Early-Onset and Severe Complex Hereditary Spastic Paraplegia Caused by De Novo Variants in SPAST.

BACKGROUND: Familial hereditary spastic paraplegia (HSP)-SPAST (SPG4) typically presents with a pure HSP phenotype. OBJECTIVE: The aim of this study was to delineate the genotypic and phenotypic spectrum of children with de novo HSP-SPAST. METHODS: This study used a systematic cross-sectional analysis of clinical and molecular features. RESULTS: We report the clinical and molecular spectrum of 40 patients with heterozygous pathogenic de novo variants in SPAST (age range: 2.2-27.7 years). We identified 19 unique variants (16/40 carried the same recurrent variant, p.Arg499His). Symptom onset was in early childhood (median: 11.0 months, interquartile range: 6.0 months) with significant motor and speech delay, followed by progressive ascending spasticity, dystonia, neurogenic bladder dysfunction, gastrointestinal dysmotility, and epilepsy. The mean Spastic Paraplegia Rating Scale score was 32.8 ± 9.7 (standard deviation). CONCLUSIONS: These results confirm that de novo variants in SPAST lead to a severe and complex form of HSP that differs from classic familial pure HSP-SPAST. Clinicians should be aware of this syndrome in the differential diagnosis for cerebral palsy. © 2022 International Parkinson and Movement Disorder Society.

Characterizing upper limb function in the context of activities of daily living in CLN3 disease.

In CLN3 disease, impairments in motor function are frequently reported to have later onset compared to visual and cognitive decline, but upper limb motor function has yet to be explored in this population. In a cohort of 22 individuals with CLN3, we used a novel application of multiple measures to (1) characterize motor function, particularly of the upper limbs, in activities of daily living (ADLs), and (2) explore associations between motor function and age as well as visual ability, disease severity, and cognitive function, as evaluated by the Unified Batten Disease Rating Scale (UBDRS), a validated CLN3 disease measure. ADLs that required coordination, speed, and fine motor control were particularly challenging for children with CLN3 based on item-level performance across direct assessments (Jebsen-Taylor Hand Function Test [JTHFT] and MyoSet Tools) and caregiver reports (Pediatric Evaluation of Disability Inventory-Computer Adaptive Testing [PEDI-CAT] and Patient-Reported Outcomes Measurement Information System [PROMIS] Pediatric Upper Extremity). Poorer visual ability, disease severity, and cognitive function were associated with worse performance on these measures, whereas age had limited impact. These findings support the need for children with CLN3 to receive skilled clinical evaluation and treatment tailored to their individual needs, particularly in the context of ADLs, as their symptom profile progresses.

Utility and practice of electrodiagnostic testing in the pediatric population: An AANEM consensus statement.

Nerve conduction studies and needle electromyography, collectively known as electrodiagnostic (EDX) studies, have been available for pediatric patients for decades, but the accessibility of this diagnostic modality and the approach to testing vary significantly depending on the physician and institution. The maturation of molecular diagnostic approaches and other diagnostic technologies such as neuromuscular ultrasound indicate that an analysis of current needs and practices for EDX studies in the pediatric population is warranted. The American Association of Neuromuscular & Electrodiagnostic Medicine convened a consensus panel to perform literature searches, share collective experiences, and develop a consensus statement. The panel found that electrodiagnostic studies continue to have high utility for the diagnosis of numerous childhood neuromuscular disorders, and that standardized approaches along with the use of high-quality reference values are important to maximize the diagnostic yield of these tests in infants, children, and adolescents.

Three-dimensional humeral morphologic alterations and atrophy associated with obstetrical brachial plexus palsy.

