Prospects of CSF shunt independence among chronically shunted patients.

BACKGROUND AND OBJECTIVES: CSF shunt placement for hydrocephalus and other etiologies has arguably been the most life-saving intervention in pediatric neurosurgery in the past 6 decades. Yet, chronic shunting remains a source of morbidity for patients of all ages. Neuroendoscopic surgery has made shunt independence possible for newly diagnosed hydrocephalic patients. In this study, we examine the prospects of shunt independence with or without endoscopic third ventriculostomy (ETV) in chronically shunted patients. METHODS: After IRB approval, a retrospective analysis was completed on patients whose shunt was ligated or removed to achieve shunt independence, with or without ETV. Clinical and imaging data were collected. RESULTS: Eighty-eight patients with CSF shunts had their shunt either ligated or removed, 57 of whom had a concomitant ETV. Original reasons for shunting included: congenital hydrocephalus 20 (23%), post-hemorrhagic hydrocephalus (PHH) of prematurity 14 (16%), aqueductal stenosis 10 (11%), intracranial cyst 8 (9%), tumor 8 (9%), infantile subdural hematomas 8 (9%), myelomeningocele 7 (8%), post-traumatic hydrocephalus 7 (8%) and post-infectious hydrocephalus 6 (7%). The decision to perform a simultaneous ETV was made based on etiology. Forty-nine (56%) patients became shunt independent. The success rate was 46% in the ETV group and 73% in the no ETV group. Using multivariate analysis and Cox Proportional Hazards models, age > 4 months at shunt placement (p = 0.032), no shunt revisions (p = 0.01), select etiologies (p = 0.043), and ETVSS > 70 (in the ETV group) (p = 0.017), were protective factors for shunt independence. CONCLUSION: Considering the long-term complications of shunting, achieving shunt independence may provide hope for improved quality of life. While this study is underpowered, it provides pilot data identifying factors that predict shunt independence in chronically shunted patients, namely age, absence of prior shunt revision, etiology, and in the ETV group, the ETVSS.

Esophageal disease and loss to follow-up are common among patients who experience non-endoscopic resolution of food impaction.

Many patients who experience esophageal food impaction (EFI) will have non-endoscopic resolution (NER) of their EFI, but this population is poorly defined. The purpose of this study is to describe the outcomes of patients with NER of EFI. A retrospective chart review from 2007 to 2017 was performed at a single tertiary care center. There were 593 patients who presented to the emergency department with EFI, defined as recent soft food ingestion and inability to tolerate oral secretions. Adequate follow-up was defined as a gastroenterology clinic visit or EGD within 6 months of EFI. Out of these, 149 patients (25.1%) had NER of their EFI. Patients with NER were less likely to have adequate follow-up than those with ER (45.0% vs. 59.5%, P = 0.003). Of those without established esophageal disease and NER, 92.5% had significant esophageal pathology on endoscopy, including stricture (34.0%), features of eosinophilic esophagitis (30.2%), and esophagitis (22.6%). Recurrent EFI occurred at a similar rate between patients with NER and ER (9.4% vs. 14.6%, P = 0.14). Patients with established esophageal disease (odds ratio [OR]: 1.51, P = 0.04) and recommendation to follow-up at time of EFI (OR: 6.06, P < 0.001) were most likely to follow up after EFI. Approximately, a quarter of patients with EFI will experience NER of their EFI. Virtually, all patients (92.5%) were found to have esophageal disease warranting longitudinal care. Importantly, follow-up rates are significantly lower in those with NER than their counterparts requiring EGD. Our study highlights the need to develop standardized protocols that improve follow-up for patients after NER of EFI.

Empiric proton pump inhibitor therapy after esophageal food impaction may mask eosinophilic esophagitis diagnosis at follow-up.

