Addressing the Value of Multidisciplinary Clinical Care in Huntington's Disease: A Snapshot of a New Huntington's Disease Center.

BACKGROUND: Clinical care for Huntington's disease (HD) is often provided in experienced centers that provide multidisciplinary care. However, the value of these centers and their uptake by HD families remain unknown. OBJECTIVE: To describe the services provided by a new HD center, including estimates of capture of the population served. METHODS: Retrospective review of a HD Center launched in 2015, including quantitative and qualitative data on clinic visits, demographic and clinical data. RESULTS: We observed a rapid and ongoing growth on the annual number of clinic encounters, with high demand for in-clinic multidisciplinary care. Using census data and estimates of HD prevalence, we determined that we served about 20% of local patients with HD. Most HD patients received pharmacological treatment for psychiatric symptoms, and over half were treated for chorea. About 25% of new HD diagnoses were on patients without family history of HD. Finally, the demand for predictive testing in at risk individuals significantly increased following the press release reporting the successful completion of the Ionis-HTTRx (RG 6042) trial. CONCLUSIONS: This report indicates a high demand for multidisciplinary care by HD families, supporting its value, providing a snapshot of the organization and function of a single center. Furthermore, it demonstrates how dissemination of news related to research advances influence clinical behavior. Reporting similar information from other HD centers to would provide us with a more global view of the status of HD care across multiple geographical areas.

Cognition, Health, and Well-Being in a Rural Sub-Saharan African Population.

Cognitive health is an important dimension of well-being in older ages, but few studies have investigated the demography of cognitive health in sub-Saharan Africa's (SSA) growing population of mature adults (= persons age 45+). We use data from the Malawi Longitudinal Study of Families and Health (MLSFH) to document the age and gender patterns of cognitive health, the contextual and life-course correlates of poor cognitive health, and the understudied linkages between cognitive and physical/mental well-being. Surprisingly, the age-pattern of decline in cognitive health is broadly similar to that observed in the U.S. We also find that women have substantially worse cognitive health than men, and experience a steeper age-gradient in cognitive ability. Strong social ties and exposure to socially complex environments are associated with higher cognitive health, as is higher socioeconomic status. Poor cognitive health is associated with adverse social and economic well-being outcomes such as less nutrition intake, lower income, and reduced work efforts even in this subsistence agriculture context. Lower levels of cognitive health are also strongly associated with increased levels of depression and anxiety, and are associated with worse physical health measured through both self-reports and physical performance. Our findings suggest that cognition plays a key-but understudied-role in shaping late-life well-being in low-income populations.

Utilization of care among drug resistant epilepsy patients with symptoms of anxiety and depression.

PURPOSE: Epilepsy patients have a significantly higher rate of anxiety and depression than the general population, and psychiatric disease is particularly prevalent among drug resistant epilepsy patients. Symptoms of anxiety and depression might serve as a barrier to appropriate epilepsy care. The aim of this study was to determine if drug resistant epilepsy patients with symptoms of anxiety and/or depression receive different epilepsy management than controls. METHOD: We identified 83 patients with drug resistant focal epilepsy seen at the Penn Epilepsy Center. Upon enrollment, all patients completed 3 self-report scales and a neuropsychiatric inventory and were grouped into those with symptoms of anxiety and/or depression and controls. Each patient's medical records were retrospectively reviewed for 1-2 years, and objective measures of outpatient and inpatient epilepsy management were assessed. RESULTS: At baseline, 53% (n=43) of patients screened positive for symptoms of anxiety and/or depression. The remaining 47% (n=38) served as controls. Patients with anxiety and/or depression symptoms had more missed outpatient visits per year compared to controls (median 0.84 vs. 0.48, p=0.02). Patients with symptoms of both anxiety and depression were more likely to undergo an inpatient admission or procedure (56% vs. 24%, p=0.02). CONCLUSION: For most measures of epilepsy management, symptoms of anxiety and/or depression do not alter epilepsy care; however, drug resistant epilepsy patients with anxiety and/or depression symptoms may be more likely to miss outpatient appointments, and those with the highest burden of psychiatric symptoms may be admitted more frequently for inpatient services compared to controls.

Epilepsy surgery: factors that affect patient decision-making in choosing or deferring a procedure.

