spastic

Scoliosis is a common complication of neuromuscular disorders. These patients are frequently recalcitrant to nonoperative treatment. When treated surgically, they have the highest risk of complications of all forms of scoliosis. While recent studies have shown an improvement in the rate of complications, they still remain high ranging from 6.3 to 75% depending upon the underlying etiology and the treatment center (Mohamad et al. in J Pediatr Orthop 27:392-397, 2007; McElroy et al. in Spine, 2012; Toll et al...

A man in his 30s was referred to neurology with right-sided paraesthesia, tremors, chest pain and lower urinary tract and erectile dysfunction. He had a medical history of left acetabular dysplasia, and subjective memory impairment, the latter being in the context of depression and chronic pain with opioid use. There was no notable family history. On examination, he had a spastic paraparesis. Imaging revealed atrophy of the thoracic spine. Lumbar puncture demonstrated a raised protein but other constituents were normal, including no presence of oligoclonal bands...

Modulation or stimulation of the nerves supplying the lower urinary tract is a possible treatment option for dysfunction of the lower urinary tract, pelvic floor and rectum if conservative or minimally invasive treatment approaches fail. This overview shows the possibilities and limitations of sacral neuromodulation, sacral deafferentation with sacral anterior root stimulation and conus deafferentation.Sacral neuromodulation (SNM) is a procedure for the treatment of refractory pelvic floor dysfunction of various origins (idiopathic, neurogenic or post-operative), such as overactive bladder, non-obstructive retention and faecal incontinence...

INTRODUCTION: Cerebral palsy (CP) is the most common cause of pediatric motor disability. While epidemiological data are widespread in high-income countries, corresponding data in low-income countries in Sub-Saharan Africa are still rare. This study aimed to estimate the prevalence of CP in Northern Benin, a French-speaking low-income country in Sub-Saharan Africa. METHODS: This study was a community-based door-to-door study involving children younger than 18 years old, in Parakou, a semi-urban city in Benin...

Casting is an effective adjunctive treatment which increases the effects of botulinum toxin on patients with spasticity. The evidence for casting as an adjunctive therapy post-botulinum toxin has been well documented in the literature. However, standardized casting protocols are lacking, with no published descriptions of upper limb casting application post-botulinum toxin injection for spasticity. In this paper we will describe our experience regarding the application of upper limb casting as an adjunctive therapy for elbow spasticity, detailing indications, contraindications and description of our protocol (materials required and technique)...

AIM: To assess the predictive capabilities of the Baby Observational Selective Control AppRaisal (BabyOSCAR) tool, administered at 3 months corrected age, in determining spastic cerebral palsy (CP) outcome, functional abilities, and body topography at 2 years of age or later. METHOD: Independent joint motions were measured at age 10 to 16 weeks from video recordings of spontaneous movement using BabyOSCAR in a sample of 75 infants. All included infants had known 2-year outcomes (45 with spastic CP and 30 without CP) including Gross Motor Functional Classification System (GMFCS) levels and CP body distribution...

PURPOSE: This bibliometric study explores cerebral palsy (CP) research from 2003 to 2022 to reveal the topic hotspots and collaborations. METHODS: We retrieved studies on CP from the Web of Science Core Collection from 2003 to 2022 and then used CiteSpace and Bibliometrix to perform a bibliometric analysis and attain knowledge mapping, including publication outputs, funding, journals, authors, institutions, countries/territories, keywords, collaborative relationships, and topic hotspots...

INTRODUCTION: Major Depressive Disorder (MDD) is one of the commonest mental disorders affecting more than 250 million people globally. Patients with chronic illnesses had higher risks for developing MDD than the general population. Neurolathyrism is a chronic illness characterized by lifelong incurable spastic paralysis of lower extremities; causing permanent disability. It is highly prevalent in Dawunt district, Ethiopia; with a point prevalence of 2.4%. Despite this, there were no previous studies assessing the prevalence of MDD among patients with neurolathyrism in Ethiopia...

INTRODUCTION: Powered robotic exoskeleton (PRE) physiotherapy programmes are a relatively novel frontier which allow patients with reduced mobility to engage in supported walking. Research is ongoing regarding their utility, risks, and benefits. This article describes the case of two fractures occurring in one patient using a PRE. CASE: We report the case of a 54 year old man who sustained bilateral tibial fractures while using a PRE, on a background of T10 AIS A SCI...