Esophageal food impaction (EFI) is often the first presentation for patients with eosinophilic esophagitis (EoE); however, there is significant heterogeneity in the management of EFI. We aimed to study the impact of EFI management, particularly post-EFI medication prescriptions on EoE diagnosis, follow-up, and recurrence in patients with endoscopic features of EoE. In our retrospective study, adults presenting between 2007 and 2017 with EFI requiring endoscopic dis-impaction with endoscopic features of EoE (furrows, rings, and/or exudates) were included. We examined the impact of demographics and EFI management on EoE diagnosis, follow-up (esophagogastroduodenoscopy [EGD] or clinic visit within 6 months), and recurrence. We identified 164 cases of EFI due to suspected EoE. Biopsy was performed in 68 patients (41.5%), and 144 patients (87.8%) were placed on proton pump inhibitor (PPI) and/or swallow corticosteroids after EFI, including 88.5% of those not biopsied. PPI use at time of biopsy was negatively associated with EoE diagnosis (odds ratio: 0.39, confidence interval: 0.17-0.85). Sixty-one (37.4%) patients were lost to follow-up at 6 months. Recurrent EFI at 1 year occurred in 3.7% of patients. Medications, most commonly PPI, are frequently prescribed after EFI when the endoscopic features of EoE are present, which may mask the diagnosis of EoE on follow-up EGD. We estimated that for every five patients biopsied on PPI, one case of EoE is masked. As recurrent EFI within 1 year is uncommon, empiric therapy should be avoided until diagnostic biopsies are obtained. Further efforts to reduce loss to follow-up after EFI are also needed.

The Transcription Factor Nfatc2 Regulates ß-Cell Proliferation and Genes Associated with Type 2 Diabetes in Mouse and Human Islets.

Human genome-wide association studies (GWAS) have shown that genetic variation at >130 gene loci is associated with type 2 diabetes (T2D). We asked if the expression of the candidate T2D-associated genes within these loci is regulated by a common locus in pancreatic islets. Using an obese F2 mouse intercross segregating for T2D, we show that the expression of ~40% of the T2D-associated genes is linked to a broad region on mouse chromosome (Chr) 2. As all but 9 of these genes are not physically located on Chr 2, linkage to Chr 2 suggests a genomic factor(s) located on Chr 2 regulates their expression in trans. The transcription factor Nfatc2 is physically located on Chr 2 and its expression demonstrates cis linkage; i.e., its expression maps to itself. When conditioned on the expression of Nfatc2, linkage for the T2D-associated genes was greatly diminished, supporting Nfatc2 as a driver of their expression. Plasma insulin also showed linkage to the same broad region on Chr 2. Overexpression of a constitutively active (ca) form of Nfatc2 induced ß-cell proliferation in mouse and human islets, and transcriptionally regulated more than half of the T2D-associated genes. Overexpression of either ca-Nfatc2 or ca-Nfatc1 in mouse islets enhanced insulin secretion, whereas only ca-Nfatc2 was able to promote ß-cell proliferation, suggesting distinct molecular pathways mediating insulin secretion vs. ß-cell proliferation are regulated by NFAT. Our results suggest that many of the T2D-associated genes are downstream transcriptional targets of NFAT, and may act coordinately in a pathway through which NFAT regulates ß-cell proliferation in both mouse and human islets.

Longitudinal growth of receptive language in children with cerebral palsy between 18 months and 54 months of age.

AIM: We examined receptive language developmental trajectories between 18 months and 54 months for three clinical speech-language profile groups of children with cerebral palsy (those with speech motor involvement, without speech motor involvement, and with anarthria) and quantified differences from age-level expectations. We identified latent classes of comprehension development, related these classes to clinical profile groups, and examined how well early receptive language predicted outcomes. METHOD: We used a prospective longitudinal design. Eighty-five children with cerebral palsy (43 females, 42 males) were followed longitudinally from 18 to 54 months of age. Children were seen two to eight times (322 data points). Children were classified into clinical profile groups. Language comprehension age-equivalent scores were the primary measures of interest. RESULTS: Children with anarthria had significant language delays, limited developmental change over time, and comprised their own latent class. Children with speech motor impairment had slight receptive language delays over time. Children with no speech motor impairment had age-appropriate receptive language over time. Early language comprehension scores were highly predictive of later latent profile group membership. INTERPRETATION: Early language comprehension abilities are highly predictive of language comprehension growth trajectory and suggest that children with early language delay, particularly those who are non-speaking, should receive language intervention to support development. WHAT THIS PAPER ADDS: There are two growth trajectories for language comprehension among children with cerebral palsy. Children with speech motor impairment had a constant 6-month receptive language delay. Children without speech motor impairment had age-appropriate receptive language. Non-speaking children had significant receptive language delay. Early language comprehension change was highly predictive of later trajectory group.

