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This study investigates the pathological toe and heel gaits seen in human locomotion using neuromusculoskeletal modelling and simulation. In particular, it aims to investigate potential cause-effect relationships between biomechanical or neural impairments and pathological gaits. Toe and heel gaits are commonly present in spinal cord injury, stroke and cerebral palsy. Toe walking is mainly attributed to spasticity and contracture at plantar flexor muscles, whereas heel walking can be attributed to muscle weakness of biomechanical or neural origin. To investigate the effect of these impairments on gait, this study focuses on the soleus and gastrocnemius muscles as they contribute to ankle plantarflexion. We built a reflex circuit model based on previous work by Geyer and Herr with additional pathways affecting the plantar flexor muscles. The SCONE software, which provides optimisation tools for 2D neuromechanical simulation of human locomotion, is used to optimise the corresponding reflex parameters and simulate healthy gait. We then modelled various bilateral plantar flexor biomechanical and neural impairments, and individually introduced them in the healthy model. We characterised the resulting simulated gaits as pathological or not by comparing ankle kinematics and ankle moment with the healthy optimised gait based on metrics used in clinical studies. Our simulations suggest that toe walking can be generated by hyperreflexia, whereas muscle and neural weaknesses partially induce heel gait. Thus, this 'what if' approach is deemed of great interest as it allows investigation of the effect of various impairments on gait and suggests an important contribution of active reflex mechanisms to pathological toe gait. KEY POINTS: Pathological toe and heel gaits are commonly present in various conditions such as spinal cord injury, stroke and cerebral palsy. These conditions present various neural and biomechanical impairments, but the cause-effect relationships between these impairments and pathological gaits are difficult to establish clinically. Based on neuromechanical simulation, this study focuses on the plantar flexor muscles and builds a new reflex circuit controller to model and evaluate the potential effect of both neural and biomechanical impairments on gait. Our results suggest an important contribution of active reflex mechanisms to pathological toe gait. This 'what if' based on neuromechanical modelling is thus deemed of great interest to target potential causes of pathological gait.

AIM To understand the long-term effects of comprehensive spasticity treatment, including selective dorsal rhizotomy (SDR), on individuals with spastic cerebral palsy. METHOD This was a pre-registered, multicenter, retrospectively matched cohort study. Children were matched on age range and spasticity at baseline. Children at one center underwent spasticity treatment including SDR (Yes-SDR, n=35) and antispastic injections. Children at two other centers had no SDR (No-SDR, n=40 total) and limited antispastic injections. All underwent subsequent orthopedic treatment. Participants returned for comprehensive long-term assessment (age ≥21y, follow-up ≥10y). Assessment included spasticity, contracture, bony alignment, strength, gait, walking energy, function, pain, stiffness, participation, and quality of life. RESULTS Spasticity was effectively reduced at long-term assessment in the Yes-SDR group and was unchanged in the No-SDR group. There were no meaningful differences between the groups in any measure except the Gait Deviation Index (Yes-SDR + 11 vs No-SDR + 5) and walking speed (Yes-SDR unchanged, No-SDR declined 25%). The Yes-SDR group underwent more subsequent orthopedic surgery (11.9 vs 9.7 per individual) and antispastic injections to the lower limbs (14.4 vs

OBJECTIVE: To determine the prevalence of bruxism in individuals with cerebral palsy (CP) and evaluating the various factors associated METHODS: One hundred and ten adults diagnosed with CP were selected from six institutions for people with special needs. Data were collected through oral examinations using the diagnostic criteria proposed by the American Academy of Sleep Medicine and the modified scale of Asworth RESULTS: Of the total sample, spastic tetraplegia was the most common type and half of the population presents severe intellectual disability. The prevalence of bruxism was 74,5%. Wear facets were observed in 67.9% of the sample, 59.1% of which were brilliant. There was a positive association between mixed bruxism (MB) and the spasticity classification. Through a logistic regression it was found that the risk of having MB and general bruxism (GB) is greater in individuals who have shiny wear facets. CONCLUSIONS: In the present study the high prevalence of GB indicates there is an urgent need for treatment options in people with CP. More studies are needed with standardized diagnostic protocols and representative samples to evaluate the factors that influence the presence of bruxism in this population and to establish an appropriate treatment planning.

Key points Damage to corticospinal axons have implications for the development of spasticity following spinal cord injury (SCI). Here, we examined to which extent residual corticospinal connections and spasticity are present in muscles below the injury (quadriceps femoris and soleus) in humans with motor complete thoracic SCI. We found three distinct sub-groups of people: participants with spasticity and corticospinal responses in the quadriceps femoris and soleus, participants with spasticity and corticospinal responses in the quadriceps femoris only, and participants with no spasticity or corticospinal responses in either muscle. Spasticity and corticospinal responses were present in the quadriceps but never only in the soleus muscle, suggesting a proximal to distal gradient of symptoms of hyperreflexia. These results suggest that concomitant patterns of residual corticospinal connectivity and spasticity exist in humans with motor complete SCI and that a clinical exam of spasticity might be a good predictor of residual corticospinal connectivity. Abstract The loss of corticospinal axons has implications for the development of spasticity following spinal cord injury (SCI). However, the extent to which residual corticospinal connections and spasticity are present across muscles below the injury remains unknown. To address this question, we tested spasticity using the Modified Ashworth Scale and transmission in the corticospinal pathway by examining motor evoked potentials elicited by transcranial magnetic stimulation over the leg motor cortex (cortical MEPs) and by direct activation of corticospinal axons by electrical stimulation over the thoracic spine (thoracic MEPs), in the quadriceps femoris and soleus muscles, in 30 individuals with motor complete thoracic SCI. Cortical MEPs were also conditioned by thoracic electrical stimulation at intervals allowing their summation or collision. We found three distinct sub-groups of participants: 47% showed spasticity in the quadriceps femoris and soleus muscle, 30% showed spasticity in the quadriceps femoris muscle only, and 23% showed no spasticity in either muscle. While cortical MEPs were present only in the quadriceps in participants with spasticity, thoracic MEPs were present in both muscles when spasticity was present. Thoracic electrical stimulation facilitated and suppressed cortical MEPs, showing that both forms of stimulation activated similar corticospinal axons. Cortical and thoracic MEPs correlated with the degree of spasticity in both muscles. These results provide the first evidence that related patterns of residual corticospinal connectivity and spasticity exist in muscles below the injury after motor complete thoracic SCI and highlight that a clinical exam of spasticity can predict residual corticospinal connectivity after severe paralysis. This article is protected by copyright. All rights reserved.

Introduction/aims It has been well established that spasticity interferes with smooth joint movements. Although the degree of spasticity is related to the excitability of anterior horn cells and is thought to improve after repetitive movements, the effect of the rhythm of repetitive movements on the excitability of anterior horn cells remains unknown. Therefore, we investigated the excitability of anterior horn cells after periodic and discrete repetitive movements using F waves. Methods Right-handed, healthy subjects were recruited for this study. Subjects then performed periodic or discrete repetitive thumb abduction movements for 10 seconds, measuring the F waves before, immediately after, and then 2 and 4 minutes after performing these movements. Specifically, the F waves were recorded from the abductor pollicis brevis muscle, after median nerve stimulation at the wrist. Next, the F/M amplitude ratio, which was used to evaluate the excitability of anterior horn cells, was compared before, immediately after, and 2 and 4 minutes after each task. Results A total of 12 subjects participated in this study. In the periodic task, the F/M amplitude ratio was found to be significantly decreased immediately after the task compared with before the task, but there was no significant difference between the other trials. Conversely, in the discrete task, there was no significant difference in the F/M amplitude ratio between trials. Discussion Periodic repetitive movements were found to temporarily reduce the excitability of anterior horn cells.