BACKGROUND: Obstetrical brachial plexus palsy (OBPP) is a common birth injury, resulting in severe functional losses. Yet, little is known about how OBPP affects the 3-dimensional (3D) humeral morphology. Thus, the purpose of this study was to measure the 3D humeral architecture in children with unilateral OBPP. METHODS: Thirteen individuals (4 female and 9 male patients; mean age, 11.8 ± 3.3 years; mean Mallet score, 15.1 ± 3.0) participated in this institutional review board approved study. A 3D T1-weighted gradient-recalled echo magnetic resonance image set was acquired for both upper limbs (involved and noninvolved). Humeral size, version, and inclination were quantified from 3D humeral models derived from these images. RESULTS: The involved humeral head was significantly less retroverted and in declination (medial humeral head pointed anteriorly and inferiorly) relative to the noninvolved side. Osseous atrophy was present in all 3 dimensions and affected the entire humerus. The inter-rater reliability was excellent (intraclass correlation coefficient, 0.96-1.00). DISCUSSION: This study showed that both humeral atrophy and bone shape deformities associated with OBPP are not limited to the axial plane but are 3D phenomena. Incorporating information related to these multi-planar, 3D humeral deformities into surgical planning could potentially improve functional outcomes after surgery. The documented reduction in retroversion is an osseous adaptation, which may help maintain glenohumeral congruency by partially compensating for the internal rotation of the arm. The humeral head declination is a novel finding and may be an important factor to consider when one is developing OBPP management strategies because it has been shown to lead to significant supraspinatus inefficiencies and increased required elevation forces.

A randomized cross-over study protocol to evaluate long-term gait training with a pediatric robotic exoskeleton outside the clinical setting in children with movement disorders.

Individuals with neuromuscular disorders display a combination of motor control deficits and lower limb weakness contributing to knee extension deficiency characterized by exaggerated stance phase knee flexion. There is a lack of evidence for long-term improvement of knee extension deficiency with currently available clinical treatment programs. Our previous work testing a wearable robotic exoskeleton with precisely timed assistive torque applied at the knee showed immediate increases in knee extension during walking for children with cerebral palsy, which continued to improve over an acute practice period. When we applied interleaved assistance and resistance to knee extension, we observed improvements in knee extension and increased muscle activation indicating the potential for muscle strengthening when used over time. There is a need for additional, high-quality trials to assess the impact of dosage, intensity and volume of training necessary to see persistent improvement in lower limb function for these patient populations. This randomized crossover study (ClinicalTrials.gov: NCT05726591) was designed to determine whether 12 weeks of overground gait training with a robotic exoskeleton outside of the clinical setting, following an initial in clinic accommodation period, has a beneficial effect on walking ability, muscle activity and overall motor function. Participants will be randomized to either complete the exoskeleton intervention or continue their standard therapy for 12 weeks first, followed by a crossover to the other study component. The primary outcome measure is change in peak knee extension angle during walking; secondary outcome measures include gait speed, strength, and validated clinical scales of motor function and mobility. Assessments will be completed before and after the intervention and at 6 weeks post-intervention, and safety and compliance will be monitored throughout. We hypothesize that the 12-week exoskeleton intervention outside the clinical setting will show greater improvements in study outcome measures than the standard therapy.

OnabotulinumtoxinA muscle injection patterns in adult spasticity: a systematic literature review.

BACKGROUND: OnabotulinumtoxinA has demonstrated significant benefit in adult focal spasticity. This study reviews the injection patterns (i.e., muscle distribution, dosing) of onabotulinumtoxinA for treatment of adult spasticity, as reported in published studies. METHODS: A systematic review of clinical trials and observational studies published between 1990 and 2011 reporting data on muscles injected with onabotulinumtoxinA in adult patients treated for any cause of spasticity. RESULTS: 28 randomized, 5 nonrandomized, and 37 single-arm studies evaluating 2,163 adult patients were included. The most frequently injected upper-limb muscles were flexor carpi radialis (64.0% of patients), flexor carpi ulnaris (59.1%), flexor digitorum superficialis (57.2%), flexor digitorum profundus (52.5%), and biceps brachii (38.8%). The most frequently injected lower-limb muscles were the gastrocnemius (66.1% of patients), soleus (54.7%), and tibialis posterior (50.5%). The overall dose range reported was 5-200 U for upper-limb muscles and 10-400 U for lower-limb muscles. CONCLUSIONS: The reviewed evidence indicates that the muscles most frequently injected with onabotulinumtoxinA in adults with spasticity were the wrist, elbow, and finger flexors and the ankle plantar flexors. OnabotulinumtoxinA was injected over a broad range of doses per muscle among the studies included in this review, but individual practitioners should be mindful of local regulatory approvals and regulations.

