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Objective: To estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2016 using data from the National ALS Registry (Registry). Established in 2009, the Registry collects data on ALS patients in the U.S. to better describe the epidemiology of ALS, examine risk factors such as environmental and occupational exposures, and characterize the demographics of those living with the disease. Methods: To identify adult prevalent cases of ALS, the Registry compiles data from three national administrative databases (maintained by the Centers for Medicare and Medicaid Services, the Veterans Health Administration, and the Veterans Benefits Administration). To ascertain cases not necessarily included in these databases and to better understand risk-factors associated with ALS and disease progression, the Registry also includes data collected from patients who voluntarily enroll via a web portal to complete online surveys. Results: In 2016, the Registry conservatively identified 16,424 adult persons who met the Registry definition of ALS for an age-adjusted prevalence rate of 5.2 per 100,000 U.S. population. The pattern of patient characteristics (e.g., age, sex, and race/ethnicity) has not changed from previous Registry reports. Overall, ALS was more common among whites, males, and persons aged 60-69 years. The age groups with the lowest number of ALS cases were persons aged 18-39 years. Males had a higher prevalence rate of ALS than females overall and across all data sources. Conclusions: Data collected by the National ALS Registry are being used to better describe the epidemiology and demographics of ALS in the U.S.

Introduction: The Centers for Disease Control and Prevention (CDC) National Amyotrophic Lateral Sclerosis (ALS) Registry is the first national registry for a chronic neurologic disease in the USA and uses a combination of case-finding methods including administrative healthcare data and patient self-registration., Methods: We applied capture-recapture methodology to estimate the completeness of the Registry for ascertaining patients with ALS for the first full year and the fourth year of the Registry (2011, 2014). The Registry uses the combination of two national administrative claims databases (Medicare and Veterans Affairs) with a self-register option at the registry portal. We conducted descriptive analyses of the demographic and clinical characteristics of the ALS cases identified by each of the sources and estimated the completeness of case ascertainment for each of the three ALS Registry sources individually, pairwise, and in all combinations., Results: Case-finding completeness was 54% in 2011 and improved to 56% in 2014. A smaller proportion of ALS patients under age 65 were ascertained than those 65 or older, and ascertainment was also lower for nonwhite than white patients. The uncorrected ALS prevalence was 4.3/100,000 in 2011 (in 2014, 5.0/100,000), but after correction for underascertainment, annual prevalence in 2011 was 7.9/100,000 (95% CI: 7.6-8.2) (in 2014 was 8.9/100,000 [95% CI: 8.7-9.2])., Discussion/conclusion: Our findings indicate that administrative healthcare databases are a very efficient method for identifying the majority of ALS prevalent cases in the National ALS Registry and that the inclusion of a web registry portal for patients to self-register is important to ensure a more representative population for estimating ALS prevalence. Nonetheless, more than 40% of ALS cases were not ascertained by the Registry, with individuals younger than age 65 and people of color underrepresented. Recommendations are provided for additional methods that can be considered to improve the completeness of case ascertainment., (© 2021 The Author(s) Published by S. Karger AG, Basel.)

Background: Adult eating disorder treatments are hampered by lack of access and limited efficacy. This open-trial study evaluated the acceptability and preliminary efficacy of a novel intervention for adults with eating disorders delivered to young adults and parent-supports in an intensive, multi-family format (Young Adult Temperament-Based Treatment with Supports; YA-TBT-S)., Methods: 38 YA-TBT-S participants (m age = 19.58; SD 2.13) with anorexia nervosa (AN)-spectrum disorders, bulimia nervosa (BN)-spectrum disorders, and avoidant/restrictive food intake disorder (ARFID) completed self-report assessments at admission, discharge, and 12-month follow-up. Assessments measured program satisfaction, eating disorder psychopathology and impairment, body mass index (BMI), and trait anxiety. Outcomes were analyzed using linear mixed effects models to examine changes in outcome variables over time., Results: Treatment was rated as highly satisfactory. 53.33% were in partial or full remission at 12-month follow-up. 56% of participants received other treatment within the 12-month follow-up period, suggesting that YA-TBT-S may be an adjunctive treatment. Participants reported reductions in ED symptomatology (AN and BN), increases in BMI (AN and ARFID), and reductions in clinical impairment (AN and ARFID) at 12-month follow-up., Conclusions: YA-TBT-S is a feasible and acceptable adjunctive treatment for young adults with a broad range of ED diagnoses and may be a method for involving parents in ED treatment in ways that are acceptable to both parents and YA. Further evaluation of efficacy is needed in larger samples, and to compare YA-TBT-S to other ED treatment approaches. Plain English summary Eating disorders are costly and dangerous psychiatric disorders that affect millions of individuals each year. Despite their risks and societal costs, currently available treatments are limited. This study examined the acceptability and efficacy of Young Adult, Temperament-Based Treatment with Supports (YA-TBT-S), a new treatment program for adults with eating disorders. YA-TBT-S was rated highly, and a significant portion of participants improved based on ratings collected 12 months after program participation. Those with anorexia nervosa (AN) and bulimia nervosa (BN) showed significant reductions in eating disorder pathology, and those with AN and avoidant/restrictive food intake disorder (ARFID) showed increases in BMI over time., (© 2021. The Author(s).)

Severe and enduring eating disorders (EDs) have the highest mortality rate of all psychiatric illnesses (Arch Gen Psychiatry, 2011, 68 , 724), especially when comorbid with treatment-resistant depression (TRD) (Psychiatr Res, 2016, 244 , 45). We report on four patients with enduring EDs and TRD treated with repeat ketamine over 12 + months, showing improvement in depression with only modest changes in ED symptoms., Competing Interests: The data that support the findings of this study are available from the corresponding author, [TS], upon reasonable request. The work described in this manuscript was performed as part of routine clinical care and was not funded by any third party. None of the authors have any conflict of interest. However, David Feifel MD PhD now owns a clinic that administers Ketamine for depression. He did not own this company during the treatment and data assessment for these case reports., (© 2021 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Background: High-quality epidemiologic data worldwide are needed to improve our understanding of disease risk, support health policy to meet the diverse needs of people with multiple sclerosis (MS) and support advocacy efforts., Objectives: The Atlas of MS is an open-source global compendium of data regarding the epidemiology of MS and the availability of resources for people with MS reported at country, regional and global levels., Methods: Country representatives reported epidemiologic data and their sources via survey between September 2019 and March 2020, covering prevalence and incidence in males, females and children, and age and MS type at diagnosis. Regional analyses and comparisons with 2013 data were conducted., Results: A total of 2.8 million people are estimated to live with MS worldwide (35.9 per 100,000 population). MS prevalence has increased in every world region since 2013 but gaps in prevalence estimates persist. The pooled incidence rate across 75 reporting countries is 2.1 per 100,000 persons/year, and the mean age of diagnosis is 32 years. Females are twice as likely to live with MS as males., Conclusions: The global prevalence of MS has risen since 2013, but good surveillance data is not universal. Action is needed by multiple stakeholders to close knowledge gaps.

Objective: To conduct educational and promotional outreach activities to general neurologists and to increase self-enrollment of persons with amyotrophic lateral sclerosis (ALS) in the National ALS Registry (Registry)., Methods: A multicomponent project to educate neurologists and increase Registry self-enrollment was delivered. Project components consisted of phone calls, mailings, train-the-trainer presentations, and key informant interviews. Project-specific metrics, continuing education enrollment, and Registry self-enrollment data were analyzed to measure project efficacy., Results: Mailings were sent to 1561 neurologists in 6 states during 2015 to 2016. Sixty-five percent of responding neurologists remembered the mailing 3 months after receipt. Of providers who saw patients with ALS in the 3-month period, 60% read the provider guide, 22% distributed a patient guide, and 15% advised a patient to self-enroll. No changes in self-enrollment rates were observed., Conclusion: Targeted mailings to providers can be used to educate them about the Registry; however, most providers did not distribute materials to patients with ALS. Increases in providers receiving Registry material did not lead to increases in patient self-enrollment., Practice Implications: General neurologists have competing priorities, and they see patients with ALS infrequently. Neurologists could be the appropriate channel to distribute Registry information to patients, but they are not the appropriate resource to assist patients with self-enrollment. Engaging the support staff of busy specialists can help increase research response rates and information distribution. The lessons learned from this project can be applied to other rare conditions and disease specialists., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2018.)

