Exploring the role of viscosity-vaso-occlusion and haemolysis-endothelial dysfunction in pain sensitization among Jamaicans with sickle cell disease.

Viscosity-vaso-occlusion (VVO) and haemolysis-endothelial dysfunction (HED) are pathophysiological mechanisms and clinical subphenotypes of sickle cell disease (SCD). Recurrent vaso-occlusive crises (VOC) may lead to neuroplastic changes and pain sensitization. Among 257 SCD participants, we assessed the relationship of subphenotypes with pain sensitivity using quantitative sensory testing to identify heat pain thresholds (HPT) and pressure pain thresholds (PPT). VOC history and sleep, social and emotional functioning were assessed using the Adult Sickle Cell Quality of Life Measurement Information System. The 'elbow method' determined the optimal number of clusters as three. Clustering was performed using K-prototypes. Among clusters 2 and 3, VOC frequency and severity were higher. Clusters 1 and 3 had lower haemoglobin, higher reticulocytes and lactate dehydrogenase and more leg ulcers. In multivariate regression, cluster 3 was associated with approximately 13.6% lower PPT compared to cluster 1, and female sex was associated with decreases in PPT and HPT at the hands and feet (p < 0.001). Hydroxyurea use and unit increases in sleep functioning and age were associated with approximately 20.1% higher foot-PPT, 6.8% higher hand-PPT and 2.5% higher hand-HPT and foot-HPT respectively. Findings suggest that a third subphenotype with mixed VVO and HED features and worse pain sensitization may exist.

Epilepsy care delivery during COVID-19 in resource-limited countries: A survey in collaboration with International Epilepsy Equity Group.

BACKGROUND: The impact of pandemic has had worse effects in countries with already stretched healthcare resources. study's The study aimed to explore changes in epilepsy care delivery in resource-limited countries during and since the acute phase of the COVID-19 pandemic. METHOD: A cross-sectional survey was conducted in 22 countries among healthcare providers (HCPs) caring for persons with epilepsy (PWE), in collaboration with newly formed global collaborators, the International Epilepsy Equity Group. Findings were compared based on the World Bank Ranking (WBR) and HCPs' practice type. Data were analyzed using Chi-square tests (α = 0.05) and pairwise multiple comparisons with α = 0.017 (Bonferroni adjustment). Open-ended responses were analyzed using thematic analysis. FINDINGS: A total of 241 HCPs participated in the study. Of these, 8.30%, 65.98%, and 21.99% were from high-income (HIC), upper-middle-income (UMIC), and lower-middle-income countries (LMICs), respectively. Among HCPs, 31.12% were adult specialists, and 43.98% were pediatric specialists. During the acute phase of the pandemic, HCPs reported that the major barrier for PWE was difficulty reaching physicians/healthcare providers. Except for difficulty reaching physicians/healthcare providers (WBR P = 0.01 HIC < LMIC), no other significant differences in barriers during the acute phase were observed. Since the acute phase of the pandemic, the major concern for PWE was fear of getting infected with the SARS-CoV-2 virus. Significant differences in concerns since the acute phase included lockdowns (WBR: P = 0.03 UMIC < LMIC), fiscal difficulties (WBR: P < 0.001 UMICs < LMICs, UMICs < HIC; practice type: P = 0.006 adult < others, pediatrics < others), clinic closure (WBR: P = 0.003 UMIC < HIC; practice type: P =< 0.001 adult < others, pediatric < others), and long waiting times (WBR: P = 0.005, LMIC < UMIC, LMIC < HIC; practice type: P = 0.006 pediatric < adults). Diagnostic services, including EEG, MRI, CT (practice type: P < 0.001, adult < others; pediatric < others), and lab work (WBR: P = 0.01 UMIC < HIC), were restricted. The telephone was the most reported teleconsultation method used. Except for SMS/texting (WBR P = 0.02 UMIC < LMIC), there were no significant differences in teleconsultation methods used. DISCUSSION: There is a high probability that the initial wave and consequent reduction of in-person care, restriction of health services, and fiscal difficulties affecting all involved in care delivery, led to the disruption of epilepsy care. Additional support are needed in resource-limited countries to cope with future pandemics.

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.

Psychedelics: Alternative and Potential Therapeutic Options for Treating Mood and Anxiety Disorders.

