Neurological Care within the Indian Health Service.

American Indians and Alaska Natives (AI/AN) are the Indigenous peoples of the United States. According to the U.S. Census Bureau, approximately 9.7 million people self-identified as AI/AN (alone or in combination with other races), representing 2.9% of the total U.S. population. These people represent diverse groups of discrete Tribes, each with their language, culture, and geographic home. As part of the conquest and settlement of North America, some Indigenous peoples signed treaties with the U.S. government, surrendering their lands in return for various government commitments, including health care. The Indian Health Service (IHS) was born out of these agreements. The IHS is an agency in the U.S. Department of Health and Human Services under the U.S. Public Health Service. The IHS provides a comprehensive health service delivery system for approximately 2.7 million AI/AN who belong to 574 federally recognized Tribes/nations in 37 states. The aim of this paper is to make recommendations regarding the initiation of sustainable neurology care in marginalized or underserved populations by reviewing 40 years of neurology care provision within the IHS. We will discuss (1) the IHS, (2) neurological care provided within the IHS, including midlevel provider extension of neurology care and traditional medical care, and (3) select neurological diagnoses within AI/AN populations. Marginalized populations, including those in the United States that are rural, remote, or low socioeconomic status, lack access to specialty neurology care. This includes many AI/AN. The IHS has developed novel solutions to promote specialty care, including neurology. Notably, initial IHS investments in full-time neurology providers have led to more robust neurology care, often receiving attention from university programs. This suggests that an initial investment in stable on-site full-time neurology services provides a path to potential sustainable care for marginalized populations.

Epilepsy-related stigma and attitudes: Systematic review of screening instruments and interventions - Report by the International League Against Epilepsy Task Force on Stigma in Epilepsy.

OBJECTIVE: This is a systematic review aimed at summarizing the evidence related to instruments that have been developed to measure stigma or attitudes toward epilepsy and on stigma-reducing interventions. METHODS: This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. A broad literature search (1985-2019) was performed in 13 databases. Articles were included if they described the development and testing of psychometric properties of an epilepsy-related stigma or attitude scale or stigma-reducing interventions. Two reviewers independently screened abstracts, reviewed full-text articles, and extracted data. Basic descriptive statistics are reported. RESULTS: We identified 4234 abstracts, of which 893 were reviewed as full-text articles. Of these, 38 met inclusion criteria for an instrument development study and 30 as a stigma-reduction intervention study. Most instruments were initially developed using well-established methods and were tested in relatively large samples. Most intervention studies involved educational programs for adults with pre- and post-evaluations of attitudes toward people with epilepsy. Intervention studies often failed to use standardized instruments to quantify stigmatizing attitudes, were generally underpowered, and often found no evidence of benefit or the benefit was not sustained. Six intervention studies with stigma as the primary outcome had fewer design flaws and showed benefit. Very few or no instruments were validated for regional languages or culture, and there were very few interventions tested in some regions. SIGNIFICANCE: Investigators in regions without instruments should consider translating and further developing existing instruments rather than initiating the development of new instruments. Very few stigma-reduction intervention studies for epilepsy have been conducted, study methodology in general was poor, and standardized instruments were rarely used to measure outcomes. To accelerate the development of effective epilepsy stigma-reduction interventions, a paradigm shift from disease-specific, siloed trials to collaborative, cross-disciplinary platforms based upon unified theories of stigma transcending individual conditions will be needed.

Systematic review of frequency of felt and enacted stigma in epilepsy and determining factors and attitudes toward persons living with epilepsy-Report from the International League Against Epilepsy Task Force on Stigma in Epilepsy.