Background Spasticity is a frequent symptom of multiple sclerosis (MS), which may negatively influence daily living activities (ADL). Objectives To (1) explore the feasibility to conduct a structured interview by specialist nurses about limitations in ADL; (2) determine the percentage of people with MS (PwMS) with limitations in ADL related to spasticity; (3) to assess the knowledge about spasticity and describe its clinical features. Design Observational, cross-sectional, multicentre study in 16 MS units of Catalonia (Spain). Participants were recruited from the outpatient facility and day-care hospital between July 2018 and June 2019 and met the following criteria: (1) age 18 or older, (2) diagnosis of MS according to McDonald criteria 2010 and (3) no clinical relapse in previous 30 days. Methods Specialist nurses conducted a structured interview divided in two parts: the assessment of (1) limitations in the ADL and (2) the presence of spasticity and associated symptoms. The usefulness of this intervention was requested. This study met the STROBE reporting guidelines checklist for observational studies. Results Three hundred sixty eight pwMS (244 women) with a mean age of 46 years and a median Expanded Disability Status Scale score of 2.5 (range, 0-8.5) were included. 262 (71%) pwMS had limitations in the ADL, and spasticity was reported as the most limiting symptom in 59 (23%). As a result of the interview, spasticity was observed in 199 (76%) participants; 47 (24%) of them were unaware that they had spasticity and 102 (51%) would not have reported it spontaneously. The level of the interview satisfaction was high (90%). Conclusions Spasticity is a complex and limiting symptom in MS. The structured interview conducted by specialist nurses is feasible and has good acceptance. Patient contribution Specialist nurses can be proactive in MS clinical assessment, which may help to detect symptoms with negative impact on quality of life.

Aim We explored the psychometric properties of the recently developed Tyneside Pegboard Test (TPT) for unimanual and bimanual dexterity in children with unilateral cerebral palsy (CP) and investigated the impact of sensorimotor impairments on manual dexterity. Method In this cross-sectional study, the TPT was assessed in 49 children with unilateral CP (mean age 9y 8mo, SD 1y 11mo, range 6-15y; 30 males, 19 females; 23 with right unilateral CP). All participants additionally underwent a standardized upper limb evaluation at body function and activity level. We investigated: (1) known-group, concurrent, and construct validity and (2) impact of sensorimotor impairments including spasticity, grip force, stereognosis, and mirror movements using analysis of covariance, Spearman's rank correlation (r), and multiple linear regression (R-2) respectively. Results TPT outcomes significantly differed according to the Manual Ability Classification System (p

AIM: To estimate yearly prevalence of ankle contractures among children with cerebral palsy (CP). Moreover, to investigate if age, gross motor function or spasticity are associated with ankle contracture.METHODS: We examined yearly prevalence of ankle contractures among 933 children based on data from a national clinical quality database from 2012 to 2019. We used the Gross Motor Function Classification System (GMFCS) and the Modified Ashworth Scale (MAS) to assess gross motor function and spasticity in the plantar flexors. Ankle contracture was defined as dorsiflexion with an extended knee equal to or below 0 degrees. Associations between age, GMFCS, spasticity and ankle contractures were analyzed using multivariable regression and presented as odds ratios (OR) with 95% confidence intervals (95%CI).RESULTS: The prevalence of ankle contracture was 32% and did not change with calendar year. GMFCS IV-V compared to I-III (40.6% vs. 28.9%, OR = 1.5 (95%CI: 1.07-2.11) and MAS 2-4 compared to 0 (44.6% vs. 24.4%, OR = 2.5 (95%CI: 1.59-3.91) were associated with a higher prevalence of ankle contracture. Age was not associated with ankle contracture.CONCLUSION: Ankle contractures are frequent among children with CP. Lower gross motor function and severe spasticity were associated with ankle contracture.

BACKGROUND Hereditary spastic paraplegia presents spasticity as the main clinical manifestation, reducing gait quality and producing incapacity. Management with botulinum toxin type A (BoNT-A) is not well elucidated. The objective of the current study was to evaluate the efficacy and safety of BoNT-A in patients with hereditary spastic paraplegias. METHODS This was a double-blind, randomized, placebo-controlled crossover trial. Each participant was randomly assigned to receive 1 injection session of either BoNT-A (100 IU/2 mL of Prosigne in each adductor magnus and each triceps surae) or saline 0.9% (2 mL). The primary outcome measure was change from baseline in maximal gait velocity, and secondary outcome measures included changes in gait at self-selected velocity, spasticity, muscle strength, Spastic Paraplegia Rating Scale, pain, fatigue, and subjective perception of improvement. We also looked at adverse events reported by the patients. RESULTS We enrolled 55 patients, 36 of whom were men and 41 with the pure phenotype. Mean age was 43 ± 13.4 years (range, 19-72 years), mean age of onset waws 27 ± 13.1 years (range

BACKGROUND AND PURPOSE Myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD) is an inflammatory autoimmune condition of the central nervous system. However, data on pain and depression have remained scarce. The aim of this study was to assess features of chronic pain and depression as well as their impact on health-related quality of life (hr-QoL) in MOGAD. METHODS Patients with MOGAD were identified in the Neuromyelitis Optica Study Group registry. Data were acquired by a questionnaire, including clinical, demographic, pain (PainDetect, Brief Pain Inventory-Short Form, McGill Pain Questionnaire-Short Form), depression (Beck Depression Inventory-II), and hr-QoL (Short Form-36 Health Survey) items. RESULTS Twenty-two of 43 patients suffered from MOGAD-related pain (11 nociceptive, eight definite neuropathic, three possible neuropathic) and 18 from depression. Patients with neuropathic pain had the highest pain intensity and most profound activities of daily living (ADL) impairment. Fifteen patients reported spasticity-associated pain, including four with short-lasting painful tonic spasms. Later disease onset, profound physical impairment, and depression were associated with chronic pain. Physical QoL was more affected in pain sufferers (p

L1syndrome is an X‐linked disorder manifesting with congenital hydrocephalus, adducted thumbs and spasticity. There are rare cases of L1 syndrome and coincident Hirschsprung disease, with mutations in the L1CAM gene thought to underlie both. We present a novel pathogenic L1CAM variant in someone with L1 syndrome and Hirschsprung disease.

RIPPLY2 is an essential part of the formation of somite patterning during embryogenesis and in establishment of rostro-caudal polarity. Here, we describe three individuals from two families with compound-heterozygous variants in RIPPLY2 (NM_001009994.2): c.238A > T, p.(Arg80*) and c.240-4 T > G, p.(?), in two 15 and 20 year old sisters, and a homozygous nonsense variant, c.238A > T, p.(Arg80*), in an 8 year old boy. All patients had multiple vertebral body malformations in the cervical and thoracic region, small or absent rib involvement, myelopathies, and common clinical features of SCDO6 including scoliosis, mild facial asymmetry, spinal spasticity and hemivertebrae. The nonsense variant can be classified as likely pathogenic based on the ACMG criteria while the splice variants must be classified as a variant of unknown significance. With this report on two further families, we confirm RIPPLY2 as the gene for SCDO6 and broaden the phenotype by adding myelopathy with or without spinal canal stenosis and spinal spasticity to the symptom spectrum. This article is protected by copyright. All rights reserved.