Amyotrophic lateral sclerosis (ALS) is a fatal, rare, and hard to diagnose neurological disease with unknown etiology. Objective : To understand the incidence, prevalence, and survival characteristics of ALS cases in the Atlanta metropolitan area. Methods : Neurologists in Clayton, Cobb, DeKalb, Fulton, and Gwinnett counties provided case reports for ALS patients under their care from 1 January 2009 to 31 December 2011. Incidence and prevalence rates were calculated for 2009, 2010, and 2011 by sex, race, and ethnicity. Using data from the National Death Index, survival time was calculated for age, sex, race, ethnicity, and El Escorial criteria. Results : There were 281 unique ALS cases reported, which is approximately 104% of the expected cases. The majority of the 281 cases were white, non-Hispanic, male, and in the 50-59 age category. The overall average incidence rate for 2009 to 2011 was 1.54 per 100,000 person-years, with higher annual incidence rates for whites, males, and non-Hispanics. The prevalence rates for 2009, 2010, and 2011 were 5.05, 5.44, and 5.56 per 100,000, respectively. Median survival time was highest for the 18-39 age group, Asians, non-Hispanics, and males. Additionally, the log-rank tests for homogeneity across strata indicate a statistical significance between strata for the age category for survival time. Conclusion : The findings for Atlanta are similar to other population-based studies in the United States. Although the Atlanta metropolitan area was selected to over-represent the minority population, the strongest predictor of survival time was age at diagnosis.

Objective: Considerable gaps exist in knowledge regarding the prevalence of neurologic diseases, such as multiple sclerosis (MS), in the United States. Therefore, the MS Prevalence Working Group sought to review and evaluate alternative methods for obtaining a scientifically valid estimate of national MS prevalence in the current health care era., Methods: We carried out a strengths, weaknesses, opportunities, and threats (SWOT) analysis for 3 approaches to estimate MS prevalence: population-based MS registries, national probability health surveys, and analysis of administrative health claims databases. We reviewed MS prevalence studies conducted in the United States and critically examined possible methods for estimating national MS prevalence., Results: We developed a new 4-step approach for estimating MS prevalence in the United States. First, identify administrative health claim databases covering publicly and privately insured populations in the United States. Second, develop and validate a highly accurate MS case-finding algorithm that can be standardly applied in all databases. Third, apply a case definition algorithm to estimate MS prevalence in each population. Fourth, combine MS prevalence estimates into a single estimate of US prevalence, weighted according to the number of insured persons in each health insurance segment., Conclusions: By addressing methodologic challenges and proposing a new approach for measuring the prevalence of MS in the United States, we hope that our work will benefit scientists who study neurologic and other chronic conditions for which national prevalence estimates do not exist., (Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease; the majority of ALS patients die within 2-5 years of receiving a diagnosis (1). Familial ALS, a hereditary form of the disease, accounts for 5%-10% of cases, whereas the remaining cases have no clearly defined etiology (1). ALS affects persons of all races and ethnicities; however, whites, males, non-Hispanics, persons aged ≥60 years, and those with a family history of ALS are more likely to develop the disease (2). No cure for ALS has yet been identified, and the lack of proven and effective therapeutic interventions is an ongoing challenge. Treatments currently available, Edaravone and Riluzole, do not cure ALS, but slow disease progression in certain patients (3,4). This report presents National ALS Registry findings regarding ALS prevalence in the United States for the period January 1-December 31, 2015. In 2015, the estimated prevalence of ALS cases was 5.2 per 100,000 population with a total of 16,583 cases identified. Overall, these findings are similar to the 2014 ALS prevalence and case count (5.0 per 100,000; 15,927 cases) (2). Prevalence rates by patient characteristics (most common in whites, males, and persons aged ≥60 years) and U.S. Census regions are consistent with ALS demographics and have not changed from 2014 to 2015 calendar years. The algorithm used to identify cases from national administrative databases was updated from the International Classification of Diseases, Ninth Revision (ICD-9) to the ICD-10 codes for claims starting on October 1, 2015, with no apparent effect on case ascertainment. Data collected by the National ALS Registry are being used to better describe the epidemiology of ALS in the United States and to facilitate research on the genetics, potential biomarkers, environmental pollutants, and etiology for ALS., Competing Interests: All authors have completed and submitted the ICMJE form for disclosure of potential conflicts of interest. No potential conflicts of interest were disclosed.

Background: Genetic factors contribute to anorexia nervosa (AN); and the first genome-wide significant locus has been identified. We describe methods and procedures for the Anorexia Nervosa Genetics Initiative (ANGI), an international collaboration designed to rapidly recruit 13,000 individuals with AN and ancestrally matched controls. We present sample characteristics and the utility of an online eating disorder diagnostic questionnaire suitable for large-scale genetic and population research., Methods: ANGI recruited from the United States (US), Australia/New Zealand (ANZ), Sweden (SE), and Denmark (DK). Recruitment was via national registers (SE, DK); treatment centers (US, ANZ, SE, DK); and social and traditional media (US, ANZ, SE). All cases had a lifetime AN diagnosis based on DSM-IV or ICD-10 criteria (excluding amenorrhea). Recruited controls had no lifetime history of disordered eating behaviors. To assess the positive and negative predictive validity of the online eating disorder questionnaire (ED100K-v1), 109 women also completed the Structured Clinical Interview for DSM-IV (SCID), Module H., Results: Blood samples and clinical information were collected from 13,363 individuals with lifetime AN and from controls. Online diagnostic phenotyping was effective and efficient; the validity of the questionnaire was acceptable., Conclusions: Our multi-pronged recruitment approach was highly effective for rapid recruitment and can be used as a model for efforts by other groups. High online presence of individuals with AN rendered the Internet/social media a remarkably effective recruitment tool in some countries. ANGI has substantially augmented Psychiatric Genomics Consortium AN sample collection. ANGI is a registered clinical trial: clinicaltrials.govNCT01916538; https://clinicaltrials.gov/ct2/show/NCT01916538?cond=Anorexia+Nervosa&draw=1&rank=3., (Copyright © 2018. Published by Elsevier Inc.)

In the United States, the past decade has seen rapid growth in treatment centers providing specialty care to patients with eating disorders. Much of this growth has been in higher levels of care, including hospital-based and residential treatment. Despite this expansion, there remains lack of agreement regarding the most important components of care, such as staff training or specifics of treatment delivery. Additionally there is no consensus on how best to assess outcome and compare performance across programs. This leaves patients, families, public and private insurance programs, and policy makers with limited information to help facilitate treatment decisions. The present paper considers implications of these changes in the eating disorder treatment landscape and examines two ideas that, if implemented, may enhance the quality of eating disorder care. First, we explore the proposal to develop a network of centers of excellence in eating disorder treatment and the value this may have for improving overall treatment quality. This idea was discussed at an expert meeting held at SAMSHA in 2017 regarding issues important to the field following passage of the 21st Century Cures Act. Second, we consider the potential utility of a study using the Delphi method to promote expert consensus regarding clinical outcome assessments., (© 2018 Wiley Periodicals, Inc.)

Disorders of the brain can exhibit considerable epidemiological comorbidity and often share symptoms, provoking debate about their etiologic overlap. We quantified the genetic sharing of 25 brain disorders from genome-wide association studies of 265,218 patients and 784,643 control participants and assessed their relationship to 17 phenotypes from 1,191,588 individuals. Psychiatric disorders share common variant risk, whereas neurological disorders appear more distinct from one another and from the psychiatric disorders. We also identified significant sharing between disorders and a number of brain phenotypes, including cognitive measures. Further, we conducted simulations to explore how statistical power, diagnostic misclassification, and phenotypic heterogeneity affect genetic correlations. These results highlight the importance of common genetic variation as a risk factor for brain disorders and the value of heritability-based methods in understanding their etiology., (Copyright © 2018 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.)

