Epidemiology of Spontaneous Cervical Artery Dissection: Population-Based Study.

BACKGROUND: Cervical artery dissection (CeAD) represents up to 15% to 25% of ischemic strokes in people under the age of 50 years. Noninvasive vessel imaging is increasingly used in clinical practice, but the impact on the frequency of detection of CeAD is unknown. In 2006, the yearly incidence rate of CeAD was estimated at 2.6 per 100 000 person-years, but the current incidence is unknown. METHODS: In this population-based retrospective observational cohort study, we utilized the resources of the Rochester Epidemiology Project to ascertain all adult residents of Olmsted County, MN, diagnosed with internal carotid artery dissection and common carotid artery dissection or vertebral artery dissection from 2002 to 2020. Patients with only intracranial involvement or CeAD following major trauma were excluded. Age-adjusted sex-specific and age- and sex-adjusted incidence rates were estimated using the US White 2010 decennial census, with rates expressed per 100 000 person-years. We assessed longitudinal trends by dividing the data into 5-year time intervals, with the last being a 4-year interval. RESULTS: We identified 123 patients with a diagnosis of CeAD. There were 63 patients with internal carotid artery dissection, 54 with vertebral artery dissection, 2 with concurrent internal carotid artery dissection and vertebral artery dissection, and 4 with common carotid artery dissection. There were 63 (51.2%) female patients and 60 (48.8%) male patients. The average age at diagnosis was 50.2 years (SD, 15.1 [95% CI, 20.1-90.5] years). The incidence rate of spontaneous CeAD encompassing all locations was 4.69 per 100 000 person-years (2.43 for internal carotid artery dissection and 2.01 for vertebral artery dissection). The incidence rate increased from 2.30 per 100 000 person-years from 2002 to 2006 to 8.93 per 100 000 person-years from 2017 to 2020 (P<0.0001). The incidence rate for female patients rose from 0.81 per 100 000 person-years from 2002 to 2006 to 10.17 per 100 000 person-years from 2017 to 2020. CONCLUSIONS: The incidence rate of spontaneous CeAD increased nearly 4-fold over a 19-year period from 2002 to 2020. The incidence rate in women rose over 12-fold. The increase in incidence rates likely reflects the increased use of noninvasive vascular imaging.

Trial Readiness of Cavernous Malformations With Symptomatic Hemorrhage, Part I: Event Rates and Clinical Outcome.

BACKGROUND: Cerebral cavernous malformation with symptomatic hemorrhage (SH) are targets for novel therapies. A multisite trial-readiness project (https://www.clinicaltrials.gov; Unique identifier: NCT03652181) aimed to identify clinical, imaging, and functional changes in these patients. METHODS: We enrolled adult cerebral cavernous malformation patients from 5 high-volume centers with SH within the prior year and no planned surgery. In addition to clinical and imaging review, we assessed baseline, 1- and 2-year National Institutes of Health Stroke Scale, modified Rankin Scale, European Quality of Life 5D-3 L, and patient-reported outcome-measurement information system, Version 2.0. SH and asymptomatic change rates were adjudicated. Changes in functional scores were assessed as a marker for hemorrhage. RESULTS: One hundred twenty-three, 102, and 69 patients completed baseline, 1- and 2-year clinical assessments, respectively. There were 21 SH during 178.3 patient years of follow-up (11.8% per patient year). At baseline, 62.6% and 95.1% of patients had a modified Rankin Scale score of 1 and National Institutes of Health Stroke Scale score of 0 to 4, respectively, which improved to 75.4% (P=0.03) and 100% (P=0.06) at 2 years. At baseline, 74.8% had at least one abnormal patient-reported outcome-measurement information system, Version 2.0 domain compared with 61.2% at 2 years (P=0.004). The most common abnormal European Quality of Life 5D-3 L domains were pain (48.7%), anxiety (41.5%), and participation in usual activities (41.4%). Patients with prospective SH were more likely than those without SH to display functional decline in sleep, fatigue, and social function patient-reported outcome-measurement information system, Version 2.0 domains at 2 years. Other score changes did not differ significantly between groups at 2 years. The sensitivity of scores as an SH marker remained poor at the time interval assessed. CONCLUSIONS: We report SH rate, functional, and patient-reported outcomes in trial-eligible cerebral cavernous malformation with SH patients. Functional outcomes and patient-reported outcomes generally improved over 2 years. No score change was highly sensitive or specific for SH and could not be used as a primary end point in a trial.

Phage K exposure generates phage-neutralizing activity in a rabbit model-humoral receptiveness may impact efficacy of phage therapy.

