A case report of a patient with primary familial brain calcification with a PDGFRB genetic variant.

Fahr's disease, or primary familial brain calcification (PFBC), is a rare genetic neurologic disease characterized by abnormal calcification of the basal ganglia, subcortical white matter and cerebellum. Common clinical features include parkinsonism, neuropsychiatric symptoms, and cognitive decline. Genes implicated in Fahr's disease include PDGFB, PDGFRB, SLC20A2, XPR1, MYORG, and JAM2. We present the case of a 51-year-old woman who developed subacute cognitive and behavioral changes primarily affecting frontal-subcortical pathways and parkinsonism in association with extensive bilateral calcifications within the basal ganglia, subcortical white matter, and cerebellum on neuroimaging. Relevant family history included a paternal aunt with parkinsonism at age 50. Normal parathyroid hormone and calcium levels in the patient's serum ruled out hypoparathyroidism or pseudohypoparathyroidism as causes for the intracranial calcifications. Genetic panel sequencing revealed a variant of unknown significance in the PDGFRB gene resulting in a p.Arg919Gln substitution in the tyrosine kinase domain of PDGFRB protein. To our knowledge this is the first report of a p.Arg919Gln variant in the PDGFRB gene associated with PFBC. Although co-segregation studies were not possible in this family, the location of the variant is within the tyrosine kinase domain of PDGFRB and pathogenicity calculators predict it is likely to be pathogenic. This report adds to the list of genetic variants that warrant functional analysis and could underlie the development of PFBC, which may help to further our understanding of its pathogenesis and the development of targeted therapies for this disorder.

Pain Influences Neuropsychological Performance Following Electrical Injury: A Cross-Sectional Study.

OBJECTIVE: Electrical injury (EI) is a significant, multifaceted trauma often with multi-domain cognitive sequelae, even when the expected current path does not pass through the brain. Chronic pain (CP) research suggests pain may affect cognition directly and indirectly by influencing emotional distress which then impacts cognitive functioning. As chronic pain may be critical to understanding EI-related cognitive difficulties, the aims of the current study were: examine the direct and indirect effects of pain on cognition following EI and compare the relationship between pain and cognition in EI and CP populations. METHOD: This cross-sectional study used data from a clinical sample of 50 patients with EI (84.0% male; Mage = 43.7 years) administered standardized measures of pain (Pain Patient Profile), depression, and neurocognitive functioning. A CP comparison sample of 93 patients was also included. RESULTS: Higher pain levels were associated with poorer attention/processing speed and executive functioning performance among patients with EI. Depression was significantly correlated with pain and mediated the relationship between pain and attention/processing speed in patients with EI. When comparing the patients with EI and CP, the relationship between pain and cognition was similar for both clinical groups. CONCLUSIONS: Findings indicate that pain impacts mood and cognition in patients with EI, and the influence of pain and its effect on cognition should be considered in the assessment and treatment of patients who have experienced an electrical injury.

Kentucky pharmacists' perceptions regarding provision of hormonal contraception.

BACKGROUND: Although Kentucky pharmacists recently gained authority to provide protocol-driven care for 13 conditions, provision of prescription hormonal contraception (HC) services is not currently authorized. A board-approved protocol allowing for provision of nonprescription over-the-counter (OTC) emergency contraception (EC) was recently approved by the Kentucky Board of Pharmacy but has yet to be implemented. OBJECTIVES: The objectives of this study were (1) to assess Kentucky pharmacists' interest in providing prescription HC and OTC EC services via protocol and (2) to identify perceived benefits/barriers regarding provision of prescription HC. METHODS: An online questionnaire was disseminated electronically to a convenience sample of Kentucky pharmacists. The questionnaire collected (1) demographic information, (2) opinions regarding provision of prescription HC and OTC EC, and (3) perceived benefits and barriers regarding provision of prescription HC. For analysis, responses were limited to pharmacists in community-based practice. McNemar's test was used to identify statistically significant differences in support by dosage form. In addition, a multivariable logistic regression model was used to examine associations between demographic factors and support for pharmacist provision of prescription HC. RESULTS: We received 151 responses from community-based pharmacists. Support for provision of prescription HC was highest for oral (61%) and transdermal (54%) forms. We found no statistically significant differences in support among demographic factors other than number of years in practice, with more recent graduates being at higher odds of support. In addition, time, reimbursement, training, and belief in the need for pelvic exams were the most commonly cited barriers to implementation. With regard to OTC EC provision, pharmacists were largely supportive (62%) and confident in their abilities. CONCLUSIONS: Community-based pharmacists in Kentucky are supportive of provision of oral, vaginal, and transdermal prescription HC as well as OTC EC via protocol. Barriers, including time, reimbursement, training, and belief in the need for pelvic exams, should be addressed to increase support for prescription HC provision.