Objective: Family-based treatment (FBT) for adolescent anorexia nervosa (AN) promotes faster weight restoration when compared to other treatments. However, the mechanisms through which this occurs are not clarified. This study explored the trajectories of parental self-efficacy and perceived family flexibility during FBT and systemic family therapy (SyFT). We also explored whether parental self-efficacy mediates the effects of treatment on weight gain early in treatment., Method: 158 adolescents (12-18 years old; 89% girls) and their parents were randomized to FBT or SyFT. Parental self-efficacy as well as adolescents' and parental perceptions of the family's flexibility were collected at baseline and at sessions 2, 4, 6, and 8., Results: Over time, only parents in FBT reported significantly greater self-efficacy. The change in maternal self-efficacy over the first 8 weeks of treatment was a significant mediator of session 10 weight gain. There were no significant group differences in perceived flexibility by session 8., Discussion: Both parents in FBT and mothers in SyFT understand early the need to change their family's rules and roles. However, the specific strategies of FBT appear to mediate early weight gain in AN., (© 2018 Wiley Periodicals, Inc.)

Background: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically results in death within 2-5 years of initial symptom onset. Multidisciplinary ALS clinics (MDCs) have been established to provide specialty care to people living with the disease., Objective: To estimate the proximity of ALS prevalence cases to the nearest MDC in the US to help evaluate one aspect of access to care., Methods: Using 2013 prevalence data from the National ALS Registry, cases were geocoded by city using geographic information system (GIS) software, along with the locations of all MDCs in operation during 2013. Case-to-MDC proximity was calculated and analyzed by sex, race, and age group., Results: During 2013, there were 72 MDCs in operation in 30 different states. A total of 15,633 ALS cases were geocoded and were distributed throughout all 50 states. Of these, 62.6% were male, 77.9% were white, and 76.2% were 50-79 years old. For overall case-to-MDC proximity, nearly half (44.9%) of all geocoded cases in the US lived >50 miles from an MDC, including approximately a quarter who lived >100 miles from an MDC. There was a statistically significant difference between distance to MDC by race and age group., Conclusions: The high percentage of those living more than 50 miles from the nearest specialized clinic underscores one of the many challenges of ALS. Having better access to care, whether at MDCs or through other modalities, is likely key to increasing survivability and obtaining appropriate end-of-life treatment and support for people with ALS.

Background: The International Classification of Disease, 10th Revision (ICD-10) did not include a code specific for Amyotrophic lateral sclerosis (ALS) until 2017. Instead, code G12.2 included both ALS and other motor neuron diseases (MND). Our objective was to determine US mortality rates for ALS exclusively by excluding other MND and progressive supranuclear palsy., Methods: All mortality data coded as G12.2 under the pre-2017 rubric were obtained for 2011-2014. Deaths without ALS listed in one of the un-coded cause-of-death fields were excluded. ALS death rates per 100,000 persons were age-adjusted to the 2000 US standard population using the direct method., Results: The proportion of excluded records coded G12.2 but not ALS was 0.21, resulting in 24,328 ALS deaths. The overall age-adjusted mortality rate was 1.70 (95% CI 1.68-1.72). The rate among males was 2.09 (95% CI 2.05-2.12) and females was 1.37 (95% CI 1.35-1.40). The overall rate among whites was 1.84, blacks 1.03, and other races 0.70. For both sexes and all races, the rate increased with age and peaked among 75-79 year-olds. Rates tended to be greater in states at higher latitudes., Conclusions: Previous reports of ALS mortality in the United States showed similar age, sex, and race distributions but with greater age-adjusted mortality rates due to the inclusion of other diseases in the case definition. When using ICD-10 data collected prior to 2017, additional review of multiple-cause of death data is required for the accurate estimation of ALS deaths., (© 2018 The Author(s) Published by S. Karger AG, Basel.)

Background: National administrative healthcare data may be used as a case-finding method for prevalence studies of chronic disease in the United States, but the completeness of ascertainment likely varies depending on the disease under study., Methods: We used 3 case-finding sources (Medicare, Medicaid, and Veterans Administration data) to estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2002-2004, and applied the capture-recapture methodology to estimate the degree of under-ascertainment when relying solely on these sources for case identification., Results: Case-finding completeness was 76% overall and did not vary by race, but was lower for males (77%) than for females (88%), and lower for patients under age 65 (66%) than patients over age 65 (79%). The uncorrected ALS prevalence ratio was 2.8/100,000 in 2002, 3.3/100,000 in 2003, and 3.7/100,000 in 2004. After correcting for under-ascertainment, the annual prevalence increased by approximately 1 per 100,000 to 3.7/100,000 in 2002 (95% CI 3.66-3.80), 4.4/100,000 in 2003 (95% CI 4.34-4.50), and 4.8/100,000 in 2004 (95% CI 4.76-4.91)., Conclusions: Federal healthcare claims databases ascertained are a very efficient method for identifying the majority of ALS-prevalent cases in the National ALS Registry, and may be enhanced by having patients self-register through the registry web portal., (© 2018 The Author(s) Published by S. Karger AG, Basel.)

Background: Current guidelines for nutritional rehabilitation in hospitalized restrictive eating disorder patients recommend a cautious approach to refeeding. Several studies suggest that higher calorie diets may be safe and effective, but have traditionally excluded severely malnourished patients. The goal of this study was to evaluate the safety of a higher calorie nutritional rehabilitation protocol (NRP) in a broad sample of inpatients with restrictive eating disorders, including those who were severely malnourished., Methods: A retrospective chart review was conducted among eating disorder inpatients between January 2015 and March 2016. Patients were started on a lower calorie diet (≤1500 kcals/day) or higher calorie diet (≥1500 kcals/day). Calorie prescription on admission was based on physician clinical judgement. The sample included patients aged 8-20 years with any DSM-5 restrictive eating disorder. Those who were severely malnourished (<75% expected body weight [EBW]) or required tube feeding during admission were included. Multivariable regression models were used to determine whether level of nutritional rehabilitation was associated with hypophosphatemia, hypomagnesemia, or hypokalemia., Results: The sample included 87 patients; mean age was 14.4 years (S.D. 32.7); 29% were <75% EBW. The majority (75.8%) was started on higher calorie diets (mean 1781 kcal/day). Controlling for rate of calorie change, initial %EBW, age, race/ethnicity, insurance, diagnosis, and NG/NJ tube placement, higher calorie diets were not associated with hypophosphatemia, hypomagnesemia, or hypokalemia on admission or within the first 72 h. Increased risk of hypophosphatemia on admission was associated with lower baseline %EBW., Conclusion: A higher calorie NRP was tolerated in this broad population of inpatients with restrictive eating disorders. Lower %EBW on admission was a more important predictor of hypophosphatemia than initial calorie level. Larger studies are required to demonstrate the safety of higher calorie diets in severely malnourished patients.

As part of our continuing effort to highlight innovative approaches to improving the health and environment of communities, the Journal is pleased to publish a bimonthly column from the Agency for Toxic Substances and Disease Registry (ATSDR). ATSDR is a federal public health agency of the U.S. Department of Health and Human Services (HHS) and shares a common office of the Director with the National Center for Environmental Health (NCEH) at the Centers for Disease Control and Prevention (CDC). ATSDR serves the public by using the best science, taking responsive public health actions, and providing trusted health information to prevent harmful exposures and diseases related to toxic substances. The purpose of this column is to inform readers of ATSDR's activities and initiatives to better understand the relationship between exposure to hazardous substances in the environment and their impact on human health and how to protect public health. We believe that the column will provide a valuable resource to our readership by helping to make known the considerable resources and expertise that ATSDR has available to assist communities, states, and others to assure good environmental health practice for all is served. The conclusions of this column are those of the author(s) and do not necessarily represent the views of ATSDR, CDC, or HHS. Kevin Horton is chief of the Environmental Health Surveillance Branch within the Division of Toxicology and Human Health Sciences at ATSDR. Wendy Kaye is a senior epidemiologist at McKing Consulting Corporation. Laurie Wagner is a research associate at McKing Consulting Corporation.