Phage therapy has not been established in the clinical routine, in part due to uncertainties concerning efficacy and immunogenicity. Here, three rabbits were immunized against staphylococcal phage K to assess viral potency in the presence of immunized serum. Three rabbits received weekly intramuscular injections of ~1010±1 pfu/mL phage K. Phage K-specific IgG formation was measured by an enzyme-linked immunosorbent assay (ELISA); phage inactivation was assessed by calculating K-rates. Using transmission electron microscopy (TEM) and immunogold labeling, antibody binding to phage K was visualized. This was numerically assessed by objective imaging analysis comparing the relative distances of each gold particle to the nearest phage head and tail structure. Immunization led to a strong IgG response, plateauing 7 days after the last phage injection. There was no significant correlation between K-rate and antibody titer over time. TEM showed IgG binding to the head structure of phage K. Image analysis showed a significant reduction in relative distances between antibodies and phage head structures when comparing samples from day 0 and day 28 (P < 0.0001). These results suggest that while individual serum analysis for antibodies against therapeutic phage bears consideration prior to and with prolonged therapy, during phage application, the formation of specific antibodies against phage may only partially explain decreased phage potency in the presence of immunized serum. Instead, other factors may contribute to an individual's "humoral receptiveness" to phage therapy. Future investigations should be directed toward the identification of the humoral factors that have the most significant predictive value on phage potency in vivo.

Use of a Shear Reduction Surface for Prehospital Transport: A Randomized Crossover Study.

OBJECTIVE: To compare the effectiveness of an antishear mattress overlay (ASMO) with a standard ambulance stretcher surface in reducing pressure and shear and increasing patient comfort. METHODS: In this randomized, crossover design, adults in three body mass index categories served as their own controls. Pressure/shear sensors were applied to the sacrum, ischial tuberosity, and heel. The stretcher was placed in sequential 0°, 15°, and 30° head-of-bed elevations with and without an ASMO. The ambulance traveled a closed course, achieving 30 mph, with five stops at each head-of-bed elevation. Participants rated discomfort after each series of five runs. RESULTS: Thirty individuals participated. Each participant had 30 runs (15 with an ASMO, 15 without), for a total of 900 trial runs. The peak-to-peak shear difference between support surfaces was -0.03 N, indicating that after adjustment for elevation, sensor location, and body mass index, peak shear levels at baseline (starting pause) were 0.03 N lower for the ASMO than for the standard surface ( P = .02). The peak-to-peak pressure difference between surfaces was -0.16 mm Hg, indicating that prerun peak-to-peak pressure was 0.16 mm Hg lower with the ASMO versus standard surface ( P = .002). The heel received the most pressure and shear. Discomfort score distributions differed between surfaces at 0° ( P = .004) and 30° ( P = .01); the overall score across all elevations was significantly higher with the standard surface than with the ASMO ( P = .046). CONCLUSIONS: The ASMO reduced shear, pressure, and discomfort. During transport, the ambulance team should provide additional heel offloading.

Sural nerve biopsy utility by masked assessment of individual histologic preparations.

BACKGROUND AND AIMS: Comprehensive study of sural nerve biopsy utility based on individual histopathologic preparations is lacking. We aimed to quantify the value of different histologic preparations in diagnosis. METHODS: One hundred consecutive sural nerves were studied by standard histological preparations plus graded teased nerve fibers (GTNF), immunohistochemistry, and epoxy-semithin morphometry. Three examiners scored the individual preparations separately by a questionnaire of neuropathic and interstitial abnormalities, masked to the biopsy number, versus a gold-standard of all preparations. Multivariate modeling was utilized to determine best approach versus the gold-standard. RESULTS: Highest confidence (range 8-9 of 10) and inter-rater reliability (99%) for fiber abnormalities came from GTNF, and interstitial abnormalities from paraffin stains (range 7-8, 99%). Vasculitic neuropathy associated with GTNF axonal degeneration (moderate to severe 79%) with OR 3.8, 95% CI (1.001-14.7), p = .04, but not significantly with the other preparations. Clinicopathologic diagnoses associated with teased fiber abnormalities in chronic inflammatory demyelinating polyradiculoneuropathy, 80% (8/10); amyloidosis, 50% (1/2); adult-onset polyglucosan disease 100% (1/1). GTNF and paraffin stains significantly correlated with fiber density determined by morphometric analysis (GTNF: OR 9.9, p < .0001, paraffin: OR 3.8, p = .03). GTNF combined with paraffin sections had highest accuracy for clinicopathologic diagnoses and fiber density with 0.86 C-stat prediction versus morphometric analysis. Pathological results lead to initiation or changes of immunotherapy in 70% (35/50; initiation n = 22, reduction n = 9, escalation n = 4) with the remaining having alternative intervention or no change. INTERPRETATION: Nerve biopsy paraffin stains combined with GTNF have highest diagnostic utility, confidence, inter-rater reliability, improving accuracy for a pathologic diagnosis aiding treatment recommendations. Immunostains and epoxy preparations are also demonstrated useful supporting consensus guidelines. This study provides class II evidence for individual nerve preparation utility.

