The rate and assessment of muscle wasting during critical illness: a systematic review and meta-analysis.

BACKGROUND: Patients with critical illness can lose more than 15% of muscle mass in one week, and this can have long-term detrimental effects. However, there is currently no synthesis of the data of intensive care unit (ICU) muscle wasting studies, so the true mean rate of muscle loss across all studies is unknown. The aim of this project was therefore to systematically synthetise data on the rate of muscle loss and to identify the methods used to measure muscle size and to synthetise data on the prevalence of ICU-acquired weakness in critically ill patients. METHODS: We conducted a systematic literature search of MEDLINE, PubMed, AMED, BNI, CINAHL, and EMCARE until January 2022 (International Prospective Register of Systematic Reviews [PROSPERO] registration: CRD420222989540. We included studies with at least 20 adult critically ill patients where the investigators measured a muscle mass-related variable at two time points during the ICU stay. We followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and assessed the study quality using the Newcastle-Ottawa Scale. RESULTS: Fifty-two studies that included 3251 patients fulfilled the selection criteria. These studies investigated the rate of muscle wasting in 1773 (55%) patients and assessed ICU-acquired muscle weakness in 1478 (45%) patients. The methods used to assess muscle mass were ultrasound in 85% (n = 28/33) of the studies and computed tomography in the rest 15% (n = 5/33). During the first week of critical illness, patients lost every day -1.75% (95% CI -2.05, -1.45) of their rectus femoris thickness or -2.10% (95% CI -3.17, -1.02) of rectus femoris cross-sectional area. The overall prevalence of ICU-acquired weakness was 48% (95% CI 39%, 56%). CONCLUSION: On average, critically ill patients lose nearly 2% of skeletal muscle per day during the first week of ICU admission.

Multimodal assessment of intensive care unit-acquired weakness in severe stroke patients.

BACKGROUND: Intensive care unit-acquired weakness (ICUAW) defines generalized muscle weakness seen in critically ill patients in the absence of other causative factors. Herein, we aimed to evaluate ICUAW in stroke patients by electrodiagnostic testing, histopathology, and assessment of respiratory complex activities (RCA), to define the frequency of ICUAW in this patient group, and to reach new parameters for early prediction and diagnosis. METHODS: We prospectively recruited twenty-four severe acute stroke patients during a sixteen-month period. In addition to serial nerve conduction studies (NCS), we performed muscle biopsy and RCA analysis on the non-paretic side when ICUAW developed. Patients undergoing orthopedic surgery without metabolic and neuromuscular diseases constituted the control group for RCA. Survival and longitudinal data were analyzed by joint modeling to determine the relationship between electrophysiological parameters and ICUAW diagnosis. RESULTS: Eight patients (33%) developed ICUAW, and six of them within the first two weeks. Extensor digitorum brevis, abductor digiti minimi (ADM), rectus femoris and vastus medialis (VM) compound muscle action potential (CMAP) amplitudes showed a significant decrease in the ICUAW group. VM CMAP amplitude (BIC = 358.1574) and ADM CMAP duration (BIC = 361.1028) were the best-correlated parameters with ICUAW diagnosis. The most informative electrophysiological findings during the entire study were obtained within the first 11 days. Muscle biopsies revealed varying degrees of type 2 fiber atrophy. Complex I (p = 0.003) and IV (p = 0.018) activities decreased in patients with ICUAW compared to controls. CONCLUSION: VM CMAP amplitude and ADM CMAP duration correlate well with ICUAW diagnosis, and may aid in the early diagnosis.

Validity and reproducibility of C-Mill walking-adaptability assessment in polio survivors.

