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1.
Muscle Nerve ; 2024 Jun 27.
Artículo en Inglés | MEDLINE | ID: mdl-38935447

RESUMEN

INTRODUCTION/AIMS: Point-of-care ultrasound of the diaphragm is highly sensitive and specific in the detection of neuromuscular diaphragmatic dysfunction. In some patients with neuromuscular diaphragmatic dysfunction, paradoxical thinning of the diaphragm during inspiration is observed on ultrasound; however, its frequency, electrodiagnostic associations, and prognostic significance remain uncertain. METHODS: Medical records of patients presenting to two electrodiagnostic laboratories (Mayo Clinic, Rochester, Minnesota and University of Alberta, Edmonton, Alberta) from January 1, 2022 to December 31, 2022, for evaluation of suspected neuromuscular respiratory failure, were reviewed. RESULTS: 214 patients were referred and 19 patients excluded due to incomplete information. Of 195 patients (384 hemidiaphragms), 104 had phrenic neuropathy, 12 had myopathy, and 79 had no evidence of neuromuscular disease affecting the diaphragm. Paradoxical thinning occurred in 31 (27%) patients with neuromuscular diaphragmatic dysfunction and was unilateral in 30, the majority (83%) having normal contralateral ultrasound. Phrenic nerve conduction studies and diaphragm electromyography results did not distinguish patients with paradoxical thinning versus without. Most patients (71%) with paradoxical thinning required non-invasive ventilation (NIV), including 16 with unilateral paradoxical thinning. Paradoxical thinning and BMI ≥30 kg/m2 were risk factors for requiring NIV in multivariable logistic regression analysis, with odds ratios of 2.887 (95% CI:1.166, 7.151) and 2.561 (95% CI: 1.186, 5.532), respectively. DISCUSSION: Paradoxical thinning of the diaphragm occurs in patients with prominent neuromuscular diaphragmatic dysfunction, most commonly from phrenic neuropathy, and is a significant risk factor for requiring NIV. Unilateral paradoxical thinning is sufficient for needing NIV. BMI ≥30 kg/m2 additionally increases risk of requiring NIV in patients with neuromuscular diaphragmatic dysfunction.

2.
Clin Auton Res ; 2024 Jun 12.
Artículo en Inglés | MEDLINE | ID: mdl-38865034

RESUMEN

PURPOSE: Prior studies reported evidence of autonomic involvement in motor neuron disease and suggested more severe dysfunction in upper motor neuron predominant syndromes. Hence, we sought to characterize autonomic impairment in primary lateral sclerosis. METHODS: Neurological evaluations, thermoregulatory sweat tests, and autonomic reflex screens were analyzed retrospectively in 34 primary lateral sclerosis patients (28 definite and 6 probable). Patients with other potential causes of autonomic failure and patients with autonomic testing results compromised by artifact were excluded. RESULTS: A total of 17 patients reported autonomic symptoms. Orthostatic lightheadedness was most frequent (8 patients), followed by bladder (7), bowel (5), and erectile dysfunction (3). The autonomic reflex screens of 33 patients were reviewed; 20 patients had abnormal studies. The thermoregulatory sweat tests of 19 patients were reviewed; 11 patients had abnormal studies. Composite Autonomic Severity Score was calculated for 33 patients and found abnormal in 20/33 patients (60.6%): 15/20 patients (75%) had mild impairment, and 5/20 patients (25%) had moderate impairment. The frequencies of testing abnormalities were: sudomotor 18/20 (90%), cardiovagal 9/20 (45%), and adrenergic 6/20 (30%). Sweat loss pattern analysis showed global, regional, and mixed patterns to be more common than length-dependent and distal patterns. CONCLUSION: We found evidence of frequent autonomic dysfunction in primary lateral sclerosis, which is generally of modest severity akin to prior reports for amyotrophic lateral sclerosis, but more commonly in a pattern consistent with preganglionic/ganglionic localization. This suggests that primary lateral sclerosis, as with amyotrophic lateral sclerosis, is a multisystem disease that affects the autonomic nervous system.