Symptoms in Patients Receiving Noninvasive Ventilation in the Intensive Care Unit.

BACKGROUND: Although a multitude of studies have demonstrated the effectiveness of noninvasive ventilation (NIV) for treatment of respiratory insufficiency, there have been few investigations of patients' experiences while receiving this common treatment. Identification of the presence, intensity, and distress of symptoms during NIV will inform the development and testing of interventions to best manage them and improve patients' intensive care unit (ICU) experiences. OBJECTIVE: The objectives of this study were (a) to identify the presence, intensity, and distress of symptoms in patients receiving NIV in the ICU using a modified version of the Edmonton Symptom Assessment Scale (MESAS) and (b) to describe the most common and distressing symptoms experienced by patients. METHODS: A cross-sectional descriptive design was used with a convenience sample of 114 participants enrolled from three ICUs at one Midwestern medical center. Participants were approached if they were English-speaking, were 18 years old or older, and had an active order for NIV; exclusions included use of personal NIV equipment, severe cognitive impairment, or problems communicating. Demographic and clinical data were obtained from the electronic health record. Presence, intensity, and distress of patient-reported symptoms were obtained once using a modified, 11-item version of the MESAS. RESULTS: The mean age of participants was 68 years old, and 54.4% were male. The primary type of NIV was bi-level positive airway pressure; a nasal/oral mask was most frequently used. The symptoms experienced by most of the participants were thirst, anxiety, tiredness, and restlessness; these symptoms were rated as moderate or severe in both intensity and distress by most participants experiencing the symptoms. DISCUSSION: Patients in the ICU experience both intense and distressful symptoms that can be severe while undergoing treatment with NIV. Future research is warranted to determine these symptoms' interrelatedness and develop interventions to effectively manage patient-reported symptoms.

Inclusion body myositis: correlation of clinical outcomes with histopathology, electromyography and laboratory findings.

OBJECTIVE: To determine whether histopathological, electromyographic and laboratory markers correlate with clinical measures in inclusion body myositis (IBM). METHODS: We reviewed our electronic medical records to identify patients with IBM according to European Neuromuscular Center (ENMC) 2011 criteria, seen between 2015 and 2020. We only included patients who had a muscle biopsy and needle electromyography (EMG) performed on the same muscle (opposite or same side). We used a detailed grading system [0 (normal) to 4 (severe)] to score histopathological and EMG findings. Clinical severity was assessed by the modified Rankin scale (mRS), muscle strength sum score (SSS), quadriceps strength and severity of dysphagia on swallow evaluation. Serum markers of interest were creatine kinase level and cN-1A antibodies. RESULTS: We included 50 IBM patients, with a median age of 69 years; 64% were males. Median disease duration at diagnosis was 51 months. On muscle biopsy, endomysial inflammation mainly correlated with dysphagia, and inversely correlated with mRS. Vacuoles and congophilic inclusions did not correlate with any of the clinical measures. On EMG, the shortness of motor un it potential (MUP) duration correlated with all clinical measures. Myotonic discharges, and not fibrillation potentials, correlated with the severity of inflammation. Serum markers did not have a statistically significant correlation with any of the clinical measures. CONCLUSIONS: Dysphagia was the main clinical feature of IBM correlating with endomysial inflammation. Otherwise, inclusion body myositis clinical measures had limited correlation with histopathological features in this study. The shortness of MUP duration correlated with all clinical measures.

Survival and associated comorbidities in inclusion body myositis.

