Background: The assessment of gait disorders in patients with neuromotor conditions, such as cerebral palsy (CP), has been a focus of clinical and research attention, with electromyography (EMG) offering a nuanced understanding of neurological and neuromuscular disorders. However, the interpretation of EMG data in the context of gait analysis remains challenging due to the complexity of neuromotor dynamics and variability in assessment methodologies.
Research Question: To which consensus can we get in a group of experts in the fields of neurological and neuromuscular disorders, biomechanics, and clinical gait analysis to establish standardized protocols and a common language for the measurement and analysis of EMG data in gait disorders, particularly in people living with CP?
Methods: A three-round Delphi process was conducted from February to September 2023 to gather opinions of 53 experts on the use of surface EMG data during gait in the context of CP.
The clinical effects of a serious game with electromyography feedback (EMGs_SG) and physical therapy (PT) was investigated prospectively in children with unilateral spastic cerebral palsy (USCP). An additional aim was to better understand the influence of muscle shortening on function. Thirty children with USCP (age 7.
View Article and Find Full Text PDFChilds Nerv Syst
April 2024
Background: Children with cerebral palsy develop foot deformities due to a combination of factors including muscle shortening, hypertonia, weakness, and cocontraction of muscles acting at the ankle joint resulting in an altered gait pattern. We hypothesized these factors affect the peroneus longus (PL) and tibialis anterior (TA) muscles couple in children who develop equinovalgus gait first followed by planovalgus foot deformities. Our aim was to evaluate the effects of abobotulinum toxin A injection to the PL muscle, in a cohort of children with unilateral spastic cerebral palsy and equinovalgus gait.
View Article and Find Full Text PDFIntroduction/aims: In adult spinal muscular atrophy (SMA), the motor unit number index (MUNIX) has been shown to be an useful electrophysiological biomarker. This study evaluated the feasibility and the clinical relevance of using the MUNIX technique for patients with pediatric SMA (Ped-SMA) and correlated MUNIX results with clinical scores.
Methods: Fourteen patients with type II Ped-SMA (11 females; median age 11 y [interquartile range (IQR), 4.
Introduction: Standing radiographs allow analysis of spinal segments and their relative positioning. However, it requires a specific positioning influencing spinal alignment. Knowledge of trunk movements when walking is therefore an essential step to evaluate dynamic sagittal balance.
View Article and Find Full Text PDFIntroduction: In adolescent idiopathic scoliosis (AIS) patients, mechanical consequences of posterior spinal fusion within the spine remain unclear. Through dynamic assessment, gait analysis could help elucidating this particular point. The aim of this study was to describe early changes within the spine following fusion with hybrid instrumentation in adolescents with idiopathic scoliosis, using gait analysis MATERIALS AND METHODS: We conducted a single-centre prospective study including AIS patients scheduled for posterior spinal fusion (PSF) using hybrid instrumentation with sublaminar bands.
View Article and Find Full Text PDFBackground: work still needs to be done to measure the impact of sialorrhea on quality of life and define the efficacy of different therapies. The Drooling Impact Scale showed good validity and sensitivity to change, especially after botulinum toxin injection. The aim of this study is to present its French translation and to explore its validity, reliability and responsiveness to change in a group of children with Cerebral Palsy.
View Article and Find Full Text PDFBackground: GLE1 (GLE1, RNA Export Mediator, OMIM#603371) variants are associated with severe autosomal recessive motor neuron diseases, that are lethal congenital contracture syndrome 1 (LCCS1, OMIM#253310) and congenital arthrogryposis with anterior horn cell disease (CAAHD, OMIM#611890). The clinical spectrum of GLE1-related disorders has been expanding these past years, including with adult-onset amyotrophic lateral sclerosis (ALS) GLE1-related forms, especially through the new molecular diagnosis strategies associated with the emergence of next-generation sequencing (NGS) technologies. However, despite this phenotypic variability, reported congenital or ALS adult-onset forms remain severe, leading to premature death.