BACKGROUND: The C-Mill interactive treadmill allows for a safe walking-adaptability assessment, unveiling reduced walking adaptability in polio survivors compared to healthy individuals, possibly related to their high fall rate. However, evidence on its validity and reproducibility is scarce. RESEARCH QUESTION: What is the validity and reproducibility of C-Mill walking-adaptability assessment in polio survivors? METHODS: Polio survivors with a history and/or fear of falling (n = 46) performed two walking-adaptability assessments, 1-2 weeks apart, including target-stepping tests (with 0%, 20% and 30% inter-target variance) and obstacle-avoidance tests (anticipatory and reactive). We examined (1) face validity by determining Group effects (for subgroups stratified for fall frequency, fear of falling and leg muscle weakness) and Condition effects (for difficulty level) on walking-adaptability outcomes, (2) construct validity by correlating walking-adaptability and balance outcomes, and (3) content validity by establishing possible ceiling effects. We determined whether face-validity findings were reproducible over test occasions and calculated Intraclass Correlation Coefficients (ICC) and the 95% Limits of Agreement (LoA) for walking-adaptability outcomes. RESULTS: Walking-adaptability outcomes differed in to-be-expected directions for subgroups stratified for fall frequency and leg muscle weakness and for difficulty levels, all reproducible over test occasions. Correlations between walking-adaptability and balance outcomes were mainly low (r < 0.587). Ceiling effects were present for anticipatory obstacle-avoidance and balance outcomes, but not for reactive obstacle avoidance. ICCs [95% confidence intervals] were good for the challenging 20% (0.80[0.67-0.88]) and 30% target-stepping conditions (0.74[0.57-0.85]) and for the reactive obstacle-avoidance (0.76[0.59-0.86]) condition, but not for 0% target-stepping and anticipatory obstacle-avoidance (ICC<0.62) conditions. Likewise, the narrowest LoA were observed for the 20% and 30% target-stepping conditions. SIGNIFICANCE: We proved face, construct and content validity of C-Mill walking-adaptability assessment in polio survivors with a history of falls and/or fear of falling. Adding walking-adaptability assessment, particularly the more challenging tests given their superior reproducibility, to currently used clinical tests could improve fall-risk evaluation in this population.

Magnetic twitch assessment of diaphragm and quadriceps weakness in critically ill mechanically ventilated patients.

Critically ill mechanically ventilated (MV) patients develop significant muscle weakness, which has major clinical consequences. There remains uncertainty, however, regarding the severity of leg weakness, the precise relationship between muscle strength and thickness, and the risk factors for weakness in MV patients. We therefore measured both diaphragm (PdiTw) and quadriceps (QuadTw) strength in MV patients using magnetic stimulation and compared strength to muscle thickness. Both PdiTw and QuadTw were profoundly reduced for MV patients, with PdiTw 19 % of normal and QuadTw 6% of normal values. There was a poor correlation between strength and thickness for both muscles, with thickness often remaining in the normal range when strength was severely reduced. Regression analysis revealed reductions in PdiTw correlated with presence of infection (p = 0.006) and age (p = 0.007). QuadTw best correlated with duration of MV (p = 0.036). Limb muscles are profoundly weak in critically ill patients, with a severity that mirrors the level of weakness observed in the diaphragm.

Radiographs assessment of changes in the psoas muscle at L4-L5 level after single-level lateral lumbar interbody fusion in patients with postoperative motor weakness.

The purposes of this study were (1) to investigate postoperative changes in cross-sectional area (CSA) and signal intensity (SI) of the psoas muscle (PS) using magnetic resonance imaging (MRI) and (2) to compare the CSA and SI of the PS between patients with and without motor weakness after single-level lateral lumbar interbody fusion (LLIF) at level L4-L5. Sixty patients were divided into two groups-those with postoperative motor weakness and those without-and the two groups were compared. Baseline demographics and clinical characteristics, such as operation time and blood loss, length of hospital stay, and postoperative complications, were recorded. The CSA and SI of the PS were obtained from the MRI regions of interest defined by manual tracing. Patients who developed motor weakness after surgery were significantly older (p = 0.040). The operation time (p = 0.868), LLIF operative time (p = 0.476), and estimated bleeding loss (p = 0.168) did not differ significantly between groups. In both groups, the CSA and SI of the left and right PS increased after surgery. The change in the CSA of the left PS was significantly higher in patients with weakness (247.6 ± 155.2 mm2) than without weakness (152.2 ± 133.1 mm2) (p = 0.036). The change in SI of the left PS did not differ between the two groups (p = 0.530). To prevent postoperative motor weakness regardless of the operation time, surgeons should be aware of the potential for surgical invasive of the PS during LLIF in older people.