The word "psychedelic" (psyche (i.e., the mind or soul) and delos (i.e., to show)) has Greek origin and was first coined by psychiatrist Humphry Osmond in 1956, who had been conducting research on lysergic acid diethylamide (LSD) at the time. Psychedelic drugs such as N,N-DMT/DMT (N,N-dimethyltryptamine), 5-MeO-DMT (5-methoxy-N,N-dimethyltryptamine), LSD (lysergic acid diethylamide), MDMA (3,4-methylenedioxymethamphetamine) and psilocybin have had significant value as an entheogen in spiritual, religious (shamanic) and sociocultural rituals in Central and South American cultures for thousands of years. In the 1960s, the globalization of these drugs and their subsequent spread outside of their indigenous, old-world cultures, led to the subsequent implementation of strict drug control laws in many Western countries. Even today, psychedelics are still classified as Schedule I drugs, resulting in a still lingering negative stigmatization/perception, vilification, and ultimate criminalization of psychedelics. This controversy still lingers and still limits scientific research and full medical acceptance. For many years up until recently, the spiritual, religious and medicinal value of these drugs could not be explored in a scientific context. More recently, a second wave of psychedelic research is now focusing on psychedelics as neuropharmaceuticals to treat alcohol and tobacco addiction, general mood and anxiety disorders and cancer-related depression. There is now a vast array of promising evidence-based data to confirm the years of anecdotal evidence of the medicinal values of psychedelics. Natural therapeutic alternatives such as psychedelic drugs may provide a safe and efficacious alternate to conventional drugs used to treat mood and anxiety disorders. In a Western context in particular, psychedelic drugs as therapeutic agents for mood and anxiety disorders are becoming increasingly of interest amidst increasing rates of such disorders globally, changing social constructions, the implementation of government regulations and increasing investment opportunities, that ultimately allow for the scientific study to generate evidenced-based data. Alternative psychotherapeutic interventions are gaining interest also, because of their low physiological toxicity, relatively low abuse potential, safe psychological effects, and no associated persisting adverse physiological or psychological effects during and after use. On the other hand, conventional psychotic drugs and anti-depressants are becoming less favorable because of their adverse side effects. Psychedelic neuropharmaceutical interventions may with medical oversight be the solution to conventional psychiatric disorders such as depression and anxiety, and an alternative to conventional psychiatric treatment options. This paper will review the therapeutic potential of psychedelic drugs as alternative therapeutic options for mood and anxiety disorders in a controlled, clinical setting, where the chances of adverse psychological episodes occurring are mitigated.

The Therapeutic Potential of Psilocybin.

The psychedelic effects of some plants and fungi have been known and deliberately exploited by humans for thousands of years. Fungi, particularly mushrooms, are the principal source of naturally occurring psychedelics. The mushroom extract, psilocybin has historically been used as a psychedelic agent for religious and spiritual ceremonies, as well as a therapeutic option for neuropsychiatric conditions. Psychedelic use was largely associated with the "hippie" counterculture movement, which, in turn, resulted in a growing, and still lingering, negative stigmatization for psychedelics. As a result, in 1970, the U.S. government rescheduled psychedelics as Schedule 1 drugs, ultimately ending scientific research on psychedelics. This prohibition on psychedelic drug research significantly delayed advances in medical knowledge on the therapeutic uses of agents such as psilocybin. A 2004 pilot study from the University of California, Los Angeles, exploring the potential of psilocybin treatment in patients with advanced-stage cancer managed to reignite interest and significantly renewed efforts in psilocybin research, heralding a new age in exploration for psychedelic therapy. Since then, significant advances have been made in characterizing the chemical properties of psilocybin as well as its therapeutic uses. This review will explore the potential of psilocybin in the treatment of neuropsychiatry-related conditions, examining recent advances as well as current research. This is not a systematic review.

Interventions for treating neuropathic pain in people with sickle cell disease.