View Article and Find Full Text PDFIntroduction: In this study we determined the clinical, paraclinical, and treatment-related features of juvenile myasthenia gravis (JMG) as well as the clinical course in a cohort of French children.
Methods: We conducted a retrospective study of 40 patients with JMG at 2 French pediatric neurology departments from April 2004 to April 2014.
Results: Among the patients, 70% had generalized JMG, 52% had positive acetylcholine receptor antibodies, 8% had muscle-specific kinase antibodies, and 40% were seronegative.
Introduction: The Mitofusin 2 gene (MFN2), which encodes a mitochondrial membrane protein, is known to be the first cause of autosomal dominant Charcot-Marie-Tooth disease type 2 (CMT2) with early onset. This gene is involved in typical CMT2A and in more atypical phenotypes as optic atrophy or spastic paraplegia. CMT2 refers to inherited axonal polyneuropathy, which associates progressive peripheral motor and sensory neuropathy, a family history consistent mainly with autosomal dominant inheritance, and normal nerve conduction velocities.
View Article and Find Full Text PDFObjectives: To describe 31 children presenting a CIDP; to compare patients with rapid-onset disease vs. patients with slow-onset disease, a rapid-onset disease being defined by a time to peak impairment of less than 8 weeks.
Study Design: A retrospective chart review identified 31 patients completing criteria for childhood CIDP, with 24 "confirmed CIDP" and 7 "possible CIDP".
Acetabular cup orientation (inclination and anteversion) is a fundamental topic in orthopaedics and depends on pelvis tilt (positional parameter) emphasising the notion of a safe range of pelvis tilt. The hypothesis was that pelvic incidence (morphologic parameter) could yield a more accurate and reliable assessment than pelvis tilt. The aim was to find out a predictive equation of acetabular 3D orientation parameters which were determined by pelvic incidence to include in the model.
View Article and Find Full Text PDFBackground: After the age of 4 years, drooling becomes pathological and impacts the quality of life of children with cerebral palsy. Intraglandular injection of Botulinum toxin is one of the treatments available to limit this phenomenon.
Aims: The objectives of this review were to validate the efficacy of Botulinum toxin injections for drooling in children with cerebral palsy, determine recommendations and identify potential side effects.
Mutations of SCN4A encoding the skeletal muscle sodium channel Nav 1.4 cause several types of disease, including sodium channel myotonias. The latter may be responsible for neonatal symptoms, including severe neonatal episodic laryngospasm (SNEL).
View Article and Find Full Text PDFWe compare adult and intact neonatal pelves, using a pelvic sagittal variable, the angle of sacral incidence, which presents significant correlations with vertebral curvature in adults and plays an important role in sagittal balance of the trunk on the lower limbs. Since the lumbar curvature develops in the child in association with gait acquisition, we expect a change in this angle during growth which could contribute to the acquisition of sagittal balance. To understand the mechanisms underlying the sagittal balance in the evolution of human bipedalism, we also measure the angle of incidence of hominid fossils.
View Article and Find Full Text PDFIn children with hemiplegia, it is important to distinguish between equinus with hindfoot varus (equinovarus) or valgus (equinovalgus). Premature onset of medial gastrocnemius (GM) EMG in individuals with equinus is well documented. Premature onset of Peroneus longus (PL) EMG has been described in neurologically impaired adults with equinovalgus, but not in children.
View Article and Find Full Text PDFThe importance of the sacral pelvic incidence (SPI) in relation to individual variations of sagittal spinal curvature has become well-recognised. We attempted to determine the relationship between SPI and acetabular orientation. The three-dimensional coordinates of 47 homologous points were observed on 51 adult anatomical pelvises (26 female and 25 male).
View Article and Find Full Text PDFThe aim of this study was to assess pelvic asymmetry (i.e. to determine whether the right iliac bone and the right part of the sacrum are mirror images of the left), both quantitatively and qualitatively, using three-dimensional measurements.
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