Spectrum of Clinical Features in X-Linked Myotubular Myopathy Carriers: An International Questionnaire Study.

OBJECTIVE: To characterize the spectrum of clinical features in a cohort of X-linked myotubular myopathy (XL-MTM) carriers, including prevalence, genetic features, clinical symptoms, and signs, as well as associated disease burden. METHODS: We performed a cross-sectional online questionnaire study among XL-MTM carriers. Participants were recruited from patient associations, medical centers, and registries in the United Kingdom, Germany, and the Netherlands. We used a custom-made questionnaire, the Checklist Individual Strength (CIS), the Frenchay Activities Index (FAI), the Short Form 12 (SF-12) health survey, and the McGill Pain Questionnaire. Carriers were classified as manifesting or nonmanifesting on the basis of self-reported ambulation and muscle weakness. RESULTS: The prevalence of manifesting carriers in this study population (n = 76) was 51%, subdivided into mild (independent ambulation, 39%), moderate (assisted ambulation, 9%), and severe (wheelchair dependent, 3%) phenotypes. In addition to muscle weakness, manifesting carriers frequently reported fatigue (70%) and exercise intolerance (49%). Manifesting carriers scored higher on the overall CIS (p = 0.001), the fatigue subscale (p < 0.001), and least severe pain subscale (p = 0.005) than nonmanifesting carriers. They scored lower on the FAI (p = 0.005) and the physical component of the SF-12 health survey (p < 0.001). CONCLUSIONS: The prevalence of manifesting XL-MTM carriers may be higher than currently assumed, most having a mild phenotype and a wide variety of symptoms. Manifesting carriers are particularly affected by fatigue, limitations of daily activities, pain, and reduced quality of life. Our findings should increase awareness and provide useful information for health care providers and future clinical trials.

High prevalence and clinical impact of dynapenia and sarcopenia in Japanese patients with type 1 and type 2 diabetes: Findings from the Impact of Diabetes Mellitus on Dynapenia study.

AIMS/INTRODUCTION: The present study aimed to clarify the prevalence and clinical characteristics of sarcopenia and dynapenia, which are muscle weakness with and without low muscle mass, respectively, in Japanese patients with type 1 diabetes mellitus and type 2 diabetes mellitus. MATERIALS AND METHODS: This cross-sectional study enrolled 1,328 participants with type 1 diabetes (n = 177), type 2 diabetes (n = 645) and without diabetes (n = 506). Sarcopenia was defined as a low grip strength and slow gait speed with low skeletal muscle mass index, whereas dynapenia was defined as low strengths of grip and knee extension with a normal skeletal muscle mass index. Participants without sarcopenia and dynapenia were defined as robust. RESULTS: Among participants aged ≥65 years, sarcopenia and dynapenia were observed in 12.2% and 0.5% of individuals without diabetes, 42.9% and 11.4% of type 1 diabetes patients, and 20.9% and 13.9% of type 2 diabetes patients. In both type 1 diabetes and type 2 diabetes patients, sarcopenic patients were significantly older and thinner, and showed a significantly higher rate of diabetic neuropathy than robust patients. In patients with type 1 diabetes and type 2 diabetes, dynapenic patients were older, and showed a higher rate of diabetic neuropathy and lower estimated glomerular filtration rate than robust patients. Patients complicated with sarcopenia and dynapenia showed a significantly lower physical quality of life and higher rate of incidental falls than robust patients. CONCLUSIONS: Sarcopenia and dynapenia were more frequent in patients with type 1 diabetes and type 2 diabetes than in individuals without diabetes, which might contribute to their impaired quality of life and incidental falls.