BACKGROUND: Pain is the hallmark of sickle cell disease (SCD) and it can be severe, frequent and unpredictable. Although nociceptive pain is more common, at times, people with SCD may have neuropathic pain. The latter can occur due to peripheral or central nerve injury. This review is focused on identifying treatment of only painful sensory neuropathy in people with SCD. OBJECTIVES: To determine the effectiveness and safety of any pharmacological or non-pharmacological therapies for treating neuropathic pain in people with SCD. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched trial registries, the reference lists of relevant articles and reviews and contacted experts in the field.Date of last search: 31 January 2019. SELECTION CRITERIA: Randomised controlled trials (RCTs) (parallel or cross-over in design), quasi-RCTs of pharmacological or non-pharmacological therapies for treating neuropathic pain in people with SCD compared to placebo or another intervention in any category (i.e. pharmacological or non-pharmacological). DATA COLLECTION AND ANALYSIS: Two review authors independently assessed all trials identified by the searches and extracted relevant data. Two authors independently assessed the risk of bias in the selected trials using the Cochrane risk of bias tool. Two review authors independently rated the quality of the evidence for each outcome using the GRADE guidelines. MAIN RESULTS: One RCT of 22 participants with SCD, conducted in the USA was included in this review. Participants were randomly assigned to either pregabalin (n = 11) or placebo (n = 11). Oral pregabalin was administered at an initial dose of 75 mg twice daily. The drug was titrated at increments of 75 mg to a maximum of 600 mg daily or decreased by 75 mg per day if necessary, based on clinical presentation and pain level. Neuropathic pain was assessed using self-reports on the Leeds Assessment of Neuropathic Symptoms and Signs (S-LANNS) scale and the Neuropathic Pain Symptom Inventory (NPSI), where higher scores were indicative of more pain. Outcomes included self-reported pain, quality of life and withdrawal due to adverse effects measured at baseline and monthly for three months post-intervention. The overall risk of bias was low with a high risk of bias due to attrition.In relation to this reviews primary outcomes, for self-reported neuropathic pain relief, given the paucity of data, we are very uncertain whether there is a difference between the pregabalin and placebo groups at the end of three months as measured by the S-LANSS scale, mean difference (MD) -2.00 (95% confidence interval (CI) -9.18 to 5.18), or the NPSI scale, MD -11.10 (95% CI -33.97 to 11.77) (very low-quality evidence). There was no report of 'Patient Global Impression of Change' in the included trial.Although the mean quality of life scores (Short Form-36) at three months showed small increases in seven of the eight domains post-intervention in the pregabalin group as compared to the placebo group, this was very low-quality evidence and we are very uncertain whether pregabalin increases quality of life. Neither of our pre-defined outcomes of 'time to improvement of symptoms' or 'changes in sleep quality', were measured in the included trial.While treatment-related adverse effects appeared higher in pregabalin group than the placebo group at three months, this was very low-quality evidence and we are very uncertain whether there is a difference, RR 1.33 (95% CI 0.39 to 4.62) (very low-quality evidence). There was one withdrawal for adverse effects in the pregabalin group while three people withdrew or dropped out from the placebo group due to adverse effects and complications and hospitalisation related to SCD. AUTHORS' CONCLUSIONS: The included trial provided very low-quality evidence. Self-reported pain relief was greater in the pregabalin group compared to the placebo control group but only using the S-LANSS scale and we are very unsure whether there is a difference. While the pregabalin group tended to have improved quality of life over the duration of the trial, this was very low-quality evidence and we are uncertain whether there is a difference. Adverse effects and withdrawals were similar across the treatment and placebo control group in trial. There are both insufficient trials addressing this review question and insufficient outcomes addressed in the single included RCT. Therefore, there is still a significant gap in evidence on interventions for neuropathic pain in people with SCD.

Global Health: Epilepsy.

Epilepsy is a frequently misunderstood and highly stigmatized condition. Major treatment gaps exist across the world, most so in areas of financial constraint. Classification permits the best approaches to treatment and to ascertaining prognosis. The International League Against Epilepsy's new classification system emphasizes clinical aspects and utilizes all available resources to determine whether it is a focal or generalized epilepsy. The most important tools are a careful history, clinical examination, electroencephalography, and appropriate neuroimaging. Inadequate, delayed, and incomplete evaluation may lead to misdiagnosis and costly mismanagement. Treatment is generally pharmacological, with approximately 20 to 30% of patients eventually proving refractory to medications and thus becoming potential surgical candidates. The type of epilepsy, age, gender, comorbidities, drug interactions, and drug cost are important factors in choosing an antiepileptic drug (AED). The teratogenic potential of some AEDs, weight gain, and menstrual hormone-related issues are important considerations in women. The impact of AEDs on bone health is critical in all age groups, particularly in the elderly. Psychiatric problems, mostly depression and anxiety, can have a great impact on seizure control and overall quality of life. Finally, effective partnerships and collaborations can bring resources, both human and financial, to regions that would otherwise find it impossible to effect change on their own.

