Botox in below knee amputation for the management of post-operative contracture: a systematic review.

During the 1970s, scientists first used botulinum toxin to treat strabismus. While testing on monkeys, they noticed that the toxin could also reduce wrinkles in the glabella area. This led to its widespread use in both medical and cosmetic fields. The objective of the study was to evaluate the potential use of Botox in managing post-operative contracture after below-knee amputation. We conducted a systematic review In Pubmed, Cochrane Library, Embase, and Google Scholar using the MESH terms Botox, botulinum toxin, post-operative contracture, amputation, and below knee amputation. Our goal was to evaluate the potential use of Botox to manage post-operative contracture in patients who have undergone below-knee amputation. Our findings show evidence in the literature that Botox can effectively manage stump hyperhidrosis, phantom pain, and jumping stump, but no clinical trial has been found that discusses the use of Botox for post-operative contracture. Botox has been used in different ways to manage spasticity. Further studies and clinical trials are needed to support the use of Botox to manage this complication.

The effect of upper limb loading with external weights on gait and trunk control in ambulatory children with spastic hemiplegic cerebral palsy: a randomized controlled trial.

OBJECTIVE: Children with hemiplegic cerebral palsy (CP) are typically ambulant with high motor functioning levels but with gait asymmetry and a greater risk of instability and falling. Physiotherapy is considered the core part of CP rehabilitation due to the risk of neurosurgery and the side effects of drug interventions. Although growing evidence has looked at the effect of upper limb loading during walking in many populations, such interventions in children with CP remain unexplored yet. The purpose of this study was to investigate if loading the upper limbs with external weights could improve walking speed, trunk control, and balance in ambulatory children with hemiplegic CP. PATIENTS AND METHODS: The following outcome measures were recorded at baseline and six weeks after the intervention: gait speed [10-Meter Walk Test (10 MWT)], trunk control [Trunk Control Measurement Scale (TCMS)], and balance [Pediatric Balance Scale (PBS)]. Multiple 2 (groups) x 2 (time-points) mixed analysis of variance models (ANOVAs) were used for analysis. RESULTS: Both groups showed a significant improvement (p < 0.001) in 10 MWT, TCMS, and PBS scores post-intervention. However, the magnitude of change in the outcome measures was higher in the intervention group (10 MWT = 0.59 m/s, TCMS = 10.41, PBS = 9.35) compared to the control group (10 MWT = 0.37 m/s, TCMS = 6.43, PBS = 4.68). CONCLUSIONS: This study demonstrated that although both control and intervention groups showed improvements in terms of gait speed, trunk control, and balance, the intervention group that had upper limb loading showed higher significant improvements.  Clinicaltrial.gov ID: NCT05444387.

RARS1-related hypomyelinating leukodystrophy-9 (HLD-9) in two distinct Iranian families: Case report and literature review.

BACKGROUND: Hypomyelinating leukodystrophy-9 (HLD-9) is caused by biallelic pathogenic variants in RARS1, which codes for the cytoplasmic tRNA synthetase for arginine (ArgRS). This study aims to evaluate the clinical, neuroradiological, and genetic characteristics of patients with RARS1-related disease and determine probable genotype-phenotype relationships. METHODS: We identified three patients with RARS1 homozygous pathogenic variants. Furthermore, we performed a comprehensive review of the literature. RESULTS: Homozygous variants of RARS1 (c.2T>C (p.Met1Thr)) were identified in three patients with HLD-9. Clinical symptoms were severe in all patients. Following the literature review, thirty HLD-9 cases from eight studies were found. The 33 patients' main symptoms were hypomyelination, language delay, and intellectual disability or developmental delay. The mean age of onset for HLD9 in the group of 33 patients with a known age of onset was 5.8 months (SD = 8.1). The interquartile range of age of onset was 0-10 months. Of the 25 variants identified, c.5A>G (p.Asp2Gly) was identified in 11 patients. CONCLUSION: Pathogenic variants in RARS1 decrease ArgRS activity and cause a wide range of symptoms, from severe, early onset epileptic encephalopathy with brain atrophy to a mild condition with relatively maintained myelination. These symptoms include the classic hypomyelination presentation with nystagmus and spasticity. Furthermore, the pathogenicity of the variation c.2T>C (p.Met1Thr) has been shown.