A call for action: Increasing the pediatric rehabilitation medicine workforce.

Pediatric Rehabilitation Medicine (PRM) is a unique blend of traditional medical rehabilitation knowledge and skills primarily focused on temporary and/or permanent disability conditions of childhood onset throughout the age continuum, with an emphasis on promoting function and participation. Although there are two established pathways to enhance knowledge and skills in PRM, one a combined residency with Pediatrics and the other a PRM fellowship, there has been a relative decline in participants in this training, as has been seen across other subspecialties in Physical Medicine and Rehabilitation (PM&R) and other medical specialties. Based on pediatric rehabilitation physician surveys and the increasing prevalence of children with disabilities, there has been a call to consider opening PRM fellowships to physicians not trained in PM&R. This commentary proposes establishing a commission to lead a transparent and inclusive process to assure that all options to address issues related to optimizing PRM care are considered and provide a course of action to address the needs of children and adults with childhood onset disabilities.

Evidence-based guideline: neuromuscular ultrasound for the diagnosis of carpal tunnel syndrome.

INTRODUCTION: The purpose of this study was to develop an evidence-based guideline for the use of neuromuscular ultrasound in the diagnosis of carpal tunnel syndrome (CTS). METHODS: Two questions were asked: (1) What is the accuracy of median nerve cross-sectional area enlargement as measured with ultrasound for the diagnosis of CTS? (2) What added value, if any, does neuromuscular ultrasound provide over electrodiagnostic studies alone for the diagnosis of CTS? A systematic review was performed, and studies were classified according to American Academy of Neurology criteria for rating articles of diagnostic accuracy (question 1) and for screening articles (question 2). RESULTS: Neuromuscular ultrasound measurement of median nerve cross-sectional area at the wrist is accurate and may be offered as a diagnostic test for CTS (Level A). Neuromuscular ultrasound probably adds value to electrodiagnostic studies when diagnosing CTS and should be considered in screening for structural abnormalities at the wrist in those with CTS (Level B).

Acceptability and potential effectiveness of a foot drop stimulator in children and adolescents with cerebral palsy.

AIM: Ankle-foot orthoses are the standard of care for foot drop in cerebral palsy (CP), but may overly constrain ankle movement and limit function in those with mild CP. Functional electrical stimulation (FES) may be a less restrictive and more effective alternative, but has rarely been used in CP. The primary objective of this study was to conduct the first trial in CP examining the acceptability and clinical effectiveness of a novel, commercially available device that delivers FES to stimulate ankle dorsiflexion. METHOD: Twenty-one individuals were enrolled (Gross Motor Function Classification System [GMFCS] levels I and II, mean age 13y 2mo). Gait analyses in FES and non-FES conditions were performed at two walking speeds over a 4 month period of device use. Measures included ankle kinematics and spatiotemporal variables. Differences between conditions were revealed using repeated measures multivariate analyses of variance. RESULTS: Nineteen individuals (nine females, 10 males; mean age 12y 11mo, range 7y 5mo to 19y 11mo; 11 at GMFCS level I, eight at level II) completed the FES intervention, with all but one choosing to continue using FES beyond that phase. Average daily use was 5.6 hours (SD 2.3). Improved dorsiflexion was observed during swing (mean and peak) and at foot-floor contact, with partial preservation of ankle plantarflexion at toe-off when using the FES at self-selected and fast walking speeds. Gait speed was unchanged. INTERPRETATION: This FES device was well accepted and effective for foot drop in those with mild gait impairments from CP.

Kinematic determinants of anterior knee pain in cerebral palsy: a case-control study.

