scholarly journals P.026 Acute lower limb spasticity: Stiff person syndrome responsive to immunomodulatory therapy in an adolescent female

2017 ◽  
Vol 44 (S2) ◽  
pp. S20-S20
Author(s):  
R Ogilvie ◽  
H Kolski
Keyword(s):  
Knee Flexion ◽  
Acute Onset ◽  
Left Knee ◽  
Stiff Person Syndrome ◽  
Limb Spasticity ◽  
Lower Limb Spasticity ◽  
Voluntary Muscle Contraction ◽  
Continuous Motor Unit Activity ◽  
Almost Continuous

Background: Stiff person syndrome (SPS) is a rare disorder presenting with progressive stiffness and spasms of the musculature of the trunk and limbs. SPS is reported very rarely in children and adolescents, with 5 cases over 25 years in a recent 99 patient cohort. Methods: Case Study Results: Herein we report a 15 year old female, presenting with acute onset of rapidly progressive spasticity of the lower extremities. Initial exam was remarkable for markedly limited left knee range of motion, in addition to asymmetrical knee spastic catch and hyper-reflexia. EMG revealed almost continuous motor unit activity which dissipated with voluntary muscle contraction. Diagnosis was confirmed by high titres of glutamate decarboxylase (GAD65) antibodies >25,000 units/ml. The patient was initially treated with IVIG, baclofen, and diazepam followed by IV methylprednisolone, with mild subjective improvements. One day following the first rituximab treatment, she achieved spontaneous knee flexion and regained the ability to ambulate independently. There is a residual spastic catch at the knees. Conclusions: This case highlights that SPS, albeit extremely rare, should be considered in the differential diagnosis of acquired spasticity in children. Also noteworthy is the relatively rapid resumption of function with aggressive immunomodulatory treatments in this historically devastating disorder.

Center of Pressure Trajectory and Spatiotemporal Gait Parameters When Walking with Limited Knee Flexion

2021 ◽  
Vol 65 (1) ◽  
pp. 1490-1493
Author(s):  
Seobin Choi ◽  
Jieon Lee ◽  
Gwanseob Shin
Keyword(s):  
Knee Flexion ◽  
Stance Phase ◽  
Center Of Pressure ◽  
Dynamic Balance ◽  
Left Knee ◽  
Step Width ◽  
Normal Walking ◽  
Gait Stability ◽  
Gait Parameters ◽  
Stiff Knee

Stiff-knee, which indicates reduced range of knee flexion, may decrease gait stability. Although it is closely related to an increase in fall risk, the effect of limited knee flexion on the balance capacity during walking has not been well studied. This study aimed at examining how walking with limited knee flexion would influence the center of pressure (COP) trajectory and spatiotemporal gait parameters. Sixteen healthy young participants conducted four different walking conditions: normal walking and walking with limited knee flexion of their left knee up to 40 and 20 degrees, respectively. Results show that the participants walked significantly (p<0.05) slower with shorter stride length, wider step width, less cadence, and decreased stance phase when walking with limited knee flexion, compared to normal walking. The increase in the asymmetry and variability of the COP was also observed. It indicates that limited knee flexion during walking might affect the dynamic balance.

Poster 75: Exploring Real-World OnabotulinumtoxinA Utilization Patterns for the Treatment of Lower Limb Spasticity: The Adult Spasticity International Registry (ASPIRE) Study

PM&R ◽  
2018 ◽  
Vol 10 ◽  
pp. S31-S31
Author(s):  
Alberto Esquenazi ◽  
Wolfgang H. Jost ◽  
Ganesh Bavikatte ◽  
Daniel S. Bandari ◽  
Michael C. Munin ◽  
...  
Keyword(s):  
Real World ◽  
Lower Limb ◽  
International Registry ◽  
Utilization Patterns ◽  
Limb Spasticity ◽  
Lower Limb Spasticity

scholarly journals Phenoconversion from Spastic Paraplegia to ALS/FTD Associated with CYP7B1 Compound Heterozygous Mutations

Genes ◽  
2021 ◽  
Vol 12 (12) ◽  
pp. 1876
Author(s):  
Julian Theuriet ◽  
Antoine Pegat ◽  
Pascal Leblanc ◽  
Sandra Vukusic ◽  
Cécile Cazeneuve ◽  
...  
Keyword(s):  
Lower Limb ◽  
Clinical Symptoms ◽  
Compound Heterozygous ◽  
Spastic Paraplegia ◽  
The Third ◽  
Limb Spasticity ◽  
Lower Limb Spasticity ◽  
Compound Heterozygous Mutations ◽  
Biallelic Mutations ◽  
Lateral Sclerosis

Biallelic mutations in the CYP7B1 gene lead to spastic paraplegia-5 (SPG5). We report herein the case of a patient whose clinical symptoms began with progressive lower limb spasticity during childhood, and who secondly developed amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD) at the age of 67 years. Hereditary spastic paraplegia (HSP) gene analysis identified the compound heterozygous mutations c.825T>A (pTyr275*) and c.1193C>T (pPro398Leu) in CYP7B1 gene. No other pathogenic variant in frequent ALS/FTD causative genes was found. The CYP7B1 gene seems, therefore, to be the third gene associated with the phenoconversion from HSP to ALS, after the recently described UBQLN2 and ERLIN2 genes. We therefore expand the phenotype associated with CYP7B1 biallelic mutations and make an assumption about a link between cholesterol dyshomeostasis and ALS/FTD.