A portable system to measure knee extensor spasticity after spinal cord injury.

BACKGROUND: The pendulum test is a quantitative method used to assess knee extensor spasticity in humans with spinal cord injury (SCI). Yet, the clinical implementation of this method remains limited. The goal of our study was to develop an objective and portable system to assess knee extensor spasticity during the pendulum test using inertial measurement units (IMU). METHODS: Spasticity was quantified by measuring the first swing angle (FSA) using a 3-dimensional optical tracking system (with external markers over the iliotibial band, lateral knee epicondyle, and lateral malleolus) and two wireless IMUs (positioned over the iliotibial band and mid-part of the lower leg) as well as a clinical exam (Modified Ashworth Scale, MAS). RESULTS: Measurements were taken on separate days to assess test-retest reliability and device agreement in humans with and without SCI. We found no differences between FSA values obtained with the optical tracking system and the IMU-based system in control subjects and individuals with SCI. FSA values from the IMU-based system showed excellent agreement with the optical tracking system in individuals with SCI (ICC > 0.98) and good agreement in controls (ICC > 0.82), excellent test-retest reliability across days in SCI (ICC = 0.93) and good in controls (ICC = 0.87). Notably, FSA values measured by both systems showed a strong association with MAS scores ( ρ  ~ -0.8) being decreased in individuals with SCI with higher MAS scores, reflecting the presence of spasticity. CONCLUSIONS: These findings suggest that our new portable IMU-based system provides a robust and flexible alternative to a camera-based optical tracking system to quantify knee extensor spasticity following SCI.

Reconsidering the Gold Standard for the Assessment of Spastic Equinus Foot.

INTRODUCTION: To determine whether differences exist in the measured range of motion (ROM) by the goniometer, equinometer, and 3-dimensional (3D) gait analysis in different settings (under general anesthesia (GA) or in the clinic) among patients with cerebral palsy. METHODS: A prospective comparative cohort study was conducted at our orthopaedics center, where 15 patients with diplegic cerebral palsy (30 limbs) were evaluated, all of whom had Gross Motor Function Classification System (GMFCS) level I and II. ROM was measured by (1) goniometer under GA (benchmark), (2) goniometer analysis during clinical examination, (3) equinometer under GA, (4) equinometer in the clinic, and (5) 3D gait analysis. ROM was measured during both knee flexion and extension. Subgroup analysis based on the GMFCS level was performed. RESULTS: Nine patients were male with GMFCS level I and a mean age of 14.3 (SD=7.2) years. Statistically significant differences were noted between all studied measurement techniques in terms of ROM, dorsiflexion, and plantarflexion during both knee flexion and extension. The GMFCS level was an effect modifier of ROM measurements. DISCUSSION: The ROM parameters during both knee flexion and extension differed from one measurement device/technique to another. The GMFCS level played a notable effect-modifying role on the ROM parameters.

IMPROVE study protocol, investigating post-stroke local muscle vibrations to promote cerebral plasticity and functional recovery: a single-blind randomised controlled trial.

INTRODUCTION: Spasticity is a frequent disabling consequence following a stroke. Local muscle vibrations (LMVs) have been proposed as a treatment to address this problem. However, little is known about their clinical and neurophysiological impacts when used repeatedly during the subacute phase post-stroke. This project aims to evaluate the effects of a 6-week LMV protocol on the paretic limb on spasticity development in a post-stroke subacute population. METHODS AND ANALYSIS: This is an interventional, controlled, randomised, single-blind (patient) trial. 100 participants over 18 years old will be recruited, within 6 weeks following a first stroke with hemiparesis or hemiplegia. All participants will receive a conventional rehabilitation programme, plus 18 sessions of LMV (ie, continuously for 30 min) on relaxed wrist and elbow flexors: either (1) at 80 Hz for the interventional group or (2) at 40 Hz plus a foam band between the skin and the device for the control group.Participants will be evaluated at baseline, at 3 weeks and 6 weeks, and at 6 months after the end of the intervention. Spasticity will be measured by the modified Ashworth scale and with an isokinetic dynamometer. Sensorimotor function will be assessed with the Fugl-Meyer assessment of the upper extremity. Corticospinal and spinal excitabilities will be measured each time. ETHICS AND DISSEMINATION: This study was recorded in a clinical trial and obtained approval from the institutional review board (Comité de protection des personnes Ile de France IV, 2021-A03219-32). All participants will be required to provide informed consent. The results of this trial will be published in peer-reviewed journals to disseminate information to clinicians and impact their practice for an improved patient's care. TRIAL REGISTRATION NUMBER: Clinical Trial: NCT05315726 DATASET: EUDRAct.

