Lower Extremity Muscle Weakness Research Articles

Ralstoniapickettii bloodstream infection in the patient with Guillain-Barre syndrome under plasmapheresis

Ralstonia pickettii is a rare Gram-negative opportunistic bacterium that causes rare infections such as bacteremia, neonatal sepsis, endocarditis, and meningitis in hospitalized or immunocompromised patients. In this study, we identified and reported bloodstream infection caused by R. pickettii in a 15 -year-old boy patient with an autoimmune disease, Guillain-Barré syndrome, under plasmapheresis and intravenous immune globulin (IVIG) therapy. He was referred for admission to the neurology center of the teaching hospital of Shiraz, Iran for inability to walk, and lower extremity muscle weakness. After he was treated with plasmapheresis once during hospitalization, and after severe fever besides shivering blood cultures using BACT/ALERT®3D instrument were positive for R. pickettii.According to antibiotic susceptibility test reports, Ciprofloxacin (5 μg) was prescribed. Fortunately, after starting antibiotic treatment, blood culture results reported no growth after 5 days. Indeed, the patient was infected with nosocomial hepatitis A and URSOBIL (300 mg/BID/Po) was administered. Hence, after reporting the infection occurrence to the hospital infection control unit, initial and possible measures such as device infection control, replacement of potentially polluted plasmapheresis fluids, disinfecting the environment and replacing old sterile washing water with new sources were carried out in plasmapheresis unit. In conclusion, R. pickettii is a rare nosocomial infection that is responsible for the contamination of medical equipment, especially in hemodialysis, plasmapheresis devices and sterile solutions. Also, it is suggested that the role and importance of rare environmental bacteria as the causative agents of human infections should not be ignored in medical centers.

Reduced oxygen desaturation in the vastus lateralis of chronic stroke survivors during graded muscle contractions

ABSTRACT Background Few studies have examined changes in skeletal muscle physiology post-stroke. This study examined changes in tissue oxygen saturation (StO2) of the vastus lateralis (VL) muscle of stroke survivors and age-matched control participants during maximal and submaximal isometric contractions of the knee extensor muscles. Objectives We hypothesized that tissue oxygen desaturation (ΔStO2) during knee extensor muscle contractions would be less in the VL in the paretic vs. the non-paretic and control legs. Methods Ten chronic stroke survivors (>6 months post-stroke) with lower extremity muscle weakness and 10 age-matched controls completed this prospective cohort study. Maximum voluntary contractions (MVCs) of the knee extensor muscles were assessed with a Biodex dynamometer and StO2 of the VL was measured using near-infrared spectroscopy. Results In the paretic leg of the stroke survivors little change in StO2 of the VL was observed during an MVC (ΔStO2 = -1.7 ± 1.8%) compared to the non-paretic (ΔStO2 = -5.1 ± 6.1%; p < 0.05) and control legs (ΔStO2 = -14.4 ± 8.8%; p < 0.05 vs. paretic and non-paretic leg). These differences remained when normalizing for strength differences between the legs. Compared to controls, both the paretic and non-paretic VL showed pronounced reductions in ΔStO2 during ramp and hold contractions equal to 20%, 40%, or 60% of the MVC (p < 0.05 vs. controls at all load levels). Conclusions These results indicate that oxygen desaturation in response to isometric muscle contractions is impaired in both the paretic and non-paretic leg muscle of stroke survivors compared to age-matched controls, and these differences are independent of differences in muscle strength.

Safety, walking ability, and satisfaction outcomes of the NEURO TRONIC stance-control knee-ankle-foot orthosis (SCKAFO): A comparative evaluation to the E-MAG active SCKAFO.