OBJECTIVE: To review the evidence of felt and enacted stigma and attitudes toward persons living with epilepsy, and their determining factors. METHODS: Thirteen databases were searched (1985-2019). Abstracts were reviewed in duplicate and data were independently extracted using a standardized form. Studies were characterized using descriptive analysis by whether they addressed "felt" or "enacted" stigma and "attitudes" toward persons living with epilepsy. RESULTS: Of 4234 abstracts, 132 met eligibility criteria and addressed either felt or enacted stigma and 210 attitudes toward epilepsy. Stigma frequency ranged broadly between regions. Factors associated with enacted stigma included low level of knowledge about epilepsy, lower educational level, lower socioeconomic status, rural areas living, and religious grouping. Negative stereotypes were often internalized by persons with epilepsy, who saw themselves as having an "undesirable difference" and so anticipated being treated differently. Felt stigma was associated with increased risk of psychological difficulties and impaired quality of life. Felt stigma was linked to higher seizure frequency, recency of seizures, younger age at epilepsy onset or longer duration, lower educational level, poorer knowledge about epilepsy, and younger age. An important finding was the potential contribution of epilepsy terminology to the production of stigma. Negative attitudes toward those with epilepsy were described in 100% of included studies, and originated in any population group (students, teachers, healthcare professionals, general public, and those living with epilepsy). Better attitudes were generally noted in those of younger age or higher educational status. SIGNIFICANCE: Whatever the specific beliefs about epilepsy, implications for felt and enacted stigma show considerable commonality worldwide. Although some studies show improvement in attitudes toward those living with epilepsy over time, much work remains to be done to improve attitudes and understand the true occurrence of discrimination against persons with epilepsy.

Validity testing in veterans with epileptic seizures and psychogenic nonepileptic seizures.

OBJECTIVE: Identification of clinically meaningful subgroups among patients with psychogenic nonepileptic seizures (PNES) or epileptic seizures (ES) is of potential value for assessing prognosis and predicting therapeutic response. Invalid performance on validity tests has been associated with noncredible complaints and worse cognitive test scores, and may be one such classification criteria. We studied invalid performance in Veterans with PNES or ES, and the association of invalid performance with cognitive test scores and subjective complaints. METHODS: Patients were consecutive admissions to three veterans affairs (VA) epilepsy monitoring units. Evaluations included two validity tests: the Test of Memory Malingering (TOMM); and the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF) over-reporting validity scales. We compared the frequency of invalid performance on the TOMM or MMPI-2-RF in patients diagnosed with PNES vs. ES. We evaluated the association of invalid performance with scores on the Repeatable Battery for Assessment of Neuropsychological Status (RBANS), and four subjective symptom measures including the Beck Depression Inventory-II, and Quality of Life in Epilepsy-31. RESULTS: Invalid TOMM performance was found in 25.3% of Veterans diagnosed with PNES and 10.8% of those with ES (p = .03). Invalid reporting on the MMPI-2-RF was found in 35.9% of the PNES group vs. 15.3% of the ES group (p = .01). Effects of valid vs. invalid reporting on external measures were similar for ES and PNES groups. Patients with invalid vs. valid TOMM performance had lower scores on the RBANS (p < .001). Patients with invalid performance had greater complaints on all subjective measures, with largest effect sizes for the MMPI-2-RF validity scales (p < .001). SIGNIFICANCE: In Veterans admitted for evaluation of poorly controlled seizures, invalid performance on validity tests was not uncommon. Cognitive test results and subjective reports from patients with invalid performance may not be credible. These observations have implications for the analysis of clinical trials, where primary and secondary outcomes often rely on self-report measures.

Health-related quality of life in Veterans with epileptic and psychogenic nonepileptic seizures.