Multimodal Assessment of the Motor System in Patients With Chronic Ischemic Stroke.

BACKGROUND AND PURPOSE: Despite continuing efforts in the multimodal assessment of the motor system after stroke, conclusive findings on the complementarity of functional and structural metrics of the ipsilesional corticospinal tract integrity and the role of the contralesional hemisphere are still lacking. This research aimed to find the best combination of motor system metrics, allowing the classification of patients into 3 predefined groups of upper limb motor recovery. METHODS: We enrolled 35 chronic ischemic stroke patients (mean 47 [26-66] years old, 29 [6-58] months poststroke) with a single supratentorial lesion and unilateral upper extremity weakness. Patients were divided into 3 groups, depending on upper limb motor recovery: good, moderate, and bad. Nonparametric statistical tests and regression analysis were used to investigate the relationships among microstructural (fractional anisotropy (FA) ratio of the corticospinal tracts at the internal capsule (IC) level (classic method) and along the length of the tracts (Fréchet distance), and of the corpus callosum) and functional (motor evoked potentials [MEPs] for 2 hand muscles) motor system metrics. Stratification rules were also tested using a decision tree classifier. RESULTS: IC FA ratio in the IC and MEP absence were both equally discriminative of the bad motor outcome (96% accuracy). For the 3 recovery groups' classification, the best parameter combination was IC FA ratio and the Fréchet distance between the contralesional and ipsilesional corticospinal tract FA profiles (91% accuracy). No other metrics had any additional value for patients' classification. MEP presence differed for 2 investigated muscles. CONCLUSIONS: This study demonstrates that better separation between 3 motor recovery groups may be achieved when considering the similarity between corticospinal tract FA profiles along its length in addition to region of interest-based assessment and lesion load calculation. Additionally, IC FA ratio and MEP absence are equally important markers for poor recovery, while for MEP probing it may be important to investigate more than one hand muscle.

Evaluation of persons with suspected lumbosacral and cervical radiculopathy: Electrodiagnostic assessment and implications for treatment and outcomes (Part I).

Persons with back, neck, and limb symptoms constitute a major referral population to specialists in electrodiagnostic (EDX) medicine. The evaluation of these patients involves consideration of both the common and less common disorders. The EDX examination with needle electromyography (EMG) is the most important means of testing for radiculopathy. This test has modest sensitivity but high specificity and well complements imaging of the spine. Needle EMG in combination with nerve conduction testing is valuable in excluding entrapment neuropathies and polyneuropathy-conditions that frequently mimic radicular symptoms. In this first of a two-part review, the optimal EDX evaluation of persons with suspected radiculopathy is presented. In part two, the implications of EDX findings for diagnosis and clinical management of persons with radiculopathy are reviewed.

Long-term consequences in survivors of critical illness. Analysis of incidence and risk factors.

AIM: This study investigates the incidence of long-term consequences in survivors of critical illness 6 months after ICU care. A retrospective analysis of the risk factors was also completed. METHODS: A mixed-method design was used. A qualitative design was used in the questionnaire study (phase 1), and a quantitative design was used for the retrospective study (phase 2). RESULTS: 116 patients were interviewed. Forty-eight patients (41.4%) reported at least one long-term consequence 6 months after ICU discharge. The most frequent consequences were anxiety (n = 33, 28.4%), depression (n = 32, 27.6%) and chronic pain (n = 24, 20.7%). The interview showed the concurrent caseness of PTSD, anxiety and depression in 14 (12.1%) patients. Observed risk factors were age > 60 years (OR = 2.65, IC = 1.23-5.69; p = 0.0119), trauma diagnosis (OR = 5.3, IC = 1.60-17.76; p = 0.0033), length of mechanical ventilation > 7 days (OR = 2.18, IC = 1-4.74; p = 0.0471) length of ICU stay > 10 days (OR = 2.47, IC = 1.16-5.26; p = 0.0185) and clinical conditions at the ICU admission. The quality of life score was lower if the respondent had long-term consequences. DISCUSSION: A high incidence of long-term consequences is found in survivors of critical illness. In future, studies that investigate interventions to prevent these issues after ICU care are need.