Stance control knee-ankle-foot orthoses (SCKAFOs) ensure knee stability by locking during stance while allowing knee flexion during swing. Differences in function of the knee joints and building principles between devices may affect their effectiveness. To investigate the preliminary effectiveness of a NEURO TRONIC on safety outcomes, net energy cost (EC), and user experiences in individuals already using an E-MAG Active SCKAFO. Prospective uncontrolled intervention study. A convenience sample of 10 subjects with flaccid lower extremity muscle weakness, including the quadriceps, due to neuromuscular disorders already using an E-MAG Active SCKAFO were provided with a newly fabricated NEURO TRONIC SCKAFO. Outcomes included knee joint locking failures and unlocking failures (ULFs) (i.e., percentage of steps the knee joint failed to lock/unlock) when walking under challenging conditions on an instrumented treadmill while wearing a safety harness; net EC (J/kg per meter) assessed with a 6-min walk test at comfortable speed; 3D gait kinematics and kinetics; and patient-reported outcomes. No differences between devices were found for knee joint locking failures (both devices 0%) and ULFs (9.9% for the NEURO TRONIC vs. 13.9% for the E-MAG Active SCKAFO). The mean (standard deviation) net EC with the NEURO TRONIC SCKAFO was 8.2% (from 3.68 [0.81] to 3.38 [0.75] J/kg per meter, p = 0.123) lower, although not significantly, compared with that with the E-MAG Active SCKAFO. Significant improvements with the NEURO TRONIC SCKAFO were found for ankle power ( p = 0.003), perceived walking effort ( p = 0.014), and reported falls ( p = 0.034). Both the NEURO TRONIC SCKAFO and the E-MAG Active SCKAFO were safe in terms of knee joint locking, while ULFs were frequent with both devices. The net EC with the NEURO TRONIC SCKAFO decreased, although not significantly, by 8.2%, likely due to insufficient power. Perceived walking effort was in favor of the NEURO TRONIC SCKAFO.

Phase II Trial of Duvelisib Maintenance after Autologous Stem Cell Transplant in T-Cell and B-Cell Non-Hodgkin Lymphomas: Results of Safety Lead in

Introduction Duvelisib is a potent phosphoinositide-3-kinase delta (PI3K-δ) and gamma (PI3K-γ) inhibitor which has demonstrated efficacy in T-cell lymphomas (TCL) and low-grade B-cell Non-Hodgkin Lymphomas (B-NHL). It exerts its effect through inhibition of malignant cell proliferation and differentiation, as well as disruption of the tumor microenvironment. We aimed to evaluate the safety and efficacy of one year of duvelisib maintenance after consolidative autologous stem cell transplant (autoSCT) in patients with TCL and B-NHL. Methods Patients with TCL or B-NHL who received standard consolidative autoSCT began duvelisib maintenance after count recovery (approximately 30 days after transplant) and continued for 11 cycles (one year after transplant). Duvelisib started at 25 mg orally twice daily for two 28-day cycles (schedule 1), followed by 25 mg twice daily for 14 days on/ 14 days off (schedule 2) if the patient was in complete remission (CR) at day +100 or if intolerable toxicities developed. Patients in partial remission (PR) at day +100 continued schedule 1, and those with stable (SD) or progressive disease (PD) were taken off study. Results Twelve patients have been enrolled, starting in July 2020. Histologic subtypes were transformed follicular lymphoma (FL, n= 5), peripheral T-cell lymphoma (PTCL, n= 1), anaplastic large cell lymphoma (ALCL, n= 3), and angioimmunoblastic T-cell lymphoma (AITL, n= 3). Seven patients were male, median age was 62 (range, 25-71), and 10 patients had stage III/IV disease at diagnosis. Pre-transplant, 11 patients were in CR and 1 patient was in PR. Median follow-up was 23.7 months and median duration of duvelisib treatment was 8.7 months. Following enrollment of 7 patients, 2 patients developed grade 3 toxicities (elevated liver enzymes and diarrhea, respectively), prompting them to come off study. Patients were enrolled at schedule 2 thereafter, with subsequent improvement in tolerability. Most common adverse events (AEs) were hematologic, gastrointestinal, and hepatic (Table. 1). Grade 2-3 AEs probably related to treatment were seen in 6 patients: neutropenia (n= 2), lymphopenia (n= 1), diarrhea (n= 2), and elevated liver enzymes (AST/ALT) (n= 1). There were no grade 4-5 AEs and no deaths related to treatment. Permanent discontinuation of duvelisib due to grade 3 AEs occurred in 3 patients: elevated liver enzymes (AST/ALT), diarrhea, and lower extremity muscle weakness, respectively. The latter was deemed unlikely related to treatment and symptoms resolved upon follow-up. Of the 12 patients enrolled, 4 have relapsed (TCL, n= 2 and B-NHL, n= 2) and proceeded to additional treatment. All patients remain alive to date. Conclusions Duvelisib maintenance for up to one year after consolidative autoSCT in patients with TCL or B-NHL is safe and well tolerated at a dose schedule of 25 mg twice daily for 14 days on/ 14 days off, of 28-day cycles. Enrollment of patients undergoing consolidative autoSCT for TCL is ongoing to evaluate the efficacy of duvelisib maintenance.

