Autonomic impairment in primary lateral sclerosis.

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).

Evolution of biomarker research in autoimmunity conditions for health professionals and clinical practice.

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.

Correlations Between Preseason Functional Test Scores and Game Performance in Female Collegiate Volleyball Players.

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.

Comparative Performance of Different Respiratory Test Parameters for Detection of Early Respiratory Insufficiency in Patients With ALS.

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 HO, and abnormal overnight pulse oximetry [OvOx]) on how they related to several clinically relevant attributes.

Assessing electrodiagnostic skills among residents and fellows: Relationships between workplace-based assessments using the Electromyography Direct Observation Tool and other measures of trainee performance.

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.

Does limited EMG denervation in early primary lateral sclerosis predict amyotrophic lateral sclerosis?

: 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. : 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.

Hip Flexion Angles During Supine Range of Motion and Bodyweight Squats.

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.

Preseason functional tests discriminate injury risk in female collegiate volleyball players.

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.

Natural History of "Pure" Primary Lateral Sclerosis.

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.

Improving accuracy of myasthenia gravis autoantibody testing by reflex algorithm.

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).

Plasma creatinine and oxidative stress biomarkers in amyotrophic lateral sclerosis.

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 ( = 355). Fasting plasma and first void urine samples were obtained. PCr, PUA, urinary 8-oxo-deoxy guanosine (8-oxodG), and 15-F-isoprostane (IsoP) were analyzed at baseline, near the midpoint of follow-up, and at the final blood draw (before death or withdrawal from study).

Corrigendum to "Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients".

[This corrects the article DOI: 10.1155/2018/5969137.].

Primary lateral sclerosis (PLS) functional rating scale: PLS-specific clinimetric scale.

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.

COMPARISON of LOWER QUARTER Y-BALANCE TEST SCORES for FEMALE COLLEGIATE VOLLEYBALL PLAYERS BASED on COMPETITION LEVEL, POSITION, and STARTER STATUS.

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.

Establishing Normal Values for F-Wave Latencies in Diagnosing Peripheral Neuropathies for the Pediatric Population.

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.

Impact of PET/CT for Restaging Patients With Locally Advanced Rectal Cancer After Neoadjuvant Chemoradiation.

Background: A major challenge in identifying candidates for nonoperative management of locally advanced rectal cancer is predicting pathologic complete response (pCR) following chemoradiation. We evaluated pre- and post-CRT PET-CT imaging to predict pCR and prognosis in this set of patients undergoing resection after neoadjuvant therapy.

Methods: We retrospectively identified patients from 2002 to 2015 with locally advanced rectal cancer who underwent CRT, pre- and post-CRT PET-CT imaging, and resection.

Nerve conduction normal values for electrodiagnosis in pediatric patients.

Introduction: Existing normal value references for pediatric nerve conduction studies (NCS) are based on limited sample sizes with uncertain reliability, suggesting a need for better normative data.

Methods: Electronic medical records were reviewed for pediatric patients (0 to <18 years) with normal findings on electromyography and NCS during the period from January 1, 1997 through September 20, 2017. Electrodiagnostic and demographic data were collected.

Revised Airlie House consensus guidelines for design and implementation of ALS clinical trials.

Objective: To revise the 1999 Airlie House consensus guidelines for the design and implementation of preclinical therapeutic studies and clinical trials in amyotrophic lateral sclerosis (ALS).

Methods: A consensus committee comprising 140 key members of the international ALS community (ALS researchers, clinicians, patient representatives, research funding representatives, industry, and regulatory agencies) addressed 9 areas of need within ALS research: (1) preclinical studies; (2) biological and phenotypic heterogeneity; (3) outcome measures; (4) disease-modifying and symptomatic interventions; (5) recruitment and retention; (6) biomarkers; (7) clinical trial phases; (8) beyond traditional trial designs; and (9) statistical considerations. Assigned to 1 of 8 sections, committee members generated a draft set of guidelines based on a "background" of developing a (pre)clinical question and a "rationale" outlining the evidence and expert opinion.

Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients.

Objective: To evaluate longitudinal cognitive/behavioral change over 12 months in participants enrolled in the ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS).

Methods: We analyzed data from 294 ALS participants, 134 of whom were studied serially. Change over time was evaluated controlling for age, sex, symptom duration, education, race, and ethnicity.