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Torticollis is encountered often in the paediatric setting and should be considered a presenting symptom, rather than a diagnosis. Aetiologies of torticollis are numerous, and the nomenclature describing underlying diagnosis can be confusing. Furthermore, children with torticollis typically present in the first instance to primary or secondary care rather than to the subspecialist. These factors can contribute to erroneous treatment of this patient-group which could be time critical in some instances. In this review, we discuss the common causes for torticollis and propose a simple clinical assessment tool and early management scheme that will assist in the differential-diagnosis and treatment pathway of this challenging condition.Conclusion: Torticollis can be the initial presentation of various conditions. The diagnosis and management tools provided in this article can aid in guiding paediatricians as to the correct initial management, imaging, and specialist referral. What is Known: • Torticollis in childhood is a very common presenting symptom with numerous aetiologies. • Management is complex, requires multiple clinical and imaging examinations, and is usually performed by non-specialized professionals. What is New: • A new, simple clinical-assessment tool under the acronym PINCH designed to aid paediatric general practitioners in diagnosing correctly the aetiology of torticollis. • A practical management scheme to aid in the treatment pathway of children with torticollis. more...

In the last 3 decades, the incidence of positional cranial deformations in infants, such as positional plagiocephaly and positional brachycephaly, has increased. The deviating shape often causes parental concern for the later psychosocial wellbeing of the child. Treatment options are nonoperative, varying from positional change to helmet therapy, of which the effect has often been debated. Multiple risk factors have been associated with an increased risk on the development of these deformations. The goal of this study was to assess the impact of known risk factors on the type and severity of resulting positional cranial deformation.Parents were asked to fill out a questionnaire regarding the presence of risk factors, such as gender, age, pregnancy duration, method of delivery and reasons for atypical deliveries, breech position, birth weight, developmental status, positional preference, family history, number of siblings, and torticollis presence. Treatment methods were documented and plagiocephalometry was used to measure the cranial proportions. All children were invited to participate in short-term follow-up. The significance of the risk factors and the effect of different kinds of therapy are discussed. Although no significant correlation was found between severity and risk factors, some risk factors could be correlated with the kind of positional cranial deformation. Of the different therapies, helmet therapy had a significant impact on the reduction of positional plagiocephaly. more...

Botulinum toxin type A (BoNTA) products are widely used for therapeutic and aesthetic indications, but there is a need for longer-lasting treatments that maintain symptom relief between injections and reduce the frequency of re-treatment. DaxibotulinumtoxinA for Injection (DAXI) is a novel BoNTA product containing highly purified 150-kDa core neurotoxin and is the first to be formulated with a proprietary stabilizing excipient peptide (RTP004) instead of human serum albumin. The positively charged RTP004 has been shown to enhance binding of the neurotoxin to neuronal surfaces, which may enhance the likelihood of neurotoxin internalization. DAXI produces robust, extended efficacy across both aesthetic and therapeutic indications. In an extensive glabellar lines clinical program, DAXI showed a high degree of efficacy, a consistent median time to loss of none or mild glabellar line severity of 24 weeks, and median time until return to baseline of up to 28 weeks. In adults with cervical dystonia, DAXI at 125 U and 250 U significantly improved Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total scores, with a median duration of efficacy of 24 and 20 weeks, respectively, which compares favorably with the 12–14 weeks’ duration reported for approved BoNTA products. Overall, DAXI was well tolerated, and the consistent extended duration of effect suggests that DAXI has the potential to improve the management of both aesthetic and therapeutic conditions., Plain Language Summary Botulinum toxin is used to block the nerve signals that cause muscles to contract. Products containing botulinum toxin are commonly given by injection to treat muscle spasms (such as cervical dystonia, a painful condition where the neck muscles contract involuntarily) and for cosmetic treatment of frown lines. However, the effects of the currently approved botulinum toxin products typically wear off about 3–4 months after injection and so the injections must be repeated regularly. A new product called DAXI (DaxibotulinumtoxinA for Injection) has been developed. In this product, the botulinum toxin is formulated with a unique protein (called RTP004) that has been designed to help deliver the botulinum toxin to the nerve cells. Research suggests that the RTP004 protein in DAXI adheres the botulinum toxin to the nerves close to the injection site, potentially making its effect last longer. To date, DAXI has been studied in over 3800 patients. The studies have shown that DAXI is effective for treating neck spasms (cervical dystonia) and for reducing the appearance of frown lines. Importantly, the effects of DAXI lasted up to 6 months, which is longer than seen with other botulinum toxin products. The side effects seen with DAXI are consistent in nature and frequency with those seen with other botulinum toxin products. These findings suggest that DAXI can improve both medical and cosmetic treatments due to its longer-lasting effect. more...

Torticollis refers to a condition in which the head is persistently tilted to one side, sometimes associated with pain. Torticollis in a child can be congenital or acquired. Torticollis as an initial manifestation of an underlying demyelinating syndrome is quite rare in children. Here, we report a 7-year-old girl who presented with persistent torticollis. Neuroimaging of the brain revealed features of a demyelinating disease. Further studies did not show any evidence of multiple sclerosis. Cerebrospinal fluid was negative for antiaquaporin-4 antibodies, antimyelin oligodendrocyte glycoprotein antibodies, and oligoclonal bands. A seronegative demyelinating disorder was considered. She was treated with pulsed methylprednisolone therapy. She responded well to steroids with no progression of illness during follow-up. Torticollis was partially improved. more...

To evaluate the synergistic occurrence of facial asymmetry and craniofacial deformities in cases of Congenital muscular torticollis (CMT) and to assess the outcome of surgical correction on these deformities. Twenty-three cases of CMT presenting in ENT OPD from January 2015 to December 2019, 9 cases requiring surgical intervention were included. Facial asymmetry and craniofacial deformities were evaluated and quantified by measuring gaze angle, translational deformity and orbito-alar distance. All cases underwent a bipolar tenotomy using harmonic scalpel. In all cases on comparison with preoperative status there was significant improvement (p more...