Right ventricular outflow tract diameter change with exercise: a prospective exercise echocardiography and invasive CPET study.

While cardiac output reserve with exercise predicts outcomes in cardiac and pulmonary vascular disease, precise quantification of exercise cardiac output requires invasive cardiopulmonary testing (iCPET). To improve the accuracy of cardiac output reserve estimation with transthoracic echocardiography (TTE), this prospective study aims to define changes in right ventricular outflow tract diameter (RVOTd) with exercise and its relationship with invasively measured haemodynamics. Twenty subjects underwent simultaneous TTE and iCPET, with data collected at rest, leg-raise, 25 W, 50 W (n = 16), 75 W (n = 14), and 100 W (n = 6). This was followed by a second exercise study with real-time RV pressure-volume loops at similar stages (except leg-raise). The overall cohort included heart failure with preserved ejection fraction (n = 12), pulmonary arterial hypertension (n = 5), and non-cardiac dyspnoea (n = 3). RVOTd was reverse engineered from the TTE-derived RVOT velocity time integral (VTI) and iCPET-derived stroke volume, using the formula: Fick stroke volume = RVOT VTI × RVOT area (wherein RVOT area = π × [RVOTd/2]2). RVOTd increased by nearly 3-4% at every 25 W increment. Using linear regression models, where each subject is treated as a categorical variable and adjusting for subject intercept, RVOTd was correlated with haemodynamic variables (cardiac output, heart rate, pulmonary artery and RV pressures). Of all the predictor haemodynamic variables, cardiac output had the highest r2 model fit (adjusted r2 = 0.68), with a unit increase in cardiac output associated with a 0.0678 increase in RVOTd (P < 0.001). Our findings indicate that RVOTd increases by 3-4% with every 25 W increment, predominantly correlated with cardiac output augmentation. These results can improve the accuracy of cardiac output reserve estimation by adjusting for RVOTd with graded exercise during non-invasive CPET and echocardiogram. However, future studies are needed to define these relationships for left ventricular outflow tract diameter.

Genetic Analysis of Obesity-Induced Diabetic Nephropathy in BTBR Mice.

Diabetic nephropathy (DN) is the leading cause of end-stage renal disease in the U.S. and has a significant impact on human suffering. Leptin-deficient BTBR (BTBRob/ob) mice develop hallmark features of obesity-induced DN, whereas leptin-deficient C57BL/6J (B6ob/ob) mice do not. To identify genetic loci that underlie this strain difference, we constructed an F2 intercross between BTBRob/ob and B6ob/ob mice. We isolated kidneys from 460 F2 mice and histologically scored them for percent mesangial matrix and glomerular volume (∼50 glomeruli per mouse), yielding ∼45,000 distinct measures in total. The same histological measurements were made in kidneys from B6 and BTBR mice, either lean or obese (Lepob/ob), at 4 and 10 weeks of age, allowing us to assess the contribution of strain, age, and obesity to glomerular pathology. All F2 mice were genotyped for ∼5,000 single nucleotide polymorphisms (SNPs), ∼2,000 of which were polymorphic between B6 and BTBR, enabling us to identify a quantitative trait locus (QTL) on chromosome 7, with a peak at ∼30 Mbp, for percent mesangial matrix, glomerular volume, and mesangial volume. The podocyte-specific gene nephrin (Nphs1) is physically located at the QTL and contains high-impact SNPs in BTBR, including several missense variants within the extracellular immunoglobulin-like domains.

