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Background Clinicians evaluating acute PE patients often have to identify risks for massive PE, a measure of hemodynamic instability and its consequence, massive PE related adverse clinical events (PEACE). We investigated the association of these risk factors with massive PE and PEACE in a consecutive PE cohort (n = 364). Methods Massive PE was defined as an acute central clot (proximal to the lobar artery) in a patient with right heart strain and systolic blood pressure ≤ 90 mg. PEACE was defined as any massive PE who died or required one or more of the following: ACLS, assisted ventilation, vasopressor use, thrombolytic therapy, or invasive thrombectomy, within seven days of PE diagnosis. Univariate and multivariate analysis assessing associations between the risk factors (age, gender, comorbidities, PE provoking risks, and whether the PE was felt to be idiopathic) and massive PE or PEACE were performed. Significance was determined at p Results Thirteen percent (n = 48) of patients presented with massive PE, and 9% (n = 32) had PEACE. In the final multivariate model, recent invasive procedure (RR = 7.4, p = 0.007), recent hospitalization (RR = 7.3, p = 0.002), and idiopathic PE (RR = 6.5, p = 0.003) were associated with massive PE. Only idiopathic PE (RR = 5.7, p = 0.005) was significantly associated with PEACE. No comorbidities or other PE provoking risks were associated with massive PE or PEACE. Conclusions As a take-home message, recent invasive procedure, recent hospitalization, and idiopathic PE were associated with massive PE, and only idiopathic PE was associated with PEACE. Simultaneously, comorbidities like age or chronic cardiopulmonary disease seem not to be associated with massive PE or PEACE.

The utility of extraocular cranial nerve electrophysiologic recordings lies primarily in the operating room during skull base surgeries. Surgical manipulation during skull base surgeries poses a risk of injury to multiple cranial nerves, including those innervating extraocular muscles. Because tumors distort normal anatomic relationships, it becomes particularly challenging to identify cranial nerve structures. Studies have reported the benefits of using intraoperative spontaneous electromyographic recordings and compound muscle action potentials evoked by electrical stimulation in preventing postoperative neurologic deficits. Apart from surgical applications, electromyography of extraocular muscles has also been used to guide botulinum toxin injections in patients with strabismus and as an adjuvant diagnostic test in myasthenia gravis. In this article, we briefly review the rationale, current available techniques to monitor extraocular cranial nerves, technical difficulties, clinical and surgical applications, as well as future directions for research.

Computed tomography pulmonary angiogram (CTPA) provides a volumetric assessment of clot burden in acute pulmonary embolism (PE). However, it is unclear if clot burden is associated with right-sided heart strain (RHS) or adverse clinical events (ACE). We prospectively enrolled Emergency Department patients with PE (in CTPA) from 2008 to 2011. We assigned 1 to 9 points as clot burden score, based on whether emboli were saddle, central, lobar, segmental, and subsegmental. We evaluated a novel score (the "CT-PASS") based on the sum (in millimeters) of the largest filling defects in the right and left pulmonary vasculature. Our primary outcome was RHS, defined by imaging (echocardiography or CTPA) or cardiac biomarkers. Our secondary outcomes included 5-day ACE. We included 271 patients (50% women), with a mean age of 59 ± 17 years. Based on CTPA, 131 patients (48%) had central PE (clot burden score ≥5 points). The median CT-PASS was 9.1 mm (interquartile range 4.9 to 16.4). In univariate analysis, higher clot burden (highest quartile CT-PASS) was associated with RHS (p = 0.003). In multivariate analysis, after adjusting for RHS, age, and gender, central PE (odds ratio [OR] 2.92, 95% confidence interval [CI] 1.10 to 7.81) and CT-PASS20 mm (OR 3.54, 95% CI 1.39 to 8.97) were significantly associated with ACE. However, this association of central PE with ACE was not statistically significant after excluding patients with shock index1 (OR 2.56, 95% CI 0.62 to 10.64). In conclusion, highest quartile CT-PASS was associated with RHS and central PE and ACE, but this association was not statistically significant in hemodynamically stable PE [corrected].

Background Electrocardiographic (ECG) changes may be seen with pulmonary emboli (PE). Whether ECG is associated with short-term adverse clinical events after PE is less well established. Hypothesis ECG findings are associated with short-term clinical deterioration after PE. Methods Consecutive adult PE patients were enrolled in an academic emergency department from 2008 to 2011. The primary outcome was right heart strain (RHS) on echocardiogram or CT pulmonary angiography, or TnT ≥0.1 ng/mL. We derived an ECG (TwiST) score that is associated with RHS and short-term adverse clinical events. Results We enrolled 298 patients with PE. On multivariate analysis, T-wave inversion in leads V1 through V3 (OR: 4.7, 95% confidence interval [CI]: 1.7-13.2), S wave in lead I (OR: 2.0, 95% CI: 1.1-3.5), and tachycardia (OR: 2.5, 95% CI: 1.3-4.8) were associated with RHS. A TwiST score ≤2 (n = 210, 72%) was 84% (95% CI: 77%-90%) sensitive for the absence of RHS, whereas a TwiST score ≥5 (n = 47, 16%) was 93% (95% CI: 88%-97%) specific for the presence of RHS. Conclusions A simple ECG (TwiST) score can identify patients likely or not likely to have RHS with >80% specificity and sensitivity and may assist in identifying patients with acute PE at risk for adverse clinical events before pursuing other advanced imaging tests.