Delayed radiation-induced motor neuron syndrome: A case report.

Delayed radiation-induced motor neuron syndrome (DRIMNS) is an atypical motor neuron disorder that develops months or years after radiation therapy. In this study we present a case of DRIMNS that developed forty years after radiotherapy and to discuss differential diagnoses. A 56-year-old male patient was admitted to our clinic with complaints of increasing difficulty in walking for the past year. He had a history of operation and radiotherapy due to testicular tumor. Electroneuromyography (ENMG) and thoracic, lumbosacral, plexus and pelvic magnetic resonance imaging (MRI) were performed considering radiculopathy, plexopathy and motor neuron disease in the differential diagnosis. MRIs revealed no abnormality. Needle EMG of lower extremity and lumbar paraspinal muscles revealed fibrillation and positive sharp waves concomitant with fasciculations and reduced recruitment suggesting anterior horn cell/root involvement. DRIMNS was considered rather than motor neuron disease based on the long duration of symptoms with slow progressive course and history of radiotherapy to the pelvic region. DRIMNS is a rare entity that should be considered in the differential diagnosis of lower extremity muscle weakness in a patient with a history of malignancy and radiotherapy. EMG findings are very valuable in making the diagnosis together with the clinical picture.

Agenesis of Pectoralis Major Muscle in Late-Onset GFPT1-Related Congenital Myasthenic Syndrome: A Case Report.

The objective of this study was to expand the phenotypic spectrum of glutamine-fructose-6-phosphate transaminase 1 (GFPT1)-related congenital myasthenia syndrome (CMS). A 61-year-old man with agenesis of the left pectoralis major muscle presented with progressive muscle weakness for a decade that transiently improved after exertion. His examination revealed proximal and distal muscle weakness in upper extremities and proximal muscle weakness in lower extremities. Muscle enzymes were elevated. An electromyogram revealed a myopathic pattern; however, a muscle biopsy of deltoid muscle and genetic testing for limb-girdle muscular dystrophies were nondiagnostic. A 3-Hz repetitive nerve stimulation of the spinal accessory nerve recording from trapezius muscle demonstrated a >20% drop in amplitude of the 5th compound motor action potential relative to 1st at both baseline and after 45-second exercise. Acetylcholine receptor binding, lipoprotein-related protein 4, muscle-specific kinase, and voltage-gated calcium channel P/Q antibodies were negative. Genetic testing targeting CMS revealed 2 likely pathogenic variants within GFPT1: novel c.7+2T>G (intron 1) that was predicted to result in a null allele and known c*22 C>A (exon 19) associated with reduced GFPT1 expression. His muscle strength dramatically improved after pyridostigmine initiation. In addition to other reported neurodevelopmental abnormalities, pectoralis major muscle agenesis (or Poland syndrome) may be a clinical manifestation of GFPT1-related CMS.

Scapular Winging In Patients With Anti-Hydroxy Methyl Glutaryl CoA Reductase (HMGCR) Antibody Positive Immune Mediated Necrotizing Myopathy (IMNM): A Case Series (P9-8.005)