4.
Artículo en Inglés | MEDLINE | ID: mdl-36576200

RESUMEN

Introduction/Aims. Primary lateral sclerosis (PLS) is exceedingly rare and has been an enigmatic disease. Recent progress has drastically changed this perception, with early biomarkers being investigated and potential medications for PLS emerging at the preclinical stage. The aim of this paper is to describe a study of PLS natural history and discuss the limitations and proposed solutions to the study of a rare and slowly progressive disease. Methods. The PLS Natural History Study is a 30-site, 24-month, prospective study that is supported by multiple funding sources. The study aims to enroll 50 early PLS (disease duration ≤4 years) and 50 definite PLS (disease duration 4 to 15 years) participants using modified PLS Diagnostic Criteria. Smartphone-based assessments including semi-quantitative and quantitative measures and patient-reported outcomes are utilized. In-person quantitative measures are also completed during site visits. The change in the PLS Functional Rating Scale score is the primary outcome. The study utilizes the NeuroBANK® patient-centric data capture and management platform. The biostatistical analysis plan has been developed. Results. In one year, 28 participants have been recruited. Enrollment has been much slower than anticipated due to the COVID-19 pandemic, the rarity of PLS, and potential study competition for internal resources from ALS clinical trials. Discussion. We discuss the need for more innovative methods to enroll and study individuals with such rare diseases and propose a number of mechanisms by which more efficient enrollment could be facilitated.


Asunto(s)
Esclerosis Amiotrófica Lateral , COVID-19 , Enfermedad de la Neurona Motora , Humanos , Enfermedad de la Neurona Motora/diagnóstico , Esclerosis Amiotrófica Lateral/diagnóstico , Esclerosis Amiotrófica Lateral/epidemiología , Esclerosis Amiotrófica Lateral/terapia , Estudios Prospectivos , Pandemias
5.
Prog Mol Biol Transl Sci ; 190(1): 219-276, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36008000

RESUMEN

Medical abzymology has made a great contribution to the development of general autoimmunity theory: it has put the autoantibodies (Ab) as the key brick of the theory to the level of physiological functionality by providing such Ab with the ability to catalyze and mediate direct and independent cytotoxic effect on cellular and molecular targets. Natural catalytic autoantibodies (abzymes) while being a pool of canonical Abs and possessing catalytic activity belong to the new group of physiologically active substances whose features and properties are evolutionary consolidated in one functionally active biomolecule. Therefore, further studies on Ab-mediated autoAg degradation and other targeted Ab-mediated proteolysis may provide biomarkers of newer generations and thus a supplementary tool for assessing the disease progression and predicting disability of the patients and persons at risks. This chapter is a summary of current knowledge and prognostic perspectives toward catalytic Abs in autoimmunity and thus some autoimmune clinical cases, their role in pathogenesis, and the exploitation of both whole molecules and their constituent parts in developing highly effective targeted drugs of the future to come, and thus the therapeutic protocols being individualized.


Asunto(s)
Anticuerpos Catalíticos , Autoinmunidad , Anticuerpos Catalíticos/metabolismo , Autoanticuerpos/metabolismo , Biomarcadores , Progresión de la Enfermedad , Humanos
6.
Int J Sports Phys Ther ; 17(5): 896-906, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35949390

RESUMEN

Background: Functional tests (FT) are assessment tools that attempt to evaluate balance, flexibility, strength, power, speed, or agility through performance of gross motor skills. FT are frequently administered by coaches or sports medicine professionals to evaluate athletic ability, to predict performance, to identify athletes at risk for injury, or to evaluate an athlete's ability to return to sport after injury. Functional tests which can provide accurate or predictive information regarding athletic ability would be advantageous to coaching staffs or medical professionals. Purpose: The primary purpose of this study was to identify correlations between preseason FT scores and in-season game statistics in a cohort of female collegiate level volleyball (VB) players. A secondary purpose was to present FT descriptive data for this cohort based on level of competition, player position, and starter status. Study Design: prospective cohort; correlational. Methods: One hundred and thirty-one female collegiate VB players representing three levels of competition completed four FT [standing long jump (SLJ), single-leg hop (SLH), lower extremity functional test (LEFT), and the Y-Balance Test - Lower Quarter (YBT-LQ)] at the start of the preseason. Player statistics were collected from team records at the completion of the season. Results: Starters performed significantly better on all tests. There were moderate negative correlations between LEFT scores and game statistics for liberos, defensive specialists, and outside hitters. There were moderate positive correlations between YBT-LQ composite scores and game statistics for liberos, defensive specialists, hitters, and middle blockers. There were also low to moderate level positive correlations between SLJ and SLH scores and game performance for outside hitters. There were low to moderate level positive correlations between SLH scores and game performance for middle blockers and opposite side hitters. Conclusions: The results of this study indicate that there are low to moderate correlations between some preseason functional test scores and some game statistics. The SLJ, SLH, LEFT, and YBT-LQ tests may help coaches with talent identification and/or may influence training strategies. Level of Evidence: 3.