INTRODUCTION: Individuals with cerebral palsy (CP) often present with altered motor control. This can be assessed selectively during sitting/lying with the Selective Control Assessment of the Lower Extremity (SCALE), or dynamically with the dynamic motor control index during walking (walk-DMC). Both approaches suggest that altered selective motor control relate to larger gait deviations. RESEARCH QUESTION: Does the walk-DMC provide valuable information in addition to the SCALE for estimating gait deviations in individuals with CP...

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Gangliosides, sialic acid-containing glycosphingolipids, are abundant in cell membranes and primarily involved in controlling cell signaling and cell communication. The altered ganglioside pattern has been demonstrated in several neurodegenerative diseases, characterized during early-onset or infancy, emphasizing the significance of gangliosides in the brain. Enzymes required for the biosynthesis of gangliosides are linked to several devastating neurological disorders, including Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegia (HSP)...

INTRODUCTION: Recently, interest in quantifying upper limb function in cerebral palsy has grown. However, the lack of reference tasks and protocols, have hindered the development of quantified movement analysis in clinical practice. This study aimed to evaluate existing instrumented assessments of upper limb function in cerebral palsy, with a focus on their clinical applicability, to identify reasons for the lack of adoption and provide recommendations for improving clinical relevance and utility...

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...

The most severe form of spastic cerebral palsy (CP), which affects the arms and legs and often the face, is known as spastic quadriplegia. In addition to other developmental disabilities such as intellectual disability and seizures, it can cause difficulty in walking. Children with CP often have seizures as a result of brain injury, and spastic quadriplegic CP is typically associated with global developmental delay. For the purpose of addressing the unique motor and functional challenges associated with spastic quadriplegia, neurophysiotherapy is essential...

BACKGROUND: The significance of HTLV-1 proviral load as a prognostic biomarker in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) has been a subject of controversy. This study aims to assess the impact of HTLV-1 proviral load (PVL) on the clinical outcome in patients with HAM/TSP. METHODS: An absolute quantitative HTLV-1 PVL RT-qPCR, TaqMan method was developed with 100% sensitivity and specificity. Then, from 2005-2018, the HTLV-1 PVL of 90 eligible newly diagnosed HAM/TSP patients were assessed for demographic, clinical symptoms and their associations with HTLV-1-PVL...

Lower limb spasm and spasticity may develop following spinal cord injury (SCI), causing hyper-excitability and increased tone, which can impact function and quality of life. Pharmaceutical interventions for spasticity may cause unwanted side effects such as drowsiness and weakness. Invasive and non-invasive electrical stimulation has been shown to reduce spasticity without these side effects. The aim of this study was to investigate the effect of sacral afferent stimulation (SAS), through surface electrical stimulation of the dorsal genital nerve ( N  = 7), and through implanted electrodes on the sacral afferent nerve roots, on lower limb spasm and spasticity ( N  = 2)...

UNLABELLED: Lysosomes and related precursor organelles robustly build up in swollen axons that surround amyloid plaques and disrupted axonal lysosome transport has been implicated in worsening Alzheimer's pathology. Our prior studies have revealed that loss of Adaptor protein-4 (AP-4) complex function, linked primarily to Spastic Paraplegia (HSP), leads to a similar build of lysosomes in structures we term "AP-4 dystrophies". Surprisingly, these AP-4 dystrophies were also characterized by enrichment of components of APP processing machinery, β-site cleaving enzyme 1 (BACE1) and Presenilin 2...

AIM: To describe the development of an observational measure of spontaneous independent joint motion in infants with spastic cerebral palsy (CP), the Baby Observational Selective Control AppRaisal (BabyOSCAR), and to test its convergent validity and reliability. METHOD: A retrospective sample of 75 infants (45 with spastic CP and 30 without CP) at 3 months of age were scored with the BabyOSCAR and compared with diagnosis of spastic CP, limbs affected, and Gross Motor Function Classification level at 2 years of age or later for convergent validity using t-tests, Kruskal-Wallis tests, and Spearman's rank correlation coefficients...

INTRODUCTION: Spastic paraplegia (SPG) is a heterogenous group of neurodegenerative disorders, that may include ocular involvement. Here we report the clinical data of a patient with late-onset Kjellin syndrome, a peculiar form of hereditary SPG with macular dystrophy. MATERIALS AND METHODS: Clinical, functional and multimodal retinal imaging data were collected. Genetic testing was performed by Whole Exome Sequencing (WES). RESULTS: A 52-year-old female patient with SPG of unknown origin was referred for a progressive visual acuity loss...