RATIONALE: Health-related quality of life (HRQoL) is compromised in civilians with epileptic seizures (ES) or psychogenic nonepileptic seizures (PNES). U.S. Veterans are a distinct patient group with regard to gender, age, and background. We studied HRQoL in Veterans and asked the following: (1) Is there a difference in HRQoL in Veterans with ES vs. PNES?; (2) What factors influence HRQoL in each group?; (3) What factors influenced the difference between seizure groups? METHODS: We studied consecutive Veterans entering the epilepsy monitoring units (EMUs) of three VA Epilepsy Centers of Excellence. Patients underwent continuous video-EEG monitoring. Seizure diagnoses followed established criteria. Health-related quality of life was measured with the Quality of Life in Epilepsy Inventory-31 (QOLIE-31). Evaluations included the Structured Clinical Interview for Diagnostic and Statistical Manual-IV (DSM IV), the posttraumatic stress disorder (PTSD) Checklist (PCL), the Beck Depression Inventory II (BDI-II), and the Minnesota Multiphasic Personality Inventory-2 Restructured form (MMPI-2RF). Between-group differences were tested with Wilcoxon tests. Nested regression analysis was used to evaluate the influence of demographic, social, military, seizure-related, and psychological factors on QOLIE-31 scores. RESULTS: The median QOLIE-31 total score was 14 points lower in Veterans with PNES vs. ES (p < 0.001; Cohen's d = 0.73). Within each seizure group, psychological factors accounted for ≥50% of the variance in QOLIE scores while combined demographic, social, and seizure-related factors accounted for 18% (group with ES) and 7% (PNES). Psychological measures, particularly PCL and the BDI-II scores, accounted for all of the difference in QOLIE-31 total scores between Veterans with ES and those with PNES. CONCLUSIONS: Health-related quality of life as measured by the QOLIE-31 is worse in Veterans with PNES as compared with those with ES. Psychological factors account for the most of the variance in QOLIE-31 scores regardless of seizure type and also account for the difference between groups with PNES and ES. Demographic, military, social, and seizure-related factors have minimal influence on HRQoL. These results in U.S. Veterans are similar to those found in civilians despite differences in patient age, gender, and background.

Psychiatric comorbidity and traumatic brain injury attribution in patients with psychogenic nonepileptic or epileptic seizures: A multicenter study of US veterans.

OBJECTIVE: To determine the frequency and severity of psychiatric disorders and attribution of seizures to traumatic brain injury (TBI) in veterans with verified psychogenic nonepileptic seizures (PNES) versus epileptic seizures (ES). METHODS: We studied 333 consecutive admissions to the monitoring units of three Veterans Administration epilepsy centers. All patients underwent continuous video-electroencephalographic recording to define definite PNES or ES. Evaluations included the Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders, 4th edition, PTSD Checklist, Beck Depression Inventory II, and Patient Seizure Etiology Questionnaire. Interviews and questionnaires were completed prior to final seizure type diagnosis and patient debriefing. The primary outcome measure was a comparison of Axis I psychiatric diagnoses in patients diagnosed with PNES versus ES. RESULTS: A total of 81 patients were diagnosed with PNES, and 70 with ES. PTSD was the most frequent Axis I diagnosis in veterans with PNES (64%, vs 13% of those with ES; P < 0.001). Posttraumatic stress disorder (PTSD) was common regardless of deployment to a war theater or combat exposure. Mood, substance abuse, and anxiety disorders were also more common in the PNES group. TBI was cited as a likely cause of seizures by 47% of veterans with PNES versus 25% of those with ES (P = 0.01). PTSD and attribution of seizures to TBI were found in 30% of veterans with PNES versus 3% of those with ES (P < 0.001). SIGNIFICANCE: In veterans referred for inpatient seizure evaluation, PTSD was strongly associated with a diagnosis of PNES versus ES. The association of PNES with PTSD, attribution of seizures to TBI, or both, may prompt early consideration of PNES.

The primary prevention of epilepsy: A report of the Prevention Task Force of the International League Against Epilepsy.