Introduction OnabotulinumtoxinA treatment for spasticity varies according to numerous factors and is individualized to meet treatment goals. Objective Explore real-world onabotulinumtoxinA utilization and effectiveness in patients with lower limb spasticity from the Adult Spasticity International Registry (ASPIRE) study. Design 2-year, multicenter, prospective, observational registry (NCT01930786). Setting 54 international clinical sites. Patients Adults (naive or non-naive to botulinum toxin[s] treatment for spasticity, across multiple etiologies) with lower limb spasticity related to upper motor neuron syndrome. Interventions OnabotulinumtoxinA administered at the clinician's discretion. Main outcome measures OnabotulinumtoxinA treatment utilization, clinician- and patient-reported satisfaction. Results In ASPIRE, 530 patients received ≥1 onabotulinumtoxinA treatment for lower limb spasticity (mean age, 52 years; stroke, 49.4%; multiple sclerosis 20.4%). Equinovarus foot was treated most often (80.9% of patients), followed by flexed knee (26.0%), stiff extended knee (22.5%), and flexed toes (22.3%). OnabotulinumtoxinA doses ranged between 10-1100 U across all presentations. Electromyography (EMG) was most commonly used for injection localization (≥41.1% of treatment sessions). Despite low patient response on the satisfaction questionnaire, clinicians (94.6% of treatment sessions) and patients (84.5%) reported satisfaction/extreme satisfaction that treatment helped manage spasticity, and clinicians (98.3%) and patients (91.6%) would probably/definitely continue onabotulinumtoxinA treatment. These data should be interpreted with care. 21 adverse events (AEs) in 18 patients (3.4%) were considered treatment-related. 67 patients (12.6%) reported 138 serious AEs; 3 serious AEs in 2 patients (0.4%) were considered treatment-related. No new safety signals were identified. Conclusions ASPIRE provides long-term observational data on the treatment of lower limb spasticity with onabotulinumtoxinA. Real-world data from this primary analysis can help to guide the clinical use of onabotulinumtoxinA to improve spasticity management. This article is protected by copyright. All rights reserved.

In the current proposed analysis, a new, feasible, and selective fluorimetric approach was designed for baclofen assay. Baclofen is a medication prescribed as a therapy for muscle spasticity that originated from multiple sclerosis or a spinal cord injury, and other cases such as hiccups. The analytical approach relies on the use of ninhydrin to form a fluorescent derivative that was monitored at λex 386 nm or λem 480 nm. Under suitable reaction conditions, the primary amino moiety in baclofen was condensed with ninhydrin and phenylacetaldehyde in the presence of Teorel buffer as a buffered medium. The method exhibited linearity when baclofen concentration was plotted against response in the range 1-10 μg ml-1 . Adjustment of the reaction variables and study of validation parameters according to ICH directives were performed correctly. An interference study was implemented to ensure that no discrepancy from the excipient had occurred. Finally, the proposed method was applied successfully for baclofen assay in dosage form and extended to test Mylobac content uniformity.

Aicardi Goutieres syndrome (AGS) is an autosomal recessive disorder characterized by severe pre- and postnatal occurring neurological symptoms, e.g. spasticity, dystonia and psychomotoric retardation, abnormalities in cerebrospinal fluid and specific alterations in neuroimaging, e.g. calcification of basal ganglia, white matter degeneration and brain atrophy(1). Successively, disease-causing mutations within genes involved in nucleic acid metabolism (TREX1, RNASEH2A/B/C, SAMHD1, ADAR1), or signaling (IFIH1) were identified.

Key points Baclofen is a GABAB agonist prescribed as a treatment for spasticity in stroke, brain injury and multiple sclerosis patients, who are often undergoing concurrent motor rehabilitation. Decreasing GABAergic inhibition is a key feature of motor learning and so there is a possibility that GABA agonist drugs, like baclofen, could impair these processes, potentially impacting rehabilitation. Here we examined the effect of 10mg baclofen, in 20 young healthy individuals, and found that the drug impaired retention of visuomotor learning with no significant effect on motor sequence learning. Overall baclofen did not alter TMS-measured GABAB inhibition, but the change in GABAB inhibition correlated with aspects of visuomotor learning retention. Further work is needed to investigate whether taking baclofen impacts motor rehabilitation in patients. Abstract The GABAB agonist baclofen is taken daily as a treatment for spasticity by millions of stroke, brain injury and multiple sclerosis patients, many of whom are also undergoing motor rehabilitation. However, multiple studies suggest that decreases in GABA are a key feature of human motor learning (Floyer-Lea et al. 2006; Stagg et al. 2011; Kolasinski et al. 2018), which raises questions about whether drugs increasing GABAergic activity may impair motor learning and rehabilitation. In this double-blind, placebo-controlled study we investigate whether a single 10mg dose of the GABAB agonist baclofen impaired motor sequence learning and visuomotor learning in 20 young healthy participants of both sexes. Participants trained on visuomotor and sequence learning tasks using their right hand. Transcranial magnetic stimulation (TMS) measures of corticospinal excitability, GABAA (SICI2.5ms ) and GABAB (LICI150ms ) receptor activation were recorded from left M1. Behaviourally, baclofen caused a significant reduction of visuomotor aftereffect (F(1,137.8) = 6.133, p = 0.014) and retention (F(1,130.7) = 4.138, p = 0.044), with no significant changes to sequence learning. There were no overall changes to TMS measured GABAergic inhibition with this low dose of baclofen. This result confirms the causal importance of GABAB inhibition in mediating visuomotor learning and suggests that chronic baclofen use could negatively impact aspects motor rehabilitation. This article is protected by copyright. All rights reserved.

Objective Recessive mutations in the CAPN1 gene have recently been identified in spastic paraplegia 76 (SPG76), a complex hereditary spastic paraplegia (HSP) that is combined with cerebellar ataxia, resulting in an ataxia‐spasticity disease spectrum. This study aims to assess the influence of CAPN1 variants on the occurrence of SPG76 and identify factors potentially contributing to phenotypic heterogeneity. Methods We screened a cohort of 240 unrelated HSP families for variants in CAPN1 using high‐throughput sequencing analysis. We described in detail the clinical and genetic features of the SPG76 patients in our cohort and summarized all reported cases. Results Six unreported CAPN1‐associated families containing eight patients with or without cerebellar ataxia were found in our cohort of HSP cases. These patients carried three previously reported homozygous truncating mutations (p.V64Gfs*103, c.759+1G>A, and p.R285*), and three additional novel compound heterozygous missense mutations (p.R481Q, p.P498L, and p.R618W). Lower limbs spasticity, hyperreflexia, and Babinski signs developed in about 94% of patients, with ataxia developing in 63% of cases. In total, 33 pathogenic mutations were distributed along the three reported functional domains of calpain‐1 protein, encoded by CAPN1, with no hotspot region. A comparison of gender distribution between the two groups indicated that female SPG76 patients were significantly more likely to present with complicated HSP than male patients (P = 0.015). Interpretation Our study supports the clinically heterogeneous inter‐ and intra‐family variability of SPG76 patients, and demonstrates that gender and calpain‐1 linker structure may contribute to clinical heterogeneity in SPG76 cases.