Surgical resection for well-selected patients with refractory epilepsy provides seizure freedom approximately two-thirds of the time. Despite this, many good candidates for surgery, after a presurgical workup, ultimately do not consent to a procedure. The reasons why patients decline potentially effective surgery are not completely understood. We explored the socio cultural, medical, personal, and psychological differences between candidates who chose (n = 23) and those who declined surgical intervention (n = 9). We created a novel questionnaire addressing a range of possible factors important in patient decision making. We found that patients who declined surgery were less bothered by their epilepsy (despite comparable severity), more anxious about surgery, and less likely to listen to their doctors (and others) and had more comorbid psychiatric disease. Patients who chose surgery were more embarrassed by their seizures, more interested in being "seizure-free", and less anxious about specific aspects of surgery. Patient attitudes, beliefs, and anxiety serve as barriers to ideal care. These results can provide opportunities for education, treatment, and intervention. Additionally, patients who fit a profile of someone who is likely to defer surgery may not be appropriate for risky and expensive presurgical testing.

Depression in HIV-positive women in Gaborone, Botswana.

This cross-sectional study measured prevalence of depression and suicide ideation in 62 randomly selected HIV-positive (HIV+) women in Botswana, a resource-limited country at the center of the HIV/AIDS epidemic. They were administered two screening measures of depression, an inventory of activities of daily living (ADL), and subjective questionnaire of cognitive functioning. Results show that the two screening measures are useful for detecting depression in women infected with HIV in resource-limited countries. Diagnosis of depression is of great importance, not only clinically, but also to ensure judicious allocation of scarce medical resources in the regions worst affected by the HIV epidemic.

Rapid psychosocial function screening test identified treatment failure in HIV+ African youth.

Psychosocial dysfunction in older children and adolescents is common and may lead to nonadherence to HIV treatments. Poor adherence leads to HIV treatment failure and the development of resistant virus. In resource-limited settings where treatment options are typically limited to only one or two available lines of therapy, identification of individuals at highest risk of failure before failure occurs is of critical importance. Rapid screening tools for psychosocial dysfunction may allow for identification of those children and adolescents who are most likely to benefit from limited psychosocial support services targeted at preventing HIV treatment failure. The Pediatric Symptom Checklist (PSC) is used in high resource settings for rapid identification of at-risk youth. In 692 HIV-infected treated children (ages of 8-< 17 years) in Botswana, having a high score on the PSC was associated with having virologic failure (OR 1.7, 95% CI: 1.1-2.6). The PSC may be a useful screening tool in pediatric HIV.

The King-Devick test and sports-related concussion: study of a rapid visual screening tool in a collegiate cohort.

OBJECTIVE: Concussion, defined as an impulse blow to the head or body resulting in transient neurologic signs or symptoms, has received increasing attention in sports at all levels. The King-Devick (K-D) test is based on the time to perform rapid number naming and captures eye movements and other correlates of suboptimal brain function. In a study of boxers and mixed martial arts (MMA) fighters, the K-D test was shown to have high degrees of test-retest and inter-rater reliability and to be an accurate method for rapidly identifying boxers and mixed martial arts fighters with concussion. We performed a study of the K-D test as a rapid sideline screening tool in collegiate athletes to determine the effect of concussion on K-D scores compared to a pre-season baseline. METHODS: In this longitudinal study, athletes from the University of Pennsylvania varsity football, sprint football, and women's and men's soccer and basketball teams underwent baseline K-D testing prior to the start of the 2010-11 playing season. Post-season testing was also performed. For athletes who had concussions during the season, K-D testing was administered immediately on the sidelines and changes in score from baseline were determined. RESULTS: Among 219 athletes tested at baseline, post-season K-D scores were lower (better) than the best pre-season scores (35.1 vs. 37.9s, P=0.03, Wilcoxon signed-rank test), reflecting mild learning effects in the absence of concussion. For the 10 athletes who had concussions, K-D testing on the sidelines showed significant worsening from baseline (46.9 vs. 37.0s, P=0.009), with all except one athlete demonstrating worsening from baseline (median 5.9s). CONCLUSION: This study of collegiate athletes provides initial evidence in support of the K-D test as a strong candidate rapid sideline visual screening tool for concussion. Data show worsening of scores following concussion, and ongoing follow-up in this study with additional concussion events and different athlete populations will further examine the effectiveness of the K-D test.