<h3>Objective:</h3> To report examination finding of scapular winging in two patients with Anti-hydroxymethylglutaryl CoA reductase (HMGCR) antibody positive immune mediated necrotizing myopathy (IMNM). <h3>Background:</h3> IMNM is a disabling auto-immune myopathy characterized by severe proximal muscle weakness, markedly elevated creatine kinase (CK) and muscle necrosis. Diagnosis is made via muscle biopsy and antibody testing in a patient with a suggestive history, examination and laboratory abnormalities. <h3>Design/Methods:</h3> Case Series <h3>Results:</h3> Patient 1, a 46 year old male presented for evaluation of muscular dystrophy. He had proximal lower extremity muscle weakness, dysphagia and 20 lbs weight loss over 1 year duration. He was on statin in the past which was discontinued after he was found to have elevated liver enzymes. Examination was notable for mild eye closure weakness, bilateral scapular winging, leg &gt; arm, proximal &gt; distal weakness and abdominal weakness. CK was elevated to 12, 000 u/L. HMCCoA Ab was positive with elevated titer to 435.8. He was started on methotrexate and IVIG Q2 weeks. CK level down trended to 2652 U/L. Patient had significant improvement in his symptoms. Patient 2, a 58 year-old male presented with proximal upper and lower extremity muscle weakness, dysphagia and 10 lbs weight loss of over 4 month duration. He was on atorvastatin for over 3 years which was discontinued 1 month after symptom onset. Examination was notable for proximal muscle weakness in bilateral upper and lower extremities, right scapular winging and neck flexion weakness. CK was elevated to 7500. HMG COA reductase antibody was positive with elevated titer to 169.4. He was started on methotrexate and IVIG Q2 weeks. He also noted improvement in his symptoms. <h3>Conclusions:</h3> Scapular winging can be seen with HMGCR Ab+ IMNM. Such patients can be misdiagnosed having muscular dystrophy. Neurologists should be aware of this clinical phenotype as these patients may respond favorably to immunotherapy. <b>Disclosure:</b> Dr. Katyal has nothing to disclose. Dr. Goyal has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Goyal has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB. An immediate family member of Dr. Goyal has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Goyal has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion.

Non-5q Spinal Muscular Atrophy in a Patient With a Novel BICD2 Missense Variant

Variants in BICD cargo adapter 2 (BICD2) cause autosomal dominant spinal muscular atrophy with lower extremity dominance (SMALED2) which is characterized with lower extremity muscle weakness and atrophy. We describe a novel, heterozygous BICD2 variant (c.1661T&gt;C, [p.Leu554Pro]) in a 21-month-old female patient with a more severe phenotypic presentation than the typical SMALED2 expression including arthrogryposis multiplex congenita, absent deep tendon reflexes, respiratory insufficiency, and cerebral depression. The variant p.Leu554Pro is located just outside of a domain that interacts with the motor protein KIF5A. The detailed neuro-phenotyping and clinical course presented here expand the understanding of BICD2 related disease.

Safety and efficacy of electrical stimulation for lower-extremity muscle weakness in intensive care unit 2019 Novel Coronavirus patients: A phase I double-blinded randomized controlled trial.

Intensive care unit (ICU) prolonged immobilization may lead to lower-extremity muscle deconditioning among critically ill patients, particularly more accentuated in those with 2019 Novel Coronavirus (COVID-19) infection. Electrical stimulation (E-Stim) is known to improve musculoskeletal outcomes. This phase I double-blinded randomized controlled trial examined the safety and efficacy of lower-extremity E-Stim to prevent muscle deconditioning. Critically ill COVID-19 patients admitted to the ICU were randomly assigned to control (CG) or intervention (IG) groups. Both groups received daily E-Stim (1 h) for up to 14 days on both gastrocnemius muscles (GNMs). The device was functional in the IG and non-functional in the CG. Primary outcomes included ankle strength (Ankles) measured by an ankle-dynamometer, and GNM endurance (GNMe) in response to E-Stim assessed with surface electromyography (sEMG). Outcomes were measured at baseline, 3 and 9 days. Thirty-two (IG = 16, CG = 16) lower extremities in 16 patients were independently assessed. The mean time between ICU admission and E-Stim therapy delivery was 1.8 ± 1.9 days (p = 0.29). At 3 days, the IG showed an improvement compared to the CG with medium effect sizes for Ankles (p = 0.06, Cohen's d = 0.77) and GNMe (p = 0.06, d = 0.69). At 9 days, the IG GNMe was significantly higher than the CG (p = 0.04, d = 0.97) with a 6.3% improvement from baseline (p = 0.029). E-Stim did not alter vital signs (i.e., heart/respiratory rate, blood saturation of oxygen), showed no adverse events (i.e., pain, skin damage, discomfort), nor interfere with ICU standard of care procedures (i.e., mechanical ventilation, prone rotation). This study supports the safety and efficacy of early E-Stim therapy to potentially prevent deterioration of lower-extremity muscle conditions in critically ill COVID-19 patients recently admitted to the ICU. If confirmed in a larger sample, E-Stim may be used as a practical adjunctive therapy. [https://clinicaltrials.gov/], identifier [NCT04685213].