7.
Neurology ; 99(7): e743-e750, 2022 08 16.
Artículo en Inglés | MEDLINE | ID: mdl-35584920

RESUMEN

BACKGROUND AND OBJECTIVES: To compare the performance of different respiratory function testing parameters in a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic. METHODS: Demographics, clinical data, and respiratory testing parameters were abstracted from the medical records of patients who attended a multidisciplinary ALS clinic from 2008 to 2016. We compared the performance of the 3 primary respiratory test parameters used by Medicare for the initiation of noninvasive ventilation (NIV) (forced vital capacity [FVC] < 50% predicted, maximum inspiratory pressure [MIP] < 60 cm H2O, and abnormal overnight pulse oximetry [OvOx]) on how they related to several clinically relevant attributes. RESULTS: Four hundred seventy-six patients were identified who underwent at least 1 respiratory test. Abnormalities of OvOx, MIP, and FVC occurred at a median of 1.6, 1.5, and 3.8 years from disease onset, respectively (p < 0.00001). Patients with bulbar-onset ALS exhibited earlier abnormalities in MIP and FVC than in spinal-onset ALS (p < 0.005). The median survival after an abnormal OvOx, MIP, or FVC test was 1.4, 1.4, and 0.9 years, respectively (p < 0.0001). Using the ALS Functional Rating Score respiratory subscales, at the time of reported respiratory symptoms there were abnormalities in OvOx (60%), MIP (69%), and FVC (19%). Conversely, when respiratory parameter abnormalities preceded reported respiratory symptoms, this occurred with frequencies in OvOx (79%), MIP (42%), or FVC (24%). Four hundred forty-three patients (93.1%) developed at least 1 abnormal respiratory measure meeting Medicare criteria for NIV consideration, but fewer than 50% in our cohort demonstrated NIV use. Improved survival in subjects using NIV was statistically significant in patients with bulbar-onset ALS. DISCUSSION: Abnormalities in OvOx and MIP perform better than FVC at early detection of neuromuscular respiratory weakness in ALS. Initiation of NIV in patients with respiratory insufficiency may improve the overall survival in ALS. In the setting of the COVID-19 pandemic, FVC and MIP have not been routinely performed because of infectious aerosol generation. OvOx, which we now routinely mail to patients' homes, has been used exclusively during the COVID-19 pandemic and allows for continued remote monitoring of the respiratory status of patients with ALS. CLASSIFICATION OF EVIDENCE: This cohort study provides Class III evidence that in people with ALS, OvOx and MIP are valuable respiratory parameters for the detection of early respiratory insufficiency.


Asunto(s)
Esclerosis Amiotrófica Lateral , COVID-19 , Ventilación no Invasiva , Insuficiencia Respiratoria , Anciano , Esclerosis Amiotrófica Lateral/complicaciones , Esclerosis Amiotrófica Lateral/diagnóstico , Estudios de Cohortes , Humanos , Medicare , Pandemias , Insuficiencia Respiratoria/diagnóstico , Insuficiencia Respiratoria/etiología , Estados Unidos , Capacidad Vital
8.
Muscle Nerve ; 66(6): 671-678, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-35470901