OBJECTIVE: To quantify the role patellofemoral and tibiofemoral kinematics may play in development of anterior knee pain (AKP) in individuals with cerebral palsy (CP). DESIGN: Case-control. SETTING: Clinical research center. PARTICIPANTS: Knees from individuals with diagnosed CP (n=20) and control knees (n=40) were evaluated. Controls were matched for sex and age based on the group average. Matching by height and weight was a secondary priority. Subjects in the control cohort were asymptomatic with no history of lower leg abnormalities, surgery, or major injury. Only individuals who were physically capable of sustaining slow, cyclic knee flexion-extension for 2.5 minutes and had no contraindications to magnetic resonance imaging were enrolled. Both groups were samples of convenience. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Three-dimensional patellofemoral and tibiofemoral joint kinematics, acquired during active leg extension, under volitional control. RESULTS: Participants with CP and AKP (n=8) demonstrated significantly greater patellofemoral extension, valgus rotation, and superior and posterior displacement relative to controls and to the subgroup of participants with CP and no AKP (n=12). Patellofemoral extension discriminated AKP in individuals with CP with 100% accuracy. CONCLUSIONS: In quantifying the 3-dimensional, in vivo knee joint kinematics during a volitional extension task, kinematic markers that discriminate AKP in individuals with CP were identified. This provides an ability to predict which individuals with CP are most likely to develop AKP and could enable aggressive conservative treatment, aimed at reducing patella alta and excessive patellofemoral extension, to be prescribed before considering surgical options. The current findings will likely lead to improved clinical diagnostics and interventions for individuals with CP, with the ultimate goal of helping maintain, if not improve, functional mobility throughout the lifespan.

Functional and Structural Brain Connectivity in Children With Bilateral Cerebral Palsy Compared to Age-Related Controls and in Response to Intensive Rapid-Reciprocal Leg Training.

Background: Compared to unilateral cerebral palsy (CP), less is known about brain reorganization and plasticity in bilateral CP especially in relation or response to motor training. The few trials that reported brain imaging results alongside functional outcomes include a handful of studies in unilateral CP, and one pilot trial of three children with bilateral CP. This study is the first locomotor training randomized controlled trial (RCT) in bilateral CP to our knowledge reporting brain imaging outcomes. Methods: Objective was to compare MRI brain volumes, resting state connectivity and white matter integrity using DTI in children with bilateral CP with PVL and preterm birth history (<34 weeks), to age-related controls, and from an RCT of intensive 12 week rapid-reciprocal locomotor training using an elliptical or motor-assisted cycle. We hypothesized that connectivity in CP compared to controls would be greater across sensorimotor-related brain regions and that functional (resting state) and structural (fractional anisotropy) connectivity would improve post intervention. We further anticipated that baseline and post-intervention imaging and functional measures would correlate. Results: Images were acquired with a 3T MRI scanner for 16/27 children with CP in the trial, and 18 controls. No conclusive evidence of training-induced neuroplastic effects were seen. However, analysis of shared variance revealed that greater increases in precentral gyrus connectivity with the thalamus and pons may be associated with larger improvements in the trained device speed. Exploratory analyses also revealed interesting potential relationships between brain integrity and multiple functional outcomes in CP, with functional connectivity between the motor cortex and midbrain showing the strongest potential relationship with mobility. Decreased posterior white matter, corpus callosum and thalamic volumes, and FA in the posterior thalamic radiation were the most prominent group differences with corticospinal tract differences notably not found. Conclusions: Results reinforce the involvement of sensory-related brain areas in bilateral CP. Given the wide individual variability in imaging results and clinical responses to training, a greater focus on neural and other mechanisms related to better or worse outcomes is recommended to enhance rehabilitation results on a patient vs. group level.

A scoping review of methods used in musculoskeletal soft tissue and nerve shear wave elastography studies.