Introduction To detect ideal locations for botulinum toxin (BoNT) injection by exploring the intramuscular nerve arborization of the psoas major and iliacus muscles. Method A modified Sihler's method was performed on the psoas major and iliacus muscles (16 specimens each). Intramuscular nerve arborization was recorded according to the most prominent point of the anterior superior iliac spine (ASIS), the posterior superior iliac spine (PSIS), the lesser trochanter (LT), and the transverse process of the 12th thoracic vertebra. Results Intramuscular nerve arborization of the psoas major muscle was the largest from 1/5 to 3/5 the distance from the transverse process of the 12th thoracic vertebra to the PSIS, and the tendinous portion of the muscle occupied from 3/5 to 5/5 this distance. In terms of the plane of the ASIS, the PSIS, and the LT, the arborization of the iliacus muscle was the largest from 1/5 to 3/5 the horizontal distance and 0 to 1/3, the distance longitudinally, and from 1/5 to 2/5, the horizontal distance and 1/3 to 2/3, the longitudinal distance. Discussion These results suggest that an injection of BoNT to the psoas major and iliacus muscle should be applied in specific areas. Additionally, the posterior approach is an ideal method for targeting only the psoas major because the injection point is above the PSIS. However, when treating both the psoas major and iliacus muscles, the proximal anterior approach is an ideal method according to the arborization patterns.

Homozygous recessive or compound heterozygous mutations in SLC13A5-gene as a cause of Early Infantile Epileptic Encephalopathy subtype 25 (OMIM 615905) were published in 2014. Previous clinical reports describe young patients, aged

Key points Cutaneous reflexes were tested to examine the neuronal mechanisms contributing to muscle spasms in humans with chronic spinal cord injury (SCI). Specifically, we tested the effect of Achilles and tibialis anterior tendon vibration on the early and late components of the cutaneous reflex and reciprocal Ia inhibition in the soleus and tibialis anterior muscles in humans with chronic SCI. We found that tendon vibration reduced the amplitude of later but not earlier cutaneous reflex in the antagonist but not in the agonist muscle relative to the location of the vibration. In addition, reciprocal Ia inhibition between antagonist ankle muscles increased with tendon vibration and participants with a larger suppression of the later component of the cutaneous reflex had stronger reciprocal Ia inhibition from the antagonistic muscle. Our study is the first to provide evidence that tendon vibration attenuates late cutaneous spasm-like reflex activity, likely via reciprocal inhibitory mechanisms, and may represent a method, when properly targeted, for controlling spasms in humans with SCI. Abstract The neuronal mechanisms contributing to the generation of involuntary muscle contractions (spasms) in humans with spinal cord injury (SCI) remain poorly understood. To address this question, we examined the effect of Achilles and tibialis anterior tendon vibration at 20, 40, 80 and 120 Hz on the amplitude of the long-polysynaptic (LPR, from reflex onset to 500 ms) and long-lasting (LLR, from 500 ms to reflex offset) cutaneous reflex evoked by medial plantar nerve stimulation in the soleus and tibialis anterior, and reciprocal Ia inhibition between these muscles, in 25 individuals with chronic SCI. We found that Achilles tendon vibration at 40 and 80 Hz, but not other frequencies, reduced the amplitude of the LLR in the tibialis anterior, but not the soleus muscle, without affecting the amplitude of the LPR. Vibratory effects were stronger at 80 than 40 Hz. Similar results were found in the soleus muscle when the tibialis anterior tendon was vibrated. Notably, tendon vibration at 80 Hz increased reciprocal Ia inhibition between antagonistic ankle muscles and vibratory-induced increases in reciprocal Ia inhibition were correlated with decreases in the LLR, suggesting that participants with a larger suppression of later cutaneous reflex activity had stronger reciprocal Ia inhibition from the antagonistic muscle. Our study is the first to provide evidence that tendon vibration suppresses late spasm-like activity in antagonist but not agonist muscles, likely via reciprocal inhibitory mechanisms, in humans with chronic SCI. We argue that targeted vibration of antagonistic tendons might help to control spasms after SCI.

To assess the efficacy, safety, and tolerability of oromucosal nabiximols cannabinoid medicine as adjunct therapy for children with spasticity due to cerebral palsy/traumatic central nervous system injury with inadequate response to existing treatment.Overall, 72 patients (mean [SD] age 12y 4mo [3y 1mo], range 8-18y) were randomized at a ratio of 2:1 to receive nabiximols (n=47; 29 males, 18 females) or placebo (n=25; 15 males, 10 females) for 12 weeks (12 sprays/day max. based on clinical response/tolerability). The primary outcome was change from baseline in level of spasticity on a 0 to 10 Numerical Rating Scale (NRS), assessed by the primary caregiver at 12 weeks. Secondary outcomes included additional measures for spasticity, sleep quality, pain, health-related quality of life, comfort, depression, and safety.There was no significant difference in the spasticity 0 to 10 NRS between nabiximols versus placebo groups after 12 weeks. No statistically significant differences were observed for any secondary endpoint. Adverse events were predominantly mild or moderate in severity; however, three cases of hallucinations were reported.Nabiximols was generally well tolerated; however, neuropsychiatric adverse events were observed. No significant reduction in spasticity with nabiximols treatment versus placebo was observed.Oromucosal nabiximols is generally well tolerated by paediatric patients. However, three cases of hallucinations were observed, one of which involved auditory hallucinations and a suicide attempt. Oromucosal nabiximols versus placebo did not reduce cerebral palsy/central nervous system injury-related spasticity.Evaluar la eficacia, seguridad y tolerabilidad del cannabinoides nabiximol de aplicación en spray bucal como terapia complementaria para niños con espasticidad debido a parálisis cerebral o lesión traumática del sistema nervioso central que no responden al tratamiento convencional. MÉTODO: En total, 72 pacientes (media [DE] edad 12 años 4 meses [3 años 1 mes], rango 8-18 años) fueron aleatorizados en una proporción de 2: 1 para recibir nabiximol spray (n = 47; 29 masculinos, 18 femeninos) o placebo (n = 25; 15 masculinos, 10 femeninos) durante 12 semanas (12 aplicaciones de spray / día máx basados en la respuesta clínica / tolerabilidad). El resultado primario fue el cambio en el nivel basal de espasticidad en una Escala de Calificación Numérica (NRS) de 0 a 10, evaluada por el cuidador primario a las 12 semanas. Los resultados secundarios incluyeron medidas adicionales para la espasticidad, la calidad del sueño, el dolor, la calidad de vida relacionada con la salud, el confort, la depresión y la seguridad.No hubo diferencias significativas en la espasticidad de 0 a 10 NRS entre los grupos de que recibieron nabiximol versus el grupo placebo después de 12 semanas. No se observaron diferencias estadísticamente significativas para ningún criterio de valoración secundario. Los eventos adversos fueron predominantemente leves o moderados en severidad; sin embargo, se informaron tres casos de alucinaciones. INTERPRETACIÓN: El nabiximol fue generalmente bien tolerado; sin embargo, se observaron eventos adversos neuropsiquiátricos. No se observó una reducción significativa en la espasticidad con el tratamiento con nabiximoles versus placebo.Avaliar a eficácia, segurança e tolerabilidade do medicamento oromucosal nabiximols canabinóide como terapia adjunta para crianças com espasticidade devida a paralisia cerebral/lesão traumática do sistema nervoso central com resposta inadequada a tratamentos existentes. MÉTODO: Em geral, 72 pacientes (média [DP] idade 12a 4m [3a 1m], variação 8-18a) foram randomizados em uma taxa de 2:1 para receber nabiximols (n=47; 29 sexo masculino, 18 sexo feminino) ou placebo (n=25; 15 sexo masculino, 10 sexo feminino) por 12 semanas (12 sprays/dia máx. com base na resposta clínica/tolerabilidade). O resultado primário foi mudança com relação a linha de base no nível de espasticidade um uma Escala Numérica de Pontuação (ENP) de 0 a 10, avaliada pelo cuidador primário com 12 semanas. Resultados secundários incluíram medidas adicionais de espasticidade, qualidade do sono, dor, qualidade de vida relacionada à saúde, conforto, depressão, e segurança.Não houve diferença significativa na espasticidade Segundo a ENP 0 a 10 entre os grupos nabiximols versus placebo após 12 semanas. Nenhuma diferença estatisticamente significativa foi observada para nenhum desfecho secundário. Eventos adversos foram predominantemente leves ou moderados em severidade; no entanto, três casos de alucinações foram reportados. INTERPRETAÇÃO: Nabiximols foi em geral bem tolerado; no entanto eventos adversos neuropsiquiátricos foram observados. Nenhuma redução significativa na espasticidade com o tratamento com nabiximols versus placebo foi observada.