RESUMEN

INTRODUCTION/AIMS: Graduate medical education programs must ensure residents and fellows acquire skills needed for independent practice. Workplace-based observational assessments are informative but can be time- and resource-intensive. In this study we sought to gather "relations-to-other-variables" validity evidence for scores generated by the Electromyography Direct Observation Tool (EMG-DOT) to inform its use as a measure of electrodiagnostic skill acquisition. METHODS: Scores on multiple assessments were compiled by trainees during Clinical Neurophysiology and Electromyography rotations at a large US academic medical center. Relationships between workplace-based EMG-DOT scores (n = 298) and scores on a prerequisite simulated patient exercise, patient experience surveys (n = 199), end-of-rotation evaluations (n = 301), and an American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) self-assessment examination were assessed using Pearson correlations. RESULTS: Among 23 trainees, EMG-DOT scores assigned by physician raters correlated positively with end-of-rotation evaluations (r = 0.63, P = .001), but EMG-DOT scores assigned by technician raters did not (r = 0.10, P = .663). When physician and technician ratings were combined, higher EMG-DOT scores correlated with better patient experience survey scores (r = 0.42, P = .047), but not with simulated patient or AANEM self-assessment examination scores. DISCUSSION: End-of-rotation evaluations can provide valid assessments of trainee performance when completed by individuals with ample opportunities to directly observe trainees. Inclusion of observational assessments by technicians and patients provides a more comprehensive view of trainee performance. Workplace- and classroom-based assessments provide complementary information about trainee performance, reflecting underlying differences in types of skills measured.


Asunto(s)
Internado y Residencia , Humanos , Estados Unidos , Competencia Clínica , Lugar de Trabajo , Electromiografía , Educación de Postgrado en Medicina
9.
Artículo en Inglés | MEDLINE | ID: mdl-35170382

RESUMEN

Objective: We assessed whether a cohort of patients with primary lateral sclerosis (PLS) and limited electromyography (EMG) motor unit denervation changes evolve into amyotrophic lateral sclerosis (ALS) with prolonged follow-up. Methods: We initially ascertained all PLS patients diagnosed at Mayo Clinic-Rochester (1990-2016). Of 64 total cases, 43 had normal EMGs ("pure" PLS) during the first 4 years after symptom onset and were the focus of a prior publication, documenting absence of evolution to ALS. The remaining 21 patients had limited motor unit changes on EMG needle examination (denervation and most with fibrillation or fasciculation potentials) but insufficient to raise a strong suspicion of ALS; these 21 patients were followed to determine whether they evolved into ALS. Results: Of these 21 patients, the median follow-up was 7 years' disease duration (range: 4-27 years; IQR 5-8.5). They included 11 females (52%) with median onset-age of 57 years (range: 42-72 years). Two patients (10%) subsequently met revised El Escorial criteria for ALS after 7 and 13 years, respectively. The remainder had stable EMG changes with a persistent PLS phenotype. Among these remaining 19 patients, the PLS course was somewhat more aggressive than our previously reported series of 43 patients devoid of EMG denervation. The paraparetic variant was more common than the hemiparetic and bulbar variants, similar to "pure" PLS. Conclusions: Among PLS patients with definite but limited EMG denervation, 2/21 (10%) later developed ALS. The patients in this series had a more progressive clinical course compared to our previously reported pure PLS cases.


Asunto(s)
Esclerosis Amiotrófica Lateral , Femenino , Humanos , Esclerosis Amiotrófica Lateral/diagnóstico , Esclerosis Amiotrófica Lateral/cirugía , Electromiografía , Estudios de Cohortes , Edad de Inicio , Desnervación
10.
Int J Exerc Sci ; 14(1): 912-918, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34567352

RESUMEN

During the lowering phase of a squat, it has been observed that a posterior pelvic tilt (PPT) may occur when squatting to full depth. Research suggests that defaulting to compensatory movement strategies, such as PPT, during the squat may correlate with risk of lower extremity and trunk pathology. The purpose of this study was to examine hip flexion (HF) angles at the point when PPT occurs among three conditions: standard squats, heel raise squats, and supine passive HF; analyzing the differences in depth between standard and heel raise squats; and calculating differences in knee angles and ankle excursion between standard and heel raise squats. 28 participants performed bodyweight squats and underwent supine passive HF while outfitted with 32 retroreflective motion capture markers. Hip, knee, and ankle joint angles were extracted at the point of PPT. A one-way repeated measures ANOVA was used to determine differences in hip joint angles between conditions, and a paired sample t-test was used to compare knee angles, ankle excursion, and squat depth between standard and heel raise squats. HF angles at PPT remained unchanged across all conditions. However, significantly greater knee flexion, ankle excursion, and squat depth were observed in the heel raise squats compared to the standard squats. Results suggest that PPT is a compensatory movement that occurs as the femur compresses into the acetabulum once hip flexion has been exhausted.