This scoping review of shear wave elastography (SWE) articles in musculoskeletal soft tissue and nerve research demonstrates methodological heterogeneity resulting from a lack of standardized data collection and reporting requirements. Seven literature databases were searched for original articles published in English from 2004-2020 that examine human skeletal muscles, tendons, and nerves in vivo. Although 5,868 records were initially identified, only 375 reports met inclusion criteria. Of the 375 articles, 260 examined 89 unique muscles, 94 examined 14 unique tendons, and 43 examined 8 unique nerves. Cohorts were often small (n = 11-20) and young (mean = 20-29 years), and participants were typically tested in the prone position. Regarding equipment, a variety of ultrasound systems (n = 11), ultrasound models (n = 18), and transducers (n = 19) were identified. Only 11% of articles contained information on the use of electromyography to confirm absence of muscle activity, and only 8% reported measurement depth. Since musculoskeletal soft tissue and nerve stiffness can vary significantly based on data collection methods, it is essential to standardize SWE collection and reporting procedures. This will allow SWE to serve as a valid and reproducible tool for assessing tissue pathology, disease progression, and response to intervention within a variety of musculoskeletal and nerve-related disorders.

Tibialis anterior architecture, strength, and gait in individuals with cerebral palsy.

INTRODUCTION: The relationship of tibialis anterior (TA) muscle architecture, including muscle thickness (MT), cross-sectional area (CSA), pennation angle (PA), and fascicle length (FL), to strength and ankle function was examined in ambulatory individuals with CP and unilateral foot drop. METHODS: Twenty individuals with CP participated in muscle ultrasound imaging, unilateral strength testing, and three-dimensional gait analysis. RESULTS: Muscle size (MT and CSA) was positively related to strength, fast gait velocity, and ankle kinematics during walking. Higher PA was related to a more dorsiflexed ankle position at initial contact and inversely with fast gait velocity. FL was related to strength, fast velocity, and step length at a self-selected speed. CONCLUSIONS: Muscle architecture partially explains the degree of impairment in strength and ankle function in CP. Treatments to increase TA size and strength may produce some gait improvement, but other factors that may contribute to ankle performance deficits must be considered.

Efficacy and safety of onabotulinumtoxinA with standardized occupational therapy for treatment of pediatric upper limb spasticity: Phase III placebo-controlled randomized trial.

BACKGROUND: This is the first large study of onabotulinumtoxinA as treatment for pediatric upper limb spasticity. OBJECTIVE: Evaluate efficacy and safety of a single treatment with onabotulinumtoxinA plus occupational therapy (OT). METHODS: In this registrational phase III, multinational study (NCT01603602), participants were randomized 1:1:1 to onabotulinumtoxinA 3 U/kg/OT, 6 U/kg/OT, or placebo/OT. Primary endpoint was average change from baseline at weeks 4 and 6 in Modified Ashworth Scale-Bohannon (MAS) score. Secondary endpoints included Modified Tardieu Scale (MTS), Clinical Global Impression of Change (CGI) and functional Goal Attainment Scale (GAS). RESULTS: 235 participants were randomized. At weeks 4 and 6, onabotulinumtoxinA groups had greater mean reductions in MAS (both -1.9; p < 0.001) versus placebo (-1.2). OnabotulinumtoxinA doses improved dynamic tone per MTS. Mean CGI at weeks 4 and 6 was unchanged in the overall population, but improved in a post hoc analysis of patients with a single affected upper limb (UL) muscle group (elbow or wrist). GAS score for passive goals was significantly higher for 6 U/kg versus placebo at week 12. Most AEs were mild/moderate in severity; overall incidence was similar between groups. CONCLUSIONS: OnabotulinumtoxinA (3 and 6 U/kg) was safe and effective in reducing upper limb spasticity in pediatric participants.

Adolescents and adults with patellofemoral pain exhibit distinct patellar maltracking patterns.