Epilepsy care in the southern Caribbean.

Very little has been reported about the health resources available for patients with epilepsy in the five English-speaking southern Caribbean countries of Trinidad and Tobago, Barbados, Grenada, Saint Vincent and the Grenadines, and Saint Lucia. There is no comprehensive resource describing their health systems, access to specialty care, antiepileptic drug (AED) use, and availability of brain imaging and EEG. The purpose of this study was to profile epilepsy care in these countries as an initial step toward improving the standard of care and identifying gaps in care to guide future policy changes. In each southern Caribbean country, we conducted study visits and interviewed health-care providers, government health ministers, pharmacy directors, hospital medical directors, pharmacists, clinic staff, radiologists, and radiology and EEG technicians. Health-care providers completed extensive epilepsy care surveys. The five countries all have integrated government health systems with clinics and hospitals that provide free or heavily subsidized care and AEDs for patients with epilepsy. Only Trinidad and Tobago and Barbados, however, have neurology specialists. The three smaller countries lack government imaging and EEG facilities. Trinidad had up to one-year waits for public MRI/EEG. Government formularies in Grenada, Saint Vincent and the Grenadines, and Saint Lucia are limited to first-generation AEDs. One or more second-line agents are formulary in Trinidad and Barbados. Nonformulary drugs may be obtained for individual patients in Barbados. Grenada, Saint Lucia, and Saint Vincent and the Grenadines participate in an Organization of Eastern Caribbean States formulary purchasing system, which added levetiracetam following the survey. Newer generic AED formulations with the lowest risks for pregnancy malformation were not in use. In conclusion, patients with epilepsy in the southern Caribbean have excellent access to government clinics and hospitals, but AED choices are limited. Local medical providers reported that the major limitations in care were lack of specialty care, lack of imaging and EEG services, financial barriers to care, long wait times for care, and limited access to additional AEDs.

Digital measures in epilepsy in low-resourced environments.

INTRODUCTION: Digital measures and digital health-care delivery have been rarely implemented in lower-and-middle-income countries (LMICs), contributing to worsening global disparities and inequities. Sustainable ways to implement and use digital approaches will help to improve time to access, management, and quality of life in persons with epilepsy, goals that remain unreachable in under-resourced communities. As under-resourced environments differ in human and economic resources, no one approach will be appropriate to all LMICs. AREAS COVERED: Digital health and tools to monitor and measure digital endpoints and metrics of quality of life will need to be developed or adapted to the specific needs of under-resourced areas. Portable technologies may partially address the urban-rural divide. Careful delineation of stakeholders and their engagement and alignment in all efforts is critically important if these initiatives are to be successfully sustained. Privacy issues, neglected in many regions globally, must be purposefully addressed. EXPERT OPINION: Epilepsy care in under-resourced environments has been limited by the lack of relevant technologies for diagnosis and treatment. Digital biomarkers, and investigative technological advances, may finally make it feasible to sustainably improve care delivery and ultimately quality of life including personalized epilepsy care.

Telemedicine for Individuals with epilepsy: Recommendations from the International League Against Epilepsy Telemedicine Task Force.

Worldwide, People with Epilepsy (PWE) are confronted with several barriers to face-to-face consultations. These obstacles hamper appropriate clinical follow-up and also increase the treatment gap for Epilepsy. Telemedicine holds the potential to enhance management as follow-up visits for PWE are focused on more on clinical history and counselling rather than physical examination. Besides consultation, telemedicine can also be used for remote EEG diagnostics and tele-neuropsychology assessments. In this article, the Telemedicine Task Force of the International League Against Epilepsy (ILAE) outlines recommendations regarding optimal practice in utilizing in the management of individuals with epilepsy. We formulated recommendations for minimum technical requirements, preparing for the first tele-consultation and the specificities for follow-up consultations. Special considerations are necessary for specific populations, including paediatric patients, patients who are not conversant with tele-medicine and those with intellectual disability. Telemedicine for individuals with epilepsy should be vigorously promoted with the aim of improving the quality of care and ultimately reduce the wide clinician access related treatment gap across several regions of the globe.