Among the causes of epilepsy are several that are currently preventable. In this review, we summarize the public health burden of epilepsy arising from such causes and suggest priorities for primary epilepsy prevention. We conducted a systematic review of published epidemiologic studies of epilepsy of 4 preventable etiologic categories-perinatal insults, traumatic brain injury (TBI), central nervous system (CNS) infection, and stroke. Applying consistent criteria, we assessed the quality of each study and extracted data on measures of risk from those with adequate quality ratings, summarizing findings across studies as medians and interquartile ranges. Among higher-quality population-based studies, the median prevalence of active epilepsy across all ages was 11.1 per 1000 population in lower- and middle-income countries (LMIC) and 7.0 per 1000 in high-income countries (HIC). Perinatal brain insults were the largest attributable fraction of preventable etiologies in children, with median estimated fractions of 17% in LMIC and 15% in HIC. Stroke was the most common preventable etiology among older adults with epilepsy, both in LMIC and in HIC, accounting for half or more of all new onset cases. TBI was the attributed cause in nearly 5% of epilepsy cases in HIC and LMIC. CNS infections were a more common attributed cause in LMIC, accounting for about 5% of all epilepsy cases. Among some rural LMIC communities, the median proportion of epilepsy cases attributable to endemic neurocysticercosis was 34%. A large proportion of the overall public health burden of epilepsy is attributable to preventable causes. The attributable fraction for perinatal causes, infections, TBI, and stroke in sum reaches nearly 25% in both LMIC and HIC. Public health interventions addressing maternal and child health care, immunizations, public sanitation, brain injury prevention, and stroke prevention have the potential to significantly reduce the burden of epilepsy.

Attributing seizures to TBI: Validation of a brief patient questionnaire.

PURPOSE: Traumatic brain injury (TBI) is an important cause of epilepsy and has also been associated with psychogenic nonepileptic seizures (PNES). We designed a brief questionnaire assessing patient beliefs regarding TBI as the cause of their seizures (Patient Seizure Etiology Questionnaire; PSEQ). This study reports content validity for the PSEQ. METHODS: Ninety Veterans undergoing comprehensive evaluation at 3 VA epilepsy centers completed the PSEQ, a series of questions regarding possible causes for their seizures, including TBI. The PSEQ was scored as YES vs. NO for TBI as the proposed cause of seizures. For each patient, two expert reviewers independently completed a structured chart review to determine whether TBI was the proposed cause of seizures (n=180 reviews). Kappa statistic was used to assess agreement between the PSEQ and each chart review and between the PSEQ and combined chart reviews where both reviewers agreed on a TBI seizure etiology. RESULTS: The PSEQ scored higher overall rates for a TBI seizure etiology than did expert chart reviews (40% vs. 28%; p<0.001). The PSEQ agreed with 82% of 180 independent chart reviews (sensitivity 88%; specificity 79%). Kappa statistic for agreement was 0.60. The two reviewers agreed on a probable TBI seizure etiology for 83% of chart reviews. The PSEQ sensitivity increased to 100% when both reviewers were in agreement. CONCLUSION: The PSEQ provides a direct, standardized measure of patient beliefs regarding TBI as the cause of their seizures and has moderate-substantial agreement with expert chart reviews.

Clinical approach to posttraumatic epilepsy.

Traumatic brain injury (TBI) is one of the most common causes of acquired epilepsy, and posttraumatic epilepsy (PTE) results in significant somatic and psychosocial morbidity. The risk of developing PTE relates directly to TBI severity, but the latency to first seizure can be decades after the inciting trauma. Given this "silent period," much work has focused on identification of molecular and radiographic biomarkers for risk stratification and on development of therapies to prevent epileptogenesis. Clinical management requires vigilant neurologic surveillance and recognition of the heterogeneous endophenotypes associated with PTE. Appropriate treatment of patients who have or are at risk for seizures varies as a function of time after TBI, and the clinician's armamentarium includes an ever-expanding diversity of pharmacological and surgical options. Most recently, neuromodulation with implantable devices has emerged as a promising therapeutic strategy for some patients with refractory PTE. Here, we review the epidemiology, diagnostic considerations, and treatment options for PTE and develop a roadmap for providers encountering this challenging clinical entity.

Multicentric Castleman's disease with voltage-gated potassium channel antibody-positive limbic encephalitis: a case report.