Establishing the base rate of performance invalidity in a clinical electrical injury sample: Implications for neuropsychological test performance.

Objective: The base rate of neuropsychological performance invalidity in electrical injury, a clinically-distinct and frequently compensation-seeking population, is not well established. This study determined the base rate of performance invalidity in a large electrical injury sample, and examined patient characteristics, injury parameters, and neuropsychological test performance based on validity status.Method: This cross-sectional study included data from 101 patients with electrical injury consecutively referred for post-acute neuropsychological evaluation. Eighty-five percent of the sample was compensation-seeking. Multiple performance validity tests (PVTs) were administered as part of standard clinical evaluation. For patients with four or more PVTs, valid performance was operationalized as less than or equal to one PVT failure and invalid performance as two or more failures.Results: Frequency analysis revealed 66% (n = 67) had valid performance while 29% (n = 29) demonstrated probable invalid performance; the remaining 5% (n = 5) had indeterminate validity. No significant differences in demographics or injury parameters emerged between validity groups (0 vs. 1 vs. ≥2 PVT failures). In contrast, the electrical injury group with invalid performance performed significantly worse across tests of processing speed and executive abilities than those with valid performance (ps< .05, ηp2 = .19-.25).Conclusions: The current study is the first to establish the base rate of neuropsychological performance invalidity in electrical injury survivors using empirical methods and current practice standards. Patient and clinical variables, including compensation-seeking status, did not differ between validity groups; however, neuropsychological test performance did, supporting the need for multi-method, objective performance validity assessment.

The relationship between neuropsychological dispersion, processing speed and memory after electrical injury.

Introduction: Previous studies of neuropsychological performance in electrical injury (EI) patients have produced evidence of deficits in various cognitive domains, but studies have yet to investigate relationships among performance in cognitive domains post-EI. This study examined whether dispersion among neuropsychological test scores was associated with injury parameters and neuropsychological performance in EI patients. Additionally, we examined whether dispersion, processing speed and/or executive abilities explain variance in episodic verbal and visual memory performance among EI patients.Method: Data from 52 post-acute EI patients undergoing outpatient evaluation with objectively-verified valid neuropsychological test performance were examined. Tests included measures of verbal and visual memory, processing speed, and executive functioning. Dispersion was calculated from executive functioning and processing speed scores.Results: Dispersion was not related to mean performance or injury characteristics, but was significantly negatively correlated with performance on a test of processing speed, suggesting that increased dispersion is associated with reduced cognitive efficiency post-EI. Delayed visual memory was related to both dispersion scores and processing speed. Stepwise regression equations predicting delayed memory determined that processing speed most significantly predicted delayed visual memory, even after controlling for immediate visual memory. No significant relationships emerged between verbal memory and non-memory neuropsychological scores.Conclusions: This is the first study to examine neuropsychological dispersion and relationships among domains of cognitive functioning in EI. Current results suggested that neuropsychological dispersion is not a marker of general functioning or severity of injury in EI patients, but may represent more specific processing speed abilities. Processing speed predicts delayed visual memory performance in EI patients, which should be considered in interpreting test scores during evaluations.

Development of the Barriers to Error Disclosure Assessment Tool.