Epilepsy awareness in a Jamaican community: driven to change!

There are relatively few published studies on epilepsy-related knowledge, attitudes, and practices (KAP) from developing countries and none from Jamaica. A questionnaire-based, cross-sectional study of 320 individuals was performed in a small community in Kingston. Residents and employees were comparable in age, sex, and personal and family history of epilepsy, but differed in attained education and occupation. Persons with postsecondary education were less likely to believe that epilepsy is a mental disorder (9% vs 24.8%, P<0.001), is due to demonic possession (8% vs 18.2%, P<0.01), or is contagious (2.5% vs 23%, P<0.001). Overall, 73% felt that people with epilepsy should not drive. The results of this Jamaican KAP study differ from those in other developing countries. There appears to be less societal stigma in Jamaica; however, there is widespread reluctance to allow PWE to drive. This represents a substantial challenge to the current initiative to change existing driving regulations that currently bar people with epilepsy from driving.

Delivering epilepsy care in low-resource settings: the role of technology.

INTRODUCTION: The implementation of technology in the field of epileptology has traditionally focused on its use for diagnosis and treatment and has, unsurprisingly, been capital-intensive, making it therefore mainly implementable in advanced high-income countries. Because of technological innovations over the past 20 years there has been almost a paradigm shift, particularly in access to and the potential for implementing relevant technology in lesser developed environments. Nearly 80% of people living with epilepsy live in low and middle-income countries. AREAS COVERED: The challenge and the purpose of this paper is to discuss how technology can be implemented into lesser-resourced contexts not only cost-effectively but in a cost-saving way while also building capacity and thus sustainability. EXPERT OPINION: The rate of technological advancement presents the risk of progressive widening of the technology and care gaps between advanced and lesser developed regions. Implementing technology is both about finding relevant appropriate technologies for the individual contexts of a diverse range of countries but also about repurposing low-tech technologies for application in epilepsy care in these areas. Finally exciting advances such as autonomous driving, digital twinning and robotic surgery will likely transform epilepsy care in several lower-resourced settings in the next 5-10 years.

Sickle Cell Disease and Pain: Is it all Vaso-occlusive Crises?

OBJECTIVES: Acute pain is the main complication of sickle cell disease. Chronic pain (CP) and neuropathic pain (NP) may also be experienced, but have not been formally described in Jamaican patients. A cross-sectional study was conducted to determine their prevalence and characteristics, and to determine the common pain locations and modalities of management. MATERIALS AND METHODS: All well individuals with sickle cell disease patients 14 years and older, not pregnant and without a history of clinical stroke were consecutively recruited. Anthropometric measurements, hematology studies, an analgesia checklist, and the Adult Sickle Cell Quality of Life Measurement Information System questionnaire were completed. The painDETECT questionnaire was completed to describe NP and pain patterns-from which CP was defined. RESULTS: There were 257 patients in total, with 55.6% being females; the mean age of the patients was 31.7±12 years, and 75% had the SS genotype. Almost all patients (92.6%) had had an acute pain crisis in their lifetime and 72.4% in the last year. The mean severity at last attack was 6.8±3.1 on a scale of 0 to 10. The prevalences of CP and NP were 21.5% and 17.9%, respectively. Female sex, the presence of current leg ulcers, and the use of a strong opioid in the last 4 weeks produced higher odds of NP, whereas older age, milder genotypes, and daily analgesic use had the highest odds of CP. Opioids were used by 40.1% of the patients in the previous 4 weeks, whereas nonpharmacological treatments such as physiotherapy was less used, but reported to be very effective. DISCUSSION: CP and NP should be assessed during routine care of sickle cell pain so that targeted therapies can be applied.

Health-related quality of life and neuropathic pain in sickle cell disease in Jamaica.