Background: Brain-gut axis signaling modifies gastrointestinal symptomatology. Altered neural processing of intestinal pain signals involves interoceptive brain regions in adults with functional and inflammatory gastrointestinal disorders. Although these disorders frequently present in childhood, there are no published studies in youth. We determined whether neural processing of somatic pain stimuli differs in adolescents and young adults (AYA) with irritable bowel syndrome (IBS) and inflammatory bowel disease (IBD), as compared to healthy controls (HC)., Methods: IBS and IBD AYA (16-20 years) underwent anticipated and thermal pain stimuli of low and high intensity on their forearm and simultaneous blood oxygen level-dependent functional magnetic resonance imaging. Data from adult HC were used for comparison. Subjects answered surveys evaluating alexithymia, anxiety, depression, and pain catastrophizing. Group data were compared using linear mixed effects and analysis of variance., Results: Study groups were similar by sex but not age. Significant group by pain condition interactions were observed in interoceptive brain regions during pain anticipation, and within perceptual brain regions during perceived pain. Higher activation within interoceptive brain regions during anticipated pain was observed in IBS compared with IBD and HC subjects. IBD patients demonstrated increased activation in perceptual brain regions during experienced pain as compared to IBS and HC., Conclusions: IBS and IBD AYA demonstrate altered neural processing of somatic pain compared with each other and with HC. Our results suggest that neuromodulatory interventions targeting interoceptive brain circuits in IBS and perceptual brain regions in IBD may be effective., Competing Interests: Conflicts of Interests/Disclosures: All authors do not have any potential conflicts that are relevant to the manuscript.

Objective: We examined the timing and number of days of hospitalization during the course of treatment, hospitalization effects on outcome, and predictors and moderators of the use of hospitalization in adolescents with anorexia nervosa (AN)., Method: Data used in this study were collected from 158 adolescents (ages 12 to 18 years of age) who met DSM-IVTR criteria for AN (exclusive of the amenorrhea criteria) randomized to receive either Family Based Treatment (FBT) or Systemic Family Therapy (SyFT) in a 7 site study., Results: The trajectory of hospital day use is similar in the first 5 weeks irrespective of treatment allocation. However, days of hospitalization continued to increase throughout SyFT but leveled off in FBT after ∼5 weeks of treatment. Early hospitalization was a negative predictor for improvements in percent weight change for both treatment groups (t(1)=2.6, p = 0.011). Co-morbid psychopathology predicted early hospital use in both treatments. Higher levels of eating related obsessions and depression moderated hospitalization rates suggesting that FBT reduces early hospitalization rates compared to SyFT for these subgroups., Discussion: These data support and extend findings from previous studies by identifying patterns of hospital use, and predictors and moderators of treatment effect for early hospitalization use in adolescent AN. © 2016 Wiley Periodicals, Inc.(Int J Eat Disord 2016; 49:891-894)., (© 2016 Wiley Periodicals, Inc.)

Problem/condition: Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease for which no cure or viable treatment has been identified. ALS, like most noncommunicable diseases, is not a nationally notifiable disease in the United States. The prevalence of ALS in the United States during 2010-2011 was estimated to be 3.9 cases per 100,000 persons in the general population. Updated prevalence estimates are needed to help monitor disease status, better understand etiology, and identify risk factors for ALS., Period Covered: 2012-2013., Description of System: The National ALS Registry, established in 2009, collects data on ALS patients in the United States to better describe the incidence and prevalence of ALS, examine risk factors such as environmental and occupational exposures, and characterize the demographics of those living with ALS. To identify prevalent cases of ALS, data are compiled from four national administrative databases (maintained by the Centers for Medicare and Medicaid Services, the Veterans Health Administration, and the Veterans Benefits Administration). To identify cases not included in these databases and to better understand risk-factors associated with ALS and disease progression, the Registry also includes data that are collected from patients who voluntarily enroll and complete online surveys., Results: During 2012 and 2013, the Registry identified 14,713 and 15,908 persons, respectively, who met the surveillance case definition of ALS. The estimated ALS prevalence rate was 4.7 cases per 100,000 U.S. population for 2012 and 5.0 per 100,000 for 2013. Due to revisions to the algorithm and use of death data from the National Death Index, an updated prevalence estimate has been calculated retrospectively for October 19, 2010-December 31, 2011. This updated estimate showed a prevalence rate of 4.3 per 100,000 population and a total of 13,282 cases. Since the inception of the Registry, the pattern of characteristics (e.g., age, sex, and race/ethnicity) among persons with ALS have remained unchanged. Overall, ALS was more common among whites, males, and persons aged 60-69 years. The age groups with the lowest number of ALS cases were persons aged 18-39 years and those aged ≥80 years. Males had a higher prevalence rate of ALS than females overall and across all data sources. These findings remained consistent during October 2010-December 2013., Interpretation: The Registry is the only available data source that can be used to estimate the national prevalence for ALS in the United States. Use of both administrative national databases and self-report from patients enables a comprehensive approach to estimate ALS prevalence. The overall increase in the prevalence rate from 4.3 per 100,000 persons (revised) during 2010-2011 to 4.7 and 5.0 per 100,000 persons, respectively, during 2012-2013 likely is not an actual increase in the number of ALS cases. Rather, this increase might be attributed to improved case ascertainment due to the refinement of the algorithm used to identify definite ALS cases, along with an increased public awareness of the Registry. Registry estimates of ALS prevalence are consistent with findings from long-established ALS registries in Europe and from smaller-scale epidemiologic studies previously conducted in the United States., Public Health Actions: Data collected by the National ALS Registry are being used to better describe the epidemiology of ALS in the United States and to help facilitate research. The combined approach of using national administrative databases and a self-enrollment web portal to collect data is novel and potentially could be used for other non-notifiable diseases such as Parkinson's disease or multiple sclerosis. Increased public awareness of the Registry might lead to more ALS cases being identified from the secure web portal (https://www.cdc.gov/als), which can ascertain cases apart from the national administrative databases. For example, in 2014, the ALS Ice Bucket Challenge, a social media-centered campaign, received extensive public visibility and created increased awareness of ALS. The Agency for Toxic Substances and Disease Registry (ATSDR) works closely with ALS advocacy and support groups, researchers, health care professionals, and others to promote the National ALS Registry and to identify all cases of ALS in the United States. In addition to estimating the prevalence of ALS, the Registry is being used to collect specimens from patient enrollees through a new biorepository, connect patient enrollees with new clinical trials and epidemiologic studies, and fund studies to help learn more about the etiology of ALS. Additional information about the National ALS Registry is available at http://www.cdc.gov/als or by calling toll-free at 1-877-442-9719.

Background: The National ALS Registry is made up of two components to capture amyotrophic lateral sclerosis (ALS) cases: national administrative databases (Medicare, Medicaid, Veterans Health Administration and Veterans Benefits Administration) and self-identified cases captured by the Registry's web portal. This study describes self-reported characteristics of U.S. adults with ALS using the data collected by the National ALS Registry web portal risk factor surveys only from October 19, 2010 through December 31, 2013., Objective: To describe findings from the National ALS Registry's web portal risk factor surveys., Measurements: The prevalence of select risk factors among adults with ALS was determined by calculating the frequencies of select risk factors-smoking and alcohol (non, current and former) histories, military service and occupational history, and family history of neurodegenerative diseases such as ALS, Alzheimer's and/or Parkinson's., Results: Nearly half of survey respondents were ever smokers compared with nearly 41% of adults nationally. Most respondents were ever drinkers which is comparable to national estimates. The majority were light drinkers. Nearly one-quarter of survey respondents were veterans compared with roughly 9% of US adults nationally. Most respondents were retired or disabled. The industries in which respondents were employed for the longest time were Professional and Scientific and Technical Services. When family history of neurodegenerative diseases in first degree relatives was evaluated against our comparison group, the rates of ALS were similar, but were higher for Parkinson's disease, Alzheimer's disease and any neurodegenerative diseases., Conclusions: The National ALS Registry web portal, to our knowledge, is the largest, most geographically diverse collection of risk factor data about adults living with ALS. Various characteristics were consistent with other published studies on ALS risk factors and will allow researchers to generate hypotheses for future research.