OBJECTIVE: To evaluate survival and associated comorbidities in inclusion body myositis (IBM) in a population-based, case-control study. METHODS: We utilized the expanded Rochester Epidemiology Project medical records-linkage system, including 27 counties in Minnesota and Wisconsin, to identify patients with IBM, other inflammatory myopathies (IIM), and age/sex-matched population-controls. We compared the frequency of various comorbidities and survival among groups. RESULTS: We identified 50 IBM patients, 65 IIM controls and 294 population controls. Dysphagia was most common in IBM (64%) patients. The frequency of neurodegenerative disorders (dementia/parkinsonism) and solid cancers was not different between groups. Rheumatoid arthritis was the most common rheumatic disease in all groups. A total of 36% of IBM patients had a peripheral neuropathy, 6% had Sjögren's syndrome and 10% had a haematologic malignancy. T-cell large granular lymphocytic leukaemia was only observed in the IBM group. None of the IBM patients had hepatitis B or C, or HIV. IBM patients were 2.7 times more likely to have peripheral neuropathy, 6.2 times more likely to have Sjögren's syndrome and 3.9 times more likely to have a haematologic malignancy than population controls. IBM was associated with increased mortality, with a 10-year survival of 36% from index, compared with 67% in IIM and 59% in population controls. Respiratory failure or pneumonia (44%) was the most common cause of death. CONCLUSIONS: IBM is associated with lower survival, and higher frequency of peripheral neuropathy, Sjögren's syndrome and haematologic malignancies than the general population. Close monitoring of IBM-related complications is warranted.

Cancer and immune-mediated necrotizing myopathy: a longitudinal referral case-controlled outcomes evaluation.

OBJECTIVES: To investigate immune-mediated necrotizing myopathy (IMNM) association with cancer and its clinical implications. METHODS: IMNM cases were identified 1 January 2000 to 31 December 2020 matching sex and age controls (4:1). RESULTS: A total of 152 patients with IMNM were identified and among serologically tested, 60% (83/140) were HMGCR-IgG+, 14% (20/140) were SRP-IgG+ and 26% (37/140) were seronegative. Cancer rates were not significantly different between serological subgroups; 18.1% (15/83) HMGCR-IgG+, 25% (5/20) SRP-IgG+ and 30% (11/37) seronegative (P = 0.34). Cancer screening was performed within 12 months from IMNM diagnosis in 88% (134/152) (whole-body CT plus FDG-PET CT in 53, CT alone in 72 and FDG-PET alone in 9). FDG-PET/CT was positive in 73% (25/34) of cancers. Increasing age was the only risk associated with cancer (P = 0.02). The odds of developing cancer at ±3 or ±5 years from IMNM diagnosis was not higher than controls (OR = 0.49; CI: 0.325-0.76). Lifetime IMNM diagnosis of cancer was less compared with controls (OR = 0.5 CI: 0.33-0.78, P = 0.002). Most patients responded to treatment (137/147, P < 0.001). Death and treatment response did not significantly differ between cancer [23% (8/34); 88% (29/33)] and non-cancer patients [19% (23/118); 92% (108/118)]. In total, 13% (20/152) of patients died during follow-up compared with 14% (41/290) of medicine and 16% (46/290) of neurology controls (P = 0.8). Seropositives had greater life expectancy than seronegatives (P = 0.01). CONCLUSIONS: Greater cancer risk is not observed in IMNM vs controls. Cancer screening in IMNM should be individualized based on age-personal and family history, including consideration of FDG-PET/CT. Immune-treatment response did not differ with cancer.

Systemic manifestations and symptom burden of facioscapulohumeral muscular dystrophy in a referral cohort.

INTRODUCTION/AIMS: The full spectrum of the clinical phenotype of facioscapulohumeral muscular dystrophy (FSHD), beyond skeletal muscle weakness, remains poorly characterized. In this study, we describe systemic manifestations and symptom burden in a large series of FSHD patients. METHODS: We performed a retrospective chart review of FSHD patients seen at our institution between 2000 and 2017. We reviewed patients' responses to a comprehensive review of symptoms and the results of diagnostic testing for sensorineural hearing loss, cardiac disease, dysphagia, ocular abnormalities, and respiratory insufficiency. We assessed the association between disease manifestations and age of onset, genetic profile, and disease duration. RESULTS: We identified 87 patients with FSHD. The most common reported symptoms included pain (71%), difficulty sleeping (41%), headaches (27%), and altered mood (24%). When tested, 7 of 16 (44%) patients had sensorineural hearing loss, 20 of 60 (33%) had cardiac arrhythmias or conduction defects, 17 of 45 (38%) had echocardiogram abnormalities, 12 of 25 (48%) had reduced forced vital capacity, and 4 of 10 (40%) had oropharyngeal dysphagia. However, patients with these abnormalities represented 8%, 23%, 20%, 14%, and 5% of total number of patients, respectively, as uniform screening was lacking. Ocular pathology attributable to FSHD was not detected. DISCUSSION: FSHD demonstrates a broad clinical phenotype. Increased vigilance among neurologists to screen for systemic manifestations of the disease is warranted. More uniform screening and future population-based studies are needed to compare findings in FSHD patients with the general population.

Utility of Carpal Tunnel Release and Ulnar Decompression in CMT1A and HNPP.