scholarly journals The effectiveness of additional core stability exercises in improving dynamic sitting balance, standing balance, lower-limb spasticity, falls and gait in subacute stroke patients (CORE-trial). Study protocol for a Randomized Controlled Trial

2021 ◽  
Author(s):  
Rosa Cabanas-Valdés ◽  
Lidia Boix-Sala ◽  
Montserrat Grau-Pellicer ◽  
Juan Antonio Guzmán-Bernal ◽  
Fernanda Maria Caballero-Gómez ◽  
...  
Keyword(s):  
Lower Limb ◽  
Daily Living ◽  
Controlled Trial ◽  
Core Stability ◽  
Standing Balance ◽  
Stroke Patients ◽  
Randomized Controlled ◽  
Limb Spasticity ◽  
Lower Limb Spasticity ◽  
Dynamic Sitting

Abstract BackgroundTrunk impairment produces disorders of motor control, balance, and gait that are correlated with increased risk of falls and reduced mobility in stroke survivors. This creates disability and dependency to perform their activities of daily living. Alterations in body alignment occur, requiring treatment strategies focused on improving the postural control. bearing. Core stability exercises (CSE) are a good strategy to improve local strength of trunk, dynamic sitting, standing balance, and gait. There is some evidence about its effectiveness but it is still necessary to run a large multicenter trial to ratify that existing evidence.MethodsThis is a single-blind multicenter randomized controlled trial. Two parallel groups are compared and both perform the same type of therapy. A control group (CG) (n=110) performs conventional physiotherapy (CP) (1 hour per session) focused on improving balance. An experimental group (EG) (n=110) performs CSE (30 minutes) in addition to CP (30 minutes) (1 hour/session in total). EG is divided in two subgroups, in which only half of patients (n=55) perform CSE plus transcutaneous electrical nerve stimulation (TENS). Primary outcome measures are dynamic sitting, assessed by Spanish-version of Trunk Impairment Scale and stepping, assessed by Brunel Balance Assessment. Secondary outcomes are postural control, assessed by Postural Assessment Scale for Stroke patients; standing balance and risk of fall assessed by Berg Balance Scale; gait speed by BTS G-Walk (accelerometer); rate of falls, lower-limb spasticity by Modified Ashworth Scale; activities of daily living by Barthel Index; and quality of life by EQ-5D-5L. These are evaluated at baseline (T0), at 3 weeks (T1), at 5 weeks -at the end of the intervention (T2), at 17 weeks (T3) and at 29 weeks (T4). Study duration per patient is 29 weeks (a 5-week intervention, followed by a 24-week post-intervention). DiscussionThe study will provide useful information on the short and long term effects of a physiotherapy rehabilitation program based on core stability exercises performed in subacute phase.Trial registrationClinicalTrials.gov Identifier NCT03975985. Data registration June 5th, 2019. Retrospectively registered. Date of registration in primary registry: June 5, 2019. Protocol version 1

scholarly journals Beneficial effects of combined rTMS and physical rehabilitation for spasticity in multiple sclerosis

2021 ◽  
Author(s):  
Frederico Arriaga Criscuoli de Farias ◽  
Carolina Matté Dagostini ◽  
Carolina Dalla Santa Dal Moro ◽  
Carolina Odorizzi Magno Nunes ◽  
Mariana Moreira Rizzolli ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Physical Therapy ◽  
Repetitive Transcranial Magnetic Stimulation ◽  
Knee Extension ◽  
Modified Ashworth Scale ◽  
Relapsing Remitting ◽  
Limb Spasticity ◽  
Lower Limb Spasticity ◽  
Partial Weight

Background: spasticity affects up to 90% of multiple sclerosis (MS) patients, being a major cause of disability. Repetitive transcranial magnetic stimulation (rTMS) appears to be a potential treatment, but literature is scarce. Objectives: to evaluate the effects of combined rTMS and physical therapy for spasticity in MS patients. Methods: relapsing-remitting MS patients suffering from lower limb spasticity were included. The rTMS protocol consisted of 20 sessions of 18 trains of 50 stimuli at 5Hz and at 100% of rest potential threshold, with 10 seconds per train and 40 second pauses, amounting for 900 pulses (15 minutes) with a Neurosoft- MS/D double coil. Optimal coil positioning was determined by motor responses in the contralateral soleus muscle. The physical therapy protocol included active exercising – stationary bicycle, step climbing with partial weight suspension – followed by mild gradual resistance exercising for knee extension and hip and foot flexion, proprioceptive exercises and active stretching. The primary outcome was improvement on the Modified Ashworth Scale (MAS) score at baseline and 4 weeks after treatment. Results: five female patients were recruited. At the 4-week follow-up, 4 out the 5 patients had a significant improvement regarding spasticity. There was a reduction in mean MAS score from 2.30 to 1.42. Patient 1 had a MAS score reduction from 2 to 1 (50%). Patient 2 from 3 to 1 (66%), patient 3 from 4 to 3 (25%) and patient 4 from 0.5 to 0.125 (75%). Conclusion: combined rTMS and physical therapy protocols presents as a beneficial treatment modality for MS spasticity.

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