Background Botulinum toxin injections are effective in relieving focal spasticity resulting from upper motor neuron injuries. Doses approved in the United States for onabotulinumtoxinA and incobotulinumtoxinA are up to 400 units, yet higher doses are often used. Objective To determine differences in risk of adverse events when using higher (>600 units) as compared to lower doses within clinically applicable categories; the difference in adverse events between types of botulinum toxin-A, and any association of the injection of cervical muscles with increased risk for adverse events. Design and setting Retrospective analysis of injections performed over a 3-year period at a freestanding rehabilitation hospital network. Participants Persons with spasticity or dystonia undergoing ona- and/or incobotulinumtoxinA injections. Interventions Not applicable. Main outcome measures Adverse events for injections were divided into the three dose ranges (≤400 units, 401-600 units, or > 600 units). Results 889 injections in 342 patients met inclusion criteria with 65% ≤400 units, 21% 401-600 units, and 14% >600 units. Adverse events were not significantly increased in doses of 401-600 units relative to ≤400 units (OR 0.97, 95% CI 0.31, 2.98). Doses of toxin over 600 units were associated with significantly increased relative risk of adverse events (OR 2.98, 95% CI 1.12, 8.13). There were no significant differences between adverse event rates for onabotulinumtoxinA or incobotulinumtoxinA (P >.99). Inclusion of cervical muscles in isolation did significantly increase the risk of adverse events (OR 4.21, 95% CI 1.15, 15.46). Conclusion Risk for adverse events were not significantly increased in doses of ona- and/or incobotulinumtoxinA up to 600 units, suggesting that the current 400 units upper bound of approved dose may need to be reexamined. Doses above 600 units were found to increase the rate of adverse effects and clinical benefit versus risk should be taken into account. Level of evidence III.

To assess the neural and non-neural contributions to spasticity in the impaired ankle of children with cerebral palsy (CP).Instrumented tapping of the Achilles tendon was done isometrically to minimize non-neural contributions and elicit neural contributions. Robot-controlled ankle stretching was done at various velocities, including slow stretching, with minimized neural contributions. Spasticity was assessed as having neural (phasic and tonic stretch reflex torque, tendon reflex gain, contraction rate, and half relaxation rate) and non-neural origin (elastic stiffness and viscous damping) in 17 children with CP (six females and 11 males; mean age [SD] 10y 8mo [3y 11mo], range 4y-18y) and 17 typically developing children (six females and 11 males; mean age [SD] 12y 7mo [2y 9mo], range 7y-18y). All torques were normalized to weight×height.Children with CP showed increased phasic and tonic stretch reflex torque (p=0.004 and p=0.001 respectively), tendon reflex gain (p=0.02), contraction rate (p=0.038), half relaxation rate (p=0.02), elastic stiffness (p=0.01), and viscous damping (p=0.01) compared to typically developing children.Controlled stretching and instrumented tendon tapping allow the systematic quantification of various neural and non-neural changes in CP, which can be used to guide impairment-specific treatment.Ankle spasticity is associated with increased phasic and tonic stretch reflexes, tendon reflex gain, and contraction and half relaxation rates. Ankle spasticity is also associated with increased elastic stiffness and viscous damping.Contribuciones neuronales y no neuronales a la espasticidad del tobillo en niños con parálisis cerebral OBJETIVO: Evaluar las contribuciones neurales y no neurales a la espasticidad en el tobillo comprometido de niños con parálisis cerebral (PC). MÉTODO: La percusión instrumentada en el tendón de Aquiles se realizó de forma isométrica para minimizar las contribuciones no neurales y un tirón del tendón exagerado, para obtener contribuciones neurales. El estiramiento del tobillo controlado por robot se realizó a varias velocidades, incluido el estiramiento lento, con contribuciones neurales minimizadas. Se evaluó la espasticidad como neural (torque reflejo de estiramiento fásico y tónico, ganancia del reflejo tendinoso, tasa de contracción y media tasa de relajación) y origen no neural (rigidez elástica y amortiguación viscosa) en 17 niños con PC (seis mujeres y 11 varones; edad media [DE] 10a 8m [3a 11m], rango 4a-18a) y 17 niños con desarrollo típico (seis mujeres y 11 hombres; edad media [SD] 12a 7m [2a 9m], rango 7a-18a). Todos los pares de torsion se normalizaron al peso × altura. RESULTADOS: Los niños con PC mostraron un aumento del torque reflejo de estiramiento fásico y tónico (p = 0,004 y p = 0,001 respectivamente), ganancia refleja del tendón (p = 0,02), tasa de contracción (p = 0,038), tasa de relajación media (p = 0,02), rigidez elastica (p = 0,01) y amortiguación viscosa (p = 0,01) en comparación con los niños con desarrollo normal. INTERPRETACIÓN: El estiramiento controlado y la percusión instrumentada del tendón, permiten la cuantificación sistemática de varios cambios neuronales y no neuronales en la PC, que pueden usarse para guiar el tratamiento específico de la discapacidad.Contribuições neurais e não neurais para a espasticidade do tornozelo em crianças com paralisia cerebral OBJETIVO: Avaliar as contribuições neurais e não-neurais para a espasticidade no tornozelo comprometido de crianças com paralisia cerebral (PC). MÉTODO: O golpeamento instrumentalizado do tendão de Aquiles foi realizado isometricamente para minimizar as contribuições não-neurais e um desvio exagerado do tendão, e assim eliciar as contribuições neurais. O alongamento do tornozelo controlado por um robô foi realizado em várias velocidades, incluindo alongamento lento, com contribuições neurais limitadas. A espasticidade foi avaliada como tendo origem neural (torque do reflexo fásico e tônico, ganho do reflexo tendinoso, taxa de contração, e taxa de meio relaxamento) e não-neural (rigidez elástica e amortecimento viscoso) em 17 crianças com PC (seis do sexo feminino e 11 do sexo masculino; média de idade [DP] 10a 8m [3 11m], variação 4a-18a) e 17 crianças com desenvolvimento típico (seis do sexo feminino e 11 do sexo masculino; média de idade [DP] 12a 7m [2a 9m], variação 7a-18a). Todos os torques foram normalizados para peso x altura. RESULTADOS: Crianças com PC mostraram aumento do torque do reflexo tônico e fásico e (p=0,004 e p=0,001 respectivamente), ganho do reflexo tendinoso (p=0,02), taxa de contração (p=0,038), taxa de meio relaxamento (p=0,02), rigidez elástica (p=0,01), e amortecimento viscoso (p=0,01) em comparação com as crianças com desenvolvimento típico. INTERPRETAÇÃO: O alongamento controlado e o golpeamento instrumentalizado do tendão permitem quantificação sistemática de várias mudanças neurais e não-neurais em PC, as quais podem ser usadas para guiar tratamento específico para a deficiência observada.