INTRODUCTION/AIMS: Carpal and cubital tunnel syndrome (CTS, CuTS) are common among patients with hereditary neuropathy with liability to pressure-palsies (HNPP) and Charcot-Marie-Tooth type 1A (CMT1A) and may impact quality of life. We aimed to evaluate the utility of nerve decompression surgeries in these patients. METHODS: Medical records were reviewed for patients with PMP22 mutations confirmed in Mayo Clinic laboratories from January 1999 to December 2020, who had CTS and CuTS and underwent surgical decompression. RESULTS: CTS occurred in 53.3% of HNPP and 11.5% of CMT1A, while CuTS was present in 43.3% of HNPP and 5.8% of CMT1A patients. CTS decompression occurred in 10-HNPP and 5-CMT1A patients, and CuTS decompression with/without transposition was performed in 5-HNPP and 1-CMT1A patients. In HNPP, electrodiagnostic studies identified median neuropathy at the wrist in 9/10 patients and ultrasound showed focal enlargements at the carpal and cubital tunnels. In CMT1A, median and ulnar sensory responses were all absent, and the nerves were diffusely enlarged. After CTS surgery, pain, sensory loss, and strength improved in 4/5 CMT1A, and 6/10 HNPP patients. Of clinical, electrophysiologic and ultrasound findings, only activity-provoked features significantly correlated with CTS surgical benefit in HNPP patients (odds ratio = 117.0:95% confidence interval, 1.94 > 999.99, p = 0.01). One CMT1A and one HNPP patient improved with CuTS surgery while 2 HNPP patients worsened. DISCUSSION: CTS symptom improvement post-surgery can be seen in CMT1A and (less frequent) in HNPP patients. CuTS surgery commonly worsened course in HNPP. Activity-provoked symptoms in HNPP best informed benefits from CTS surgery.

CONNECT: A pragmatic clinical trial testing a remotely provided linkage to service coordination after hospitalization for TBI.

OBJECTIVE: To test whether a complex behavioral intervention delivered remotely to connect individuals to clinical resources after hospitalization for TBI improved their quality of life. DESIGN/METHODS: Community-based randomized pragmatic clinical trial. Main measures TBI-QOL, Activity Measure for Post-Acute Care (AM-PAC), Clinical Satisfaction and Competency Rating Scale. RESULTS: 332 individuals ≥18 years-old hospitalized for TBI in four upper Midwest states were randomized to Remote (n = 166) and Usual Care (n = 166) groups. The groups were equivalent and representative of their state population's racial and ethnic composition, age, and proportion living in rural communities. There were no significant differences within or between experimental groups over the study period in TBI-QOL t-scores. There was a significant improvement in AM-PAC Daily Activities within the Remote group and a significant between-group improvement in clinical satisfaction for the Remote group. CONCLUSION: Enrolling a representative, regional community-based sample of individuals with TBI can be successful, and delivering a customized complex behavioral intervention remotely is feasible. The overall lack of intervention effectiveness was likely due to enrolling individuals without pre-identified clinical needs, initiating intervention after the immediate post-acute phase when needs are often highest, inability to provide direct clinical care remotely, and potential lack of outcome measure responsiveness in our sample.

Hospital to Hospital Transfers of Cerebral Hemorrhage: Characteristics of Early Withdrawal of Life-Sustaining Treatment.

BACKGROUND: Large intracerebral hemorrhages (ICHs) are associated with significant morbidity and mortality. Patient transfer to higher level centers is common, but care in these centers rarely demonstrably improves morbidity or reduces mortality. Patients may rapidly progress to brain death, but a large number die shortly after transferring because of withdrawal of life-sustaining treatment (WOLST). This outcome may result in poor resource use and unnecessary cost to patients, families, and institutions. We sought to determine clinical and radiographic predictors of early death or WOLST that may alter potential transfer. METHODS: We performed a retrospective review of patients admitted from outside medical centers to the neurosciences intensive care unit at Saint Marys Mayo Clinic Hospital in Rochester, MN, from January 2014 to December 2019. Patients ≥ 18 years old with a spontaneous ICH were included. Exclusion criteria included trauma, subarachnoid hemorrhage, and subdural hematoma. We identified patients who died or underwent WOLST within 24 h of transfer. Descriptive characteristics of patients and ICH were collected. Data were analyzed with univariable, multivariable, and logistic regression. Predictive modeling was performed. An additional case-matched study was completed to evaluate for characteristics further. RESULTS: A total of 317 consecutive patients were identified. Forty-two patients were found with early death or WOLST within 24 h of transfer. Do not resuscitate/do not intubate (DNR/DNI) code status (odds ratio [OR] 5.23, confidence interval [CI] 3.31-8.28), anticoagulation use (OR 2.11, CI 1.09-4.09), and lower level of consciousness at presentation based on Glasgow Coma Score (OR 1.41, CI 1.29-1.54) and Full Outline of Unresponsiveness (FOUR) score (OR 1.34, CI 1.26-1.46) were associated with WOLST. Associated characteristics on the computed tomography scan included midline shift (OR 4.64, CI 2.32-9.29), hydrocephalus (OR 9.30, CI 4.56-18.96), and intraventricular extension (OR 5.27, CI 2.60-10.68). Case matching restricted to midline shift demonstrated similarity between patients with aggressive care and WOLST. DNR/DNI code status, warfarin use, ICH score, and composite FOUR score were the best predictive characteristics (area under the curve 0.942). CONCLUSIONS: Early death or WOLST after ICH within 24 h of presentation was most associated with DNR/DNI code status, warfarin use, ICH score, and lower level of consciousness at presentation. These characteristics may be used by clinicians to guide conversations prior to transfer to tertiary care centers.