INTRODUCTION: In dystonia, dopaminergic alterations are considered to be responsible for the motor symptoms. Recent attention for the highly prevalent non-motor symptoms suggest also a role for serotonin in the pathophysiology. In this study we investigated the dopaminergic, serotonergic and noradrenergic metabolism in blood samples of dystonia patients and its relation with (non-)motor manifestations.METHODS: Concentrations of metabolites of dopaminergic, serotonergic and noradrenergic pathways were measured in platelet-rich plasma in 41 myoclonus-dystonia (M-D), 25 dopa-responsive dystonia (DRD), 50 cervical dystonia (CD) patients and 55 healthy individuals. (Non-)motor symptoms were assessed using validated instruments, and correlated with concentrations of metabolites.RESULTS: A significantly higher concentration of 3-methoxytyramine (0.03 vs. 0.02 nmol/L, p < 0.01), a metabolite of dopamine, and a reduced concentration of tryptophan (50 vs. 53 μmol/L, p = 0.03), the precursor of serotonin was found in dystonia patients compared to controls. The dopamine/levodopa ratio was higher in CD patients compared to other dystonia groups (p < 0.01). Surprisingly, relatively high concentrations of levodopa were found in the untreated DRD patients. Low concentrations of levodopa were associated with severity of dystonia (rs = -0.3, p < 0.01), depression (rs = -0.3, p < 0.01) and fatigue (rs = -0.2, p = 0.04).CONCLUSION: This study shows alterations in the dopaminergic and serotonergic metabolism of patients with dystonia, with dystonia subtype specific changes. Low concentrations of levodopa, but not of serotonergic metabolites, were associated with both motor and non-motor symptoms. Further insight into the dopaminergic and serotonergic systems in dystonia with a special attention to the kinetics of enzymes involved in these pathways, might lead to better treatment options. more...

Introduction In patients with cervical dystonia (CD), pain is a major contributor to disability and social isolation and is often the main reason patients seek treatment. Surveys evaluating patient perceptions of their CD symptoms consistently highlight pain as a troublesome and disabling feature of their condition with significant impact on daily life and work. Areas covered In this article, the authors review the epidemiology, assessment, possible mechanisms and treatment of pain in CD, including a meta-analysis of randomized controlled trial data with abobotulinumtoxinA. Expert opinion Mechanisms of pain in CD may be muscle-based and non-muscle based. Accumulating evidence suggests that non-muscle-based mechanisms (such as abnormal transmission and processing of nociceptive stimuli, dysfunction of descending pain inhibitory pathways as well as structural and network changes in the basal ganglia, cortex and other areas) may also contribute to pain in CD alongside prolonged muscle contraction. Chemodenervation with botulinum toxin is considered the first-line treatment for CD. Treatment with botulinum toxin is usually effective, but optimization of the injection parameters should include consideration of pain as a core symptom in addition to the motor problems. more...

Acquired cervical scoliosis previously has been reported in dogs as a clinical sign associated with Chiari‐like malformation and syringomyelia but has not been described with inflammatory central nervous system disease. A 9‐month‐old Flat‐Coated Retriever was presented with an acute onset of cervical scoliosis with no other neurological deficits. Magnetic resonance imaging identified a focal, poorly defined intramedullary lesion within the cranial cervical spinal cord. Cerebrospinal fluid (CSF) analysis indicated mononuclear pleocytosis consistent with a diagnosis of meningomyelitis of unknown etiology. A second dog, a 3‐year‐old female spayed German Shepherd, developed an acute onset of cervical scoliosis with mild generalized proprioceptive ataxia 2 months after commencing immunosuppressive corticosteroid treatment for presumed steroid‐responsive meningitis‐arteritis. Magnetic resonance imaging at the time of diagnosis disclosed a similar intramedullary lesion within the cranial cervical spinal cord, with a neutrophilic pleocytosis on CSF analysis. Both dogs were treated with immunosuppressive dosages of prednisolone, along with cytosine arabinoside in the first dog, with resolution of cervical scoliosis seen in both. To our knowledge, this is the first report of acute onset acquired, reversible cervical scoliosis in dogs with presumed immune‐mediated meningomyelitis. more...

Background Cervical dystonia (CD) is the most common form of focal dystonia with involuntary movements and postures of the head. The pathogenesis and neural mechanisms underlying CD have not been fully elucidated. Methods Twenty-seven newly drug-naive patients with CD and 21 healthy controls (HCs) were recruited with clinical assessment and resting-state functional magnetic resonance imaging (rs-fMRI) scanning. Severity of CD was measured by Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and Tsui scores. Whole-brain voxel-wise intrinsic connectivity (IC) and seed-based functional connectivity (FC) analyses were performed for detection of changes in the CD group relative to HCs, controlling for age, gender, and global time series correlation, followed by correlation analyses of IC, seed-based FC and clinically relevant features, respectively. Results In comparison with HCs, CD patients showed significantly increased IC measurement in the anterior part of the left supramarginal gyrus and extended to the inferior left postcentral gyrus (AL-SMG/IL-PCG). With this cluster as a seed, decreased FC was found in the right precentral and postcentral gyrus. Moreover, the regional IC value in the AL-SMG/IL-PCG was significantly positively correlated with TWSTRS-1 (severity) score, and significantly negatively correlated with the associated seed-based FC strength. Conclusions Our results showed signs of both hyper- and hypo-connectivity in bilateral regions of the sensorimotor network related to CD. The imbalance of functional connectivity (both hyper- and hypo-) may hint both overloading and disrupted somatosensory or sensorimotor integration dysfunction within the sensorimotor network underlying the pathophysiology of CD, thus providing a network target for future therapies. more...

Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has become an accepted treatment for severe cervical dystonia (CD). Assessment of therapeutic efficacy of DBS mostly focused on head position at rest but hardly on limitations of head and neck mobility, which represent a functionally important impairment in CD. We aimed to determine prospectively head and neck range of motion (ROM) preoperatively and during chronic bilateral GPi DBS in a series of 11 patients with idiopathic CD or segmental dystonia with prominent CD using a computerized motion analysis. Maximum horizontal rotation of the head in the transverse plane and lateral inclination in the frontal plane were measured preoperatively and at a median of 7 months of chronic GPi DBS, using an ultrasound-based three-dimensional measuring system combined with surface electromyography of cervical muscles. Horizontal rotation of the head increased from 78.8° ± 31.5° (mean ± SD) preoperatively to 100.7° ± 24.7° with GPi DBS (p more...

Introduction. The relevance of the feet planovalgus deformity problem is caused by its prevalence and tendency to progression, insufficient knowledge of a number of aspects, and the complexity of treatment. Another urgent pediatric orthopedics problem is the placement torticollis in infants, and the possible relationship of this problem with feet planovalgus deformity. Among the least studied aspects of these diseases categories it is necessary to highlight the problem of the peculiarities of the osteopathic status in children with feet planovalgus deformity with a history of placement torticollis, and the dynamics of clinical manifestations during their osteopathic correction.The aim of the research was to study the features of the osteopathic status in patients with planovalgus deformity of the feet in combination with a history of placement torticollis and without such a combination, and to evaluate the clinical efficacy of complex therapy, including osteopathic correction.Materials and methods. The study involved 60 patients aged 7–14 years with planovalgus deformity of the feet. The study participants were divided into two groups. The first group included 30 patients with planovalgus deformity of the feet and a history of placement torticollis. The second group included 30 patients with planovalgus deformity of the feet, who did not have a history of torticollis. All study participants received complex therapy (orthopedic treatment and osteopathic correction). At the beginning and at the end of the course of osteopathic correction, the condition of the arch of the foot (the severity of fl at feet) and osteopathic status were assessed in all study participants.Results. In the first group there were prevailed somatic dysfunctions (SD) of the head region, the detection rate was 86%, the pelvic region (somatic component — C), 76 %, and the neck region (C), 13 %. Among the second group participants there were prevailed SD of the pelvic region (C) — the detection rate was 100 %, the lower extremities, 43 %, and the lumbar region (C), 13 %. The SD in the pelvic region was the most typical; however, more often (ppppppConclusion. The revealed differences between the participants of the groups in the prevalence and number of regional dysfunctions and the prevalence of local SD could, presumably, reflect the relationship between the placement torticollis and planovalgus deformity of the feet. Based on the absence of differences in the degree of severity of flat feet between the participants of the groups, it can be assumed that the history of the placement torticollis is not associated by itself with the aggravation of the severity of feet planovalgus deformity. more...

Introduction Isolated head tremor, a pathological condition characterized by head tremor without dystonic postures or tremor in other body parts, has recently been suggested to be a form of dystonia. It is however still unclear whether isolated head tremor precedes dystonia or remains unmodified overtime. Methods We enrolled 20 patients with isolated head tremor. For each patient, we assessed videos recorded at enrollment and after 5 years. The videotapes were reviewed by two independent experienced movement disorder specialists who evaluated and scored tremor and CD severity using the Fahn-Tolosa-Marin Clinical Rating Scale for Tremor and the revised Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), respectively. Results Upon enrollment, all 20 patients showed isolated head tremor. Mean tremor severity was 2.7 ± 0.9 as measured using the Fahn-Tolosa-Marin Clinical Rating Scale for Tremor total score. At the 5-year follow-up examination, 15 (75%) of the 20 patients with isolated head tremor showed dystonic postures in the neck, while the remaining 5 patients (25%) had only isolated head tremor. Mean severity of dystonia as measured using the TWSTRS-2 total score was 11.8 ± 3.6. Head tremor severity was unchanged between baseline and the 5-year follow-up examination (p > 0.05). At the follow-up examination, no patients had tremor or dystonia in a body part other than the neck, nor did they develop bradykinesia or other parkinsonian signs. Conclusions Our longitudinal study demonstrated that patients with isolated head tremor may develop cervical dystonia over time. more...

Case report. Juvenile rheumatoid arthritis (JRA) typically presents with fever, rash, anterior uveitis, and/or joint pain. We present three cases with initial torticollis due to rotatory subluxation of C1–C2 as an initial sign of JRA. Three girls, ages 5–9, presented with C1-2 rotatory subluxation. Traction was able to reduce the atlanto-axial joint in all cases. Based on imaging, history, exam, and laboratory results, they were diagnosed with JRA. After reduction of the atlantoaxial joint, they were transitioned to a halo vest and disease-modifying antirheumatic drugs (DMARDs). The older 2 children underwent C1-2 fusion. The younger child has minimal symptoms and has not undergone surgical intervention 4 years from initial presentation. Rotatory subluxation can be the first presenting sign of JRA. Younger children may be able to be treated conservatively with traction and medication, while older children may require occiput to C2 fusion due to bony destruction and basilar invagination. IV. more...

Background The dentatorubrothalamic tract (DRTT) remains understudied in idiopathic cervical dystonia (CD), despite evidence that the pathway is relevant in the pathophysiology of the disorder. Objective The aim of this study was to examine the DRTT in patients with CD using diffusion tensor imaging (DTI)-based tractography. Methods Magnetic resonance imaging scans from 67 participants were collected to calculate diffusion tractography metrics using a binary tractography-based DRTT template. Fractional anisotropy and diffusivity measures of left and right DRTT were computed and compared between 32 subjects with CD and 35 age-matched healthy volunteers. Results Fractional anisotropy of right DRTT and mean and axial diffusivity of left DRTT were significantly reduced in patients with CD. Similar abnormalities were observed in patients with focal CD and patients with CD without tremor. DTI metrics did not correlate with disease duration or severity. Conclusions Significant reductions in DTI measures suggest microstructural abnormalities within the DRTT in CD, characterized by a tractography pattern consistent with decreased axonal integrity. © 2021 International Parkinson and Movement Disorder Society. more...