Longitudinal Assessment of Quadriceps Muscle Morphology Before and After Anterior Cruciate Ligament Reconstruction and Its Associations With Patient-Reported Outcomes.

BACKGROUND: Reductions in muscle size are common after anterior cruciate ligament reconstruction (ACLR) and may contribute to suboptimal patient outcomes. However, few studies have quantified postoperative alterations in muscle quality and evaluated its associations with patient-reported function. HYPOTHESES: Rectus femoris cross-sectional area (CSA) will decrease postoperatively but improve at return to activity (RTA), rectus femoris muscle quality (percentage fat [PF]) will increase postoperatively and be greater at RTA compared with preoperative values, and rectus femoris CSA and PF will be associated with International Knee Documentation Committee (IKDC) scores at both postoperative time points. STUDY DESIGN: Case series. LEVEL OF EVIDENCE: Level 4. METHODS: A total of 26 individuals who sustained an ACL injury and underwent reconstructive surgery were evaluated preoperatively (T0), 9 weeks post-ACLR (T1), and at RTA. Rectus femoris CSA and PF were evaluated bilaterally via ultrasound imaging, and patient-reported function was assessed using the IKDC score. RESULTS: Bilateral reductions in rectus femoris CSA were noted from T0 to T1 (P < 0.01). Only the uninvolved limb returned to preoperative CSA (P = 0.80), as the involved limb failed to return to preoperative levels at RTA (P = 0.04). No significant changes in rectus femoris PF were observed across time points (P > 0.05). Lesser PF (P < 0.01) but not CSA (P = 0.75) was associated with higher IKDC score at T1. Lesser PF (P = 0.04) and greater CSA (P = 0.05) was associated with higher IKDC score at RTA. CONCLUSION: Substantial atrophy occurs bilaterally after ACLR, and the involved limb does not return to preoperative muscle size despite the patient completing rehabilitation. Quadriceps muscle morphology is associated with patient-reported function and may be an important rehabilitation target after ACLR. CLINICAL RELEVANCE: Quadriceps atrophy and poor muscle quality may contribute to suboptimal patient functioning and quadriceps dysfunction and may be important in RTA decision making. Assessing muscle morphology using ultrasound may be a feasible and clinically beneficial tool in patients after ACLR.

Peak Cough Flow in Children with Neuromuscular Disorders.

PURPOSE: Patients with neuromuscular disease (NMD) experience weakened cough due to progressive respiratory muscle weakness. Peak cough flow (PCF) measurements derived from adult populations are used to recommend initiation of assisted cough therapies. The objective of this study was to characterize PCF values among pediatric patients with NMD. METHODS: Retrospective chart review was performed for patients seen in the multidisciplinary pediatric muscular dystrophy clinic from 2010 to 2016. Clinical and demographic variables included age, gender, ambulation status, and PCF measurements. RESULTS: 366 patients with an established diagnosis of NMD (median age 11.8 years) were included in this study. 102 (27.8%) out of the 366 patients were affected by Duchenne muscular dystrophy (DMD), 42 (11.5%) by congenital muscular dystrophy (CMD), 42 (11.5%) by Charcot Marie Tooth disease (CMT) and 24 (6.5%) by Becker's muscular dystrophy (BMD). The mean PCF values in DMD (255.8 L/min) and CMD (249.1 L/min) were lower than CMT (321.5 L/min) with p-values of 0.007 and 0.02, respectively. The mean PCF of BMD (333.3 L/min) was higher than that of DMD and CMD but the difference was not statistically significant. PCFs were not statistically different between ambulatory and non-ambulatory status (263.0 L/min versus 290.8 L/min, p = 0.12). Children under 10 years of age had lower PCF relative to older subjects (179.5 L/min versus 300.9 L/min, p < 0.0001). CONCLUSION: Baseline PCF values in young children are below the adult-specific values suggested for starting assisted cough techniques. Further longitudinal trials are required to derive pediatric-specific reference values for PCF in patients with NMD.