Racial disparities in post-operative pain experience and treatment following cesarean birth.

OBJECTIVE: To evaluate racial/ethnic differences in post-operative pain experience and opioid medication use (morphine milligram equivalent) in the first 24 h following cesarean birth. METHODS: This study was a single-center retrospective cohort of birthing persons who underwent cesarean deliveries between 1/1/16 and 12/31/17. A total of 2,228 cesarean deliveries were analyzed. The primary outcome was average pain, which was the mean of all documented self-reported pain scores (0-10 scale) during the first 24 h post-delivery. The secondary outcome included oral morphine equivalents used in the first 24 h post-delivery. Linear regression was performed to examine whether the race/ethnicity of the birthing parent was associated with mean pain scores and oral morphine equivalents, controlling for confounding variables. RESULTS: In multivariate analyses non-Hispanic Black birthing persons reported higher mean pain scores (Coefficient: 0.61, 95% confidence interval [0.39-0.82], p < .001]) than non-Hispanic White birthing persons, but received similar quantities of morphine milligram equivalent (Coefficient: -0.98 mg, 95% confidence interval [-5.93-3.97], p = .698]). Non-Hispanic Asian birthing persons reported similar reported mean pain scores to those of non-Hispanic White birthing persons (Coefficient: 0.02 mg, 95% confidence interval [-0.17-0.22], p = .834]), but received less morphine milligram equivalent (Coefficient: -5.47 mg, 95% confidence interval [-10.05 to -0.90], p = .019). When controlling for reported mean pain scores, both non-Hispanic Black (Coefficient: -6.36 mg, 95% confidence interval [-10.97 to -1.75], p = .007) and non-Hispanic Asian birthing persons (Coefficient: -5.66 mg, 95% confidence interval [-9.89 to -1.43], p = .009) received significantly less morphine milligram equivalents. CONCLUSION: Despite reporting higher mean pain scores, non-Hispanic Black birthing persons did not receive higher quantities of morphine milligram equivalent. Non-Hispanic Asian birthing persons received lower quantities of morphine milligram equivalent despite reporting similar pain scores to non-Hispanic White birthing persons. These differences suggest disparities in post-operative pain management for birthing persons of color in our study population.

The impact of preexisting maternal anxiety on pain and opioid use following cesarean delivery: a retrospective cohort study.

BACKGROUND: Anxiety disorders are the most common mental health condition. They are associated with negative pain experiences and can hinder rehabilitation in the hospital setting. Anxiety has been shown to be predictive of increased postoperative pain in patients undergoing nonobstetrical surgery. OBJECTIVE: To evaluate the impact of preexisting maternal anxiety disorders on average self-reported pain scores and opioid use in the first 24 hours following cesarean delivery STUDY DESIGN: This was a single-center retrospective cohort study of cesarean deliveries between January 1, 2016 and December 31, 2017. The primary outcome was average pain, calculated by averaging all documented self-reported pain scores (0-10 scale) during the first 24 hours postdelivery. The secondary outcome included the oral morphine milligram equivalents used in the first 24 hours postdelivery. Analysis of the impact of anxiety disorders on these outcomes was performed using multivariable linear regression to control for confounding variables. RESULTS: A total of 2228 cesarean deliveries were analyzed, of which 578 (25.9%) had an anxiety disorder documented. Women with a diagnosis of anxiety had higher average pain scores (3.9 vs 3.5; P<.001) and morphine milligram equivalents use (110.4 mg vs 102.2 mg; P<.001) than women without anxiety. CONCLUSION: Patients with preexisting anxiety diagnoses reported higher average pain scores and opioid pain medication use in the first 24 hours following cesarean delivery.

The effect of remote scribes on primary care physicians' wellness, EHR satisfaction, and EHR use.