KIF1A-related disorders (KRD) were first described in 2011 and the phenotypic spectrum has subsequently expanded to encompass a range of central and peripheral nervous system involvement. Here we present a case series demonstrating the range of clinical, neurophysiological, and radiological features which may occur in childhood-onset KRD. We report on all the children and young people seen at a single large tertiary centre. Data were collected through a retrospective case-notes review. Twelve individuals from 10 families were identified. Eight different mutations were present, including four novel mutations. Two patients displayed a very severe phenotype including congenital contractures, severe spasticity and/or dystonia, dysautonomia, severe sensorimotor polyneuropathy and optic atrophy, significant white matter changes on brain MRI, respiratory insufficiency, and complete lack of neurodevelopmental progress. The remaining 10 patients represented a spectrum of severity with common features including a movement disorder with spasticity and/or dystonia, subtle features of dysautonomia, sensory axonal neuropathy, varying degrees of optic atrophy and of learning and/or behavioural difficulties, and subtle or absent-but sometimes progressive-changes in white matter on MRI. Epilepsy was common among the more severely affected children. This case series demonstrates that KRD comprise a range of neurological disorders, with both the milder and the more severe forms combining central and peripheral (including autonomic) nervous system deficits.

Background/objective Chronic pain is commonly reported in individuals with spinal cord injuries (SCIs), with recent prevalence reported as high as 80%. Uncontrolled pain is known to decrease quality of life, attenuate mood, and impact sleep. Spinal cord stimulation (SCS) for the treatment of refractory pain was first used in the SCI population in 1972. To date there have been no randomized controlled trials examining the effect of SCS on neuropathic pain post-SCI. A literature review in 2009 identified 27 studies, the majority prior to 2000, that included at least 1 patient with SCI. Given the significant advancements in the field of SCS, this review examines the updated evidence of SCS for the treatment of neuropathic pain in individuals with SCI and provides guidance on future investigations. Methods MEDLINE and EMBASE databases were searched. All published reports, case series, and clinical trials reviewing SCS for neuropathic pain that included at least 1 individual with SCI were included. Results The initial search identified 376 reports, of which 22 met inclusion criteria, for a total of 69 patients. All reports were of very low quality. A majority of the reported patients were male, underwent tonic stimulation, and reportedly experienced improvement in pain and spasticity, with decreased use of pain medication. Conclusions The synthesized findings from primarily case studies support the safety of SCS in SCI with the suggestion of potential pain relief benefit; however, data from low-quality studies are insufficient for informing clinical practice. A well-designed, prospective clinical trial is proposed to further investigate this indication.

BACKGROUND Poststroke spasticity affects motor function and the ability to perform activities of daily living, with the potential to affect quality of life (QoL) and increase caregiver burden. OBJECTIVE To investigate the effect of repeated incobotulinumtoxinA treatment on spasticity-associated functional disability, caregiver burden, and QoL in the 36-week open-label extension of the phase 3 PURE study (NCT01392300). DESIGN Open-label extension period of a prospective, double-blind, placebo-controlled, randomized, multicenter study. SETTING Forty-six investigation sites in seven countries (Czech Republic, Germany, Hungary, India, Poland, Russia, United States). PARTICIPANTS Adults, aged 18-80 years, ≥12 months since last botulinum neurotoxin injection or entirely toxin naive, with median poststroke upper-limb spasticity of >2 years' duration. METHODS Participants who completed the 12-week, double-blind main period could enter the open-label extension and receive up to three additional incobotulinumtoxinA treatments (fixed total dose 400 U at 12-week intervals) into the affected muscles of one upper limb. MAIN OUTCOME MEASURES Functional disability (Disability Assessment Scale; DAS), caregiver burden (Carer Burden Scale), and quality of life (QoL; EuroQol [EQ] 5-dimensions three-level [EQ-5D-3L]). RESULTS The open-label extension included 296 treated patients. Mean DAS score for the principal target domain improved significantly from the main period baseline to the end-of-study visit (P

INTRODUCTION The Ankle Lunge Test (ALT) is a reliable method of measuring weight bearing ankle dorsiflexion range, a key component of gait, in healthy ankles and a range of musculoskeletal conditions. The aim of this study is to evaluate the reliability and concurrent validity of the ALT in people with stroke and investigate whether change in ALT is observed following treatment of spasticity with Botulinum Toxin. METHODOLOGY Repeated Measure Design: Forty stroke survivors were recruited for reliability testing. Twenty three stroke survivors were evaluated with the ALT before and after treatment of the lower limb with Botulinum Toxin. Measurement with the ALT was performed for all participants. For the reliability group, two physiotherapists evaluated the ALT on Day 1 and one therapist re-evaluated the ALT one to 5 days later. For the change evaluation group, the ALT was tested prior to and six to eight weeks after intervention. Other measures evaluated were velocity, the Functional Ambulation Category, Timed Up and Go and the Modified Tardieu Scale. RESULTS Intra-rater, inter-rater and test-retest reliability yielded intra-class correlation coefficients at or above 0.92. Moderate correlations were found between the ALT and velocity r = 0.605 (p

A 14‐year‐old girl with cerebral palsy presented for bilateral lower limb surgery for spasticity. A lumbar epidural catheter was sited under general anaesthesia on the third attempt and used intra‐operatively with good effect. A local anaesthetic infusion was used for postoperative analgesia but was noted to be leaking under the dressing with a patchy, unilateral block. The catheter was therefore removed on the second postoperative day. Following discharge, the patient progressively developed new back and leg pain for which she was re‐admitted seven weeks later. This was investigated and initially thought to be myositis of the erector spinae muscles on magnetic resonance imaging. When the patient failed to respond to treatment, a muscle biopsy demonstrated desmoid fibromatosis. Trauma may cause or accelerate the development of desmoid fibromatosis, which has also been theorised to arise from scar tissue in previously injured areas. We hypothesise that challenging epidural placement or the leakage of the local anaesthetic agent into the surrounding muscular tissue, inducing local myonecrosis, could have been the triggering or accelerating event in tumour development. This may be the first reported case of extra‐abdominal desmoid fibromatosis in association with epidural placement.

This is the first case report describing a laparoscopic fundoplication in a child with an intrathecal Baclofen pump which was inserted because of severe spasticity secondary to cerebral palsy. The child had symptoms of gastroesophageal reflux with recurrent episodes of aspiration pneumonia. These were managed with a gastrostomy and conservative therapy with no success. The presence of an intrathecal Baclofen pump makes abdominal surgery challenging and carries the risk of pump infection with its associated sequelae. However, we performed a successful laparoscopic fundoplication with no intraoperative complications and the child was asymptomatic at 18 months follow-up.