Patient-reported disease burden in oculopharyngeal muscular dystrophy.

INTRODUCTION: There is currently little evidence regarding oculopharyngeal muscular dystrophy (OPMD) disease burden reported by patients. In this study we aim to elicit direct patient input regarding OPMD disease burden. METHODS: We conducted semistructured interviews with 25 participants with genetically confirmed OPMD and a wide range of disease duration (15 ± 8 years). Using the Framework Technique, themes and categories were then extracted. RESULTS: Analyses revealed 7 themes (physical impact, mental impact, social impact, perception of progression, treatment perceptions, coping strategies, and access to disease information), encompassing 27 categories of OPMD disease burden. The most frequent categories were related to dysphagia, coping strategies for dysphagia, and impaired mobility. DISCUSSION: This study demonstrates the importance of considering, when providing clinical care, the broad range of coping strategies patients use to deal with OPMD symptoms, especially dysphagia, to properly assess limitations and monitor real disease progression.

Patient-reported study of the impact of pediatric-onset myotonic dystrophy.

INTRODUCTION: The prevalence and impact of symptoms affecting individuals with pediatric forms of myotonic dystrophy type-1 (DM1) are not well understood. METHODS: Patients from the United States, Canada, and Sweden completed a survey that investigated 20 themes associated with pediatric-onset DM1. Participants reported the prevalence and importance of each theme affecting their lives. Surveys from participants were matched with surveys from their caregivers for additional analysis. RESULTS: The most prevalent symptomatic themes included problems with hands or fingers (79%) and gastrointestinal issues (75%). Problems with urinary/bowel control and gastrointestinal issues were reported to have the greatest impact on patients' lives. Responses from participants and their caregivers had varying levels of agreement among symptomatic themes. DISCUSSION: Many symptoms have meaningful impact on disease burden. The highest levels of agreement between caregivers and individuals with pediatric forms of myotonic dystrophy were found for physical activity themes.

[Assessment of the respiratory muscles strength at various stages of ischemic stroke]. / Otsenka sily dykhatel'nykh myshts na razlichnykh stadiiakh ishemicheskogo insul'ta.

Power characteristics of the respiratory muscles (RM) in the dynamics of ischemic stroke (IS) is an important tool for the early diagnosis of RM dysfunction and development of individual programs for treatment at different stages of rehabilitation. AIM: To assess the power of RM in patients with IS at different stages of the disease. MATERIAL AND METHODS: Power characteristics of RM of 56 patients with IS with different severity of the disease were examined after 2-4, 5-6 and 13-14 months of IS onset. Maximum inspiratory (MIP) and expiratory (MEP) pressures in the oral cavity, maximum rate of pressure development (MRPD) and sniff nasal inspiratory pressure (SNIP) were determined. RESULTS AND CONCLUSION: A decrease in expiratory and inspiratory RM strength was shown in the early and late periods of IS, severity of which was depended on the severity of neurologic disorders. The bronchial obstruction resulted in RM dysfunction. Expiratory muscles weakness only was observed in the remote period while one third of patients had obstructive disorders of breathing. The functional activity of different RM at different IS stages was correlated with the smoking index, forced expiratory volume in the first second, forced vital capacity, skeletal muscles mass and severity of dyspnea, asthenia and disturbances of vital functions. These differences were the most obvious in elderly group of people. RM power examination of IS patients is an important mechanism of early diagnosis and rehabilitation.