Effects of dry needling on spasticity, cortical excitability, and range of motion in a patient with multiple sclerosis: a case report.

BACKGROUND: Dry needling is an intervention used by physiotherapists to manage muscle spasticity. We report the effects of three sessions of dry needling on ankle plantar flexor muscle spasticity and cortical excitability in a patient with multiple sclerosis. CASE PRESENTATION: The patient was a 40-year-old Iranian woman with an 11-year history of multiple sclerosis. The study outcomes were measured by the modified modified Ashworth scale, transcranial magnetic stimulation parameters, and active and passive ankle range of motion. They were assessed before (T0), after three sessions of dry needling (T1), and at 2-week follow-up (T2). Our result showed: the modified modified Ashworth scale was improved at T2 from, 2 to 1. The resting motor threshold decreased from 63 to 61 and 57 at T1 and T2, respectively. The single test motor evokes potential increased from 76.2 to 78.3. The short intracortical inhibition increased from 23.6 to 35.4 at T2. The intracortical facilitation increased from 52 to 76 at T2. The ankle active and passive dorsiflexion ROM increased ~ 10° and ~ 6° at T2, respectively. CONCLUSION: This case study presented a patient with multiple sclerosis who underwent dry needling of ankle plantar flexors with severe spasticity, and highlighted the successful use of dry needling in the management of spasticity, ankle dorsiflexion, and cortical excitability. Further rigorous investigations are warranted, employing randomized controlled trials with a sufficient sample of patients with multiple sclerosis. Trial registration IRCT20230206057343N1, registered 9 February 2023, https://en.irct.ir/trial/68454.

The state of the art in therapeutic administration of botulinum toxin in children with cerebral palsy: an integrative review.

OBJECTIVE: To describe the current state of the art in the therapeutic administration of botulinum toxin with indications, efficacy, and safety profile for children and adolescents with cerebral palsy. DATA SOURCE: An integrative review was conducted. The MEDLINE/PubMed database was searched twice within the last decade using distinct terms, and only studies written in the English language were included. The study population was limited to those aged 0-18 years. Articles that were duplicates or lacked sufficient methodology information were excluded. DATA SYNTHESIS: We found 256 articles, of which 105 were included. Among the included studies, most were conducted in developed countries. Botulinum toxin demonstrated good safety and efficacy in reducing spasticity, particularly when administered by a multidisciplinary rehabilitation team. It is primarily utilized to improve gait and upper limb function, facilitate hygiene care, reduce pain, prevent musculoskeletal deformities, and even decrease sialorrhea in patients without a functional prognosis for walking. CONCLUSIONS: The administration of botulinum toxin is safe and efficacious, especially when combined with a multi-professional rehabilitation team approach, which increases the probability of functional improvement. It can also be beneficial for patients with significant functional impairments to help with daily care tasks, such as hygiene, dressing, and reducing sialorrhea. Pediatricians must be familiar with this treatment and its indications to attend to and refer patients promptly when necessary, and to exploit their neuroplasticity. Further research on this topic is required in developing countries.

Real-world differences in dosing and clinical utilization of OnabotulinumtoxinA and AbobotulinumtoxinA in the treatment of upper limb spasticity.