Recent studies have highlighted differences in how older patients respond to high-risk pulmonary embolism (PE) and treatment. However, guidelines for PE risk stratification and treatment are not based on age, and data are lacking for older patients. We characterized the impact of age on clinical features, risk stratification, treatment, and outcomes in a sample of patients with PE in the emergency department. We performed an observational cohort study of 547 consecutive patients with PE in the emergency department from 2005 to 2011 in an urban tertiary hospital. We used bivariate proportions and multivariable logistic regression to compare clinical presentation, risk category, treatment, and outcomes in patients ≥65 years with those65 years. The mean age was 58 ± 17 years, 276 (50%) were women, and 210 (38%) were ≥65 years. PE was more severe in patients ≥65 years (massive 14% vs 5%, submassive 48% vs 25%, and low risk 38% vs 70%, p0.0001), with submassive PE being the most common presentation in patients ≥65 years. However, subanalysis removing natriuretic peptides from the definition of submassive PE negated this finding. Treatment with parenteral anticoagulation (88% vs 90%, p = 0.32), thrombolytic therapy (5% vs 4%, p = 0.87), and inferior vena cava filter (4% vs 4%, p = 0.73) were similar among age groups. Patients ≥65 years had higher 30-day mortality (11% vs 3%, p0.001). In conclusion, patients ≥65 years present with more severe PE and have higher mortality, although treatment patterns were similar to younger patients. Age-specific guideline definitions of submassive PE may better identify high-risk patients.

Several factors have been associated with mortality in the months after PE. Factors associated with short-term clinical deterioration or need for hospital-based intervention are less well known.We prospectively enrolled consecutive emergency department patients with PE and recorded clinical, biomarker and radiographic data. We assessed hospitalised patients daily to identify clinical deterioration or need for hospital-based intervention for 5 days after PE. We captured postdischarge events via 5-day and 30-day interviews. We used univariate and multivariable models to assess associations with clinical deterioration, severe clinical deterioration and 30-day all-cause mortality. We also assessed the test characteristics of three published clinical decision rules.We enrolled 298 patients with PE: mean age 59 (SD±17) years; 152 (51%) male and 268 (90%) white race. 101 (34%) patients clinically deteriorated or required a hospital-based intervention within 5 days, and 197 (66%) did not. 27 (9%) patients suffered severe clinical deterioration and 12 died within 30 days. Factors independently associated with clinical deterioration were hypotension (p=0.001), hypoxia (p0.001), coronary disease (p=0.004), residual deep vein thrombosis (p=0.006) and right heart strain on echocardiogram (p0.001). In contrast, factors associated with 30-day all-cause mortality were active malignancy (p0.001) and congestive heart failure (p=0.009). The sensitivity of clinical decision rules was moderate (39-80%) for 5-day clinical deterioration but higher (67-100%) for 30-day mortality.Most patients do not clinically deteriorate after PE diagnosis. Several factors are associated with short-term clinical deterioration, but these factors differ from those associated with 30-day mortality.

Evoked potentials are time-locked electrophysiologic potentials recorded in response to standardized stimuli using scalp electrodes. These responses provide good temporal resolution and have been used in various clinical and intraoperative settings. Olfactory evoked potentials (OEPs) may be used as an adjunct tool in identifying patients of Parkinson disease and Alzheimer dementia. In clinical practice, visual evoked potentials (VEPs) are particularly useful in identifying subclinical cases of optic neuritis and in treatment surveillance. In recent times, pattern electroretinograms and photopic negative response have been gaining attention in identifying glaucoma suspects. During surgical manipulation, there is a risk of damage to optic or olfactory nerve. Intraoperative neurophysiologic monitoring can provide information regarding the integrity of olfactory or visual pathway. OEPs and VEPs, however, show high degree of variability and are not reliable tools because the responses are extremely susceptible to volatile anesthetic agents. Newer techniques that could possibly circumvent these drawbacks have been developed but are not used extensively. In this article, we briefly review the available techniques to obtain OEPs and VEPs, diagnostic applications, the utility of intraoperative monitoring, the limitations of the current techniques, and the future directions for research.

Sjogren-Larsson syndrome is an autosomal recessive disorder characterized by defective activity of fatty aldehyde dehydrogenase. It presents as a triad of congenital ichthyosis, spastic diplegia, and mental retardation. The pathology behind this syndrome is the failure of degradation of fatty aldehydes. This case is presented for its rarity.