BACKGROUND: Castleman's disease is a rare lymphoproliferative disorder which occurs in localized and multicentric forms and can mimic lymphoma. Despite its well-known association with certain autoimmune diseases, including paraneoplastic pemphigus and myasthenia gravis, Castleman's disease has not previously been associated with limbic encephalitis. CASE PRESENTATION: We report the case of a 47-year old Caucasian man who presented with subacute onset of constitutional symptoms, diffuse lymphadenopathy, and stereotyped spells involving olfactory aura, nausea, disorientation, and unresponsiveness. He was found to have focal dyscognitive seizures of temporal lobe origin, cerebrospinal fluid with lymphocytic pleocytosis, hyponatremia, and serum positive for voltage-gated potassium channel antibodies, consistent with limbic encephalitis. An extensive infectious workup was unrevealing, but lymph node biopsy revealed multicentric Castleman's disease. His symptoms improved with antiepileptic drugs and immunotherapy. CONCLUSION: This case highlights the clinical diversity of voltage-gated potassium channel autoimmunity and expands the association of Castleman's disease and autoimmune syndromes to include limbic encephalitis. Clinicians should be aware that paraneoplastic disorders of the central nervous system can be related to underlying hematologic disorders such as Castleman's disease.

Antiepileptic drug prescribing patterns in Iraq and Afghanistan war veterans with epilepsy.

OBJECTIVE: We examined patterns of antiepileptic drug (AED) use in a cohort of Iraq/Afghanistan war veterans (IAVs) who were previously identified as having epilepsy. We hypothesized that clinicians would be more likely to prescribe newer AEDs and would select specific AEDs to treat seizures based on patient characteristics including gender and comorbidities. METHODS: From the cohort of IAVs previously identified with epilepsy between fiscal years 2009 and 2010, we selected those who received AEDs from the Veterans Health Administration in FY2010. Regimens were classified as monotherapy or polytherapy, and specific AED use was examine overall and by gender. Multivariable logistic regression examined associations of age; gender; race/ethnicity; medical, psychiatric, and neurological comorbidities; and receipt of neurology specialty care associated with the six most commonly used AEDs. RESULTS: Among 256,284 IAVs, 2123 met inclusion criteria (mean age: 33years; 89% men). Seventy-two percent (n=1526) received monotherapy, most commonly valproate (N=425) and levetiracetam (n=347). Sixty-one percent of those on monotherapy received a newer AED (levetiracetam, topiramate, lamotrigine, zonisamide, oxcarbazepine). Although fewer women than men received valproate, nearly 90% (N=45) were of reproductive age (≤45years). Antiepileptic drug prescribing patterns were associated with posttraumatic stress disorder, bipolar disorder, cerebrovascular disease, dementia/cognitive impairment, headache, and receipt of neurological specialty care (all p<0.01). SIGNIFICANCE: In this cohort of veterans with epilepsy, most received AED monotherapy and newer AEDs. Prescribing patterns were different for men and women. The patterns observed between AEDs and neurological/psychiatric comorbidities suggest that clinicians are practicing rational prescribing.

Standards for epidemiologic studies and surveillance of epilepsy.

Worldwide, about 65 million people are estimated to have epilepsy. Epidemiologic studies are necessary to define the full public health burden of epilepsy; to set public health and health care priorities; to provide information needed for prevention, early detection, and treatment; to identify education and service needs; and to promote effective health care and support programs for people with epilepsy. However, different definitions and epidemiologic methods complicate the tasks of these studies and their interpretations and comparisons. The purpose of this document is to promote consistency in definitions and methods in an effort to enhance future population-based epidemiologic studies, facilitate comparison between populations, and encourage the collection of data useful for the promotion of public health. We discuss: (1) conceptual and operational definitions of epilepsy, (2) data resources and recommended data elements, and (3) methods and analyses appropriate for epidemiologic studies or the surveillance of epilepsy. Variations in these are considered, taking into account differing resource availability and needs among countries and differing purposes among studies.

Prevalence of epilepsy and seizures in the Navajo Nation 1998-2002.