OBJECTIVES: An interprofessional group of health colleges' faculty created and piloted the Barriers to Error Disclosure Assessment tool as an instrument to measure barriers to medical error disclosure among health care providers. METHODS: A review of the literature guided the creation of items describing influences on the decision to disclose a medical error. Local and national experts in error disclosure used a modified Delphi process to gain consensus on the items included in the pilot. After receiving university institutional review board approval, researchers distributed the tool to a convenience sample of physicians (n = 19), pharmacists (n = 20), and nurses (n = 20) from an academic medical center. Means and SDs were used to describe the sample. Intraclass correlation coefficients were used to examine test-retest correspondence between the continuous items on the scale. Factor analysis with varimax rotation was used to determine factor loadings and examine internal consistency reliability. Cronbach α coefficients were calculated during initial and subsequent administrations to assess test-retest reliability. RESULTS: After omitting 2 items with intraclass correlation coefficient of less than 0.40, intraclass correlation coefficients ranged from 0.43 to 0.70, indicating fair to good test-retest correspondence between the continuous items on the final draft. Factor analysis revealed the following factors during the initial administration: confidence and knowledge barriers, institutional barriers, psychological barriers, and financial concern barriers to medical error disclosure. α Coefficients of 0.85 to 0.93 at time 1 and 0.82 to 0.95 at time 2 supported test-retest reliability. CONCLUSIONS: The final version of the 31-item tool can be used to measure perceptions about abilities for disclosing, impressions regarding institutional policies and climate, and specific barriers that inhibit disclosure by health care providers. Preliminary evidence supports the tool's validity and reliability for measuring disclosure variables.

Examination of the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF) validity and substantive scales in patients with electrical injury.

Objective: Electrical injury (EI) is a distinct subtype of traumatic injury that often results in a unique constellation of cognitive sequelae and unusual sensory experiences due to peripheral nervous system injury that are uncommon in general medical/neurological populations and have been unexplored with the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF). Method: This study examined performance patterns on MMPI-2-RF validity and substantive scales among 62 EI patients who underwent neuropsychological evaluation, of which 46 demonstrated valid symptom reporting and neurocognitive test performance via multiple independent validity indicators and were retained for analysis. Results: Valid EI patients scored significantly higher than the MMPI-2-RF normative sample on several validity scales with the largest effect sizes on F-r (Infrequent Responses), Fs (Infrequent Somatic Responses), FBS-r (Symptom Validity), and RBS (Response Bias), and ≥33% obtaining elevated scores on these scales per standard interpretive criteria. Review of item content on these scales revealed several reflect disturbances in sensation, physical functioning, and/or cognition that are not infrequent in this population. Further, MMPI-2-RF clinical profiles did not reveal generalized distress or noncredible over-reporting. Rather, similar to the MMPI-2, valid EI patients had a specific pattern related to physical/sensory symptoms and reduced positive emotions with elevations on restructured clinical (RC) scale 1 (somatic complaints), somatic/cognitive specific problem scales, and low positive emotions (RC2). Conclusions: Elevations on some MMPI-2-RF validity scale may capture some degree of actual EI sequela that neuropsychologists need to consider to prevent erroneously concluding that a credible EI patient is over-reporting when s/he is reporting bona fide, EI-related symptoms.

Psychiatric Outcome Over a Decade After Electrical Injury: Depression as a Predictor of Long-Term Adjustment.

Electrical injury (EI) produces a variety of physical, cognitive, and emotional consequences. Psychiatric and neurocognitive symptoms may complicate survivors' psychosocial adjustment and ability to return to work. However, due to a paucity of longitudinal research, the long-term course of EI remains poorly understood. The purpose of this study was to investigate psychiatric and functional status in EI patients over a decade after injury. Fourteen EI patients who originally underwent baseline neuropsychological evaluation participated in this long-term follow-up. Participants completed a telephone survey of functional status, neuropsychological symptom checklist, and the Psychosocial Adjustment to Illness Scale Self-Report. Participants were grouped according to baseline Beck Depression Inventory (BDI) scores. After an average of 12.36 years postinjury, participants with elevated baseline BDI scores experienced difficulty across multiple domains of psychosocial adjustment at follow-up. This group was also less likely to return to work and exhibited a significant increase in psychological distress. EI results in significant chronic psychiatric complaints for many survivors. In the current sample, psychiatric sequelae of EI continue to persist over a decade after injury. Moreover, elevated baseline BDI scores predicted worse outcomes for vocational and psychosocial adjustment. Findings underscore the impact of emotional symptoms on recovery and need for specialized psychiatric intervention immediately following injury.

Direct-to-Consumer Advertising: Implications for the Pharmacist and Other Health Professionals.