According to prescribing information, potency units are not interchangeable between botulinum toxin A products. This exploratory study compared real-world dosing and utilization of onabotulinumtoxinA and abobotulinumtoxinA in adults with upper limb spasticity. In this retrospective study, 101 clinicians provided chart data via online surveys for 215 US post-stroke patients treated for upper limb spasticity with ≥3 onabotulinumtoxinA or abobotulinumtoxinA doses (phase 1: 9/18/2020-12/10/2020; phase 2: 9/30/2021-12/7/2021). Most participating clinicians were physicians (70.3%) specializing in neurology (71.3%) or physiatry (20.8%). In the onabotulinumtoxinA (n = 107) and abobotulinumtoxinA (n = 108) groups, ∼75% of patients had moderate-to-severe spasticity. A range of onabotulinumtoxinA:abobotulinumtoxinA dose ratios (1:2.2 [95% CI: 1.8, 2.6] to 1:4.1 [95% CI: 3.0, 6.0]) was observed across muscles. For the most recent dose, mean number of muscles injected was greater for onabotulinumtoxinA (4.3) versus abobotulinumtoxinA (3.1; P = 0.0003). For onabotulinumtoxinA versus abobotulinumtoxinA, the proportion of injections was 81.3% versus 63.9% (P = 0.0067) in forearm muscles and 23.4% versus 3.7% (P = 0.0001) in hand muscles. Mean injection intervals were similar (onabotulinumtoxinA: 102.0 days; abobotulinumtoxinA: 99.1 days). Differences in real-world dosing and utilization of onabotulinumtoxinA and abobotulinumtoxinA for upper limb spasticity were observed. There was no standard dose-conversion ratio, consistent with each product's prescribing information.

[Analysis of 9 patients with adolescence-onset methylenetetrahydrofolate reductase deficiency].

Objective: To explore the diagnosis and treatment of adolescence-onset methylenetetrahydrofolate reductase (MTHFR) deficiency. Methods: This was a retrospective case study. Nine patients with adolescence-onset MTHFR deficiency were diagnosed at Peking University First Hospital from January 2016 to December 2022, and followed up for more than 1 year. Their general information, clinical manifestations, laboratory tests, cranial images, MTHFR gene variants, diagnosis, treatment, and outcome were analyzed retrospectively. Results: The 9 patients came from 8 families. They had symptoms at age of 8.0 years to 17.0 years and diagnosed at 9.0 years to 17.5 years. Eight were male and 1 was female. Two patients were brothers, the elder brother developed abnormal gait at 17.0 years; and the younger brother was then diagnosed at 15.0 years of age and treated at the asymptomatic stage, who was 18.0 years old with normal condition during this study. The main manifestations of the 8 symptomatic patients included progressive dyskinesia and spastic paralysis of the lower limbs, with or without intellectual decline, cognitive impairment and behavioral abnormalities. Totally, 15 variants of MTHFR gene were identified in the 9 patients, including 8 novel variants. Five patients had brain image abnormalities. Increased plasma total homocysteine level (65-221 µmol/L) was found in all patients, and decreased to 20-70 µmol/L after treatment with betaine and calcium folinate. Besides, the 8 symptomatic patients had their behavior and cognitive problems significantly improved, with a legacy of lower limb motor disorders. Conclusions: Late-onset MTHFR deficiency can occur in adolescence. The diagnosis is usually delayed because of non-specific clinical symptoms. The test of blood total homocysteine could be used as a selective screening test. Eight novel varients of MTHFR gene were identified. Timely treatment can improve clinical condition significantly, and pre-symptomatic treatment may prevent brain damage.

Cervical dorsal rhizotomy for upper limbs spasticity. Case report.

Spasticity is a prevalent symptom of upper motor neuron syndrome, becoming debilitating when hindering voluntary movement and motor function and causing contractures and pain. Functional neurosurgery plays a crucial role in treating severe spasticity. Despite extensive literature on SDR for lower limb spasticity, there is a scarcity of papers regarding the procedure in the cervical region to alleviate upper limb spasticity. This case report details a cervical dorsal rhizotomy (CDR) performed for upper limb spasticity, resulting in significant improvement in spasticity with sustained outcomes and low complication rates. Neuroablative procedures like CDR become an option to treat spasticity.

Current perspectives on neuromodulation in ALS patients: A systematic review and meta-analysis.