Quantitative grip force assessment of muscular weakness in chronic inflammatory demyelinating polyneuropathy.

BACKGROUND: In patients suffering from Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) disease severity is assessed by Medical Research Counsil (MRC) Scale or Inflammatory Neuropathy Cause and Treatment (INCAT) disability score. However, none of these methods is appropriate to objectively assess muscle weakness or to detect very small subclinical changes. More objective and quantitative measures are needed in order to evaluate treatment efficiency or to detect subclinical affection of upper limps for early diagnosis. The goal of our study was to objectively quantify muscular weakness in CIDP patients with the non-invasive Quantitative Motor (Q-Motor) test of Grip Force Assessment (QGFA) as well as the Involuntary Movement Assessment (QIMA) and to search for differences between typical and atypical CIDP variants. In addition, we hypothesized that Q-Motor findings correlate with disease severity scales such as MRC or INCAT score. METHODS: In this cross-sectional exploratory proof-of-concept study subjects with confirmed diagnosis of typical or atypical CIDP were examined and compared to healthy controls (HC). For Q-Motor tests all subjects had to lift a device (250 g and 500 g) equipped with an electromagnetic sensor that measured grip force (GF) and three-dimensional changes in position and orientation. The measures "grip force variability" (GFV), "position index" (PI) and "orientation index" (OI) were provided to assess involuntary movements due to muscular weakness. RESULTS: 33 patients with CIDP and 28 HC were included. All measures were significantly elevated in CIDP patients for both devices in the right and left hand compared to healthy controls. Subgroup analysis revealed no differences between typical and atypical CIDP variants. INCAT score only weakly correlated with OI and PI. However, there was a stronger correlation between MRC and QIMA parameters in both hands. CONCLUSION: Q-Motor assessments were capable to objectively assess muscular weakness in CIDP. In particular, QIMA measures detected subclinical generalized muscle weakness even in patients with milder disability. Sensitivity and rater-independence of Q-Motor assessments support a further exploration of QIMA measures as potential endpoints for future clinical trials in CIDP.

Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

Quantitative Assessment of Muscle Strength Following "Slow" Surgical Lengthening of the Medial Hamstring Muscles in Children With Cerebral Palsy.

BACKGROUND: Classic teaching for surgical lengthening of muscle contractures in children with cerebral palsy (CP) has emphasized complete correction of the deformity acutely, with immobilization of the targeted muscles in the fully corrected position. Clinical experience has led to the impression that the muscles are invariably weakened by this approach. We have developed an alternative technique for correction of contractures called slow surgical lengthening (SSL). The goal of the study was to determine the physical examination, kinematic, and muscle strength outcomes following SSL of the medial hamstring muscles in children with CP. METHODS: The study group included 41 children with CP who underwent SSL of the medial hamstring muscles as part of a comprehensive single-event multilevel surgery, who had preoperative and 1-year postoperative evaluations in our Motion Analysis Center, which included quantitative assessment of isometric and isokinetic muscle strength. RESULTS: All subjects were Gross Motor Function Classification System I and II. Mean age at the time of surgery was 10.8 years. The mean popliteal angle improved by 16.2 degrees (P<0.001) following SSL of the medial hamstrings. Sagittal plane kinematics following SSL of the medial hamstrings showed improvement of knee extension at initial contact of 10.2 degrees (P<0.001), decrease of peak knee flexion in mid-swing of 3.6 degrees (P=0.014), improved minimum knee flexion in stance of 4.9 degrees (P=0.002), and no significant change in mean anterior pelvic tilt (P=0.123). Mean peak isometric knee flexion torque remained unchanged from preoperative to postoperative studies (P=0.154), whereas mean peak isokinetic knee flexion torque significantly increased by 0.076 Nm/kg (P=0.014) following medial hamstring SSL. DISCUSSION: SSL was developed based upon clinical experience and improved understanding of the pathophysiology of skeletal muscle in children with CP. The SSL technique allows the tendinous tissue to separate spontaneously at the time of recession, but does not force further acute lengthening by intraoperative manipulation, thereby minimizing the damage to the underlying muscle. It is broadly believed that muscle weakness is inevitable following surgical lengthening. The current study shows that the SSL technique does not cause weakness. LEVEL OF EVIDENCE: Level IV-therapeutic.