Direct-to-consumer advertising of prescription-only medications is big business for pharmaceutical manufacturers and has altered the relationship between patients and health professionals. Seeing promotional messages from the manufacturer of a pharmaceutical can have both positive and negative impacts. These are discussed along with current efforts to control activities in this area as well as possible future developments.

Mood and cognition after electrical injury: a follow-up study.

Individuals who have experienced an electrical injury have been reported to demonstrate both acute and delayed cognitive and psychiatric symptoms. The present study assessed 20 electrically injured patients who underwent neuropsychological evaluations twice following their injury. Time since injury, time between assessments, and longitudinal mood changes were evaluated for their potential impact on simple and complex attention outcomes. As an overall group, there was little change over time from low average to average baseline attention/concentration performance. However, results indicated that longitudinal increases in depressive symptoms were consistently associated with poorer performance on a measure of simple and complex attention. Loss of consciousness, litigation status, baseline injury status (acute vs. post-acute), and time between evaluations were not significant predictors of changes in cognitive performance. Implications for the treatment of comorbid psychiatric issues and for future research on victims of electrical trauma are discussed.

Pharmacists on Facebook: online social networking and the profession.

OBJECTIVE: To provide a brief history of Facebook and online social networking and discuss how it has contributed and can contribute in the future to a paradigm change in social communications. SUMMARY: When student pharmacists complete school and enter practice, they encounter enhanced expectations to act appropriately and professionally. Facebook expands the dilemma of separating private and public life--a challenge for individuals in all professions. From the standpoint of a professional association, Facebook provides a tremendous opportunity to reach out to members in an unprecedented way. Pharmacy organizations are beginning to use these new tools to increase communication and dissemination of information. CONCLUSION: The popularity of Facebook has brought the issue of online social networking to the forefront of professional and organizational discussions. The issues of privacy, identity protection, and e-professionalism are likely to reappear as pharmacists and student pharmacists continue to communicate via online networks. The potential exists for organizations to harness this organizational and communication power for their own interests. Further study is needed regarding the interaction between online social networking applications and the profession of pharmacy.

State boards of pharmacy regulation of the supervision of pharmacist interns.

OBJECTIVES: To compare the regulations of state boards of pharmacy for pharmacist intern supervision and review publications of service-learning experiences in pharmacy curricula for methods of supervision. METHODS: Online state pharmacy statutes and board of pharmacy regulations were searched to characterize which states' regulations included provisions for the supervision of pharmacist interns, permitted nonpharmacist supervision for student volunteers, and included provisions on interns participating in the practice of pharmacy. Additionally, a PubMed search was conducted for articles describing the supervision of service-learning experiences of pharmacy students at various colleges and schools of pharmacy. RESULTS: The state boards of pharmacy in all 51 jurisdictions included regulations for the supervision of pharmacist interns. Regulations specifically permitted only pharmacist supervision of interns in 45 (88%) jurisdictions, and 3 (6%) states included provisions allowing nonpharmacist supervision of pharmacist interns. Provisions allowing nonpharmacist supervision on a case-by-case basis existed in 6 (12%) jurisdictions. Among the 32 identified reports of service-learning experiences offered in pharmacy curricula, 14 contained the words "supervision" or "supervise," and 9 indirectly described methods of student supervision. CONCLUSIONS: State boards of pharmacy regulations largely prohibited nonpharmacist supervision of pharmacy students, and reports of pharmacy student service-learning experiences frequently omitted descriptions of student supervision. Boards of pharmacy should consider revising existing regulations to address the growing need for service-learning in pharmacy curricula.

Legal and ethical issues regarding social media and pharmacy education.

Widespread use of social media applications like Facebook, YouTube, and Twitter has introduced new complexities to the legal and ethical environment of higher education. Social communications have traditionally been considered private; however, now that much of this information is published online to the public, more insight is available to students' attitudes, opinions, and character. Pharmacy educators and administrators may struggle with the myriad of ethical and legal issues pertaining to social media communications and relationships with and among students. This article seeks to clarify some of these issues with a review of the legal facets and pertinent court cases related to social media. In addition, 5 core ethical issues are identified and discussed. The article concludes with recommendations for pharmacy educators with regard to preparing for and addressing potential legal issues pertaining to social media.

Educational innovations: Categories of bulletin board postings designed to increase awareness of contemporary pharmaceutical policy issues.