BACKGROUND: Physician burnout is a major problem in the United States. Small studies suggest scribes can improve clinician satisfaction, but scribe programs have not been evaluated using separate control groups or structured measures of electronic health record (EHR) use. METHODS: We conducted a pre-post, non-randomized controlled evaluation of a remote scribe pilot program introduced in September 2019 in an academic primary care practice. Scribes were paired with physicians via an audio-only cellphone connection to hear and document in real-time. Physician wellness was measured with the 10-item Mini-Z and 16-item Professional Fulfillment Index. EHR use was measured using vendor-derived platforms that provide routine EHR-related data. RESULTS: 37 of 38 scribe users (97.4%) and 68 of 160 potential control physicians (42.5%) completed both pre and post intervention questionnaires. Compared with controls, scribe users had improvements in Mini-Z wellness metrics including Joyful Workplace (mean improvement 2.83, 95%CI 0.60, 5.06) and a single-item dichotomized burnout measure (OR 0.15, 95%CI 0.03, 0.71). There were significant reductions among scribe users compared to controls in total EHR time per 8 scheduled hours (-1.14 h, 95%CI -1.55, -0.72), and an increase in the percentage of orders with team contribution (10.4%, 95%CI 5.2, 15.6). These findings remained significant in adjusted analyses. CONCLUSIONS/IMPLICATIONS: A remote scribe program was associated with improvements in physician wellness and reduced EHR use. Healthcare organizations can consider scribe programs to help improve wellness among their physician workforce.

The Effect of Blue-Enriched Lighting on Medical Error Rate in a University Hospital ICU.

BACKGROUND: Fatigue-related errors that occur during patient care impose a tremendous socioeconomic impact on the health care system. Blue-enriched light has been shown to promote alertness and attention. The present study tested whether blue-enriched light can help to reduce medical errors in a university hospital adult ICU. METHODS: In this interventional study, a blue-enriched white light emitting diode was used to enhance traditional fluorescent light at the nurse workstation and common areas in the ICU. Medical errors were identified retrospectively using an established two-step surveillance process. Suspected incidents of potential errors detected on nurse chart review were subsequently reviewed by two physicians blinded to lighting conditions, who made final classifications. Error rates were compared between the preintervention fluorescent and postintervention blue-enriched lighting conditions using Poisson regression. RESULTS: The study included a total of 1,073 ICU admissions, 522 under traditional and 551 under interventional lighting (age range 17-97 years, mean age ± standard deviation 58.5 ± 15.8). No difference was found in overall medical error rate (harmful and non-harmful) pre- vs. postintervention, 45.5 vs. 42.7 per 1,000 patient-days (rate ratio: 0.94, 95% confidence interval = 0.71-1.23, p = 0.64). CONCLUSION: Interventional lighting did not have an effect on overall medical error rate. The study was likely underpowered to detect the 25% error reduction predicted. Future studies are required that are powered to assess more modest effects for lighting to reduce the risk of fatigue-related medical errors and errors of differing severity.

Ventriculoperitoneal Shunt Drainage Increases With Gravity and Cerebrospinal Fluid Pressure Pulsations: Benchtop Model.

BACKGROUND: There have been few improvements in cerebrospinal fluid (CSF) shunt technology since John Holter introduced the silicon valve, with overdrainage remaining a major source of complications. OBJECTIVE: To better understand why valves are afflicted by supra-normal CSF flow rates. We present in Vitro benchtop analyses of flow through a differential pressure valve under simulated physiological conditions. METHODS: The pseudo-ventricle benchtop valve testing platform that comprises a rigid pseudo-ventricle, compliance chamber, pulsation generator, and pressure sensors was used to measure flow rates through a differential pressure shunt valve under the following simulated physiological conditions: orientation (horizontal/vertical), compliance (low/medium/high), and pulsation generator force (low/medium/high). RESULTS: Our data show that pulse pressures are faithfully transmitted from the ventricle to the valve, that lower compliance and higher pulse generator forces lead to higher pulse pressures in the pseudo-ventricle, and that both gravity and higher pulse pressure lead to higher flow rates. The presence of a valve mitigates but does not eliminate these higher flow rates. CONCLUSION: Shunt valves are prone to gravity-dependent overdrainage, which has motivated the development of gravitational valves and antisiphon devices. This study shows that overdrainage is not limited to the vertical position but that pulse pressures that simulate rhythmic (eg, cardiac) and provoked (eg, Valsalva) physiological CSF pulsations increase outflow in both the horizontal and vertical positions and are dependent on compliance. A deeper understanding of the physiological parameters that affect intracranial pressure and flow through shunt systems is prerequisite to the development of novel valves.