Validity of a cardiology fellow performance assessment: reliability and associations with standardized examinations and awards.

BACKGROUND: Most work on the validity of clinical assessments for measuring learner performance in graduate medical education has occurred at the residency level. Minimal research exists on the validity of clinical assessments for measuring learner performance in advanced subspecialties. We sought to determine validity characteristics of cardiology fellows' assessment scores during subspecialty training, which represents the largest subspecialty of internal medicine. Validity evidence included item content, internal consistency reliability, and associations between faculty-of-fellow clinical assessments and other pertinent variables. METHODS: This was a retrospective validation study exploring the domains of content, internal structure, and relations to other variables validity evidence for scores on faculty-of-fellow clinical assessments that include the 10-item Mayo Cardiology Fellows Assessment (MCFA-10). Participants included 7 cardiology fellowship classes. The MCFA-10 item content included questions previously validated in the assessment of internal medicine residents. Internal structure evidence was assessed through Cronbach's α. The outcome for relations to other variables evidence was overall mean of faculty-of-fellow assessment score (scale 1-5). Independent variables included common measures of fellow performance. FINDINGS: Participants included 65 cardiology fellows. The overall mean ± standard deviation faculty-of-fellow assessment score was 4.07 ± 0.18. Content evidence for the MCFA-10 scores was based on published literature and core competencies. Cronbach's α was 0.98, suggesting high internal consistency reliability and offering evidence for internal structure validity. In multivariable analysis to provide relations to other variables evidence, mean assessment scores were independently associated with in-training examination scores (beta = 0.088 per 10-point increase; p = 0.05) and receiving a departmental or institutional award (beta = 0.152; p = 0.001). Assessment scores were not associated with educational conference attendance, compliance with completion of required evaluations, faculty appointment upon completion of training, or performance on the board certification exam. R2 for the multivariable model was 0.25. CONCLUSIONS: These findings provide sound validity evidence establishing item content, internal consistency reliability, and associations with other variables for faculty-of-fellow clinical assessment scores that include MCFA-10 items during cardiology fellowship. Relations to other variables evidence included associations of assessment scores with performance on the in-training examination and receipt of competitive awards. These data support the utility of the MCFA-10 as a measure of performance during cardiology training and could serve as the foundation for future research on the assessment of subspecialty learners.

Burden and causes of readmissions following initial discharge after aortic syndromes.

BACKGROUND: Aortic syndromes, including aortic dissection (AD), intramural hematoma (IMH), and penetrating aortic ulcer (PAU), carry significant morbidity and mortality; few data exist regarding burden and causes of related rehospitalizations following initial discharge. METHODS: The study was conducted using the Rochester Epidemiology Project. All adult residents (age ≥18 years) with an incident diagnosis of AD, IMH, and PAU (1995-2015) were identified from the Rochester Epidemiology Project using the International Classification of Diseases, Ninth Revision and Tenth Revision codes and Hospital Adaptation of the International Classification of Diseases, second edition, codes. Assessment of any-cause (aortic and cardiovascular), aorta-related, or cardiovascular-related readmissions was determined following date of hospital discharge or diagnosis date (ie, the index event). RESULTS: A total of 117 patients of 130 cases of AD, IMH, and PAU included in the initial study population survived the index event and were evaluated. The median age of diagnosis was 74 years, and 70 (60%) were male. A total of 79 patients (68%) experienced at least one readmission. The median time to first any-cause, cardiovascular, and aortic readmission was 143, 861, and 171 days, respectively. The cumulative incidence of any-cause readmissions at 2, 4, and 10 years was 45%, 55%, and 69%, respectively. The cumulative incidence of cardiovascular readmissions at 2, 4, and 10 years was 15%, 20%, and 28%, respectively. The cumulative incidence of aortic readmissions at 2, 4, and 10 years was 38%, 46%, and 59%, respectively. Overall survival for the entire cohort at 2, 4, and 10 years was 84%, 75%, and 50%, respectively. CONCLUSIONS: Readmissions following initial discharge after diagnosis of aortic syndrome are common and not different across specific disease types. Whereas aorta-related rehospitalizations occur in more than half of patients but tend to be earlier, cardiovascular-related rehospitalizations tend to happen later in about one-third of patients. This may suggest the need for early follow-up focused on aortic complications, whereas later follow-up should address cardiovascular events.