Effects of Compression Stockings on Body Balance in Hemiplegic Patients with Subacute Stroke

(1) Background: Stroke patients with hemiplegia have an increased risk of developing deep vein thrombosis (DVT). DVT increases the risk of life-threatening pulmonary embolism and is associated with poor prognosis. The early wearing of compression stockings can help prevent DVT. This study aimed to assess the impact of compression stockings on body balance in stroke patients with unilateral lower extremity muscle weakness; (2) Methods: Hemiplegic stroke patients in the subacute phase who were able to walk with assistance were recruited. The patients were divided into two groups: one group received rehabilitation treatment with compression stockings, and the other received treatment without compression stockings. The rehabilitation treatment involved hospitalization for 4 weeks, the Trunk Control Test (TCT), the Trunk Impairment Scale (TIS), and the Berg Balance Scale (BBS). The patients were evaluated before and 4 weeks after the start of treatment. The differences in BBS, TCT, and TIS before and after treatment between the two groups were compared; (3) Results: Altogether, 236 hemiplegic stroke patients were recruited. There was an improvement in body balance after treatment in both groups, and BBS, TCT, and TIS scores significantly increased in the group that received rehabilitation treatment with compression stockings; (4) Conclusions: In patients with hemiplegic stroke in the subacute period, rehabilitation while wearing compression stockings appears to improve body balance.

Knee Extensor Muscle Strength to Measure the Ability of Five Times Sit to Stand Independently in Patients with Incomplete Spinal Cord Injury.

Patients with incomplete spinal cord injury (iSCI) and lower extremity muscle weakness often fall while standing up from a chair. The sit-to-stand (STS) task primarily uses the strength of the knee extensor muscles. The five times sit-to-stand test (FTSST) is often applied to determine lower limb function and the results are related to lower extremity muscle strength. This study explored the cut-off point for knee extensor muscle strength in patients with iSCI to independently determine their FTSST results and the correlation between knee extensor muscle strength and FTSST results. Forty-four participants were assessed for knee extensor muscle strength using a hand-held dynamometer (HHD) and the FTSST. The data indicated that knee extensor muscle strength ≥ 53.06 Newton was the best independent predictor of the FTSST results (sensitivity 72.7%, specificity 72.7%). Moreover, knee extensor muscle strength was significant and correlated with the FTSST results (r = -0.45, P = 0.035). The findings offer a cut-off point for the knee extensor muscle strength measured while standing up from a chair that may help medical professionals set rehabilitation goals for patients with iSCI.

Pain Management After Open Liver Resection: Epidural Analgesia Versus Ultrasound-Guided Erector Spinae Plane Block.

Background: Multimodal analgesia techniques, including regional analgesia, have been shown to provide effective analgesia and minimize opioid consumption after liver resection surgery. While thoracic epidural analgesia (TEA) is considered the gold standard, its role in the current era of enhanced recovery after surgery (ERAS) has been questioned. Erector spinae plane blocks (ESPBs) have the potential to provide effective postoperative analgesia without the risks associated with epidural analgesia. The primary aim of this quality improvement project was to evaluate the analgesic efficacy of ultrasound-guided ESPB in comparison with TEA in patients undergoing open liver resection.Methods: Fifty patients who underwent open liver resection and received TEA (n=25) or ESPB (n=25) as part of an ERAS pathway were retrospectively identified. The primary outcome measure was cumulative postoperative opioid consumption at 24 hours. Secondary outcomes included opioid consumption, pain scores, the incidence of nausea and vomiting requiring antiemetics, lower extremity muscle weakness, and occurrence of hypotension requiring treatment on arrival to the post-anesthesia care unit and at 2, 6, 12, 24 hours, and daily through postoperative day 7. Results: Opioid requirements were significantly lower in the TEA group compared to the ESPB group. Postoperative pain scores at rest and with deep inspiration were significantly lower in the TEA group through postoperative day 5. There were no differences in other outcome measures.Conclusions: These findings suggest that compared with ESPB, TEA provides superior pain relief after open liver resection.