11.
Phys Ther Sport ; 51: 79-84, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34274871

RESUMEN

OBJECTIVE: Preseason functional tests have been previously associated with noncontact time-loss lower extremity injuries in a variety of athletic populations. This study aimed to determine the effectiveness of one or more functional tests to discriminate injury risk in a cohort of female collegiate volleyball players in the United States. DESIGN: Prospective cohort study. SETTING: National Collegiate Athletic Association Division II & III, National Association of Intercollegiate Athletics female volleyball players; PARTICIPANTS: 130 female volleyball players (mean age: 19.31 ± 1.1 years). MAIN OUTCOME MEASURE: Lower extremity functional test, single limb hop for distance, standing long jump, previous injury history, lower quarter Y-balance test, and limb symmetry index were measured prior to the season beginning. Noncontact time-loss lower quarter injuries were tracked during the season. RESULTS: Athletes with suboptimal scores on the single limb hop test (<70% of height) and standing long jump (<80% of height), combined with a previous history of injury, were three times more likely to sustain an injury during the season (OR = 3.0; 95% CI: 1.09 to 8.30). Individual functional tests did not discriminate injury risk. CONCLUSIONS: A battery of preseason functional tests and injury history discriminates injury risk in female collegiate volleyball players.


Asunto(s)
Traumatismos en Atletas , Voleibol , Adolescente , Adulto , Atletas , Traumatismos en Atletas/diagnóstico , Femenino , Humanos , Estudios Prospectivos , Estados Unidos , Universidades , Adulto Joven
12.
Muscle Nerve ; 64(1): 50-58, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-33651404

RESUMEN

OBJECTIVE: Gray scale ultrasound (US) has been demonstrated to be a sensitive and specific tool in the diagnosis of pediatric neuromuscular disease (NMD). With recent advances in genetic testing, the diagnostic work up for NMD has evolved. The purpose of this study was to compare the current diagnostic value of gray scale US to previously defined sensitivities and specificities to determine when this test can add value to a patient's diagnostic workup. METHODS: Standardized quantitative gray scale US imaging was performed on 148 pediatric patients presenting for electrodiagnostic testing to evaluate for NMD. Patients were categorized as having an NMD, a non-NMD, or as "uncertain." The US results were defined as normal, borderline or abnormal based on echointensity values. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of the test were calculated. RESULTS: Forty-five percent of the patients had an NMD, 54% a non-NMD, and in 1% the diagnosis remained uncertain. US was abnormal in 73% of myopathies, 63% of neuromuscular junction disorders, 60% of generalized neuropathies and 58% of focal neuropathies. After excluding patients in whom muscle US was not expected to be abnormal (eg, sensory neuropathy), sensitivity was 83%, specificity 79%, PPV 75%, NPV 86%, and accuracy 81%. CONCLUSIONS: Quantitative gray scale muscle US still has good diagnostic value as a screening tool in pediatric NMD. As with any diagnostic test, muscle US is best used in conjunction with history and physical examination to increase specificity and diagnostic yield.


Asunto(s)
Músculo Esquelético/diagnóstico por imagen , Enfermedades Neuromusculares/diagnóstico por imagen , Ultrasonografía Intervencional/normas , Adolescente , Niño , Preescolar , Estudios de Cohortes , Estudios Transversales , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Ultrasonografía Intervencional/métodos
13.
Neurology ; 96(17): e2231-e2238, 2021 04 27.
Artículo en Inglés | MEDLINE | ID: mdl-33637635