Individuals with anorexia nervosa (AN) restrict eating and become emaciated. They tend to have an aversion to foods rich in fat. Because epoxide hydrolase 2 (EPHX2) was identified as a novel AN susceptibility gene, and because its protein product, soluble epoxide hydrolase (sEH), converts bioactive epoxides of polyunsaturated fatty acid (PUFA) to the corresponding diols, lipidomic and metabolomic targets of EPHX2 were assessed to evaluate the biological functions of EPHX2 and their role in AN. Epoxide substrates of sEH and associated oxylipins were measured in ill AN, recovered AN and gender- and race-matched controls. PUFA and oxylipin markers were tested as potential biomarkers for AN. Oxylipin ratios were calculated as proxy markers of in vivo sEH activity. Several free- and total PUFAs were associated with AN diagnosis and with AN recovery. AN displayed elevated n-3 PUFAs and may differ from controls in PUFA elongation and desaturation processes. Cytochrome P450 pathway oxylipins from arachidonic acid, linoleic acid, alpha-linolenic acid and docosahexaenoic acid PUFAs are associated with AN diagnosis. The diol:epoxide ratios suggest the sEH activity is higher in AN compared with controls. Multivariate analysis illustrates normalization of lipidomic profiles in recovered ANs. EPHX2 influences AN risk through in vivo interaction with dietary PUFAs. PUFA composition and concentrations as well as sEH activity may contribute to the pathogenesis and prognosis of AN. Our data support the involvement of EPHX2-associated lipidomic and oxylipin dysregulations in AN, and reveal their potential as biomarkers to assess responsiveness to future intervention or treatment.

Adaptive decision making to eat is crucial for survival, but in anorexia nervosa, the brain persistently supports reduced food intake despite a growing need for energy. How the brain persists in reducing food intake, sometimes even to the point of death and despite the evolution of multiple mechanisms to ensure survival by governing adaptive eating behaviors, remains mysterious. Neural substrates belong to the reward-habit system, which could differ among the eating disorders. The present review provides an overview of neural circuitry of restrictive food choice, binge eating, and the contribution of specific serotonin receptors. One possibility is that restrictive food intake critically engages goal-directed (decision making) systems and "habit," supporting the view that persistent caloric restriction mimics some aspects of addiction to drugs of abuse., Significance Statement: An improved understanding of the neural basis of eating disorders is a timely challenge because these disorders can be deadly. Up to 70 million of people in the world suffer from eating disorders. Anorexia nervosa affects 1-4% of women in United States and is the first cause of death among adolescents in Europe. Studies relying on animal models suggest that decision making to eat (or not) can prevail over actual energy requirements due to emotional disturbances resulting in abnormal habitual behavior, mimicking dependence. These recent studies provide a foundation for developing more specific and effective interventions for these disorders., (Copyright © 2015 the authors 0270-6474/15/3513868-11$15.00/0.)

Objectives: Amyotrophic lateral sclerosis (ALS) is a rare motor neuron disease with incidence rates ranging from 1 to 2/100,000 person-years. The Texas Department of State Health Services previously conducted surveillance for ALS in three metropolitan areas of Texas. This project provides an update to this research, while expanding its scope to the entire state., Methods: The Texas Department of State Health Services contacted neurologists throughout Texas to determine whether they diagnose or treat patients with ALS. Those neurologists who cared for Texas residents with ALS between 2009 and 2011 were asked to complete a one-page case reporting form for each patient., Results: A total of 1422 unique cases were received. The average crude annual incidence rate was 1.30/100,000 person-years and the 2009 period prevalence rate was 2.92/100,000 individuals. Reported cases were most likely to be 60 to 69 years old, non-Hispanic, white, and men., Conclusions: This project provides an update to previously published Texas-specific epidemiological data regarding ALS; also, we note that our findings are consistent with previously published studies.

Our objective was to provide demographic profiles and incidence estimates of amyotrophic lateral sclerosis (ALS) in two diverse California metropolitan areas: Los Angeles County (LA) and the San Francisco Bay Area (SFBA). Data were retrospectively collected from multiple sources. Case eligibility criteria included residency in SFBA or LA, and treatment for or diagnosis of ALS between 1 January 2009 and 31 December 2011. Overall incidence rates as well as age-, gender-, race- and ethnicity-specific rates were calculated. We identified 539 ALS cases in SFBA and 545 in LA; 618 were incident cases. Cases were more likely to be male and white. There were considerably more cases (p < 0.05) in LA who were foreign-born (LA, 22%; SFBA, 15%), black (LA, 10%; SFBA, 6%) or Hispanic (LA, 19%; SFBA, 10%). Conversely, the age adjusted incidence rates (per 100,000) were higher in SFBA for whites (LA, 1.40; SFBA, 2.49) and Hispanics (LA, 0.66; SFBA, 1.57) compared with LA. General case demographics and incidence rates in these two areas were similar to published studies. However, the differences between the two areas raise questions about how factors such as geography, access to care, and referral patterns may affect case ascertainment and diagnosis.

Our objective was to describe racial and ethnic differences of amyotrophic lateral sclerosis (ALS) in distinct geographic locations around the United States (U.S.). ALS cases for the period 2009-2011 were identified using active case surveillance in three states and eight metropolitan areas. Of the 5883 unique ALS cases identified, 74.8% were white, 9.3% were African-American/black, 3.6% were Asian, 12.0% were an unknown race, and 0.3% were marked as some other race. For ethnicity, 77.5% were defined as non-Hispanic, 10.8% Hispanic, and 11.7% were of unknown ethnicity. The overall crude average annual incidence rate was 1.52 per 100,000 person-years and the rate differed by race and ethnicity. The overall age-adjusted average annual incidence rate was 1.44 per 100,000 person-years and the age-adjusted average incidence rates also differed by race and ethnicity. Racial differences were also found in payer type, time from symptom onset to diagnosis, reported El Escorial criteria, and age at diagnosis. In conclusion, calculated incidence rates demonstrate that ALS occurs less frequently in African-American/blacks and Asians compared to whites, and less frequently in Hispanics compared to non-Hispanics in the U.S. A more precise understanding of racial and ethnic variations in ALS may help to reveal candidates for further studies of disease etiology and disease progression.

Background: The Agency for Toxic Substances and Disease Registry established surveillance projects to determine the incidence, prevalence, and demographic characteristics of persons with Amyotrophic Lateral Sclerosis (ALS) in defined geographic areas. There is a need to characterize and account for the survival and prognostic factors among a population-based cohort of ALS cases in the United States., Methods: A cohort of incident cases diagnosed from 2009-2011 in New Jersey was followed until death or December 31, 2013, whichever happened first. Survival was assessed using Kaplan-Meier curves and Cox proportional hazards regression was used to identify prognostic factors., Results: Sixty-four percent of incident cases died between 2009 and 2013, 93.7% specifically from ALS. Among the 456 cases studied in the survival analysis, the median survival from diagnosis was 21 months; 46% of cases survived longer than two years from diagnosis. Older age predicted shorter survival. While there is some indication of differences because of sex, race, and ethnicity, these differences were not statistically significant when accounting for age., Conclusions: New Jersey mortality data were queried to determine the vital status of a cohort of incident ALS cases and used to investigate relationships between demographic factors and survival. Results are consistent with other population-based studies. Older age was a strong predictor of shorter survival time. Additional follow-up time is needed to characterize longer-term survival., (© 2015 S. Karger AG, Basel.)

Unlabelled: Our objective was to develop state and metropolitan area-based surveillance projects to describe the characteristics of those with ALS and to assist with evaluating the completeness of the National ALS Registry. Because the literature suggested that ethnic/racial minorities have lower incidence of ALS, three state and eight metropolitan areas were selected to over-represent ethnic/racial minorities to have a sufficient number of minority patients. Project activities relied on reports from medical providers and medical records abstraction. The project areas represented approximately 27% of the U.S., Population: The combined racial and ethnic distribution of these areas is 64.4% white, 16.0% African-American, 6.7% Asian, and 28.3% Hispanic. Most neurologists did not diagnose or provide care for ALS patients. The number of unique patients reported was close to expected (5883 vs. 6673). Age and gender distribution of patients was similar to the literature. The crude average annual incidence rate was 1.52 per 100,000 person-years, CI 1.44-1.61, and the 2009 prevalence rate was 3.84 per 100,000 population, CI 3.70-3.97. In conclusion, this study represents the largest number of clinically diagnosed ALS patients reported by neurologists in the U.S. Comparison of these data with those in the National ALS Registry will help evaluate the completeness of administrative databases.