Watering flowers in the rain: The elusive nature of executive dysfunction in HIV.

Individuals infected with human immunodeficiency virus (HIV) frequently experience both neurocognitive and psychiatric dysfunction. Apathy is a prominent neuropsychiatric symptom associated with HIV and is related to neurologic dysfunction. In contrast, depression is independent of neurocognitive impairment in HIV. This case report illustrates the importance of behavioral observations from family members of HIV-positive (HIV+) individuals as a valuable source of information. These behavioral observations can be particularly important in rural resource-limited settings, where cognitive testing is often limited to standardized mental status examinations.

Neurobehavioral effects in HIV-positive individuals receiving highly active antiretroviral therapy (HAART) in Gaborone, Botswana.

OBJECTIVE: To explore the prevalence and features of HIV-associated neurocognitive disorders (HANDS) in Botswana, a sub-Saharan country at the center of the HIV epidemic. DESIGN AND METHODS: A cross sectional study of 60 HIV-positive individuals, all receiving highly active antiretroviral therapy (HAART), and 80 demographically matched HIV-seronegative control subjects. We administered a comprehensive neuropsychological test battery and structured psychiatric interview. The lowest 10(th) percentile of results achieved by control subjects was used to define the lower limit of normal performance on cognitive measures. Subjects who scored abnormal on three or more measures were classified as cognitively impaired. To determine the clinical significance of any cognitive impairment, we assessed medication adherence, employment, and independence in activities of daily living (ADL). RESULTS: HIV+ subjects were impaired for all cognitive-motor ability areas compared with matched, uninfected control subjects. Thirty seven percent of HIV+ patients met criteria for cognitive impairment. CONCLUSION: These findings indicate that neurocognitive impairment is likely to be an important feature of HIV infection in resource-limited countries; underscoring the need to develop effective treatments for subjects with, or at risk of developing, cognitive impairment.

Depression among HIV-positive individuals in Botswana: a behavioral surveillance.

This study examined incidence of depression in HIV-positive individuals in Botswana. One hundred and twenty HIV-positive individuals were administered a measure of daily activities and two measures of depression. Twenty four to 38% were diagnosed with depression, suicidal ideation ranged from 9 to 12%, with a positive correlation between scores on the two depression measures. Depression was associated with greater impairment in activities of daily living, especially the ability to take medication. These instruments can diagnose depression in persons living with HIV in developing countries, which will help to target those at risk for poor adherence, and will enable better allocation of limited resources.

Validation of the Pediatric Symptom Checklist in HIV-infected Batswana.

OBJECTIVE: To determine the validity of the Pediatric Symptom Checklist (PSC), a brief measure of psychosocial health, for screening HIV+ Batswana children. METHOD: Setswana versions of the parent and child PSC were administered to 509 HIV+ Batswana children (age 8-16) and their parents/guardians. Test properties were evaluated and cut-off scores were derived using receiver operating characteristic curve analysis. Scores on the parent-completed PSC and the child-completed PSC-Y were compared to parental and clinic staff reports of concern about the child's psychosocial health and to scores on the Children's Depression Inventory and the Revised Children's Manifest Anxiety Scale. RESULTS: The Setswana PSC has high internal consistency (Cronbach's alpha 0.87 for the parent-completed version). Comparing PSC scores to parental reports of concern and child-reported depression symptoms, a cut-off score of 20 on the PSC and PSC-Y maximised the sensitivity and specificity. CONCLUSIONS: The PSC performed well in Setswana-speaking children and is a promising screening tool for paediatric psychosocial problems in busy clinical settings. Screening with the PSC may allow for early detection and treatment of psychosocial problems. This is likely to be of particular value for HIV+ children for whom HIV treatment non-adherence may result from untreated psychosocial dysfunction.

NeuroAIDS in Africa.

In July 2009, the Center for Mental Health Research on AIDS at the National Institute of Mental Health organized and supported the meeting "NeuroAIDS in Africa." This meeting was held in Cape Town, South Africa, and was affiliated with the 5th IAS Conference on HIV Pathogenesis, Treatment and Prevention. Presentations began with an overview of the epidemiology of HIV in sub-Saharan Africa, the molecular epidemiology of HIV, HIV-associated neurocognitive disorders (HANDs), and HAND treatment. These introductory talks were followed by presentations on HAND research and clinical care in Botswana, Cameroon, Ethiopia, The Gambia, Kenya, Malawi, Nigeria, Senegal, South Africa, Uganda, and Zambia. Topics discussed included best practices for assessing neurocognitive disorders, patterns of central nervous system (CNS) involvement in the region, subtype-associated risk for HAND, pediatric HIV assessments and neurodevelopment, HIV-associated CNS opportunistic infections and immune reconstitution syndrome, the evolving changes in treatment implementation, and various opportunities and strategies for NeuroAIDS research and capacity building in the region.