BACKGROUND Spasticity and pain frequently co-occur in persons with spinal cord injury (SCI), yet, how these sequelae interact in daily life is unclear. Additionally, little is known about how psychological factors relate to the perception of spasticity and its impacts on daily life. OBJECTIVES (1) Characterize relationships between spasticity and chronic pain with regard to perceived severity, difficulty dealing, and life interference. (2) Determine the extent to which perceived spasticity severity and physiological, psychological, and pain-related factors contribute to impacts of spasticity on daily life (difficulty in dealing, life interference). (3) Determine the effects of painful spasticity on aspects of chronic pain and spasticity (severity, life interference, interference with sleep, and spasm duration). DESIGN Observational study. SETTING University laboratory. PARTICIPANTS Twenty participants with SCI and lower extremity spasticity. METHODS Measures included International SCI Pain Basic Data Set, Pain and Spasticity Inventories, Difficulty Dealing with Pain/Spasticity, SCI-Spasticity Evaluation Tool, Connor-Davidson Resilience and Moorong Self-Efficacy Scales, Spinal Cord Assessment Tool for Spastic Reflexes, spasm duration, and injury-related and demographic factors. Bivariate correlations, multiple regression analyses, and pairwise comparisons were performed. RESULTS Spasticity and chronic pain were directly related, with respect to perceived severity, difficulty dealing, and life interference (rho = 0.514-0.673, P

Background When managing patients with focal spasticity, phenol neurolysis is often avoided largely because of its presumed poor adverse effect profile. It is suggested that dysesthesias may be more common with phenol neurolysis of the mixed sensorimotor nerves (eg, radial, median, and ulnar nerves) compared to neurolysis of pure motor nerves. However, these risks may be mitigated with precise localization of pure motor branches by ultrasound and electrical stimulation (EStim). Objective To explore practice patterns of phenol neurolysis to distal upper extremity mixed sensorimotor nerves with ultrasound and EStim guidance. Design A retrospective analysis of all neurolysis procedure records at a single institution from January 2013 to February 2018. Demographic and clinical variables including primary neurological diagnosis, concurrent spasticity treatments, nerves injected, phenol dosage and adverse events were abstracted from the electronic medical records. Participants 57 patients who received phenol neurolysis with ultrasound and EStim guidance for spasticity management to radial, median, or ulnar nerves. Main outcome measures Reported adverse effects. Results A total of 57 patients who collectively received neurolysis to 139 nerves across 102 encounters, met inclusion criteria. Most prevalent diagnoses included traumatic brain injury (N = 27, 47.4%) and stroke (N = 18, 31.6%), with a smaller subset having spinal cord injury (N = 10, 17.5%). Most patients received concomitant chemodenervation with botulinum toxin (N = 44, 77.2%). The average phenol dosage per nerve was 1.8 mL, with a range of 0.5-9.0 mL. Reported adverse effects included three cases of prolonged pain, but no dysesthesias were reported during the follow-up period (>40 days). Conclusions Phenol neurolysis with ultrasound and EStim guidance was successfully and safely used to manage focal spasticity in the distal upper extremity. Level of evidence III.

Comprehensive instrumented muscle and joint assessments should be considered when prescribing Botulinum NeuroToxin-A (BoNT-A) treatment in spastic paresis. In a child with spastic paresis, comprehensive evaluation following treatment with BoNT-A, serial casting, and physiotherapy showed that short-term improvements in gait occurred without changes in muscle morphology. Rather, foot flexibility increased. ispartof: Clinical Case Reports Wiley vol:7 issue:9 pages:1-10 ispartof: location:England status: Published online

Key points In people or animals with incomplete spinal cord injury (SCI), changing a spinal reflex through an operant conditioning protocol can improve locomotion.All previous studies conditioned the reflex during steady‐state maintenance of a specific posture. By contrast, the present study down‐conditioned the reflex during the swing‐phase of locomotion in people with hyperreflexia as a result of chronic incomplete SCI. The aim was to modify the functioning of the reflex in a specific phase of a dynamic movement.This novel swing‐phase conditioning protocol decreased the reflex much faster and farther than did the steady‐state protocol in people or animals with or without SCI, and it also improved locomotion.The reflex decrease persisted for at least 6 months after conditioning ended.The results suggest that conditioning reflex function in a specific phase of a dynamic movement offers a new approach to enhancing and/or accelerating recovery after SCI or in other disorders. Abstract In animals and people with incomplete spinal cord injury, appropriate operant conditioning of a spinal reflex can improve impaired locomotion. In all previous conditioning studies, the reflex was conditioned during steady‐state maintenance of a stable posture; this steady‐state protocol aimed to change the excitability of the targeted reflex pathway; reflex size gradually changed over 8–10 weeks. The present study introduces a new protocol, comprising a dynamic protocol that aims to change the functioning of the reflex pathway during a specific phase of a complex movement. Specifically, we down‐conditioned the soleus H‐reflex during the swing‐phase of locomotion in people with hyperreflexia as a result of chronic incomplete SCI. The swing‐phase H‐reflex, which is absent or very small in neurologically normal individuals, is abnormally large in this patient population. The results were clear. With swing‐phase down‐conditioning, the H‐reflex decreased much faster and farther than did the H‐reflex in all previous animal or human studies with the steady‐state protocol, and the decrease persisted for at least 6 months after conditioning ended. The H‐reflex decrease was accompanied by improvements in walking speed and in the modulation of locomotor electromyograph activity in proximal and distal muscles of both legs. These results provide new insight into the factors controlling spinal reflex conditioning; they suggest that the conditioning protocols targeting reflex function in a specific movement phase provide a promising new opportunity to enhance functional recovery after SCI or in other disorders., Key points In people or animals with incomplete spinal cord injury (SCI), changing a spinal reflex through an operant conditioning protocol can improve locomotion.All previous studies conditioned the reflex during steady‐state maintenance of a specific posture. By contrast, the present study down‐conditioned the reflex during the swing‐phase of locomotion in people with hyperreflexia as a result of chronic incomplete SCI. The aim was to modify the functioning of the reflex in a specific phase of a dynamic movement.This novel swing‐phase conditioning protocol decreased the reflex much faster and farther than did the steady‐state protocol in people or animals with or without SCI, and it also improved locomotion.The reflex decrease persisted for at least 6 months after conditioning ended.The results suggest that conditioning reflex function in a specific phase of a dynamic movement offers a new approach to enhancing and/or accelerating recovery after SCI or in other disorders.

Background Extracorporeal shock wave therapy (ESWT) has been suggested as a useful treatment for spasticity in patients with stroke. To date, most studies have used clinical or biomechanical assessments to determine the effects of treatment. Objectives To assess the effect of a single session of ESWT through ultrasonographic assessment on spasticity in patients with chronic stroke. Design Randomized controlled trial. Setting Rehabilitation center. Participants Eighteen patients with chronic stroke. Methods Participants were randomly assigned to an ESWT group (n = 9) or control group (n = 9). In the ESWT group, a single session of ESWT was given in the medial head of the gastrocnemius muscle of the spastic side at 4 Hz, 2000 shots with intensity of stimulation using energy of 0.1 mJ/mm 2 . Sham stimulation was provided by only making sound without putting the device into contact with the skin in the control group. The clinical trial registration number of this study is KCT0002582. Main Outcome Measures Participants were evaluated prior to the treatment, 30 minutes, 1 week, and 4 weeks after treatment. Clinical measures included Modified Ashworth Scale (MAS), passive range of motion (PROM), and Fugl-Meyer Assessment (FMA). As the primary outcome measure, the ultrasonographic measures were Achilles tendon length (ATL), muscle fascicle length (MFL), muscle thickness (MT), and pennation angle (PA). Results There were no significant differences between the 2 groups for any demographic or baseline measures. At all follow-up evaluations, improvement was shown in MAS as well as changes from baseline of ultrasonographic measures in the ESWT group compared to the control group. In the case of ultrasonographic measures, the difference between the group was greatest at 4 weeks. At follow-up of 4 weeks, between-group difference was 0.66 points ( P = .04) on MAS scores, 5.45 points ( P = .004) on ATL scores, 4.95 points ( P = .002) on MFL scores, 1.83 points ( P P Conclusions Some ultrasonographic measures of spasticity as well as MAS were improved after a single session of ESWT. Future studies with larger numbers of subjects compared with other spasticity metrics are necessary to further evaluate the treatment effect of ESWT. Level of Evidence I