Cervical Dystonia (CD) and Parkinson's disease, particularly tremor-dominant motor phenotype (TD-PD), showed a selective deficit of time-based prospective memory (TBPM). The two movement disorders are mainly characterized by dysfunctions of basal-ganglia and prefrontal cortex but it is reported that cerebellum also plays a key role in their pathogenesis. These cerebral structures are specifically involved in TBPM rather than in event-based PM (EBPM), but until now no study directly compared these two components of PM between CD and TD-PD patients. Therefore, the present study aimed at investigating if differences in PM functioning between CD and TD-PD patients might exist and if the type of movement disorder moderated the relationship between deficit of PM and deficit of executive functions and retrospective memory. Thirty TD-PD, 27CD patients and 29 healthy subjects (HCs), matched for demographic features, underwent neuropsychological tests for PM, executive functions, retrospective memory and self-rated questionnaires. The three groups did not differ on neuropsychological variables except for TBPM where TD-PD and CD patients performed worse than HCs; moreover, TD-PD performed worse than CD patients. Moderation analysis indicated that the type of movement disorder moderated the relationship between executive dysfunction and TBPM, but not EBPM. In conclusion, selective deficit of TBPM characterizes both CD and TD-PD but it is associated with executive dysfunction only in TD-PD. It might be possible to speculate that the involvement of the cerebellum, responsible for internal timing processes, could explain the impairment of TBPM in both movement disorders. This issue deserves to be explored in future neuroimaging studies. more...

Grisel’s syndrome (GS) is a rare syndrome which refers only to non-traumatic atlantoaxial subluxation. This syndrome predominantly occurs in young children following an upper respiratory infection or otolaryngologic procedures. An eight-year-old girl with a delayed diagnosis of GS was admitted to our outpatient clinic with complaints of painful torticollis and neck stiffness. Three-dimensional computed tomography revealed rotatory atlantoaxial subluxation. After consulting with the neurosurgery department, the patient underwent surgery. The significance of this patient was that she was unable to be diagnosed early and atlantoaxial subluxation remained hidden for five years without any complications. In conclusion, this rare case highlights the importance of delayed diagnosis of GS and clinicians should be aware of this syndrome. more...

Dystonia is conceptualized as a network disorder involving basal ganglia, thalamus, sensorimotor cortex and the cerebellum. The cerebellum has been implicated in dystonia pathophysiology, but studies testing cerebellar function in dystonia patients have provided equivocal results. This study aimed to further elucidate motor network deficits in cervical dystonia with special interest in the role of the cerebellum. To this end we investigated motor learning tasks, that differ in their dependence on cerebellar and basal ganglia functioning. In 18 cervical dystonia patients and 18 age matched healthy controls we measured implicit motor sequence learning using a 12-item serial reaction time task mostly targeting basal ganglia circuitry and motor adaptation and eyeblink conditioning as markers of cerebellar functioning. ANOVA showed that motor sequence learning was overall impaired in cervical dystonia (p = 0.01). Moreover, unlike healthy controls, patients did not show a learning effect in the first part of the experiment. Visuomotor adaptation and eyeblink conditioning were normal. In conclusion, these data lend support to the notion that motor learning deficits in cervical dystonia relate to basal ganglia-thalamo-cortical loops rather than being a result of defective cerebellar circuitry. more...

In cervical dystonia, functional MRI (fMRI) evidence indicates changes in several resting state networks, which revert in part following the botulinum neurotoxin A (BoNT) therapy. Recently, the involvement of the cerebellum in dystonia has gained attention. The aim of our study was to compare connectivity between cerebellar subdivisions and the rest of the brain before and after BoNT treatment. Seventeen patients with cervical dystonia indicated for treatment with BoNT were enrolled (14 female, aged 50.2 ± 8.5 years, range 38–63 years). Clinical and fMRI examinations were carried out before and 4 weeks after BoNT injection. Clinical severity was evaluated using TWSTRS. Functional MRI data were acquired on a 1.5 T scanner during 8 min rest. Seed-based functional connectivity analysis was performed using data extracted from atlas-defined cerebellar areas in both datasets. Clinical scores demonstrated satisfactory BoNT effect. After treatment, connectivity decreased between the vermis lobule VIIIa and the left dorsal mesial frontal cortex. Positive correlations between the connectivity differences and the clinical improvement were detected for the right lobule VI, right crus II, vermis VIIIb and the right lobule IX. Our data provide evidence for modulation of cerebello-cortical connectivity resulting from successful treatment by botulinum neurotoxin. more...

Torticollis is a clinical diagnosis with heterogeneous causes. We present an unusual case of acquired torticollis in an 8-month-old female infant with a large cerebellopontine angle arachnoid cyst. Symptoms resolved after surgical fenestration. Non-traumatic acquired or new-onset torticollis requires brain imaging, and posterior fossa lesions are an important entity in the differential for pediatric clinicians. more...

Acquired torticollis is not a separate disease rather a warning sign of an underlying disorder. The etiology of torticollis in children is quite wide. Hematogenous osteomyelitis of the first cervical vertebra (the atlas) is an extremely rare and potentially dangerous pathology. The aim is to acquaint physicians of different specialties with an extremely rare condition – acute hematogenous osteomyelitis of the atlas. Case Report. A case of osteomyelitis of the first cervical vertebra in a 7-year-old boy is presented. An important sign of the disease is torticollis. Conclusions. Hematogenous osteomyelitis of the atlas is an extremely rare and potentially dangerous pathology. Regardless of its incidence, it must be considered when examining a child with acquired wryneck. All the ENT specialists must possess the knowledge of this disease, as they are most likely the first doctors to see such a child. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: torticollis, osteomyelitis of the atlas, C1 osteomyelitis, vertebral osteomyelitis, children. more...