RESUMEN

OBJECTIVE: To assess whether primary lateral sclerosis (PLS), classified as pure when the EMG is normal, converts to amyotrophic lateral sclerosis (ALS) after longitudinal follow-up. METHODS: Retrospective chart review was performed of patients with pure PLS at Mayo Clinic in Rochester, MN (1990-2016). Inclusion criteria required a normal EMG during the first 4 years of symptoms. RESULTS: Forty-three patients had pure PLS (25 female, 58%) with a median onset age of 50 years (range 38-78 years) and median follow-up at 9 years' disease duration (range 4-36 years). The ascending paraparesis phenotype (n = 30, 70%) was most common, followed by hemiparetic onset (n = 9, 21%) and bulbar onset (n = 4, 9%). Among the 30 paraparetic-onset cases, bladder symptoms (n = 18, 60%) and dysarthria (n = 15, 50%) were more common than pseudobulbar affect (n = 9, 30%) and dysphagia (n = 8, 27%). By the last follow-up, 17 of 30 (56%) used a cane and 6 (20%) required a wheelchair. The paraparetic variant, compared with hemiparetic and bulbar onset, had the youngest onset (48 vs 56 vs 60 years, respectively; p = 0.02). Five patients died; 1 patient required a feeding tube; and none required permanent noninvasive ventilation. Two patients developed an idiopathic multisystem neurodegenerative disorder, which surfaced after 19 and 20 years. Two patients developed minor EMG abnormalities. The remainder 39 had persistently normal EMGs. CONCLUSIONS: Pure PLS did not convert to ALS after a median of 9 years' disease duration follow-up in our study population. The ascending paraparetic phenotype was most common, with earlier onset and frequent bladder involvement. After years of pure PLS, <5% develop a more pervasive neurodegenerative disorder.


Asunto(s)
Esclerosis Amiotrófica Lateral/patología , Progresión de la Enfermedad , Enfermedad de la Neurona Motora/patología , Fenotipo , Adulto , Anciano , Esclerosis Amiotrófica Lateral/diagnóstico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/diagnóstico , Estudios Retrospectivos
14.
Neurology ; 95(22): e3002-e3011, 2020 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-32938782

RESUMEN

OBJECTIVE: To improve myasthenia gravis (MG) autoantibody testing. METHODS: MG serologic tests with confirmatory or refuting clinical-electrodiagnostic (EDX) testing and cancer evaluations were reviewed over 4 years (2012-2015). All patients had acetylcholine receptor-binding (AChR-Bi), modulating (AChR-Mo), and striational (STR) autoantibody testing, and negatives reflexed to muscle-specific kinase (MuSK). Thymoma and cancer occurrences were correlated with STR and reflexed glutamic acid decarboxylase 65 (GAD65), ganglionic acetylcholine receptor (α3), collapsin response mediating protein-5, and voltage-gated potassium channel complex autoantibodies. RESULTS: Of 433 samples tested, 133 (31%) met clinical-EDX criteria for MG. Best sensitivity (90%) occurred at AChR-Bi >0.02 nmol/L, leaving 14 negative (6 ocular MG, 7 generalized MG, 1 MuSK MG) with specificity 90% (31 false-positives). Using AChR-Mo antibodies (>20% loss), specificity was better (92%, 24 false-positives), but sensitivity dropped (85%). Specificity improved (95%) by testing AChR-Mo when AChR-Bi are positive, resulting in 45% reduction of false-positives (31-17), maintaining AChR-Bi 90% sensitivity. Cutoff values recommended by area under the curve analysis did not outperform this approach. AChR-Bi and AChR-Mo values were significantly higher in true-positives. CT evaluations in 121 MG samples revealed 16 thymomas. Historical or subsequent cancers occurred in 22. STR and reflexed autoantibodies were not more common in MG with thymoma or other cancers. Full-body CT (n = 34) was performed in those with STR and reflex autoantibody positivity, but without additional cancers found. CONCLUSION: Accuracy of MG serologic testing is improved by reflexing AChR-Bi-positive cases to AChR-Mo. STR and other reflexed cancer evaluation autoantibodies did not provide value beyond standard CT chest imaging at the time of MG diagnosis. Diagnostic certainty is informed by AChR-Bi and AChR-Mo with higher values increasing specificity.