OBJECTIVE: The goal of this project was to categorize and classify bulletin board postings pertaining to pharmaceutical policy from both the professional and lay press. METHODS: Bulletin board postings were used to supplement in-class discussion to keep students, faculty and staff up-to-date on emerging trends. A bulletin board located in the main classroom area of the College of Pharmacy Building where students would pass by on the way to class and congregate during break periods was used to display articles from various sources concerning topics related to pharmaceutical policy. Information is presented about the primary subject matters addressed in the articles, the types of publications from which they were drawn, and the top ten sources of articles displayed. RESULTS: This project showed that coverage of issues related to pharmacists is predominantly seen in newspapers and most pertinent issues are business related. CONCLUSIONS: It can be seen from this analysis that the issues facing pharmacists are varied. The pharmaceutical policy field is transforming and many of these changes are very relevant to the general population. This is seen from the coverage of all of these issues in the lay press.

Psychiatric morbidity following electrical injury and its effects on cognitive functioning.

OBJECTIVE: This study examines the prevalence of psychiatric morbidity in a large sample of electrical injury (EI) patients in three phases of recovery and its effects on cognitive functioning. METHODS: Eight-six self-referred EI patients received psychiatric and neuropsychological evaluations. Descriptive statistics were conducted to examine the prevalence of psychiatric morbidity. Polytomous logistic regression was used to identify predictors of psychiatric diagnosis. Between-subjects analysis of variances (ANOVA) was conducted to examine the effects of psychiatric morbidity on cognitive functioning. RESULTS: Seventy-eight percent of subjects warranted a psychiatric diagnosis. Long-term patients compared to acute patients were more likely to be diagnosed with two diagnoses than not having any diagnosis (OR=14.30, 95% CI 1.40-38.71). Patients with two diagnoses performed worse than both patients with a single or no diagnosis on all cognitive outcome measures (P<.05). Voltage level, chronic pain and litigation status did not predict psychiatric morbidity. CONCLUSIONS: Psychiatric difficulties commonly emerge and persist following EI. EI patients with psychiatric conditions exhibited poorer cognitive performance as compared to EI patients with no post-injury psychiatric difficulties. Health care professionals need to devote careful attention to psychiatric and cognitive status when treating survivors of EI.

Alteration in functional brain systems after electrical injury.

Neuropsychological studies in electrical injury patients have reported deficits in attention, learning, and working memory, but the neural substrates of these deficits remain poorly characterized. In this study we sought to examine whether electrical injury subjects demonstrate abnormal patterns of brain activation during working memory and procedural learning tasks. Fourteen electrical injury subjects and fifteen demographically matched healthy control subjects performed a spatial working memory paradigm and a procedural learning paradigm during functional MRI studies. For the spatial working memory task, electrical injury patients exhibited significantly greater activation in the middle frontal gyrus and motor and posterior cingulate cortices. Increased activation in EI subjects also was observed on a visually-guided saccade task in several sensorimotor regions, including the frontal and parietal eye fields and striatum. On the procedural learning task, electrical injury patients exhibited significantly less activation in the middle frontal gyrus, anterior cingulate cortex, and frontal eye fields than controls. This is the first study to document task-dependent, system-level cortical and subcortical dysfunction in individuals who had experienced an electrical shock trauma.

Alternative ways to resolve contract disputes: implications for pharmacists, facilities, and patients.

Alternative dispute resolution (ADR) has been developed as an alternative to going to court to resolve disputes. Arbitration and mediation are the two most common forms, with arbitration involving a neutral third party who decides the controversy, the award being either binding or nonbinding on the parties base d on prior agreement. Mediation also involves a neutral third party, but the decision comes from the parties themselves reaching an agreement, with the mediator focused more on the process of securing a meeting of the minds. With the growing prevalence of ADR, pharmacists may encounter provisions in contracts-in either professional or personal life-so it is important that they understand the implications of such provisos. Pharmacists who serve patients in long-term care facilities also should be aware of the increasing use of provisions in patients' contracts with the institution and know how courts view those agreements. ADR is being encountered with increasing frequency, and by being familiar with the two most common approaches, pharmacists can keep abreast of developments and work to avoid pitfalls while adopting these approaches to resolve disputes in appropriate circumstances.