 week after the operation. No operation-related complications were observed during the follow-up period, which ranged from 2 months to 3 years., Conclusions: The early effect of MVD for some patients with spasmodic torticollis was satisfactory, but the long-term results need to be assessed further.

It is unclear how the torticollis occasionally observed in patients with progressive supranuclear palsy (PSP) relates to vestibulo-collic reflex mechanisms. We report here the results of vestibular evoked myogenic potentials (VEMPs) in a PSP patient with forced head deviation in the opposite direction of turning, leading to torticollis for a few seconds. As VEMPs were normal bilaterally we conclude that an enhanced vestibulo-collic reflex per se is not the cause of the torticollis in our patient. The abnormal head deviation induced by turning in some PSP patients is best explained by damage to reticular nuclei responsible for resetting eye and head saccades. When such mechanisms are defective, unopposed vestibulo-collic reflexes can lead to eye and head deviations in the opposite direction of body turns.

Two 8-year-old boys presented with complaints of torticollis and pain on neck turning. Both patients had a history of throat infection. Radiography and computed tomography demonstrated atlanto-axial subluxation. The patients were treated under diagnoses of Grisel's syndrome, unilateral or bilateral subluxation of the atlas on the axis associated with infection in the head or neck. Both patients were treated conservatively with bed rest, muscle relaxants, non-steroid anti-inflammatory agents, and immobilization with a soft cervical collar and cervical halter traction. The torticollis had resolved and reduction of atlanto-axial subluxation was confirmed within 2 weeks in both patients. Early diagnosis of Grisel's syndrome with appropriate antibiotics and a cervical collar, with halter traction if necessary, can achieve good outcome. Surgical treatment for the reduction of atlanto-axial subluxation should be reserved for persistent or recurrent cases.

Infants with congenital muscular torticollis (CMT) often have an imbalance in muscle function in the lateral flexors of the neck, and the need for a valid and reliable assessment tool to determine muscle function in these muscles is essential. The lateral uprighting response is used to examine and to strengthen the sternocleidomastoid muscle. A Muscle Function Scale (MFS) has been refined and used for several years in a clinic for infants with CMT. The MFS describes an infant's muscle function in the lateral flexors of the neck through ordered categorical scores. The aim of this study was to find out if the muscle function scale (MFS) is valid and reliable. A panel of experts examined validity, and the kappa statistic and intraclass correlation coefficient were calculated for interrater and intrarater reliability. The MFS is found to be a valid tool to measure the muscle function of the lateral flexors of the neck in infants with CMT. The interrater and intrarater reliability is high for both novice and experienced physiotherapists (kappa>0.9; ICC>0.9).

Sensory gestes (SG) are a pathognomonic sign of dystonia, which can be detected in up to two thirds of patients with cervical dystonia (CD). They reduce dystonia severity markedly but transiently. We report a patient whose CD substantially worsened with sensory input to the back of the head and neck in different body postures, a phenomomen recently termed "reverse" sensory geste (rSG) in craniocervical dystonia. In a cohort of CD outpatients, screening for "reverse" effects of SG on dystonia yielded a prevalence of 12.8% (n = 6/47). The most frequent rSG pattern was increased dystonic activity in a supine, resting position while trying to fall asleep. The response to rSG persisted throughout the course of the disease arguing for an impairment of central integration of neck proprioception. Assessment of rSG should be included in the routine examination of CD patients, since BTX treatment may have to beadjusted accordingly to be efficacious.