OBJECTIVEThe primary treatment for atlantoaxial rotatory fixation (AARF) remains controversial. The aim of this study was to investigate the primary treatment for AARF and create an algorithm for primary treatment.METHODSThe authors analyzed the data of 125 pediatric patients at four medical institutions from April 1989 to December 2018. The patients were reported to have neck pain, torticollis, and restricted neck range of motion and were diagnosed according to the Fielding classification as type I or II. As a primary treatment, 88 patients received neck collar fixation, and 28 of these patients did not show symptom relief and required Glisson traction. Thirty-seven patients were primarily treated with Glisson traction. In total, 65 patients, including neck collar treatment failure patients, underwent Glisson traction in hospitals.RESULTSThe success rate of treatment was significantly higher in the Glisson traction group (97.3%) than in the neck collar fixation group (68.2%) (p = 0.0001, Wilcoxon test). In the neck collar effective group, Fielding type I was more predominant (p = 0.0002, Wilcoxon test) and the duration from onset to the first visit was shorter (p = 0.02, Wilcoxon test) than that in the neck collar ineffective group. Using multivariate logistic regression analysis with the above items, the authors generalized from the estimated formula: logit [p(success group by neck collar fixation group)|duration from onset to the first visit (x1), Fielding type (x2)] = 0.4(intercept) − 0.15x1 + 1.06x2, where x1 is the number of days and x2 = 1 (for Fielding type I) or −1 (for Fielding type II). In cases for which the score is a positive value, the neck collar should be chosen. Conversely, in cases for which the score is a negative value, Glisson traction should be the first choice.CONCLUSIONSAccording to this formula, in patients with Fielding type I AARF, neck collar fixation should be allowed only if the duration from onset is ≤ 10 days. In patients with Fielding type II, because the score would be a negative value, Glisson traction should be performed as the primary treatment. more...

Background Although several brain networks play important roles in cervical dystonia (CD) patients, regional homogeneity (ReHo) changes in CD patients have not been clarified. We investigated to explore ReHo in CD patients at rest and analyzed its correlations with symptom severity as measured by Tsui scale. Methods A total of 19 CD patients and 21 gender-, age-, and education-matched healthy controls underwent fMRI scans at rest state. Data were analyzed by ReHo method. Results Patients showed increased ReHo in the right cerebellum crus I and decreased ReHo in the right superior medial prefrontal cortex (MPFC). Moreover, the right precentral gyrus, right insula, and bilateral middle cingulate gyrus also showed increased ReHo values. A significantly positive correlation was observed between ReHo value in the right cerebellum crus I and symptom severity (p Conclusions Our investigation suggested abnormal ReHo existed in brain regions of the “pain matrix” and salience network (the right insula and bilateral middle cingulate gyrus), the motor network (the right precentral gyrus), the cerebellum and MPFC and further highlighted the significance of these networks in the pathology of CD. more...

Sternocleidomastoid (SCM) pseudotumors, also known as fibromatosis colli or congenital torticollis, are painless benign neck lumps found in newborns. Whilst unilateral cases are relatively common, bilateral SCM pseudotumors are a rare phenomenon with only a handful of cases reported internationally. We present the case of a 5-week-old infant who was brought to the emergency department with painless, bilateral, palpable anterior neck masses following a slightly traumatic but otherwise uncomplicated spontaneous delivery. An ultrasound scan of his neck revealed well-defined soft tissue lesions within both of the SCM muscles. He was subsequently diagnosed with bilateral SCM pseudotumors. This case emphasizes the importance of considering this entity as a differential diagnosis in infants presenting with bilateral palpable neck masses. more...

Background To observe the characteristics of binocular integration and stereopsis in children with television torticollis. Methods A retrospective study was carried out, where data were collected from 25 children with television torticollis as the disease group after refractive error correction and 25 normal children as the control group. A virtual reality system was used to assess and analyze the characteristics of binocular integration by a contrast balance test and binocular stereopsis. Results The 25 children in the disease group included 17 males and 8 females with an average age of 7.5 ± 1.9 years old and an average binocular spherical equivalent of − 0.35 ± 1.46D. The 25 children in the control group were also 17 males and 8 females with an average age of 7.3 ± 2.2 years old and the average binocular spherical equivalent of − 0.48 ± 0.93D. No significant differences were found in the horizontal bar contrast balance test between the 2 groups at near and far distances. Near-distance vertical bar contrast balance test was normal in 23 subjects and suppressed in 2 subjects in the control group, while it was normal in 13 subjects and suppressed in 12 subjects in the disease group, which showed a statistically significant difference (P = 0.002). Far distance vertical bar contrast balance test was normal in 24 subjects and suppressed in 1 subject in the control group, normal in 7 subjects and suppressed in 18 subjects in the disease group, showing a statistically significant difference (P = 0.000). All subjects in the 2 groups showed 100〞 as near distance stereoacuity. At far distance, the mean stereoacuity was 176.00〞 ± 92.56〞 in the control group, and 352.00〞 ± 270.99〞 in the disease group, with a statistically significant difference (P = 0.011). Conclusion By using virtual reality technology, defects in binocular visual function were found in children whose television torticollis persisted after regular refractive error correction. Television torticollis may be associated with the deficit of binocular integration for vertical bars and far distance stereopsis. more...

In a recent workshop on "Defining research priorities in dystonia,", there was absolutely no reference to sex as a factor in disease pathogenesis. In this viewpoint paper, we argue that the most distinctive aspects of adult onset isolated focal dystonia are the marked sex-related differences demonstrated by epidemiological, clinical, and laboratory studies in patients with adult onset dystonia, particularly in cervical dystonia, the most common presentation. We propose that the future focus of research should be on neurobiological mechanisms underlying the profound sexual dimorphism in this disorder. Targeting research into gamma aminobutyric acid (GABA)ergic function, which also shows similar sexual dimorphism, would be most productive in elucidating the pathogenesis of adult onset dystonia. © 2021 International Parkinson and Movement Disorder Society. more...