Exploratory analysis using machine learning of predictive factors for falls in type 2 diabetes

We aimed to investigate the status of falls and to identify important risk factors for falls in persons with type 2 diabetes (T2D) including the non-elderly. Participants were 316 persons with T2D who were assessed for medical history, laboratory data and physical capabilities during hospitalization and given a questionnaire on falls one year after discharge. Two different statistical models, logistic regression and random forest classifier, were used to identify the important predictors of falls. The response rate to the survey was 72%; of the 226 respondents, there were 129 males and 97 females (median age 62 years). The fall rate during the first year after discharge was 19%. Logistic regression revealed that knee extension strength, fasting C-peptide (F-CPR) level and dorsiflexion strength were independent predictors of falls. The random forest classifier placed grip strength, F-CPR, knee extension strength, dorsiflexion strength and proliferative diabetic retinopathy among the 5 most important variables for falls. Lower extremity muscle weakness, elevated F-CPR levels and reduced grip strength were shown to be important risk factors for falls in T2D. Analysis by random forest can identify new risk factors for falls in addition to logistic regression.

The Relationship between the Activity Balance Confidence and Mobility Tests among Older Adults in Indonesia.

Introduction Unsteady gait, instability, and lower extremity muscle weakness are some of the risk factors for falls. Reduced balance is a further precursor of falls, and injuries adversely affect the instability. In doing an activity without losing their balance, confidence among older adults is also crucial because it will influence their mobility. Objectives The objective of this study is to examine the association between activity balance confidence and functional mobility, including gait, balance, and strength, among older adults. Methods A cross-sectional study was conducted among older adults living in long-term care facilities and community dwellings. A total of 326 older adults (>60 years old) participated in this study from three provinces in Java Island, Indonesia. The inclusion criteria were older adults living independently and without obstacles in communication, who have no hearing loss, and who agreed to be respondents. The activity-specific balance confidence (ABC) scale determines the level of confidence. The participants were asked about their balance confidence not to lose their balance while doing 16 activities. The dependent variable is the mobility test, including a gait test using TUG (times up and go) to see how the subjects stand, walk, and turn around; a balance test (four stages); and a strength test (30-second chair stand). Results The results of the ABC scale showed the respondents felt the most confidence not to lose their balance when they walk around the house (82.01%) and the less confidence when they stepped onto or off an escalator while holding onto a railing (37.7%). The gait, balance, and strength test revealed that 51.2% of the respondents showed an unsteady gait, 63.8% showed instability that felt awkward and unusual when standing on one leg, and 60.1% of the participants showed muscle weakness. The bivariate analysis significantly correlated the ABC scale test and all mobility tests. The older adult participants who are not confident will have 12.03 times higher the unstable result of the gait test, 8.4 times higher the unstable result of the balance test, and 7.47 times higher the less strength result of the strength test who are confident. Conclusion Older adults who lack balance confidence showed significantly poorer results in mobility tests.

Thyrotoxic Periodic Paralysis: A Case Report and Discussion of Clinical and Imaging Features

Thyrotoxic Periodic Paralysis (TPP) is a rare manifestation of thyrotoxicosis, resulting in periodic episodes of acute onset muscle weakness in the setting of hypokalemia. The thyrotoxic form of Hypokalemic Periodic Paralysis (HPP) is less studied than the more well-known familial form due to fewer reported cases and smaller prevalence. This case study presents a 30-year-old African American male with multiple episodes of acute lower extremity muscle weakness, tachycardia, and a history of heat intolerance. Abnormal findings on thyroid ultrasound coupled with increased thyroid related immunoglobulins led to a diagnosis of TPP related to exacerbation of newly-found Graves’ disease. The case study will further discuss the importance of imaging in assessing the etiology of TPP with review of relevant literature.