Onset of progressive motor impairment in patients with critical central nervous system demyelinating lesions.

OBJECTIVE: To compare progressive motor impairment onset attributable to a "critical" central nervous system (CNS) demyelinating lesion in patients with highly restricted versus unlimited magnetic resonance imaging (MRI) lesion burden. METHODS: We identified 135 patients with progressive motor impairment for ⩾1 year attributable to a "critical" demyelinating lesion with: MRI burden of 1 lesion ("progressive solitary sclerosis"), 2-5 lesions ("progressive paucisclerosis"), or unrestricted (>5) lesions and "progressive unilateral hemiparesis." Neuroradiology review of brain and spinal cord MRI documented unequivocally demyelinating lesions. RESULTS: A total of 33 (24.4%) patients had progressive solitary sclerosis; 56 (41.5%) patients had progressive paucisclerosis; and 46 (34.1%) patients had progressive unilateral hemiparesis. Median age at onset of progressive motor impairment was younger in progressive solitary sclerosis (49 years; range 24-73) and progressive paucisclerosis (50 years; range 30-64) than in progressive unilateral hemiparesis (54 years; range 39-77; p = 0.02 and p = 0.003, respectively). Within progressive unilateral hemiparesis, motor-progression onset was similar between those with 4-10, 11-20, or >20 brain lesions (55, 54, 53 years of age, respectively; p = 0.44). CONCLUSION: Motor-progression age is similar, but paradoxically earlier, in cohorts with highly restricted CNS lesion burden than in those with unrestricted lesion burden with progressive unilateral hemiparetic MS. The "critical" demyelinating lesion rather than total brain MRI lesion burden is the major contributor to motor-progression onset in these cohorts.

Cause of Death after Traumatic Brain Injury: A Population-Based Health Record Review Analysis Referenced for Nonhead Trauma.

INTRODUCTION: Traumatic brain injury (TBI) is a leading cause of disability and is associated with decreased survival. Although it is generally accepted that TBI increases risk of death in acute and postacute periods after injury, causes of premature death after TBI in the long term are less clear. METHODS: A cohort sample of Olmsted County, Minnesota, residents with confirmed TBI from January 1987 through December 1999 was identified. Each case was assigned an age- and sex-matched non-TBI referent case, called regular referent. Confirmed TBI cases with simultaneous nonhead injuries were identified, labeled special cases. These were assigned 2 age- and sex-matched special referents with nonhead injuries of similar severity. Underlying causes of death in each case were categorized using death certificates, International Classification of Diseases, Ninth Revision, International Statistical Classification of Diseases, Tenth Revision, and manual health record review. Comparisons were made over the study period and among 6-month survivors. RESULTS: Case-regular referent pairs (n = 1,257) were identified over the study period, and 221 were special cases. In total, 237 deaths occurred among these pairs. A statistically significant difference was observed between total number of deaths among all cases (n = 139, 11%) and regular referents (n = 98, 8%) (p = 0.006) over the entire period. This outcome was not true for special cases (32/221, 14%) and special referents (61/441, 14%) (p = 0.81). A greater proportion of deaths by external cause than all other causes was observed in all cases (52/139, 37%) versus regular referents (3/98, 3%) and in special cases (13/32, 41%) versus special referents (5/61, 8%) (p < 0.001 for both). Among all case-referent pairs surviving 6 months, no difference was found between total number of deaths (p = 0.82). The underlying cause of death between these 2 groups was significantly different for external causes only (p < 0.01). For special cases surviving 6 months versus special referents, no difference was observed in total number of deaths (p = 0.24) or underlying causes of death (p = 1.00) between groups. DISCUSSION/CONCLUSION: This population-based case-matched referent study showed that increased risk of death after TBI existed only during the first 6 months after injury, and the difference was due to external causes.