BACKGROUND: Chronic idiopathic patellofemoral pain is associated with patellar maltracking in both adolescents and adults. To accurately target the underlying, patient-specific etiology, it is crucial we understand if age-of-pain-onset influences maltracking. METHODS: Twenty adolescents (13.9 ± 1.4 years) and 20 adults (28.1 ± 4.9 years) female patients with idiopathic patellofemoral pain (age-of-pain-onset: < 14 and > 18 years of age, respectively) formed the patient cohort. Twenty adolescents and 20 adults (matched for gender, age, and body mass index) formed the control cohort. We captured three-dimensional patellofemoral kinematics during knee flexion-extension using dynamic MRI. Patellar maltracking (deviation in patient-specific patellofemoral kinematics, relative to their respective age-controlled mean values) was the primary outcome measure, which was compared between individuals with adolescent-onset and adult-onset patellofemoral pain using ANOVA and discriminant analysis. FINDINGS: The female adolescent-onset patellofemoral pain cohort demonstrated increased lateral (P = 0.032), superior (P = 0.007), and posterior (P < 0.001) maltracking, with increased patellar flexion (P < 0.001) and medial spin (P = 0.002), relative to the adult-onset patellofemoral pain cohort. Post-hoc analyses revealed increased lateral shift [mean difference ± 95% confidence interval = -2.9 ± 2.1 mm at 10° knee angle], posterior shift [-2.8 ± 2.1 mm, -3.3 ± 2.3 mm & -3.1 ± 2.4 mm at 10°, 20°& 30°], with greater patellar flexion [3.8 ± 2.6 mm & 5.0 ± 2.8 mm, at 20°& 30°] and medial spin [-2.2 ± 1.7 mm & -3.4 ± 2.3 mm at 20°& 30°]. Axial-plane maltracking accurately differentiated the patient age-of-pain-onset (60-75%, P < 0.001). INTERPRETATION: Age-of-pain-onset influences the maltracking patterns seen in patients with patellofemoral pain; with all, but 1, degree of freedom being unique in the adolescent-onset-patellofemoral pain cohort. Clinical awareness of this distinction is crucial for correctly diagnosing a patient's pain etiology and optimizing interventional strategies.

Q-angle and J-sign: indicative of maltracking subgroups in patellofemoral pain.

Mechanical factors related to patellofemoral pain syndrome and maltracking are poorly understood. Clinically, the Q-angle, J-sign, and lateral hypermobility commonly are used to evaluate patellar maltracking. However, these measures have yet to be correlated to specific three-dimensional patellofemoral displacements and rotations. Thus, we tested the hypotheses that increased Q-angle, lateral hypermobility, and J-sign correlate with three-dimensional patellofemoral displacements and rotations. We also determined whether multiple maltracking patterns can be discriminated, based on patellofemoral displacements and rotations. Three-dimensional patellofemoral motion data were acquired during active extension-flexion using dynamic MRI in 30 knees diagnosed with patellofemoral pain and at least one clinical sign of patellar maltracking (Q-angle, lateral hypermobility, or J-sign) and in 37 asymptomatic knees. Although the Q-angle is assumed to indicate lateral patellar subluxation, our data supported a correlation between the Q-angle and medial, not lateral, patellar displacement. We identified two distinct maltracking groups based on patellofemoral lateral-medial displacement, but the same groups could not be discriminated based on standard clinical measures (eg, Q-angle, lateral hypermobility, and J-sign). A more precise definition of abnormal three-dimensional patellofemoral motion, including identifying subgroups in the patellofemoral pain population, may allow more targeted and effective treatments.

Functional near-infrared spectroscopy to assess sensorimotor cortical activity during hand squeezing and ankle dorsiflexion in individuals with and without bilateral and unilateral cerebral palsy.