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons, resulting in muscle weakness, paralysis, and eventually patient mortality. In recent years, neuromodulation techniques have emerged as promising potential therapeutic approaches to slow disease progression and improve the quality of life of ALS patients. A systematic review was conducted until August 8, 2023, to evaluate the neuromodulation methods used and their potential in the treatment of ALS. The search strategy was applied in the Cochrane Central database, incorporating results from other databases such as PubMed, Embase, CTgov, CINAHL, and ICTRP. Following the exclusion of papers that did not fulfil the inclusion criteria, a total of 2090 records were found, leaving a total of 10 studies. R software was used to conduct meta-analyses based on the effect sizes between the experimental and control groups. This revealed differences in muscle stretch measures with manual muscle testing (p = 0.012) and resting motor threshold (p = 0.0457), but not with voluntary isometric contraction (p = 0.1883). The functionality of ALS was also different (p = 0.007), but not the quality of life. Although intracortical facilitation was not seen in motor cortex 1 (M1) (p = 0.1338), short-interval intracortical inhibition of M1 was significant (p = 0.0001). BDNF showed no differences that were statistically significant (p = 0.2297). Neuromodulation-based treatments are proposed as a promising therapeutic approach for ALS that can produce effects on muscle function, spasticity, and intracortical connections through electrical, magnetic, and photonic stimulation. Photobiomodulation stands out as an innovative approach that uses specific wavelengths to influence mitochondria, with the aim of improving mitochondrial function and reducing excitotoxicity. The lack of reliable placebo controls and the variation in stimulation frequency are some of the drawbacks of neuromodulation.

Utilizing combination intrathecal baclofen and analgesic medication to manage spasticity and pain in patients with pediatric-onset disability: Case series.

PURPOSE: The use of intrathecal medications for the management of spasticity and various pain syndromes in the adult population has been previously reported. However, no evidence-based guidelines currently exist in the pediatric population. This case series presents patients (n = 8) with pediatric-onset disability who underwent placement of intrathecal baclofen pumps initially for management of severe spasticity. Despite titration of dose and use of oral analgesia, their uncontrolled pain persisted. Each patient was transitioned to a combination of baclofen and analgesic intrathecal therapy. The outcome in pain improvement and quality of life, as reported by patients and/or caregivers, were retrospectively reviewed. METHODS: Retrospective review of the background and decision-making process regarding transition to combination intrathecal therapy identified patient selection characteristics. Each patient and/or their caregivers completed a survey regarding improvements in pain, spasticity, function, and quality of life following initiation of combination intrathecal medications. RESULTS: Survey results revealed improvements in functional and pain assessments after initiation of combination baclofen and analgesic intrathecal medication. Patients and caregivers reported decreases in pain and oral spasticity medications. CONCLUSION: Use of pumps with antispasmodic and analgesic medication for combination intrathecal medication delivery should be considered in the management of patients with childhood-onset disabilities who have both severe spasticity and pain.

Pediatric Spastic Wrist Contractures Can Be Well Managed With Wrist Arthrodesis.

BACKGROUND: Severe spastic wrist contractures secondary to cerebral palsy (CP) or alike can have significant implications for patient hand function, hygiene, skin breakdown, and cosmesis. When these contractures become rigid, soft tissue procedures alone are unable to obtain or maintain the desired correction. In these patients' wrist arthrodesis is an option-enabling the hand to be stabilized in a more functional position for hygiene, dressing, and general cosmesis, though are patients satisfied? METHODS: All children who had undergone a wrist arthrodesis for the management of a severe wrist contracture at Shriners Hospital, Portland between January 2016 and January 2021 were identified (n=23). A chart review was undertaken to obtain data-demographic, operative, clinical, and radiographic. All patients were then contacted to participate in 2 patient-reported outcome questionnaires (74% response agreement), a numerical rating scale (NRS), based on the visual analog scale (VAS) and the disability analog scale (DAS). Results were assessed with the aid of descriptive statistics, means and percentages with the primary focus of determining overall patient satisfaction with the procedure. RESULTS: Twenty-three patients were included in the review, and 74% took part in the prospective survey. Included were 10 patients with hemiplegia, 4 with triplegia, 7 with quadriplegia, 1 with a diagnosis of Rhett syndrome, and 1 with a history of traumatic brain injury. All patients achieved radiologic union by a mean of 8 weeks, and 87% obtained a neutral postoperative wrist alignment. The NRS showed 88% of patients were highly satisfied with their results; specifically, 82% had an improved appearance, 53% improved function, 71% improved daily cares, and 65% improved hygiene. The postoperative DAS score averaged 4.7 of 12 indicating mild disability. When looking at how a patient's diagnostic subtype affected outcome results, patients with triplegia reported less improvement and greater disability on the NRS and DAS, averaging 9.5 (severe disability on the DAS). The GMFCS classification had less correlation with outcomes. CONCLUSION: Wrist arthrodesis is a good option for the management of pediatric spastic wrist contractures, with limited complications and an overall high patient/carer satisfaction rate. LEVEL OF EVIDENCE: Level IV-case series.