As the Public Health Accreditation Board (PHAB) launched the nation's only accreditation program for state, local, tribal, and territorial health departments in September 2011, attention to the issues facing the public health workforce in these health departments was included. PHAB developed several measures in the accreditation standards and measures related to public health workforce development. The accreditation process calls upon health departments to focus more intentionally on their current workforce, while also supporting the development of future public health workers. Working with a group of public health workforce thought leaders, PHAB developed a long-range plan for the expectation of accredited health departments in workforce development. Beginning with the development of intentional standardization in workforce development and moving into future challenges and issues, PHAB uses its platform of quality improvement to bring emphasis on the current and future public health workforce. This article describes the development of the workforce components of public health department accreditation as well as future plans to ensure that the momentum continues. Using data from the accredited health departments at the time of article submission, PHAB also describes some of the approaches that governmental public health departments that have completed the accreditation process are using to develop their own workforce and support the development of the future public health workforce. Challenges faced by health departments in these areas are also described., (Copyright © 2014. Published by Elsevier Inc.)

Importance: Anorexia nervosa (AN) is a serious disorder with high rates of morbidity and mortality. Family-based treatment (FBT) is an evidence-based therapy for adolescent AN, but less than half of those who receive this approach recover. Hence, it is important to identify other approaches to prevent the development of the chronic form of AN for which there is no known evidence-based treatment., Objective: To compare FBT with systemic family therapy (SyFT) for the treatment of adolescent-onset AN., Design, Setting, and Participants: Research in Anorexia Nervosa (RIAN) is a 2-group (FBT and SyFT) randomized trial conducted between September 2005 and April 2012. Interviewers were blinded to the treatment condition. A total of 564 adolescents receiving care at 6 outpatient clinics experienced in the treatment of AN were screened. Of these, 262 adolescents did not meet the inclusion criteria and 138 declined to participate; hence, 164 adolescents (aged 12-18 years) of both sexes meeting the criteria for Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, AN (except for amenorrhea) were enrolled. Three participants were withdrawn from FBT and 7 were withdrawn from SyFT after serious adverse events occurred., Interventions: Two manualized family therapies with 16 one-hour sessions during 9 months. Family-based therapy focuses on the facilitation of weight gain, whereas SyFT addresses general family processes., Main Outcomes and Measures: The primary outcomes were percentage of ideal body weight (IBW) and remission (≥95% of IBW). The a priori hypothesis was that FBT would result in faster weight gain early in treatment and at the end of treatment (EOT)., Results: There were no statistically significant differences between treatment groups for the primary outcome, for eating disorder symptoms or comorbid psychiatric disorders at the EOT or follow-up. Remission rates included FBT, 33.1% at the EOT and 40.7% at follow-up and SyFT, 25.3% and 39.0%, respectively. Family-based therapy led to significantly faster weight gain early in treatment, significantly fewer days in the hospital, and lower treatment costs per patient in remission at the EOT (FBT, $8963; SyFT, $18 005). An exploratory moderator analysis found that SyFT led to greater weight gain than did FBT for participants with more severe obsessive-compulsive symptoms., Conclusions and Relevance: The findings of this study suggest that FBT is the preferred treatment for adolescent AN because it is not significantly different from SyFT and leads to similar outcomes at a lower cost than SyFT. Adolescents with more severe obsessive-compulsive symptoms may receive more benefits with SyFT., Trial Registration: clinicaltrials.gov Identifier NCT00610753.

Problem/condition: Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease for which no cure has been identified. Although ALS has no known definitive cause, familial ALS (a hereditary form) occurs in 5%-10% of cases. Many hypotheses have been formulated about what causes ALS, including chemical exposures, occupational exposure, military service, infectious agents, nutritional intake, physical activity, and trauma. Worldwide, ALS affects white males aged >60 years more often than any other group. In the United States, ALS surveillance is necessary to estimate the incidence and prevalence of ALS and collect data on risk factors. ALS is not a nationally notifiable condition in the United States (i.e., it is not a reportable condition in all jurisdictions), and individual state reporting requirements differ, with Massachusetts being the only state that mandates reporting., Period Covered: October 19, 2010-December 31, 2011., Description of System: In 2009, the federal Agency for Toxic Substances and Disease Registry (ATSDR) implemented the National ALS Registry to collect and analyze data regarding persons with ALS in the United States. The main goals of the Registry, as defined by the 2008 ALS Registry Act, are to describe the incidence and prevalence of ALS better, examine risk factors such as environmental and occupational exposures, and characterize the demographics of those living with ALS. The Registry uses a two-pronged approach to identify all cases of ALS. The first approach uses four existing national administrative databases (maintained by Medicare, Medicaid, the Veterans Health Administration, and the Veterans Benefits Administration) to identify prevalence of ALS. The second approach uses a secure web portal (http://www.cdc.gov/als) that was launched to the public on October 19, 2010, to identify cases not included in the four national administrative databases and to collect risk-factor data on known ALS cases. ALS patients who have registered via the web portal can complete brief risk-factor surveys online that are intended to attain a better understanding of ALS (e.g., genetics and environmental and occupational exposures) and help determine disease progression., Results: During October 19, 2010-December 31, 2011, a total of 12,187 persons meeting the surveillance case definition of definite ALS were identified by the Registry, for a prevalence of 3.9 cases of ALS per 100,000 persons in the U.S. general population. Incidence cannot be measured because the date of diagnosis was not noted in all patient records. Overall, ALS was more common among white males, non-Hispanics, and persons aged 60-69 years. The age groups with the lowest number of persons with ALS were age 18-39 years and age >80 years. Males had a higher prevalence rate of ALS than females overall and across all data sources., Interpretation: This is the first (and to date the only) effort to estimate the national prevalence of ALS in the United States. Using the combined approach of the national databases and the web-based portal enables researchers to estimate ALS prevalence more accurately. Registry findings for the prevalence of ALS are consistent with findings from long-established ALS registries in Europe and from smaller-scale epidemiologic studies conducted previously in the United States. Although incidence cannot be measured with Registry data at this time, incidence is being measured in smaller geographic areas that have participated in ATSDR's State and Metropolitan Area ALS surveillance projects., Public Health Actions: Data collected by the National ALS Registry are being used to better describe the prevalence of ALS in the United States and to help facilitate research. The combined approach of using national administrative databases and a self-enrollment web portal to collect data is novel and potentially could be used for other non-notifiable diseases such as Parkinson's disease or multiple sclerosis. ATSDR is working closely with ALS advocacy and support groups, researchers, health-care professionals, and others to promote the National ALS Registry in order to capture all cases of ALS. To further enhance and strengthen the Registry, ATSDR is 1) adding new modules to the portal to examine other potential risk factors, 2) launching a feasibility study for a novel ALS biorepository (available at http://wwwn.cdc.gov/als/ALSBioRegistry.aspx) linked to the Registry that would potentially provide biologic specimens from patient enrollees to help researchers learn more about disease etiology, 3) engaging in surveillance activities in selected states and large metropolitan areas to help test the completeness of the Registry as well as calculating incidence in these areas, and 4) using the Registry to recruit patient enrollees for new clinical trials and epidemiologic studies. Additional information about the National ALS Registry is available at http://www.cdc.gov/als or by calling toll-free at 1-877-442-9719.

Anorexia nervosa (AN) and related eating disorders are complex, multifactorial neuropsychiatric conditions with likely rare and common genetic and environmental determinants. To identify genetic variants associated with AN, we pursued a series of sequencing and genotyping studies focusing on the coding regions and upstream sequence of 152 candidate genes in a total of 1205 AN cases and 1948 controls. We identified individual variant associations in the Estrogen Receptor-ß (ESR2) gene, as well as a set of rare and common variants in the Epoxide Hydrolase 2 (EPHX2) gene, in an initial sequencing study of 261 early-onset severe AN cases and 73 controls (P=0.0004). The association of EPHX2 variants was further delineated in: (1) a pooling-based replication study involving an additional 500 AN patients and 500 controls (replication set P=0.00000016); (2) single-locus studies in a cohort of 386 previously genotyped broadly defined AN cases and 295 female population controls from the Bogalusa Heart Study (BHS) and a cohort of 58 individuals with self-reported eating disturbances and 851 controls (combined smallest single locus P<0.01). As EPHX2 is known to influence cholesterol metabolism, and AN is often associated with elevated cholesterol levels, we also investigated the association of EPHX2 variants and longitudinal body mass index (BMI) and cholesterol in BHS female and male subjects (N=229) and found evidence for a modifying effect of a subset of variants on the relationship between cholesterol and BMI (P<0.01). These findings suggest a novel association of gene variants within EPHX2 to susceptibility to AN and provide a foundation for future study of this important yet poorly understood condition.