The term "cerebral palsy mimic" is used to describe a number of neurogenetic disorders that may present with motor symptoms in early childhood, resulting in a misdiagnosis of cerebral palsy. Cerebral palsy describes a heterogeneous group of neurodevelopmental disorders characterized by onset in infancy or early childhood of motor symptoms (including hypotonia, spasticity, dystonia, and chorea), often accompanied by developmental delay. The primary etiology of a cerebral palsy syndrome should always be identified if possible. This is particularly important in the case of genetic or metabolic disorders that have specific disease-modifying treatment. In this article, we discuss clinical features that should alert the clinician to the possibility of a cerebral palsy mimic, provide a practical framework for selecting and interpreting neuroimaging, biochemical, and genetic investigations, and highlight selected conditions that may present with predominant spasticity, dystonia/chorea, and ataxia. Making a precise diagnosis of a genetic disorder has important implications for treatment, and for advising the family regarding prognosis and genetic counseling. © 2019 International Parkinson and Movement Disorder Society.

This review summarizes studies that examined the effectiveness of cannabinoids in treating spasticity, with a focus on understanding the relevance of the existing evidence to paediatric populations. MEDLINE, Embase, PsycINFO, and the Cochrane Library were searched to identify studies that examined the use of cannabinoids in spasticity. We identified 32 studies in adult and paediatric populations. Results were summarized by condition, with adult and paediatric studies considered separately. There is evidence from randomized controlled clinical trials that cannabinoids are more effective than placebo in reducing symptoms of spasticity in adults with multiple sclerosis. Most positive effects were based on patient-rated rather than clinician-rated measures, were modest in size, and should be considered in the context of the narrow therapeutic index of cannabinoids for spasticity and adverse effects. There were comparatively few, and no large studies, of spasticity in conditions other than multiple sclerosis. Few studies have been conducted in paediatric populations. Paediatric studies of spasticity provide low quality evidence and are inadequate to inform clinical practice. Cannabinoids have modest efficacy in reducing muscle spasticity in adults with multiple sclerosis. There is limited evidence of efficacy for cannabinoid use in other conditions, particularly in paediatric populations. Studies in paediatric populations have been of low quality and are insufficient to inform clinical practice.Esta revisión resume los estudios que examinaron la efectividad de los cannabinoides en el tratamiento de la espasticidad, con un enfoque en la comprensión de la relevancia de la evidencia existente para las poblaciones pediátricas. Se realizaron búsquedas en Medline, Embase, PsycINFO y la Biblioteca Cochrane para identificar estudios que examinaron el uso de cannabinoides en la espasticidad. Se identificaron 32 estudios en poblaciones adultas y pediátricas. Los resultados se resumieron por condición, con estudios en adultos y pediátricos considerados por separado. Existe evidencia de ensayos clínicos controlados aleatorios de que los cannabinoides son más efectivos que el placebo para reducir los síntomas de la espasticidad en adultos con esclerosis múltiple. La mayoría de los efectos positivos se basaron en las medidas clasificadas por el paciente en lugar de las clasificadas por el médico, fueron de tamaño modesto y deben considerarse en el contexto del estrecho índice terapéutico de los cannabinoides para la espasticidad y los efectos adversos. Hubo comparativamente pocos, y no hay estudios grandes, de espasticidad en afecciones distintas a la esclerosis múltiple. Se han realizado pocos estudios en poblaciones pediátricas. Los estudios pediátricos de espasticidad proporcionan evidencia de baja calidad y son inadecuados para informar la práctica clínica.CANABINÓIDES PARA O TRATAMENTO DA ESPASTICIDADE: Esta revisão sintetiza estudos que examinaram a efetividade de canabinóides no tratamento da espasticidade, com foco na compreensão da relevância da evidência existente para populações pediátricas. Medline, Embase, PsycINFO, e Cochrane Library foram pesquisados para identificar estudos que examinaram o uso de canabinóides na espasticidade. Identificamos 32 estudos em populações adultas e pediátricas. Os resultados foram sintetizados por condição com estudos em adultos e pediátricos considerados separadamente. Há evidência de ensaios clínicos randomizados controlados de que os canabinóides são mais efetivos do que placebos na redução de sintomas de espasticidade em adultos com esclerose múltipla. A maioria dos efeitos positivos foram baseados em medidas fornecidas por pacientes e não por clínicos, eram de tamanho modesto, e devem ser considerados no contexto do estreito índice terapêutico dos canabinóides para espasticidade e efeitos adversos. Houve comparativamente menos, e nenhum grande estudo, da espasticidade em condições diferentes da esclerose múltipla. Poucos estudos foram conduzidos em populações pediátricas. Estudos pediátricos da espasticidade fornecem baixa evidência de qualidade e são inadequados para informar a prática clínica.

Key Clinical Message MED23 deficiency causes the autosomal recessive Intellectual Disability (ID). Here we report an Iranian case with nonsyndromic ID presenting with developmental delay, microcephaly, hypotonia, severe ID, speech delay, and spasticity, who was homozygous for the novel MED23 c.670C>G variant. These results expand the clinical and mutation spectrum of MED23 deficiency.

This case describes a 16-year-old boy who suffered a severe traumatic brain injury. The patient gradually recovered but developed debilitating spasticity mainly in left triceps muscle, abnormal positioning of shoulder girdle complex, and shoulder pain. Phenol (6%) was injected to axillary and radial nerves under the guidance of electrical stimulation and ultrasound imaging at 10 weeks after the initial injury. After injection, triceps spasticity and shoulder pain were immediately reduced, and abnormal positions of shoulder girdle complex and elbow joint gradually returned to functional limits over 2 weeks. This case suggests that phenol neurolysis for spasticity management in early recovery could yield functional recovery.Level of Evidence: V.

Shoulder pain is commonly associated with spasticity of the rotator cuff muscles including the subscapularis (SSC). The aim of this study was to elucidate the intramuscular innervation pattern of the SSC using the modified Sihler's staining technique to facilitate the targeting of botulinum neurotoxin (BoNT) injections to alleviate shoulder spasticity. Ten SSC specimens (mean age, 81.5 years) were used in this study. Modified Sihler's staining was used to clarify the muscle and to stain the intramuscular nerves. Their extramuscular and intramuscular innervation patterns were examined. The upper subscapular, lower subscapular, thoracodorsal, and axillary nerves (USN, LSN, TDN, and AXN) innervated the SSC in 100%, 80%, 20%, and 40% of specimens, respectively. There was an anastomosis between the USN and LSN in the central portion of the SSC in more than half of the cases. The USN innervated the overall portion of the muscle. In contrast, the additional branches from the TDN and AXN innervated the inferior SSC portion. The superficial branches of the USN were mostly distributed in the superior SSC portion while the deep branches were distributed in the inferior portion. As a major intramuscular nerve within the SSC, the USN should be targeted by a BoNT injection. Regarding the USN distribution, the aim should be to spread the BoNT injectate within the central SSC portion. For supplementary injection to the AXN, the lateral approach would be more appropriate than alternatives. A physician performing a BoNT injection should consider the intramuscular innervation of the SSC portion. Clin. Anat. 32:110-116, 2019. © 2018 Wiley Periodicals, Inc.