Neurocognitive impairment among HIV-positive individuals in Botswana: a pilot study.

BACKGROUND: The primary objective of this study was to determine the prevalence of neurocognitive impairment among HIV-positive individuals in Botswana, using the International HIV Dementia Scale (IHDS). We also compared performance on the IHDS with performance on tests of verbal learning/memory and processing speed, and investigated the association between performance on the IHDS and such variables as depression, age, level of education and CD4 count. METHODS: We conducted a cross-sectional study of 120 HIV-positive individuals randomly selected from an outpatient HIV clinic in Gaborone, Botswana. Patients provided a detailed clinical history and underwent neuropsychological testing; measures of depression, daily activities and subjective cognitive complaints were recorded. RESULTS: Despite the fact that 97.5% of subjects were receiving highly active antiretroviral therapy (HAART), 38% met criteria for dementia on the IHDS, and 24% were diagnosed with major depressive disorder. There was a significant association between neurocognitive impairment as measured by the IHDS and performance on the other two cognitive measures of verbal learning/memory and processing speed. Level of education significantly affected performance on all three cognitive measures, and age affected processing speed and performance on the IHDS. Depression and current CD4 count did not affect performance on any of the cognitive measures. CONCLUSIONS: The prevalence of neurocognitive impairment in HIV-positive individuals in Botswana is higher than expected, especially since almost all of the subjects in this study were prescribed HAART. This suggests the need to reconsider the timing of introduction of antiretroviral therapy in developing countries where HAART is generally not administered until the CD4 cell count has dropped to 200/mm3 or below. The contribution of other factors should also be considered, such as poor central nervous system penetration of some antiretrovirals, drug resistance, potential neurotoxicity, and co-morbidities. Memory impairment and poor judgment may be underlying causes for behaviours that contribute to the spread of HIV and to poor adherence. It is important to identify these neurobehavioural complications of HIV so that effective treatments can be developed.

Hippocampal volumetry and functional MRI of memory in temporal lobe epilepsy.

This study examined the utility of structural and functional MRI at 1.5 and 3T in the presurgical evaluation and prediction of postsurgical cognitive outcome in temporal lobe epilepsy (TLE). Forty-nine patients undergoing presurgical evaluation for temporal lobe (TL) resection and 25 control subjects were studied. Patients completed standard presurgical evaluations, including the intracarotid amobarbital test (IAT) and neuropsychological testing. During functional imaging, subjects performed a complex visual scene-encoding task. High-resolution structural MRI scans were used to quantify hippocampal volumes. Both structural and functional imaging successfully lateralized the seizure focus and correlated with IAT memory lateralization, with improvement for functional imaging at 3T as compared with 1.5 T. Ipsilateral structural and functional MRI data were related to cognitive outcome, and greater functional asymmetry was related to earlier age at onset. These findings support continued investigation of the utility of MRI and fMRI in the presurgical evaluation of TLE.

The Philadelphia Face Perception Battery.

The Philadelphia Face Perception Battery (PFPB) tests four aspects of face perception: discrimination of facial similarity, attractiveness, gender, and age. Calibration with 116 neurologically intact subjects yielded average performance of approximately 90%. Across subjects, there was a low correlation (<0.22) in performance between the tests (with the exception of the attractiveness and age discrimination tests) suggesting that the tests measure independent aspects of face perception. There were modest effects of subject demographic factors upon performance, and test-retest reliability scores (between 0.37 and 0.75) were comparable to other neuropsychological batteries. Modification of the stimuli to obscure internal facial features lowered performance on the age, gender, and attractiveness discrimination tests between 2 and 4 standard deviations. The clinical sensitivity of the battery was demonstrated by testing a patient with acquired prosopagnosia. She showed performance impairments of between 2 and 4 standard deviations on all sub-tests. The PFPB is freely available for non-commercial use.