Background: Limited epidemiological data exist about amyotrophic lateral sclerosis (ALS) in the United States (US). The Agency for Toxic Substances and Disease Registry maintains the National ALS Registry and funded state and metropolitan surveillance projects to obtain reliable, timely information about ALS in defined geographic areas., Methods: Neurologists submitted case reports for ALS patients under their care between January 1, 2009 and December 31, 2011 who were New Jersey residents. A medical record verification form and electromyogram (EMG) report were requested for a sample of case reports. Incidence rates were standardized to the 2000 US Standard Population., Results: The average crude annual incidence rate was 1.87 per 100,000 person-years, the average age-adjusted annual incidence rate was 1.67 per 100,000 person-years, and the point prevalence rate on December 31, 2011 was 4.40 per 100,000 persons. Average annual incidence rates and point prevalence rates were statistically higher for men compared with women; Whites compared with Blacks/African Americans and Asians; and non-Hispanics compared with Hispanics., Conclusions: The project findings contribute new, population-based, state-specific information to epidemiological data regarding ALS. The findings are generally consistent with previously published surveillance studies conducted in the US and abroad., (© 2014 S. Karger AG, Basel.)

Individuals with anorexia nervosa (AN) restrict food consumption and become severely emaciated. Eating food, even thinking of eating food, is often associated with heightened anxiety. However, food cue anticipation in AN is poorly understood. Fourteen women recovered from AN and 12 matched healthy control women performed an anticipation task viewing images of food and object images during functional magnetic resonance imaging. Comparing anticipation of food versus object images between control women and recovered AN groups showed significant interaction only in the right ventral anterior insula, with greater activation in recovered AN anticipating food images. These data support the hypothesis of a disconnect between anticipating and experiencing food stimuli in recovered AN. Insula activation positively correlated with pleasantness ratings of palatable foods in control women, while no such relationship existed in recovered AN, which is further evidence of altered interoceptive function. Finally, these findings raise the possibility that enhanced anterior insula anticipatory response to food cues in recovered AN could contribute to exaggerated sensitivity and anxiety related to food and eating., (© 2013 Elsevier Ireland Ltd. All rights reserved.)

Objective. To evaluate the validity of proxy interviews in obtaining information on persons with rapidly fatal diseases such as malignant mesothelioma (MM). Methods. Persons with MM diagnosed in 2002 through 2005 in New York and New Jersey and 1997-2004 in Wisconsin were eligible for inclusion in the project. Persons with MM and their family member proxy were interviewed using the same questionnaire designed by ATSDR to collect information on potential direct or indirect occupational and environmental exposure to asbestos, genetic, and health related malignancy predisposition, and exposure to tobacco products. Descriptive statistics and the McNemar/Durkalski test were used to analyze 33 matched pairs. Results. The overall study confirmed a generally high ability of proxies to give interviews of comparable quality and completeness when asked dichotomous questions. The reliability of information collected from proxies varied by topic and family relationship. Conclusions. Family proxy interviews, using dichotomous responses, can serve as an acceptable source of information about health and exposure-related risk factors for MM.

Objective: Our objective is to determine whether quetiapine was superior to placebo in increasing weight or reducing core symptoms of anorexia nervosa as assessed by the Yale-Brown-Cornell Eating Disorder Scale and the Eating Disorder Inventory-2., Method: Participants were randomised to 8 weeks of quetiapine or placebo., Results: There are 21 participants who signed informed consent, 15 were randomised, 14 returned for at least one visit after receiving drug and 10 completed the study. There were no differences between drug and placebo in questionnaire scores, weight or measures of anxiety or depression., Discussion: There was no difference between quetiapine and placebo on weight gain or core symptoms. Small effect sizes suggest that a higher number of participants would not increase significant differences between groups., (Copyright © 2012 John Wiley & Sons, Ltd and Eating Disorders Association.)

Objective: Physiological and pharmacological studies indicate that altered brain serotonin (5-HT) activity could contribute to a susceptibility to develop appetitive and behavioral alterations that are characteristic of bulimia nervosa (BN)., Method: Eight individuals recovered from BN (REC BN) and eight healthy control women were scanned with [11C]DASB and positron emission tomography imaging of the 5-HT transporter (5-HTT). Logan graphical analysis was applied, and parametric binding potential (BP(nondisplaceable (ND)) ) images were generated. Voxel-by-voxel t-tests and a region of interest (ROI) analysis were conducted., Results: REC BN had significantly lower [11C]DASB BP(ND) in midbrain, superior and inferior cingulate and significantly higher [11C]DASB BP(ND) in anterior cingulate and superior temporal gyrus in the voxel-based analysis. ROI analysis indicated lower [11C]DASB BP(ND) in midbrain (p = .07), containing the dorsal raphe, in REC BN, consistent with our earlier studies., Discussion: These preliminary findings of a small-scale study confirm and extend previous data suggesting that ill and recovered BN have altered 5-HTT measures, which potentially contribute to BN symptomatology and/or differential responses to medication., (Copyright © 2011 Wiley Periodicals, Inc.)

Objective: To describe obstacles in the implementation of a controlled treatment trial of adolescent anorexia nervosa (AN)., Method: The original aim was to enter 240 participants with AN to one of four cells: Behavioral family therapy (BFT) plus fluoxetine; BFT plus placebo; systems family therapy (SFT) plus fluoxetine; SFT plus placebo., Results: Recruitment was delayed pending a satisfactory resolution concerning participant safety. After 6 months of recruitment it became clear that the medication was associated with poor recruitment leading to a study redesign resulting in a comparison of two types of family therapy with a projected sample size of 160. One site was unable to recruit and was replaced., Discussion: Problems with the delineation of safety procedures, recruitment, re-design of the study, and replacement of a site, were the main elements resulting in a 1-year delay. Suggestions are made for overcoming such problems in future AN trials., (Copyright © 2011 Wiley Periodicals, Inc.)

Objectives: The treatment of eating disorders is a complex process that relies not only on the use of psychotropic drugs but should include also nutritional counselling, psychotherapy and the treatment of the medical complications, where they are present. In this review recommendations for the pharmacological treatment of eating disorders (anorexia nervosa (AN), bulimia nervosa (BN), binge eating disorder (BED)) are presented, based on the available literature., Methods: The guidelines for the pharmacological treatment of eating disorders are based on studies published between 1977 and 2010. A search of the literature included: anorexia nervosa bulimia nervosa, eating disorder and binge eating disorder. Many compounds have been studied in the therapy of eating disorders (AN: antidepressants (TCA, SSRIs), antipsychotics, antihistaminics, prokinetic agents, zinc, Lithium, naltrexone, human growth hormone, cannabis, clonidine and tube feeding; BN: antidepressants (TCA, SSRIs, RIMA, NRI, other AD), antiepileptics, odansetron, d-fenfluramine Lithium, naltrexone, methylphenidate and light therapy; BED: antidepressants (TCA, SSRIs, SNRIs, NRI), antiepileptics, baclofen, orlistat, d-fenfluramine, naltrexone)., Results: In AN 20 randomized controlled trials (RCT) could be identified. For zinc supplementation there is a grade B evidence for AN. For olanzapine there is a category grade B evidence for weight gain. For the other atypical antipsychotics there is grade C evidence. In BN 36 RCT could be identified. For tricyclic antidepressants a grade A evidence exists with a moderate-risk-benefit ratio. For fluoxetine a category grade A evidence exists with a good risk-benefit ratio. For topiramate a grade 2 recommendation can be made. In BED 26 RCT could be identified. For the SSRI sertraline and the antiepileptic topiramate a grade A evidence exists, with different recommendation grades., Conclusions: Additional research is needed for the improvement of the treatment of eating disorders. Especially for anorexia nervosa there is a need for further pharmacological treatment strategies., (© 2011 Informa Healthcare)

This pilot study investigated the perception of the quality of health care received by 55 HIV-positive African Americans. A survey instrument, "The Quality of Care Through the Patient's Eyes"-HIV questionnaire (QUOTE-HIV), developed in the Netherlands, was used to collect quantitative data from the modified QUOTE-HIV. Qualitative data are from 2 focus groups' perception discussions of the applicability of the QUOTE-HIV to HIV-positive African Americans. The study's purpose was to assess the usefulness of the questionnaire to identify patients' perceptions of quality health care. Results indicated that the QUOTE-HIV is a useful tool to assess HIV-positive African Americans' satisfaction and adequately covered all areas of concern discussed by both focus groups as components for measuring perceived quality of care. Most important, the QUOTE-HIV was found to be appropriate for assessing study participants' satisfaction with quality health care and assessing the receipt of quality health care from their respective providers.