Objective The main objective of this study was to define and verify anatomo-sonographic landmarks for ultrasound-guided injection of botulinum toxin into the longissimus capitis (LC) and splenius cervicis (SC) muscles. Methods and results After a preliminary work of anatomical description of the LC and SC muscles, we identified these muscles on two cadavers and then on a healthy volunteer using ultrasound and magnetic resonance imaging (MRI) to establish a radio-anatomical correlation. We defined an anatomo-sonographic landmark for the injection of each of these muscles. The correct positioning of vascular glue into the LC muscle and a metal clip into the SC muscle of a fresh cadaver as verified by dissection confirmed the utility of the selected landmarks. Discussion For the LC muscle, the intramuscular tendon of the cranial part of the muscle appears to be a reliable anatomical landmark. The ultrasound-guided injection can be performed within the cranial portion of the muscle, between the intra-muscular tendon and insertion into the mastoid process at dens of the axis level. For the SC muscle, the surface topographic landmarks of the spinous processes of the C4–C5 vertebrae and the muscle body of the levator scapulae muscle seem to be reliable landmarks. From these, the ultrasound-guided injection can be carried out laterally by transfixing the body of the levator scapulae. Conclusion The study defined two cervical anatomo-sonographic landmarks for injecting the LC and SC muscles. more...

Background The pathophysiology of cervical dystonia is still unclear. Recent evidence points toward a network disorder affecting several brain areas. The objective of this study was to assess the saccadic inhibition as a marker of corticostriatal function in cervical dystonia. Methods We recruited 31 cervical dystonia patients and 17 matched healthy controls. Subjects performed an overlap prosaccade, an antisaccade, and a countermanding task on an eye tracker to assess automatic visual response and response inhibition. Results Cervical dystonia patients made more premature saccades (P = 0.041) in the overlap prosaccade task and more directional errors in the antisaccade task (P = 0.011) and had a higher rate of failed inhibition in the countermanding task (P = 0.001). Conclusions The results suggest altered saccadic inhibition in cervical dystonia, possibly as a consequence of dysfunctional corticostriatal networks. Further studies are warranted to confirm whether these abnormalities are affected by the available therapies and whether this type of impairment is found in other focal dystonias. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. more...

This study compared the cost of screening with outcomes and verified the utility of a screening program. This cross-sectional study included all elementary and junior high school students from Tsukuba city who underwent musculoskeletal screening in 2016; 19,583 students were included. Written marksheet type questionnaires were distributed that focused on reporting scoliosis, torticollis, flat foot, and pain, among other symptoms. Using information from the questionnaire, face-to-face screening was performed by a doctor on-campus and/or orthopedic surgeons. Students with abnormalities on screening were identified and referred for a hospital visit. The screening, questionnaire, reasons for and number of hospital visits, examination, treatment, and their individual costs were examined. Hospital visits were recommended to 532/19,583 (2.7%) students after musculoskeletal screening. Among them, 250 and 129 students visited other and our hospitals, respectively. Overall, 11 students required treatment. Costs associated with screening, hospital examination, and treatment totaled to ¥8,526,351; 129 students visited our institution, of whom 11 (8.5%) required treatment. Among them, 10/11 and 1/11 were treated using orthotic and operative treatments, respectively. If scoliosis surgery may be prevented by musculoskeletal screening and orthotic treatment, it is possible that the outcomes may justify the examination costs. more...

Descriptive, retrospective. Scientific level of evidence IV. The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine. A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2 years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed. Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5 years and 2 months (6 months to 13 years and 3 months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3 years and 5 months (range 2–12 years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment. Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results. more...

Prolonged remission of dystonia occurs rarely; however, well-documented cases are lacking. We report the clinical characteristics and course of four patients with botulinum toxin (BoNT)-associated prolonged remission of idiopathic cervical dystonia. Mean age at onset was 40 years. All had a relatively short duration of symptoms (mean 10.3 months), and with remission occurring after ≤ 3 treatments with BoNT. At last examination, the remission duration was 2–5 years. In the two cases that subsequently relapsed after 4–5 years, there was an altered phenomenology and worsened severity than at the onset. Recognizing this rare phenomenon has valuable clinical implications. more...

Background/Objective: To evaluate the success of combining ipsilateral inferior and superior oblique muscle surgery in young children with congenital unilateral superior oblique under action who present in infancy with a large socially noticeable head-tilt. Methods: A consecutive retrospective case series of young children was analysed. The success of surgery in eliminating the head-tilt was evaluated by pre- and postoperative ocular motility assessment focusing on the vertical misalignment in primary position and downgaze, the magnitude of the head-tilt in degrees and the status of the superior oblique tendon. Results: Five children had a mean age at first surgery of 41 (range 25–63) months, a mean primary position vertical deviation of 26 (25–30) prism dioptres, a head-tilt of 30 (20–35) degrees and a mean post-operative follow up of 24 (8–43) months. While there was a uniform surgical plan, nonetheless each operation required individualisation based on a spectrum of per-operative superior oblique tendon findings. The head tilt was eliminated in 40% and reduced in the remainder, to a mean of 7 (0–18) degrees and with a mean post-operative primary position vertical misalignment of 3 (range 0–10) and of 10 (range 0–40) prism dioptres in downgaze. Conclusion: Combined, ipsilateral oblique muscle surgery reduced the severe head tilt and primary position alignment to a psychosocially and functionally acceptable level. For the majority, the outcome was stable or associated with further decremental improvement. A persistent downgaze vertical tropia occurred in children with macroscopically abnormal superior oblique tendons but these cases were not identifiable clinically pre-operatively. more...