Thyrotoxic periodic paralysis mimicking hypokalemic periodic paralysis

Thyrotoxic periodic paralysis is a unique disorder that causes episodic proximal lower extremity muscle weakness in a patient who already has a hyperthyroid state and is not treated for the underlying disease state. The affected population mostly includes Asian in origin specifically the male gender (vs most thyroid disorder affects the women gender). Precipitating factors include in addition to a hyperthyroid state are strenuous exertion, a high-carbohydrate meal, and drugs like diuretics, insulin, high dose of steroids, antiretrovirals, and interferon therapy. Other genetic causes include the decreased activity of Kir2.6 mutation increased activity of Na+/K+ ATPase causing an intracellular shift of potassium that leads to hypokalaemia. The other challenging part for the physician is to differentiate it from Familial periodic paralysis an autosomal disorder seen in Caucasians and Western countries because both the disorders present similarly and differentiate between them by the presence of hyperthyroid state with hypokalaemia in Thyrotoxic periodic paralysis. The mortality is associated with respiratory paralysis hence the acute intervention includes giving nonselective beta-blockers followed by anti-thyroid medications. We present a case of an Indian adolescent who presented to us with lower muscle weakness with an underlying thyrotoxicosis state. This help physician with early diagnosis and appropriate treatment.  Â

Assessing gait, balance, and muscle strength among breast cancer survivors with chemotherapy-induced peripheral neuropathy (CIPN): study protocol for a randomized controlled clinical trial

BackgroundChemotherapy-induced peripheral neuropathy (CIPN) is a common and understudied consequence of taxane chemotherapy for breast cancer treatment. CIPN symptoms include numbness combined with tingling sensations, persistent shooting, stabbing, or burning pain even in the absence of painful stimuli, lower extremity muscle weakness, and impaired balance. CIPN symptoms often persist for a long time after completion of chemotherapy, causing significant loss of functional abilities and increased risk of falls. Persistent CIPN caused by taxanes represents a therapeutic challenge due to the limited treatment options. Resistance exercise has shown promising results; however, the effect of exercise on CIPN remains understudied. This study aims to assess the effects of exercise on gait, balance, and lower extremity muscle strength after a 16-week home-based exercise program compared to an educational attention control condition.MethodsA sample of 312 women who completed taxane-based chemotherapy for breast cancer and have symptomatic neuropathy is recruited from a community-dwelling sample. Participants are randomized to either a 16-week Home-Based Physical Activity Intervention or an Educational Attention control group. The home-based intervention protocol consists of targeted lower extremity stretches, followed by 10 min each of gait/balance and 10 min of resistive training accessed by hyperlink or DVD. An Exercise Diary records quantitative exercise data. The gait assessment includes temporospatial parameters and lower extremity joint angles using APDM motion sensors. Participants’ balance is assessed using the Sensory Organization Test (SOT) performed using a NeuroCom Balance Master. Isometric strength of hip, knee, and ankle flexor and extensor muscles is assessed using an isokinetic dynamometer, Biodex BX Advantage. In addition, we assess neuropathy symptoms using the FACT-Taxane Additional Concerns Subscale and nerve conduction velocity of the sural and peroneal nerve action potentials. Outcomes are assessed at baseline (prior to randomization) and 16 weeks.DiscussionThere are currently no evidence-based interventions that address the functional declines associated with CIPN. If successful, this program is simple and easy to implement in the standard of care for individuals with CIPN. Gait and balance training have the potential to reduce physical dysfunction associated with CIPN and reduce the burden of disease in cancer survivors.Trial registrationClinicalTrials.gov NCT04621721. Registered on August 3, 2020. ClincialTrials.gov is a primary registry of the World Health Organization International Clinical Trials Registry Platform (WHO ICTEP) network and includes all items from the WHO Trial Registration data set in Trial registration.

246 PREGNANCY COURSE AND OUTCOME IN Woman with facioscapulohumeral muscular dystrophy

Introduction and aims of the study: Facioscapulohumeral muscular dystrophy (FSHD) is a rare muscular dystrophy affecting all ages, primarily people in the second decade. The disease is initially presented with face, shoulder girdle and upper arm involvement followed by lower extremity muscle weakness. Disease progression is usually slow, although about one fifth of patients will require a wheelchair to accommodate mobility. Women with this syndrome often have poor birth outcomes, with FSHD symptom deterioration post-partum.