Healthcare costs and utilization associated with muscle weakness diagnosis codes in patients with chronic obstructive pulmonary disease: a United States claims analysis.

AIMS: Muscle weakness (MW)-attributable healthcare resource utilization (HCRU) and costs in patients with chronic obstructive pulmonary disease (COPD) have not been well-characterized in US insurance claims databases. The primary objective of this study was to estimate HCRU in patients with evidence of COPD with and without MW diagnosis codes. MATERIALS AND METHODS: This retrospective analysis used the MarketScan® Commercial Claims and Encounters and Medicare Supplemental and Coordination of Benefits databases. Between January 2007 and March 2016, we identified patients aged ≥40 years with diagnosis codes for COPD (≥1 emergency department or inpatient claim or ≥2 outpatient claims within 1 year). The cohort was divided into patients with and without ≥1 MW diagnosis code. Propensity score matching was used to generate pairs of patients with and without MW (1:1). Multivariable regression analyses were used to estimate adjusted incremental costs and utilization attributable to the presence of MW diagnosis codes among patients with COPD. RESULTS: Of 427,131 patients who met the study inclusion criteria, 14% had evidence of MW. After matching, 107,420 unique patients remained equally distributed across MW status. Patients with MW diagnosis codes had greater predicted annual HCRU, $2,465 greater total predicted annual COPD-related costs, and $15,179 greater total all-cause costs than those without MW diagnosis codes. Overall, <1% of patients received COPD-related pulmonary rehabilitation services. LIMITATIONS: Study limitations include the potential for undercoding of MW and lack of information on severity of MW in claims data. CONCLUSION: The presence of MW diagnosis codes yielded higher HCRU in this COPD population and suggests that the burden of MW affects both all-cause and COPD-related care. However, utilization of pulmonary rehabilitation, a known effective treatment for MW, remains low. Future research should expand on our results by assessing data sources that allow for clinical confirmation of MW among patients with COPD.

Assessment of Respiratory Muscle Weakness in Subjects With Neuromuscular Disease.

INTRODUCTION: Neuromuscular diseases (NMD) are a group of rare heterogeneous disorders that may be accompanied by respiratory muscle weakness. The simplest measurements of respiratory muscle strength are maximum inspiratory pressure (PImax) and maximum expiratory pressure (PEmax) of the mouth. Inspiratory muscle weakness can also be evaluated by the sniff test (sniff nasal inspiratory pressure method). This study tested the agreements in PImax and PEmax (measured by using a plethysmograph and portable equipment) as well as the correlations of PImax and PEmax by using the sniff nasal inspiratory pressure method, lung function, and arterial blood gas parameters in subjects with NMD. METHODS: This prospective, noninterventional study measured respiratory parameters in all the subjects with NMD who underwent measurement of maximum respiratory pressures. RESULTS: A total of 55 subjects with NMD were included. There were no statistically significant differences in PImax and PEmax measured by using a plethysmograph and portable equipment. Moreover, PImax showed a good correlation with the sniff nasal inspiratory pressure method. CONCLUSIONS: Measurements of PImax and PEmax by using portable equipment were equivalent to those performed by using the accepted standard, plethysmography, in the subjects with NMD. Noninvasive evaluation of the sniff test with the portable equipment correlates with PImax, which makes this approach a good method for measuring the maximum strength of inspiratory muscles in patients with NMD.