Anti-cN1A antibodies do not correlate with specific clinical, electromyographic, or pathological findings in sporadic inclusion body myositis.

BACKGROUND: Anti-cytosolic 5'-nucleotidase 1A (cN1A) antibodies are commonly detected in patients with sporadic inclusion body myositis (sIBM). However, their pathogenic role has not been established. Moreover, efforts toward identifying sIBM distinct clinicopathologic characteristics associated with these antibodies have yielded conflicting results. METHODS: We first searched for patients, seen in our clinics, tested for anti-cN1A antibodies between December 2015 and December 2019. We identified 92 patients who were diagnosed with sIBM, according to the 2011 ENMC or Griggs et al criteria. Thereafter, we reviewed and compared the clinical and investigational findings of these patients in relation to their antibody status. RESULTS: Anti-cN1A antibodies were present in 47/92 (51%) patients with sIBM. Comparison of seropositive and seronegative cohorts yielded no significant difference in clinical features, including facial weakness, oropharyngeal and respiratory involvement, or disease severity. The antibody titer did not correlate with the clinical phenotype, CK value, or presence of myotonic discharges on EMG. Anti-cN1A antibody positive patients appeared to have more frequent auto-aggressive inflammation on muscle biopsy but not as an isolated myopathological feature. CONCLUSIONS: Our study showed that anti-cN1A antibody positive and negative sIBM patients have similar clinical features and disease severity. Anti-cN1A antibodies in our sIBM cohort did not correlate with any studied clinical or laboratory parameter and, therefore, were of limited value in the patient's assessment.

Surgical Resection for Crohn's and Cancer: A Comparison of Disease-Specific Risk Factors and Outcomes.

BACKGROUND AND OBJECTIVES: The goal of this study was to compare disease-specific risk factors and 30-day outcomes between patients with Crohn's disease (CD) and colon cancer (CC) undergoing right-sided surgical resection. METHODS: The American College of Surgeons-National Surgical Quality Improvement Program (ACS-NSQIP®) was interrogated to extract all patients ≥18 years undergoing elective right-sided resection for CD versus CC. Independent risk factors for surgical complications were identified through multivariable logistic regression for both groups. In a second step, surgical and medical 30-day morbidity was compared after risk adjustment. RESULTS: The cohort consisted of 17,516 patients, of which 2,899 (16.6%) underwent surgery for CD versus 14,617 (83.4%) for CC. Independent risk factors for surgical complications in patients with CD were male gender, African American race, ASA score (III or IV), active smoking, prolonged surgery, and preoperative anemia. Independent risk factors for surgical complications in the cancer group were age ≥70 years, male gender, ASA score (III or IV), respiratory and cardiovascular comorbidities, and preoperative hypoalbuminemia (<3.5 g/dL). After risk adjustment, surgical complications (OR 1.25, p = 0.002), sepsis (OR 1.64, p = 0.012), and unplanned readmissions (OR 1.39, p = 0.004) were more common in patients with CD. Thirty-day mortality was higher in cancer patients (1.1 vs. 0.1%, p < 0.0001). CONCLUSIONS: Patients with Crohn's disease were more prone to surgical complications and postoperative sepsis compared to the cancer group undergoing the same procedure. Careful evaluation and correction of disease-specific modifiable risk factors of patients with CD and CC, respectively, are important.

Imipenem-Relebactam Susceptibility Testing of Gram-Negative Bacilli by Agar Dilution, Disk Diffusion, and Gradient Strip Methods Compared with Broth Microdilution.

This study aimed to determine whether agar dilution, research-use-only disk diffusion (Mast Group Ltd., Bootle Merseyside, UK), Etest (bioMérieux, Inc., Durham, NC), and MIC test strip (MTS) (Liofilchem, Inc., Waltham, MA) methods yield equivalent results to those of broth microdilution (BMD) for imipenem-relebactam susceptibility testing using a collection of 297 Gram-negative bacilli, including members of the order Enterobacterales and Pseudomonas aeruginosa, enriched for drug resistance. MIC and disk diameter results were interpreted using United States Food and Drug Administration breakpoints. Overall, 76.8% of the isolates tested were susceptible to imipenem-relebactam by BMD. MIC values for agar dilution, Etest, and MTS were not significantly different from that for BMD, although they tended to be 1 to 2 dilutions higher. Essential agreement was 95.6% for agar dilution, 90.6% for Etest, and 85.2% for MTS. Categorical agreement was 98.0% for agar dilution, 73.1% for disk diffusion, 96.3% for Etest, and 96.6% for MTS. In conclusion, agar dilution and Etest yielded comparable results to BMD for imipenem-relebactam.