Thyrotoxic Periodic Paralysis in Young Caucasian Male

Abstract Background: Thyrotoxic periodic paralysis (TPP) can be a medical emergency as delay in diagnosis can lead to life-threatening arrhythmia. Periodic paralysis is more prevalent in the Asian population. We report a case of thyrotoxic periodic paralysis in a young Caucasian male. Case: A 24-year-old male with a past history of Graves’ disease, hypertension, and asthma was brought to the hospital due to leg weakness and fall. He was initially diagnosed with Graves’ disease 2 years ago. The patient could not take methimazole or metoprolol due to the affordability issue for the last 18 months. On presentation, he fell on the floor while attempting to stand up from the couch. He could not stand up or pick his cell phone. He remained on the floor for 2-3 hrs. A review of the system was positive for palpitation and fatigue and negative for diarrhea, weight loss, anxiety, sleep problem, and dry eyes. On arrival, he had a pulse of 100/min, BP of 157/85 mmHg with rest of vitals signs normal. Motor strength on bilateral lower extremities were 2/5. Upper extremity strength was normal. No thyromegaly or thyroid bruit was noted in the exam. The rest of the physical exam was normal. Labs showed Potassium 1.9 with a normal reference range (RR) of 3.5 - 5.1 mmol/l. His TSH was &amp;lt; 0.01 (RR 0.35 - 5.00 MCU/ML), Free T4 was 5.0 (RR 0.6- 1.6 NG/DL), Total T3 was 425 (RR 87 - 180 NG/DL) and CK was 70 (RR 35- 232 U/L). EKG showed sinus rhythm at 90 bpm with no PR, T/ST, or QT abnormalities. He was given IV potassium and was also started on methimazole 10mg TID and metoprolol. His weakness and tachycardia were improved the next day. We discussed with him the options of medical management vs. surgery. He underwent a total thyroidectomy. Biopsy showed nodular hyperplasia consistent with graves’ disease. Discussion: Thyrotoxic periodic paralysis (TPP) is characterized by hypokalemia and episode of acute muscle weakness in lower extremities in the setting of hyperthyroidism. The pathophysiology of TPP remains uncertain. Hyperthyroidism is a hyperadrenergic state in which beta-2-adrenergic stimulation in muscle cells directly induces cellular K+ uptake by increasing cAMP, leading to activation of Na/K ATPase. The increase in the influx of intracellular K+ leads to hypokalemia and skeletal muscle weakness. Some studies show pathophysiology can be different in Caucasians compared to the Asian population that there could be abnormalities in Na and K channels other than Na/K ATPase. Potassium replacement should be done with caution as hypokalemia is due to intracellular shift and rebound hyperkalemia is common during the management. Beta-blocker may reverse adrenergic overstimulation of Na/K ATPase. It can help rapidly improve paralytic symptoms.

Microsurgery for intradural epidermoid cyst at cauda equina level in a 9-year-old child: A case report.

Introduction and importanceEpidermoid cysts are rare benign tumors. Here, we present a case of spontaneous intradural epidermoid cyst at cauda equina level in a 9-year-old patient, which we believed the first case to be reported in Vietnam.Case presentationA 9-year-old boy presented with 4 months of spontaneous left lower extremity muscle weakness and paresthesia. The MRI images suggested the diagnosis of intradural epidermoid cyst at cauda equina level. The patient underwent L5–S1 laminectomy and durotomy for tumor resection. The histology confirmed the diagnosis of epidermoid cyst. Post-operative images demonstrated total cyst removal.Clinical discussionThe epidermiology, presentation and diagnosis and strategy of treatments as well as their outcomes were discussed.ConclusionDiagnosis of spinal epidermoid cyst is often delayed for its obscure presentation. Microsurgical dissection along with intra-operative mobile C-Arms enable total tumor resection while preserving spinal stability and neurological function. Follow-up with post-operative magnetic resonance imaging and tumor marker are helpful.