Objectives: Craniovertebral junction (CVJ) abnormalities constitute a group of treatable neurological disorders, especially in the Indian subcontinent. Thus, it is essential that clinicians should be able to make a precise diagnosis of abnormalities and rule out important mimickers on multidetector computed tomography (MDCT) as this information ultimately helps determine the management, prognosis, and quality of life of patients. CVJ is the most complex part of the cervical region. Congenital malformations of this region can cause serious neurological deficit and require a surgical intervention. The present study was undertaken to know the embryological basis of the CVJ and to identify commonly observed congenital CVJ abnormalities, their frequency, and mode of presentation. Materials and Methods: Diagnosed cases of CVJ anomalies on dynamic MDCT head were reviewed at a tertiary care center between January 2014 to December 2019. Type of anomaly, clinical presentation, and associated malformations were recorded. Different types of variations were expressed in terms of percentage. Results: Congenital anomalies were seen in 42 cases. Fifteen types of anomalies were detected. Anomalies were either singly or in combination. The CVJ anomalies were more common in young adults (28%), almost equal in both sexes. The most common anomaly was basilar invagination (52.3%), followed by atlanto-occipital assimilation (33.3%), and Arnold–Chiari malformation is the most common soft tissue anomaly. In fourteen cases, additional anomalies of other vertebrae were present. The most common symptoms were weakness of extremities, neck pain, paresthesia, torticollis, and gait disturbances. About 28 patients got improved, 8 patients had residual deficit as that of preoperative status, and 4 patients got deteriorated after surgery, at 1-month follow-up. About 34 patients had improved, 5 remained static, and 3 patients got worsened at the end of 3-month follow-up. About 37 patients had improved, 4 patients remained static, and 2 patients got deteriorated at 6 months of follow-up. The patients with increased atlantodens interval 3–5 mm showed 77% improvement after surgery. Conclusion: Congenital CVJ anomalies, though rare, are fatal. CVJ abnormalities constitute an important group of treatable neurological disorders with diagnostic dilemma. The atlantodental interval is the most important preoperative prognostic marker. Dynamic CT imaging can provide additional useful information to the diagnosis of CVJ instability. To prevent long-term neurological problems, early diagnosis and treatment of congenital bony CVJ anomalies is important. more...

OBJECTIVEAtlantoaxial rotatory fixation (AARF) is an acquired fixed abnormality of C1–2 joint rotation associated with torticollis in childhood. If the condition is left uncorrected, patients are at risk for developing C1–2 fusion with permanent limitation in the cervical range of movement, cosmetic deformity, and impact on quality of life. The management of AARF and the modality of nonsurgical treatment are poorly defined in both primary care and specialized care settings, and the optimal strategy is not clear. This systematic review aims to examine the available evidence to answer key questions relating to the nonsurgical management of AARF.METHODSA systematic review was performed using the following databases: PubMed, MEDLINE, Healthcare Management Information Consortium (HMIC), EMCare, Embase, Cumulative Index to Nursing and Allied Health Literature (CINAHL), British Nursing Index (BNI), and Allied and Complementary Medicine Database (AMED). Search criteria were created and checked independently among the authors. All articles with a radiological diagnosis of AARF and primary outcome data that met the study inclusion criteria were included and analyzed by the authors.RESULTSSearch results did not yield any level I evidence such as a meta-analysis or randomized controlled trial. The initial search yielded 724 articles, 228 of which were screened following application of the core exclusion criteria. A total of 37 studies met the full criteria for inclusion in this review, consisting of 4 prospective studies and 33 retrospective case reviews. No articles directly compared outcomes between modalities of nonsurgical management. Six studies compared the outcome of AARF based on duration of symptoms before initiation of treatment. Comparative analysis of studies was hindered by the wide variety of treatment modalities described and the heterogeneity of outcome data.CONCLUSIONSThe authors did not identify any level I evidence comparing different nonsurgical management approaches for AARF. There were few prospective studies, and most studies were uncontrolled, nonrandomized case series. Favorable outcomes were often reported regardless of treatment methods, with early treatment of AARF tending to yield better outcomes independent of the treatment modality. There is a lack of high-quality data, and further research is required to determine the optimal nonsurgical treatment strategy. more...

Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid–fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis. more...

Foaling is a process of giving birth to foal which is prompt and forceful event in equine breeding. Dystocia is declared when 2nd stage of parturition exceeds from 20 min without fetal movements in mare. Dystocia is very challenging situation for both mare and veterinarian. Survivability of mare and foal, and subsequent fertility of mare depends on efficient management. Therefore, the current report described a rare case of wry neck (torticollis or unilateral bent neck) resulted in dystocia, that was efficiently resolved by partial fetotomy and repulsion technique in mare under field conditions. Intensive post operative care was performed to avoid infection and to achieve fertility subsequently. more...

Objectives Non-motor symptoms (NMS) are commonly present along with motor impairment in patients with cervical dystonia (CD) and have a significant impact on health-related quality of life (HRQoL). However, the prevalence of NMS and their association with dystonia are still unclear. The aim of our study was to assess the prevalence of depression, anxiety, fatigue, apathy, pain, sleep problems, and excessive daytime sleepiness (EDS) in CD using different evaluation approaches and to explore their association with HRQoL relative to that of motor symptoms.Materials and Methods We enrolled 102 Slovak patients with CD. The severity of both motor and non-motor symptoms was assessed using validated scales. HRQoL was determined by the 36-item Short Form Health Survey (SF-36). Association of NMS with poor HRQoL was assessed using multiple regressions.Results The most frequent NMS in our sample were sleep impairment (67.3%), anxiety (65.5%), general and physical fatigue (57.5% and 52.9%, respectively), depression (47.1%), mental fatigue (31.4%), apathy (30.4%), reduced activity (29.4%), EDS (20.2%), and reduced motivation (18.6%). Univariate analysis showed that NMS, but not motor symptoms, were significantly linked to poor HRQoL, with EDS being most commonly associated with poor HRQoL, followed by disrupted sleep, depression, and fatigue.Conclusions The prevalence of NMS among patients with CD is high, and some NMS are strongly associated with poor HRQoL, while motor impairment was not associated with the severity of NMS or poor HRQoL. Actively diagnosing and treating NMS should therefore be a routine part of the clinical management of patients with CD. more...

Non-motor symptoms (NMS) occur in patients with cervical dystonia (CD) but with variable frequencies and impact on health-related quality of life (HRQoL). To define non-motor and motor profiles and their respective impact on HRQoL in CD patients using the newly validated Dystonia Non-Motor Symptoms Questionnaire (DNMSQuest). In an observational prospective multicentre case–control study, we enrolled 61 patients with CD and 61 age- and sex-matched healthy controls (HC) comparing demographic data, motor and non-motor symptoms and HRQoL measurements. 95% CD patients reported at least one NMS. Mean total NMS score was significantly higher in CD patients (5.62 ± 3.33) than in HC (1.74 ± 1.52; p r = 0.72, EQ-5D: r = − 0.59; p more...