Longitudinal Development of Receptive Vocabulary in Children with Cerebral Palsy and Anarthria: Use of the MacArthur-Bates CDI.

OBJECTIVE: To examine receptive language growth in children with cerebral palsy (CP) and anarthria using a parent-reported measure of vocabulary. METHOD: Scores from 47 children (29 males) with CP and anarthria were obtained from the vocabulary checklists on the MacArthur-Bates Communication Development Inventories (MCDI) and analyzed to examine the distribution of receptive language growth. Linear trajectories of word composite scores were created using a linear-mixed model, incorporating between two and ten data points per child. RESULTS: Three different growth trajectories emerged: approximately 23% grew by 100 or more words per year, 13% grew by 50-100 words per year, and 64% grew by 50 words per year or less. Age-four vocabulary was strongly correlated with rate of increase in vocabulary. CONCLUSION: Receptive vocabulary scores from the MCDI are increasing at a reduced pace for most children with CP and anarthria. More sensitive measures of language assessment are necessary to gain a complete picture of their language ability levels.

Longitudinal Growth in Single-Word Intelligibility Among Children With Cerebral Palsy From 24 to 96 Months of Age: Effects of Speech-Language Profile Group Membership on Outcomes.

Purpose We examined whether there were differences among speech-language profile groups of children with cerebral palsy (CP) in age of crossing 25%, 50%, and 75% intelligibility thresholds; age of greatest intelligibility growth; rate of intelligibility growth; maximum attained intelligibility at 8 years; and how well intelligibility at 36 months predicts intelligibility at 96 months when group membership is accounted for. Profile groups were children with no speech motor impairment (NSMI), those with speech motor impairment and language comprehension that is typically developing (SMI-LCT), and those with speech motor impairment and language comprehension impairment (SMI-LCI). Method Sixty-eight children with CP were followed longitudinally between 24 and 96 months of age. A total of 564 time points were examined across children (M = 8.3 time points per child, SD = 2.6). We fitted a nonlinear random effects model for longitudinal observations, allowing for differences between profile groups. We used the fitted model trajectories to generate descriptive analyses of intelligibility growth by group and to generate simulations to analyze how well 36-month intelligibility data predicted 96-month data accounting for profile groups. Results Children with CP who have NSMI have different growth and better intelligibility outcomes than those with speech motor impairment. Children with SMI-LCT tend to have better outcomes but similar intelligibility growth as children with SMI-LCI. There may be a subset of children that cut across SMI-LCI and SMI-LCT groups who have severe speech motor involvement and show limited growth in intelligibility. Conclusions Intelligibility outcomes for children with CP are affected by profile group membership. Intelligibility growth tends to be delayed in children with speech motor impairment. Intelligibility at 3 years is highly predictive of later outcomes regardless of profile group. Intervention decision making should include consideration of early intelligibility, and treatment directions should include consideration of augmentative and alternative communication.

Resident self-assessment and self-reflection: University of Wisconsin-Madison's Five-Year Study.