Background Previous studies in post-operative orthopedic and pediatric patients suggest that erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) testing may be helpful in ruling out septic arthritis. However, these tests have not been evaluated in a population of adult Emergency Department (ED) patients. Study Objective Determine the sensitivity of ESR and CRP in patients with septic arthritis. Methods Retrospective analysis of ED patients with septic arthritis from 2003 to 2008. Eligible patients had an International Classification of Diseases - Ninth Revision diagnosis of pyogenic arthritis (711.0x) plus: positive synovial fluid culture, positive synovial Gram stain, or operative irrigation. Patients were excluded if no ESR or CRP was performed within 24 h. Sensitivity of ESR and CRP at various cutoffs was calculated with 95% confidence intervals (CI). Results We identified 167 patients with septic arthritis. We included 143 (86%) who had ESR (n = 140, 84%) or CRP (n = 96, 57%) performed. Mean age was 49 (± 22) years, and 85 (59%) were male. Race was: 125 (87%) white, 4 (3%) black, and 12 (8%) Hispanic. Thirty-five (24%) had infection of prosthetic joints. Synovial cultures were positive in 102 (71%). Sensitivity of ESR was: 98% (95% CI 94–100%) using a cutoff of ≥ 10 mm/h (n = 134) and 94% (95% CI 88–97%) using a cutoff of ≥ 15 mm/h (n = 131). The sensitivity of CRP was 92% (95% CI 84–96%) using a cutoff of ≥ 20 mg/L (n = 88). Conclusion ESR and CRP have sensitivities of > 90% for septic arthritis, but only when low thresholds are used. Further study is required to determine the clinical usefulness of ESR and CRP testing.

The original version of the article unfortunately contained an error in conflict of interest. This erratum is published with the correct conflict of interest.

Purpose Clinical decision rules for the disposition of patients with pulmonary embolism (PE) are typically validated against an outcome of 30-day mortality or disease recurrence. There is little justification for this time frame, nor is it clear whether this outcome reflects emergency department (ED) decision making. Aims To determine which outcomes emergency physicians (EP) consider most relevant to disposition decisions. Methods Survey of attending EPs in geographically diverse US states using acute PE as the diagnostic framework. Responses required single-answer multiple choice, a numerical percentage, rank-ordered responses, or a five-point Likert scale. We distributed the survey via e-mail to 608 EPs. Results We received responses from 292 (48%) EPs: 88% board certified, 91% trained in emergency medicine, and 70% work in academics. Respondents reported discharging 1% of patients with PE from the ED, but 21% reported being asked to do so by an admitting service. EPs were more interested in knowing 5-day (in hospital) outcomes [192/265, 72% (95% exact CI = 66%–78%)] than 30-day outcomes [39/261, 15% (95% exact CI = 11%–20%)] or 90-day outcomes [29/263, 11% (95% exact CI = 8%–15%)]. On a Likert scale, 212/241 (88%, 95% exact CI = 83%–92%) agreed or strongly agreed that they considered 5-day (in hospital) clinical deterioration when making a decision to admit or discharge a patient from the ED compared to 184/242 (76%, 95% exact CI = 70%–81%) and 73/242 (30%, 95% exact CI = 24%–36%) for 30 and 90 days, respectively. A wide variety of clinical outcomes beyond death or recurrent PE were considered indicative of clinical deterioration. Conclusions Five-day (in hospital) outcomes that incorporate a variety of clinical deterioration events are of interest to EPs when determining the disposition of ED patients with PE. Researchers should consider this when developing and validating clinical decision rules.

Background Electrocardiographic (ECG) changes may be seen with pulmonary emboli (PE). Whether ECG is associated with short-term adverse clinical events after PE is less well established. Hypothesis ECG findings are associated with short-term clinical deterioration after PE. Methods Consecutive adult PE patients were enrolled in an academic emergency department from 2008 to 2011. The primary outcome was right heart strain (RHS) on echocardiogram or CT pulmonary angiography, or TnT ≥0.1 ng/mL. We derived an ECG (TwiST) score that is associated with RHS and short-term adverse clinical events. Results We enrolled 298 patients with PE. On multivariate analysis, T-wave inversion in leads V1 through V3 (OR: 4.7, 95% confidence interval [CI]: 1.7-13.2), S wave in lead I (OR: 2.0, 95% CI: 1.1-3.5), and tachycardia (OR: 2.5, 95% CI: 1.3-4.8) were associated with RHS. A TwiST score ≤2 (n = 210, 72%) was 84% (95% CI: 77%-90%) sensitive for the absence of RHS, whereas a TwiST score ≥5 (n = 47, 16%) was 93% (95% CI: 88%-97%) specific for the presence of RHS. Conclusions A simple ECG (TwiST) score can identify patients likely or not likely to have RHS with >80% specificity and sensitivity and may assist in identifying patients with acute PE at risk for adverse clinical events before pursuing other advanced imaging tests.

CT Pulmonary Angiogram (CTPA) provides a volumetric assessment of clot burden associated with acute pulmonary embolism (PE). However, it is unclear if clot burden is associated with right heart strain (RHS) or clinical deterioration. We prospectively enrolled Emergency Department patients with PE

Glycine encephalopathy (GE) or nonketotic hyperglycinemia is an autosomal recessive disorder due to a primary defect in glycine cleavage enzyme system. It is characterized by elevated levels of glycine in plasma and cerebrospinal fluid usually presenting with seizures, hypotonia, and developmental delay. In our case, paradoxical increase in seizure frequency on starting sodium valproate led us to diagnose GE.