Safety of dry needling in stroke patients: a scoping review.

INTRODUCTION: Spasticity is a common problem in stroke patients. Treatments of spasticity often have side effects or are insufficiently effective. Dry needling (DN) has been proposed as a potential additional option to consider in the multimodal treatment of post-stroke spasticity, although questions about its safety remain. The goal of this study is to assess the safety of DN in stroke patients. EVIDENCE ACQUISITION: A systematic search in Medline, Embase, The Cochrane Library, Web of Science, CIHNAL and PEDro was conducted in June 2023. Two reviewers independently screened abstracts according to the eligibility criteria. EVIDENCE SYNTHESIS: Twenty-five articles were included in this review. Only six studies reported adverse events, all of which were considered minor. None of the included studies reported any serious adverse events. In four of the included studies anticoagulants were regarded as contra-indicative for DN. Anticoagulants were not mentioned in the other included studies. CONCLUSIONS: There is a paucity of literature concerning the safety of DN in stroke patients. This review is the first to investigate the safety of DN in stroke patients and based on the results there is insufficient evidence regarding the safety of DN in stroke patients. CLINICAL REHABILITATION IMPACT: Although DN could be a promising treatment in post-stroke spasticity, further research is indicated to investigate its mechanism of action and its effect on outcome. However, before conducting large clinical trials to assess outcome parameters, the safety of DN in stroke patients must be further investigated.

Identification of Neural and Non-Neural Origins of Joint Hyper-Resistance Based on a Novel Neuromechanical Model.

Joint hyper-resistance is a common symptom in neurological disorders. It has both neural and non-neural origins, but it has been challenging to distinguish different origins based on clinical tests alone. Combining instrumented tests with parameter identification based on a neuromechanical model may allow us to dissociate the different origins of joint hyper-resistance in individual patients. However, this requires that the model captures the underlying mechanisms. Here, we propose a neuromechanical model that, in contrast to previously proposed models, accounts for muscle short-range stiffness (SRS) and its interaction with muscle tone and reflex activity. We collected knee angle trajectories during the pendulum test in 15 children with cerebral palsy (CP) and 5 typically developing children. We did the test in two conditions - hold and pre-movement - that have been shown to alter knee movement. We modeled the lower leg as an inverted pendulum actuated by two antagonistic Hill-type muscles extended with SRS. Reflex activity was modeled as delayed, linear feedback from muscle force. We estimated neural and non-neural parameters by optimizing the fit between simulated and measured knee angle trajectories during the hold condition. The model could fit a wide range of knee angle trajectories in the hold condition. The model with personalized parameters predicted the effect of pre-movement demonstrating that the model captured the underlying mechanism and subject-specific deficits. Our model may help with the identification of neural and non-neural origins of joint hyper-resistance and thereby opens perspectives for improved diagnosis and treatment selection in children with spastic CP, but such applications require further studies to establish the method's reliability.

Influence of musculoskeletal pain during gait on kinematics and selective motor control in individuals with spastic cerebral palsy: A pilot study.

BACKGROUND: Individuals with cerebral-palsy commonly present with altered kinematics and selective-motor-control during gait, and may also experience musculoskeletal pain. This pilot study aims to investigate if the immediate experience of musculoskeletal pain during gait influences kinematics and selective-motor-control in individuals with spastic cerebral-palsy. METHODS: Retrospective treadmill-based gait-analysis data for 145 individuals with spastic cerebral-palsy were screened. Participants were asked about experiencing lower-extremity musculoskeletal pain immediately during gait, with 26 individuals (18%) reporting this was the case (pain-group; mean 11.55 ± 3.15 years, Gross-Motor-Function-Classification-System levels I/II/III n = 5/13/8, Uni/bilateral involvement n = 11/15). Of the 77 individuals who did not report any pain, a no-pain group (n = 26) was individually matched. Kinematics were evaluated using the Gait-Profile-Score and spatiotemporal parameters (dimensionless-walking-speed, single-leg-support percentage and step-time). Selective-motor-control was assessed using the Walking-Dynamic-Motor-Control index. FINDINGS: In the pain-group, 58% reported experiencing pain in their more-involved leg, 8% in the less-involved leg and 34% in both legs. Regarding the pain location, 38% of the pain-group reported experiencing pain in multiple locations. On a more specific level, 35%, 46% and 54% reported pain around the hip/thigh, knee/calf and ankle/ft, respectively. No significant differences were observed between the pain and no-pain groups for any of the outcome measures, in each leg or bilaterally. INTERPRETATION: No significant differences in kinematics and selective-motor-control during gait were found between individuals with spastic cerebral-palsy, with and without musculoskeletal pain. This suggests that the individuals in this study may not present with obvious antalgic gait patterns, which may relate to the pre-existing altered kinematics and selective-motor-control.