Asunto(s)
Autoanticuerpos/sangre , Pruebas Inmunológicas/normas , Miastenia Gravis/diagnóstico , Timoma/diagnóstico , Neoplasias del Timo/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Algoritmos , Electrodiagnóstico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/sangre , Miastenia Gravis/inmunología , Estudios Retrospectivos , Sensibilidad y Especificidad , Timoma/sangre , Timoma/inmunología , Neoplasias del Timo/sangre , Neoplasias del Timo/inmunología , Adulto Joven
15.
Artículo en Inglés | MEDLINE | ID: mdl-32276554

RESUMEN

Objective: To determine the associations between plasma creatinine (PCr), plasma uric acid (PUA), and urinary oxidative stress (OS) biomarkers with the ALSFRS-R at baseline and survival in a large epidemiological cohort study (ALS COSMOS) with a well-phenotyped patient population (N = 355).Methods: Fasting plasma and first void urine samples were obtained. PCr, PUA, urinary 8-oxo-deoxy guanosine (8-oxodG), and 15-F2t-isoprostane (IsoP) were analyzed at baseline, near the midpoint of follow-up, and at the final blood draw (before death or withdrawal from study). We estimated associations between these biomarkers and the ALSFRS-R at baseline and survival.Results: At baseline, PCr correlated with ALSFRS-R (Spearman r = 0.30), percent (%) FVC (r = 0.20), PUA (r = 0.37), and 8-oxodG (r = -0.13, all p < 0.05). Baseline PCr significantly predicted survival (adjusted hazard ratio 0.28, p < 0.001). Time to death from baseline was shortest for those in the lowest two PCr quartiles relative to the highest two quartiles. PCr and ALSFRS-R values were significantly correlated at all three time points (baseline: r = 0.29, midpoint: r = 0.23, final: r = 0.38, all p < 0.001). PCr and PUA significantly declined over time, whereas OS biomarkers significantly increased over time.Conclusions: To date, PCr predicted survival the best, compared to PUA, 8-oxodG, and IsoP. Although PCr represents the degree of muscle mass, it may also represent complex biochemical changes in ALS. Because the field has no reliable prognostic biomarkers, the importance of PCr warrants further investigation through clinical studies in ALS.


Asunto(s)
Esclerosis Amiotrófica Lateral/sangre , Esclerosis Amiotrófica Lateral/diagnóstico , Creatinina/sangre , Estrés Oxidativo/fisiología , Adulto , Anciano , Anciano de 80 o más Años , Esclerosis Amiotrófica Lateral/mortalidad , Biomarcadores/sangre , Estudios de Cohortes , Estudios Transversales , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Tasa de Supervivencia/tendencias , Ácido Úrico/sangre
16.
J Clin Neurophysiol ; 37(1): 68-73, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31274707

RESUMEN

PURPOSE: We present a unique technique applying F-wave latencies to assist in the diagnosis of peripheral neuropathy within the pediatric age group. METHODS: We calculated an F-wave estimated deviance adjusted for limb length measurements, distal motor conduction velocity, and distal motor latencies. We compared the F-wave estimated deviance from the normal subjects with those with peripheral neuropathy (both axonal and demyelinating) to establish diagnostic accuracy in pediatric patients. RESULTS: The normal range for the F-wave estimated deviance in the upper limb was -1 to +6 ms and in the lower limb was -8 to +5 ms. When compared with 82 subjects with peripheral neuropathy, there was a significant difference between the normal subjects and those with neuropathy (P values between 0.002 and 0.0005 for each of the individual nerves tested). CONCLUSIONS: The F-wave estimated deviance is independent of age and can be accurately applied to the electrodiagnostic testing of pediatric patients with suspected neuropathy.