We assessed the relation between season of birth and eating disorder symptoms and personality characteristics in a sample of 880 women with eating disorders and 580 controls from two Price Foundation Studies. Eating disorder symptoms were assessed using the Structured Interview of Anorexic and Bulimic Disorders and the Structured Clinical Interview for DSM-IV. Personality traits were assessed using the Temperament and Character Inventory and the Frost Multidimensional Perfectionism Scale. Date of birth was obtained from a sociodemographic questionnaire. No significant differences were observed 1) in season of birth across eating disorder subtypes and controls; nor 2) for any clinical or personality variables and season of birth. We found no evidence of season of birth variation in eating disorders symptoms or personality traits. Contributing to previous conflicting findings, the present results do not support a season of birth hypothesis for eating disorders.

Aim: To evaluate key pre-arrest factors and their collective ability to predict post-cardiopulmonary arrest mortality. CPR is often initiated indiscriminately after in-hospital cardiopulmonary arrest. Improved understanding of pre-arrest factors associated with mortality may inform advance care planning., Methods: A cohort of 49,130 adults who experienced pulseless cardiopulmonary arrest from January 2000 to September 2004 was obtained from 366 US hospitals participating in the National Registry for Cardiopulmonary Resuscitation (NRCPR). Logistic regression with bootstrapping was used to model in-hospital mortality, which included those discharged in unfavorable and severely worsened neurologic state (Cerebral Performance Category >/=3)., Results: Overall in-hospital mortality was 84.1%. Advanced age, black race, non-cardiac, non-surgical illness category, pre-existing malignancy, acute stroke, trauma, septicemia, hepatic insufficiency, general floor or Emergency Department location, and pre-arrest use of vasopressors or assisted/mechanical ventilation were independently predictive of in-hospital mortality. Retained peri-arrest factors including cardiac monitoring, and shockable initial pulseless rhythms, were strongly associated with survival. The validation model's AUROC curve (0.77) revealed fair performance., Conclusions: Predictive pre-resuscitation factors may supplement patient-specific information available at bedside to assist in revising resuscitation plans during the patient's hospitalization., (Copyright 2009. Published by Elsevier Ireland Ltd.)

Anorexia nervosa (AN) and bulimia nervosa (BN) are related disorders of unknown etiology that most commonly begin during adolescence in women. AN and BN have unique and puzzling symptoms, such as restricted eating or binge-purge behaviors, body image distortions, denial of emaciation, and resistance to treatment. These are often chronic and relapsing disorders, and AN has the highest death rate of any psychiatric disorder. The lack of understanding of the pathogenesis of this illness has hindered the development of effective interventions, particularly for AN. Individuals with AN and BN are consistently characterized by perfectionism, obsessive-compulsiveness, and dysphoric mood. Individuals with AN tend to have high constraint, constriction of affect and emotional expressiveness, ahendonia and asceticism, whereas individuals with BN tend to be more impulsive and sensation seeking. Such symptoms often begin in childhood, before the onset of an eating disorder, and persist after recovery, suggesting they are traits that create a vulnerability for developing an ED. There is growing acknowledgement that neurobiological vulnerabilities make a substantial contribution to the pathogenesis of AN and BN. Considerable evidence suggests that altered brain serotonin (5-HT) function contributes to dysregulation of appetite, mood, and impulse control in AN and BN. Brain imaging studies, using 5-HT specific ligands, show that disturbances of 5-HT function occur when people are ill, and persist after recovery from AN and BN. It is possible that a trait-related disturbance of 5-HT neuronal modulation predates the onset of AN and contributes to premorbid symptoms of anxiety, obsessionality, and inhibition. This dysphoric temperament may involve an inherent dysregulation of emotional and reward pathways which also mediate the hedonic aspects of feeding, thus making these individuals vulnerable to disturbed appetitive behaviors. Restricting food intake may become powerfully reinforcing because it provides a temporary respite from dysphoric mood. Several factors may act on these vulnerabilities to cause AN to start in adolescence. First, puberty-related female gonadal steroids or age-related changes may exacerbate 5-HT dysregulation. Second, stress and/or cultural and societal pressures may contribute by increasing anxious and obsessional temperament. Individuals with AN may discover that reduced dietary intake, by reducing plasma tryptophan availability, is a means by which they can modulate brain 5-HT functional activity and anxious mood. People with AN enter a vicious cycle which accounts for the chronicity of this disorder because caloric restriction results in a brief respite from dysphoric mood. However, malnutrition and weight loss, in turn, produce alterations in many neuropeptides and monoamine function, perhaps in the service of conserving energy, but which also exaggerates dysphoric mood. In summary, this article reviews findings in brain chemistry and neuroimaging that shed new light on understanding the psychopathology of these difficult and frustrating disorders.

Few, if any, published time series studies have evaluated the effects of particulate matter air exposures by combining hospital admissions with medical visit data for smaller populations. We investigated the relationship between daily particulate matter (<10 microm in aerometric diameter or PM10) exposures with admissions and medical visits (emergency room, urgent care, and family practice) for respiratory and cardiovascular disease in Pocatello and Chubbuck, Idaho (population about 60,000), from November 1994 through March 2000. Within generalized linear models, time, weather, influenza, and day-of-week effects were controlled. In single-pollutant models, respiratory disease admissions and visits increased (7.1-15.4% per 50 microg/m3 PM10) for each age group analyzed, with the highest increases in two groups, children and especially the elderly. Statistical analyses suggest that the results probably did not occur by chance. Sensitivity analyses did not provide strong evidence that the respiratory disease effect estimates were sensitive to reasonable changes in the final degrees of freedom choice for time and weather effects. No strong evidence of confounding by NO2 and SO2 was found from results of multi-pollutant models. Ozone and carbon monoxide data were not available to include multi-pollutant models, but evidence suggests that they were not a problem. Unexpectedly, evidence of an association between PM10 with cardiovascular disease was not found, possibly due to the lifestyles of the mostly Mormon study population. Successful time series analyses can be performed on smaller populations if diverse, centralized databases are available. Hospitals that offer urgent or other primary care services may be a rich source of data for researchers. Using data that potentially represented a wide-range of disease severity, the findings provide evidence that evaluating only hospital admissions or emergency room visit effects may underestimate the overall morbidity due to acute particulate matter exposures. Further work is planned to test this conclusion.

Objective: The current study sought to compare eating disorder symptomatology among ballet dancers and individuals with restricting anorexia nervosa (RAN), bulimia nervosa (BN), and no eating pathology., Method: Twenty-nine female ballet dancers completed assessments and were compared with an archival dataset of 26 women with RAN, 47 women with BN, and 44 women with no eating pathology. Eating disorder diagnoses and behaviors were assessed with a semi-structured clinical interview, the Eating Disorder Inventory (EDI), and a weight history interview., Results: Eighty-three percent of dancers met lifetime criteria for AN (6.9%), BN (10.3%), AN+BN (10.3%), or EDNOS (55.0%). Moreover, dancers looked more similar to eating-disordered individuals than to control individuals on measures of eating pathology., Conclusion: Despite previous emphasis on the pathology AN, the current findings suggest that dancers frequently engage in binge eating and purging behaviors. Moreover, it appears that their pathology is as severe as that of non-dancing women with eating disorders., (Copyright (c) 2006 by Wiley Periodicals, Inc.)