BACKGROUND: Chart review represents a critical cornerstone for practice-based learning and improvement in our internal medicine residency program. OBJECTIVE: To document residents' performance monitoring and improvement skills in their continuity clinics, their satisfaction with practice-based learning and improvement, and their ability to self-reflect on their performance. DESIGN: Retrospective longitudinal design with repeated measures. PARTICIPANTS: Eighty Internal Medicine residents abstracted data for 3 consecutive years from the medical records of their 4,390 patients in the University of Wisconsin-Madison (UW) Hospital and Clinics and William S. Middleton Veterans Administration (VA) outpatient clinics. MEASUREMENT: Logistic modeling was used to determine the effect of postgraduate year, resident sex, graduation cohort, and clinic setting on residents' "compliance rate" on 17 nationally recognized health screening and chronic disease management parameters from 2003 to 2007. RESULTS: Residents' adherence to national preventive and chronic disease standards increased significantly from intern to subsequent years for administering immunizations, screening for diabetes, cholesterol, cancer, and behavioral risks, and for management of diabetes. Of the residents, 92% found the chart review exercise beneficial, with 63% reporting gains in understanding about their medical practices, 26% reflecting on specific gaps in their practices, and 8% taking critical action to improve their patient outcomes. CONCLUSIONS: This paper provides support for the feasibility and practicality of this limited-cost method of chart review. It also directs our residency program's attention in the continuity clinic to a key area important to internal medicine training programs by highlighting the potential benefit of enhancing residents' self-reflection skills.

Differentiating Typical From Atypical Speech Production in 5-Year-Old Children With Cerebral Palsy: A Comparative Analysis.

Objective Early diagnosis of speech disorders in children with cerebral palsy (CP) is of critical importance. A key problem is differentiating those with borderline or mild speech motor deficits from those who are within an age-appropriate range of variability. We sought to quantify how well functional speech measures differentiated typically developing (TD) children from children with CP. Method We studied speech production in 45 children with CP (26 with clinical speech motor impairment [SMI] and 19 with no evidence of speech motor impairment [NSMI]) and in 29 TD children of the same age. Speech elicitation tasks were used. Intelligibility, speech rate, and intelligible words per minute were examined. Results All measures differentiated between all 3 groups of children with considerable precision based on area under the receiver operating characteristic curve (AUC) data. AUC was highest for overall intelligibility, which ranged from .88 to .99. Intelligible words per minute also yielded very strong AUCs, ranging from .81 to .99. In each of the receiver operating characteristic models, discrimination between groups was highest for children with speech motor impairment versus TD children. Data indicated that 90% of TD children had overall intelligibility above 87% at 5 years of age, but that no child was 100% intelligible. Furthermore, 90% children with SMI had intelligibility below 72%. Conclusion Findings suggest that functional speech measures differentiate very clearly between children with and without CP and that even children who do not show evidence of speech motor impairment have functional differences in their speech production ability relative to TD peers.

Longitudinal Growth in Single Word Intelligibility Among Children With Cerebral Palsy From 24 to 96 Months of Age: Predicting Later Outcomes From Early Speech Production.

Purpose Children with cerebral palsy (CP) are at risk for significant communication problems. Reduced speech intelligibility is common, even for those who do not have speech motor deficits. Development of intelligibility has not been comprehensively quantified in children with CP; as a result, we are currently unable to predict later speech outcomes. Such information would advance treatment decision making. We sought to examine growth in speech intelligibility among children with CP using a prospective longitudinal design, with a focus on age of crossing target intelligibility thresholds, age of greatest intelligibility growth, and how well intelligibility at 36 months predicted intelligibility at 96 months. Method Sixty-nine children with CP were followed longitudinally between 24 and 96 months of age. A total of 566 time points were examined across children ( M = 8.2 time points per child, SD = 2.6). We fitted a nonlinear random effects model for longitudinal observations and then used the fitted model trajectories to generate descriptive analyses of growth. We used results of the model to generate a set of simulations, which we analyzed to determine how well 36-month intelligibility data predicted 96-month data. Results Half of children crossed 25% and 50% intelligibility thresholds at 36 and 49 months of age, respectively. Slightly more than half of children did not reach 75% intelligibility by 96 months of age. Age of crossing 25%, 50%, and 75% intelligibility thresholds was highly negatively correlated with intelligibly at 96 months. Children had the steepest intelligibility growth at 36 months, followed by 48 and 60 months. Intelligibility at 36 months was highly predictive of intelligibility at 96 months. Conclusions The developmental window from 3 to 5 years constitutes a time of rapid growth in speech intelligibility in children with CP. Children who cross intelligibility thresholds of 25%, 50%, and 75% at earlier ages have better outcomes when they are older; early performance is highly predictive of later speech intelligibility outcomes. Children with CP as a group have delayed speech intelligibility development but are still growing through 96 months of age.

Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study.

OBJECTIVE: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction. METHODS: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx. RESULTS: Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications. CONCLUSIONS: This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Estimating the rate of progressive visual field damage in those with open-angle glaucoma, from cross-sectional data.

PURPOSE: To estimate the rate of visual field progression in open-angle glaucoma (OAG) subjects, by using data from population-based cross-sectional studies. METHODS: Subjects with OAG were identified in nine surveys of randomly sampled populations using standard criteria for glaucomatous optic neuropathy. Subjects were of European, African, Chinese, and Hispanic ethnicity. The measure of OAG damage was the mean deviation (MD) of an automated visual field test (Humphrey Field Analyzer; Carl Zeiss Meditec, Inc., Dublin, CA). The rate of progression was the mean of all subjects' damage in the worse eye divided by an average time since onset. Time since onset was estimated from age-specific prevalence rates. RESULTS: A total of 1066 subjects with OAG contributed visual field data. The mean worsening in decibels per year was: European-derived, -1.12; Hispanic, -1.26; African-derived, -1.33; and Chinese -1.56 (difference among ethnicities, P = 0.16). The mean duration of disease was lowest among Chinese persons at 10.5 years (95% CI: 8.8-12.6) and was highest in African-derived subjects at 15.4 years (95% CI: 14.6-15.9). The progression rate was not consistently related to age or gender. By combining disease duration and progression rate, the model predicted that 15% or fewer of the worse eyes would reach the end of the field damage scale in the patient's lifetime. CONCLUSIONS: The estimates of typical worsening per year in the worse eye among subjects with OAG suggested slightly more rapid progression than in some clinic-based studies. The rate did not differ significantly by ethnicity or gender, but was worse in those with known, treated OAG and in pseudophakic subjects.

Effectiveness of careful bedside examination in assessment, diagnosis, and prognosis of vestibular neuritis.

OBJECTIVE: To determine whether the use of 4 bedside tests (head-impulse, head-heave, head-shake, and vibration tests) can be as effective as the caloric test, a widely accepted standard, in the diagnosis and prediction of the time to recovery from vestibular neuritis. DESIGN: Inception cohort (1-year follow-up), criterion standard study. SETTING: Primary referral center. PATIENTS: All patients had acute vertigo, and those having a diagnosis of vestibular neuritis were eligible for inclusion in the study. Sixty-eight patients (43 men and 25 women; mean age, 54.9 years) met this criterion, and 53 of them (77.9%) completed the study. MAIN OUTCOME MEASURES: Spontaneous head-shaking and vibration-induced nystagmus elicited with a battery-powered device were tested wearing Frenzel goggles. The head-impulse and head-heave tests were performed manually. Caloric irrigation was administered with hot, cold, and ice water. RESULTS: At baseline, more than half of the patients exhibited positive signs with all 4 tests and all had caloric paralysis or paresis. Signs with the head-impulse and head-heave tests correlated highly (odds ratio, 24.9; P < .001), as did those with the head-shake and vibration tests (odds ratio, 22.8; P < .001). Patients with a positive sign with the head-impulse or vibration test were 70% less likely to recover than were those with a negative sign. Head-impulse (hazard ratio, 0.08; P = .002) and head-shake (hazard ratio, 0.23; P = .01) test results were associated with the outcome of the caloric test. CONCLUSION: Careful bedside examination of patients with vestibular neuritis has both diagnostic value in the short term and prognostic value in the long term.