Significance: Our study is the first comparison of brain activation patterns during motor tasks across unilateral cerebral palsy (UCP), bilateral cerebral palsy (BCP), and typical development (TD) to elucidate neural mechanisms and inform rehabilitation strategies. Aim: Cortical activation patterns were compared for distal upper and lower extremity tasks in UCP, BCP, and TD using functional near-infrared spectroscopy (fNIRS) and related to functional severity. Approach: Individuals with UCP ( n = 10 , 18.8 ± 6.8 years ), BCP ( n = 14 , 17.5 ± 9.6 years ), and TD ( n = 16 , 17.3 ± 9.1 years ) participated in this cross-sectional cohort study. The fNIRS was used to noninvasively monitor the hemodynamic response to task-related cortical activation. The block design involved repetitive nondominant hand squeezing and ankle dorsiflexion. Results: Individuals with UCP demonstrated the highest levels of activation for the squeeze task ( UCP > BCP q = 0.049 ; BCP > TD q < 0.001 ; and UCP > TD q = 0.001 ) and more activity in the ipsilateral versus contralateral hemisphere. Individuals with BCP showed the highest levels of cortical activation in the dorsiflexion task ( BCP > UCP q < 0.001 ; BCP > TD ). Conclusions: Grouping by CP subtype and manual function or mobility level demonstrated significant differences from TD, even for individuals with the mildest forms of CP. Hemispheric activation patterns showed hypothesized but nonsignificant trends.

Management of Anterocapitis and Anterocollis: A Novel Ultrasound Guided Approach Combined with Electromyography for Botulinum Toxin Injection of Longus Colli and Longus Capitis.

Chemodenervation of cervical musculature using botulinum neurotoxin (BoNT) is established as the gold standard or treatment of choice for management of Cervical Dystonia (CD). The success of BoNT procedures is measured by improved symptomology while minimizing side effects and is dependent upon many factors including: clinical pattern recognition, identifying contributory muscles, BoNT dosage, and locating and safely injecting target muscles. In patients with CD, treatment of anterocollis (forward flexion of the neck) and anterocaput (anterocapitis) (forward flexion of the head) are inarguably challenging. The longus Colli (LoCol) and longus capitis (LoCap) muscles, two deep cervical spine and head flexor muscles, frequently contribute to these patterns. Localizing and safely injecting these muscles is particularly challenging owing to their deep location and the complex regional anatomy which includes critical neurovascular and other structures. Ultrasound (US) guidance provides direct visualization of the LoCol, LoCap, other cervical muscles and adjacent structures reducing the risks and side effects while improving the clinical outcome of BoNT for these conditions. The addition of electromyography (EMG) provides confirmation of muscle activity within the target muscle. Within this manuscript, we present a technical description of a novel US guided approach (combined with EMG) for BoNT injection into the LoCol and LoCap muscles for the management of anterocollis and anterocaput in patients with CD.

Human body shape index based on an experimentally derived model of human growth.

OBJECTIVES: To test the assumption of geometrically similar growth by developing experimentally derived models of human body growth during the age interval of 5 to 18 years; to use these derived growth models to establish a new human body shape index (HBSI) based on natural age-related changes in human body shape (HBS); and to compare various metrics of relative body weight (body mass index [BMI], ponderal index [PI], and HBSI) in a sample of 5- to 18-year-old children. STUDY DESIGN: Nondisabled Polish children (n = 847) participated in this descriptive study. To model growth, the best fit between body height (H) and body mass (M) was calculated for each sex using the allometric equation M = m(i) H(chi). HBSI was calculated separately for girls and boys, using sex-specific values for chi and a general HBSI from combined data. The customary BMI and PI were calculated and compared with HBSI values. RESULTS: The models of growth were M = 13.11H(2.84) (R2 = 0.90) for girls and M = 13.64H(2.68) (R2 = 0.91) for boys. HBSI values contained less inherent variability and were less influenced by growth (age and height) compared with BMI and PI. CONCLUSIONS: Age-related growth during childhood is sex-specific and not geometrically similar. Therefore, indices of HBS formulated from experimentally derived models of human growth are superior to customary geometric similarity-based indices for characterizing HBS in children during the formative growth years.

Reply to Comment on Ultrasound Guidance for Botulinum Neurotoxin Chemodenervation Procedures. Toxins 2018, 10, 18-Quintessential Use of Ultrasound Guidance for Botulinum Toxin Injections.

We thank the authors for their detailed letter and salient comments related to our article on Ultrasound Guidance for botulinum toxin (BoNT) injections.[...].