Asunto(s)
Electromiografía/normas , Conducción Nerviosa/fisiología , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Adulto , Niño , Femenino , Humanos , Masculino , Valores de Referencia
17.
Muscle Nerve ; 61(2): 163-172, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31758557

RESUMEN

INTRODUCTION: Our research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS). METHODS: A prototype of the PLS Functional Rating Scale (PLSFRS) was generated. Seventy-seven participants with PLS were enrolled and evaluated at 21 sites that comprised the PLSFRS study group. Participants were assessed using the PLSFRS, Neuro-Quality of Life (QoL), Schwab-England Activities of Daily Living (ADL), and the Clinical Global Impression of Change scales. Participants completed telephone assessments at 12, 24, and 48 weeks after enrollment. RESULTS: The PLSFRS demonstrated internal consistency as well as intrarater, interrater, telephone test-retest reliability, and construct validity. Significant changes in disease progression were detected at 6 and 12 months; changes measured by the PLSFRS vs the ALSFRS-R were significantly higher. DISCUSSION: The PLSFRS is a valid tool to assess the natural history of PLS in a shorter study period.


Asunto(s)
Enfermedad de la Neurona Motora/diagnóstico , Actividades Cotidianas , Adulto , Anciano , Cuidadores , Certificación , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/fisiopatología , Enfermedad de la Neurona Motora/psicología , Variaciones Dependientes del Observador , Calidad de Vida , Reproducibilidad de los Resultados , Teléfono
18.
Res Sports Med ; 28(2): 155-167, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-31663370

RESUMEN

The prevalence of patellar tendinopathy has been reported to be as high as 50% in elite male volleyball (VB) players; however, the rate of injury in female collegiate VB athletes is unknown. The purpose of this study was to 1) identify the prevalence of ultrasonographic evidence of patellar tendon abnormality at the start of the preseason in female collegiate VB players; 2) report the incidence of tendinopathy during the season; and 3) determine if the preseason presence of tendon abnormality is associated with onset of disease. One hundred and six female collegiate VB players had both patellar tendons imaged. Incidence of patellar tendinopathy was tracked during the course of the 4-month season. Twenty-two athletes presented with ultrasonographic evidence of patellar tendon abnormality in at least one knee at the start of the preseason. The incidence of time-loss patellar tendinopathy was 0.26 (95% CI: 0.04, 0.85) per 1000 athletic exposures. This study was unable to determine if preseason presence of tendon abnormality was associated with a greater risk of tendinopathy due to power. The prevalence of tendon abnormality in the preseason and the incidence of patellar tendinopathy in female collegiate VB players are lower than that observed in other populations.


Asunto(s)
Traumatismos en Atletas/epidemiología , Ligamento Rotuliano/lesiones , Tendinopatía/epidemiología , Adolescente , Traumatismos en Atletas/diagnóstico por imagen , Femenino , Humanos , Ligamento Rotuliano/diagnóstico por imagen , Prevalencia , Tendinopatía/diagnóstico por imagen , Ultrasonografía , Voleibol , Adulto Joven
20.
Int J Sports Phys Ther ; 14(3): 415-423, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31681500

RESUMEN

BACKGROUND: The Lower Quarter Y-Balance Test (YBT-LQ) is used by sports medicine professionals to measure an athlete's dynamic balance. The YBT-LQ is used by clinicians to track recovery during clinical rehabilitation, assess an athlete's readiness to return to sport after injury, and to identify athletes potentially at-risk for a time-loss injury. Normative data for the YBT-LQ are lacking for female collegiate volleyball (VB) players. The purpose of this study was to examine preseason YBT-LQ scores and their relationships to level of competition, starter status, player position, and prior lower quadrant (i.e., low back and lower extremities) injury history. METHODS: One-hundred thirty-four female collegiate VB players (mean age = 19.3 ± 1.1 years) representing athletes from three levels of competition (D II = 32, D III = 77, NAIA = 25) participated in this study. Athletes reported their prior injury history and performed the YBT-LQ testing protocol. RESULTS: NAIA and D III athletes demonstrated significantly greater reach measures on the YBT-LQ than D II athletes in several directions. Starters demonstrated significantly greater reach measures in five out of eight reach directions. Liberos/defensive specialists/setters demonstrated significantly greater posterolateral and composite reach measures bilaterally. There was no difference in reach measures based on prior history of lower quadrant (low back and lower extremities) injury. CONCLUSION: This study provides normative data for YBT-LQ in female collegiate volleyball players. The data presented in this report may be used by coaches and rehabilitation professionals when evaluating dynamic balance in healthy volleyball players and by clinicians to compare an injured athlete's recovery to norms. LEVEL OF EVIDENCE: 3b.

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