Atlantoaxial rotatory subluxation (AARS) is a rare outcome of trauma in adults. We present a case of a 38-year-old female who presented with neck pain and stiffness after a mild trauma. On exam the patient had a “cock-robin” position, comparable to acute torticollis. Computerized tomography demonstrated findings consistent with AARS. Reduction was performed in the emergency department and the patient had no further neurological sequelae. Recognition of AARS after trauma requires a high index of suspicion and early diagnosis is important to best patient outcomes. more...

This case report presents oropharyngeal dysphagia due to oromandibular and cervical dystonia, a rare consequence of aseptic meningitis. A 19-year-old male who was diagnosed with aseptic meningitis visited the rehabilitation outpatient clinic for a sense of foreign body in his throat and odynophagia. Repetitive involuntary movements of his facial, tongue, and laryngeal muscles accompanied by lateroanterior torticollis were observed. Videofluoroscopic swallowing study showed inefficient bolus formation due to repetitive rolling of his tongue and vallecular stasis without penetration or aspiration. Dysphagia and odynophagia had brought the patient significant weight loss and frustration. We provided swallowing training to improve the efficiency and safety of swallowing. The patient’s symptoms improved gradually along with body weight gain and emotional stability. Acute-onset oropharyngeal dysphagia is devastating for young adults. A multidisciplinary approach is mandatory for optimal outcome. We share our experience as a team work and emphasize the rehabilitation aspect. more...

Spinal cord subependymomas (SCSEs) in children are extremely rare, and no reports distinguishing SCSEs from syringomyelia have been published. We report a case of a 10-year-old boy who presented with torticollis, scoliosis, as well as pain that had begun in the posterior portion of the neck and progressed to the right shoulder and upper arm. Magnetic resonance imaging showed an intramedullary cyst-like lesion with the same signal intensity as that of cerebrospinal fluid. Idiopathic syringomyelia with scoliosis was first suspected, and a syrinx-subarachnoid space shunt was performed. After surgery, the lesion was slightly smaller; however, 2 years after surgery, it had re-grown, causing excruciating pain but no other symptoms. A second surgery was performed, and gross total resection was achieved. Pathological evaluation revealed SCSE. SCSE needs to be considered as a differential diagnosis for spinal centric cyst-like lesions in children. more...

Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children. The aim of our study is to investigate all possible clinical and radiological prognostic factors in children with AARS/F. We retrospectively reviewed all cervical spine CT scans of children with AARS/F treated in our Hospital over the last 15 years. AARS/F was classified according to Fielding and Hawkins classification and C1-C2 rotation-degree was calculated. Moreover, two orthopedic surgeons reviewed all clinical reports of these children. All patients were conservatively treated (cervical traction/neck collar). An early recovery was considered in patients with complete clinical/radiological healing at 3 months follow-up, while a late recovery was considered in patients with disease persistence or relapse at 3 months follow-up or earlier. Fifty-five patients with diagnosis of AARS/F were included in the study (mean age = 8.5 years old - 25F, 30M). In 9/55 subjects (16.4%), a late recovery was observed. The presence of a concomitant infection or inflammation in the head and neck region (Grisel's syndrome) was significantly associated with a late recovery (p < .001). Also, the type of AARS/F (p = .019), according to the Fielding and Hawkins classification, and C1-C2 rotation-degree (p = .027) were significantly correlated with the recovery time.Conclusion: In patients with AARS/F, the presence of a concomitant infection/inflammation in the head and neck region is the most important prognostic factor and it is associated with a late recovery. The Fielding and Hawkins classification and C1-C2 rotation-degree well correlate with patients' recovery time. What is Known: • Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children and CT is the most useful imaging tool for diagnosis and classification of AARS/F. • Conservative treatments are effective in the majority of pediatric patients with AARS/F. What is New: • The presence of a concomitant infection/inflammation in the head and neck region associated with AARS/F (Grisel's syndrome) is the most important prognostic factor and it is associated with a late recovery. • C1-C2 rotation-degrees, as well as Fielding and Hawkins classification system, well correlate with patients' recovery time. more...

We aimed to analyze the prepulse inhibition (PPI) and recovery rate (R) of the trigeminocervical reflex (TCR) in patients with cervical dystonia (CD).We enrolled 15 patients with CD and 16 healthy subjects. TCR was recorded over splenius capitis after infraorbital nerve stimulation. For TCR-PPI, we applied a prepulse stimulus to the left second finger 100 ms prior to the test stimulus and the percentage of change of response to test stimulus was calculated. For TCR-R, we applied paired infraorbital stimuli at interstimulus interval (ISIs) of 300 ms and the percentage of change of the second compared to the first response was calculated.TCR-PPI and TCR-R values were higher (less inhibition and greater recovery) on both sides in the patient group compared to healthy subjects. There was high correlation between TCR-PPI and TCR-R on both sides in patients with dystonia (p 0.005). We did not find any significant relationship between TCR-R or TCR-PPI and side of dystonic posture.We showed disturbed modulation of TCR in CD patients. In CD, a general inhibition of the inhibitory pathways and facilitation of the excitatory pathways occur. Although TCR was recorded directly on the affected muscles in CD, symmetric abnormal TCR findings in CD suggest that these findings are probably secondary to altered function of higher order centers rather than being directly related to the pathophysiological process. more...

Purpose The primary purpose of this study was to describe the experiences of parents of infants diagnosed with congenital muscular torticollis (CMT). A secondary purpose was to compare the experiences of parents of infants with mild grades versus severe grades of involvement based on the CMT severity classification system. Methods Through semistructured interviews, a qualitative phenomenological approach of inquiry was used to investigate the lived experiences of 12 parents. Results Eight themes common to both groups of parents were identified. Findings indicated having an infant with CMT has a significant effect on the parents and other caregivers. Two themes were unique to parents of the infants with severe CMT. Conclusions Parents are faced with a diagnosis that requires regular therapy visits and a challenging home program. A multimodal approach by clinicians for teaching and supporting parents during the episode of care may best address their unique challenges and stresses. more...