Effectiveness of simple and basic home-based exercise programs including pediatric massage executed by caregivers at their homes in the management of children with spastic cerebral palsy: A randomized controlled trial.

PURPOSE: This study aimed to assess the effectiveness of simple and basic home-based exercise programs (HEPs), including pediatric massage (PM), executed by caregivers at their homes in the management of children with spastic cerebral palsy (CP). METHODS: Sixty-eight children with spastic CP (diplegia) aged 4-12 years were randomly assigned to PM and HEP groups for a randomized controlled trial continuing from November 01, 2021 to June 2022. Parents provided home-based exercises to both groups, five times a week for 12 weeks. However, the PM group was additionally provided with PM. Modified Ashworth Scale (MAS), Gross Motor Function Measure (GMFM-88) and Gross Motor Function Classification System (GMFCS) were used for evaluation of spasticity and gross motor activity at baseline as well as after six and 12 weeks of intervention. Comparative analysis of data was carried out with SPSS-20. RESULTS: Mean age in HEP and PM groups was 6.65±2.12 and 7.09±2.22 years respectively. Data revealed homogeneity of both groups at the beginning of study. The PM group showed a statistically significant decrease in MAS scores after six and 12 weeks of intervention (p < 0.05) when compared with the HEP group, but similar changes did not happen in GMFM scores and GMFCS levels. However, comparative analysis revealed statistically significant change in GMFM scores and GMFCS levels (p < 0.05) when compared from baseline to 12 weeks of intervention in both groups. CONCLUSION: PM along with HEPs can be used effectively to reduce spasticity and to improve gross motor ability if performed for a period of at least six and 12 weeks respectively. In conjunction with HEPs, PM has better outcomes in the management of tone and movement disorders of spastic CP than HEPs alone.

An iPSC model for POLR3A-associated spastic ataxia: Generation of three unrelated patient cell lines.

Spastic Ataxias (SA) are a group of neurodegenerative disorders with combined pyramidal and cerebellar system affection, leading to an overlap phenotype between Hereditary Spastic Paraplegias (HSP) and Cerebellar Ataxias (CA). Here we describe the generation of iPSCs from three unrelated patients with an ultra-rare subtype of SA caused by compound heterozygous mutations in POLR3A, that encodes the largest subunit of RNA polymerase III. iPSCs were reprogrammed from normal human dermal fibroblasts (NHDFs) using episomal reprogramming with integration-free plasmid vectors: HIHRSi004-A, derived from a 44 year-old male carrying the mutations c.1909 + 22G > A/c.3944_3945delTG, HIHRSi005-A obtained from a 66 year-old male carrying the mutations c.1909 + 22G > A/c.1531C > T, and HIHRSi006-A from a 27 year-old male carrying the mutations c.1909 + 22G > A/c.2472_2472delC (ENST00000372371.8).

[Selective Neurectomy in Spastic Paralysis of the Upper Extremity]. / Die hyperselektive Neurektomie bei spastischen Lähmungen der oberen Extremität.

Selective neurectomy refers to the targeted transection of motor nerve fibres at their entry into the muscle in order to reduce the increased muscle tone in cases of spastic paralysis. This procedure has regained popularity in recent years, especially in the upper extremity. First and foremost, it requires an exact knowledge of the topographical anatomy of muscle innervation. To be able to control the extent and localisation of the denervation, the terminal nerve branches must be visualized precisely during the procedure. For a meaningful reduction of muscle tone, 2/3 to 4/5 of nerve fibres must be resected. This article presents the historical development, principles and operative details of this technique as well as clinical results.