PURPOSE: To determine the prevalence of epilepsy and seizures in the Navajo. METHODS: We studied 226,496 Navajo residing in the Navajo Reservation who had at least one medical encounter between October 1, 1998 and September 30, 2002. We ascertained and confirmed cases in two phases. First, we identified patients with International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes signifying epilepsy or seizures using Indian Health Service (IHS) administrative data. Second, we reviewed medical charts of a geographic subpopulation of identified patients to confirm diagnoses and assess the positive predictive value of the ICD-9-CM codes in identifying patients with active epilepsy. RESULTS: Two percent of Navajo receiving IHS care were found to have an ICD-9-CM code consistent with epilepsy or seizures. Based on confirmed cases, the crude prevalence for the occurrence of any seizure (including febrile seizures and recurrent seizures that may have been provoked) in the geographic subpopulation was 13.5 per 1,000 and the crude prevalence of active epilepsy was 9.2 per 1,000. Prevalence was higher among males, children under 5 years of age, and older adults. DISCUSSION: The estimated prevalence of active epilepsy in the Navajo Nation is above the upper limit of the range of reported estimates from other comparable studies of U.S. communities.

Disparities in epilepsy: report of a systematic review by the North American Commission of the International League Against Epilepsy.

PURPOSE: We undertook a systematic review of the evidence on disparities in epilepsy with a focus on North American data (Canada, United States, and the English-speaking Caribbean). METHODS: We identified and evaluated: access to and outcomes following medical and surgical treatment, disability, incidence and prevalence, and knowledge and attitudes. An exhaustive search (1965-2007) was done, including: (1) disparities by socioeconomic status (SES), race/ethnicity, age, or education of subgroups of the epilepsy population; or (2) disparities between people with epilepsy (PWE) and healthy people or with other chronic illnesses. RESULTS: From 1,455 citations, 278 eligible abstracts were identified and 44 articles were reviewed. Comparative research data were scarce in all areas. PWE have been shown to have lower education and employment status; among PWE, differences in access to surgery have been shown by racial/ethnic groups. Aboriginals, women, and children have been shown to differ in use of health resources. Poor compliance has been shown to be associated with lower SES, insufficient insurance, poor relationship with treating clinicians, and not having regular responsibilities. DISCUSSION: Comprehensive, comparative research on all aspects of disparities in epilepsy is needed to understand the causes of disparities and the development of any policies aimed at addressing health disparities and minimizing their impact.

Patients optimizing epilepsy management via an online community: the POEM Study.

OBJECTIVE: The study objective was to test whether engaging in an online patient community improves self-management and self-efficacy in veterans with epilepsy. METHODS: The study primary outcomes were validated questionnaires for self-management (Epilepsy Self-Management Scale [ESMS]) and self-efficacy (Epilepsy Self-Efficacy Scale [ESES]). Results were based on within-subject comparisons of pre- and postintervention survey responses of veterans with epilepsy engaging with the PatientsLikeMe platform for a period of at least 6 weeks. Analyses were based on both completer and intention-to-treat scenarios. RESULTS: Of 249 eligible participants enrolled, 92 individuals completed both surveys. Over 6 weeks, completers improved their epilepsy self-management (ESMS total score from 139.7 to 142.7, p = 0.02) and epilepsy self-efficacy (ESES total score from 244.2 to 254.4, p = 0.02) scores, with greatest impact on an information management subscale (ESMS-information management total score from 20.3 to 22.4, p < 0.001). Results were similar in intention-to-treat analyses. Median number of logins, postings to forums, leaving profile comments, and sending private messages were more common in completers than noncompleters. CONCLUSIONS: An internet-based psychosocial intervention was feasible to implement in the US veteran population and increased epilepsy self-management and self-efficacy scores. The greatest improvement was noted for information management behaviors. Patients with chronic conditions are increasingly encouraged to self-manage their condition, and digital communities have potential advantages, such as convenience, scalability to large populations, and building a community support network. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with epilepsy, engaging in an online patient community improves self-management and self-efficacy.

Epilepsy, sleep disturbances, and psychiatric consequences.

Neurologic impairment after TBI causes serious morbidity for patients and their families. Prior articles have discussed headache and memory impairment. In this article, epilepsy, sleep disturbances, and psychiatric consequences will be covered. People who have suffered traumatic brain injury are at risk for any of these disturbances, and each person will have a constellation of neurologic symptoms spanning the spectrum from no difficulty in any area to symptoms in each of these areas.