BACKGROUND: Health related quality of Life (HRQOL) is an important consideration when managing chronic diseases, like sickle cell disease (SCD). Assessment of neuropathic pain (NP) and its association with HRQOL in SCD are rarely reported. OBJECTIVES: To examine the prevalence of NP and its association with HRQOL in adult Jamaicans with SCD. METHODS: Adult SCD patients were recruited consecutively and data were collected on socio-demographics, NP using Douleur Neuropathique 4 (DN4), and HRQOL using the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me). Means, medians, t-tests, ANOVA tests, Wilcoxon Rank-sum tests, Kruskal-Wallis tests, Pearson's correlation and multivariate linear regression analyses were performed using STATA 14.2. RESULTS: There were 236 respondents, with 56.8% female, mean age 33.2 years (SD: 11.6; range: 18-67 years), and 75% had homozygous SS genotype. NP was likely present in 26.7% of the population. The standardized ASCQ-Me (mean ± SD; ordered from lowest to best HRQOL domain scores) were: emotional impact 53.3 ± 10.1; sleep impact 56.1 ± 9.7; social function 57.7 ± 10.6; pain impact 58.6 ± 7.8; and stiffness impact 61.0 ± 7.3. On multivariate analyses, NP significantly reduced emotional and social functioning and worsened sleep and stiffness. Higher acute pain scores significantly worsened all HRQOL domains, while higher disease severity worsened all except stiffness. Obesity was associated with worse sleep and greater stiffness. Females with leg ulcers reported lower social functioning and unemployed females had greater pain impact. CONCLUSIONS: NP is increasingly prevalent in SCD and worsens HRQOL. Gender specific studies are needed to understand the significantly poorer HRQOL in women.

Seizures in the Jamaica cohort study of sickle cell disease.

Although there is some evidence that epilepsy is more common in Sickle Cell Disease (SCD), we sought to establish the incidence rates, risk factors for and specific types of seizures in a SCD cohort followed from birth, and how seizure occurrence affects morbidity and mortality. We examined all records of persons in the Jamaica cohort Study of Sickle Cell Disease (JSSCD) clinically identified as having experienced a seizure during their lifetime. At first presentation, seizures were classified as Febrile Convulsion, Acute Symptomatic Seizure or Single Unprovoked Seizure. The seizure classification was revised to include Epilepsy if seizures recurred. Thirty-eight persons in the JSSCD (N = 543) were identified with seizures. The 5-year cumulative incidence of febrile convulsions was 2·2%. The incidence rate of epilepsy (all genotypes) was 100/100 000 person-years, 139/100 000 for the SS genotype. Despite limited availability of diagnostic investigations, clinical seizures were associated with increased all-cause mortality. Male gender (Odds Ratio [OR]: 4·0[95% confidence interval [CI]; 1·03-20·0]) and dactylitis in childhood (OR: 17·4 [95% CI; 4·82-62·85]) were associated with increased risk of developing epilepsy. Epilepsy in persons with SCD is 2-3 times more common than in non-sickle populations and is associated with increased all-cause mortality in all sickle cell genotypes.

Aquaporin-4 immuneglobulin g testing in 36 consecutive jamaican patients with inflammatory central nervous system demyelinating disease.

Epidemiological studies of neuromyelitis optica (NMO) in Jamaica are lacking. Here we reviewed the clinical records of 700 patients undergoing neurological evaluation at the Kingston Public Hospital, the largest tertiary institution in Jamaica over a 4 month period. We investigated the diagnostic utility of Aquaporin-4 ImmuneglobulinG (AQP4-IgG) testing in 36 consecutive patients with a diagnosis of an inflammatory demyelinating disorder (IDD) of the central nervous system (CNS). Patients were classified into 3 categories: i) NMO, n=10; ii) multiple sclerosis (MS), n=14 and iii) unclassified IDD (n=12). All sera were tested for AQP-IgG status by cell binding assay (Euroimmun). No MS cases were positive. Ninety per cent of NMO cases were positive. Four of 12 patients with unclassified IDD tested positive for AQP4-IgG. AQP4-IgG seropositivity was associated with a lower socioeconomic status, higher EDSS (P=0.04) and lower pulmonary function than the seronegative cases (P=0.007). Aquaporin-4 autoimmunity may account for a significant proportion of Jamaican CNS IDDs.

Chronic inflammatory demyelinating polyneuropathy in a patient infected with human T lymphotropic virus type I.

A 32-year-old Afro-Caribbean woman presented with a 1-year history of slowly progressive sensory and motor symptoms initially affecting the legs and later involving the arms. Clinical examination demonstrated a mainly distal pattern of weakness with little objective sensory impairment. The clinical features suggested the possibility of chronic inflammatory demyelinating polyneuropathy. This diagnosis was supported by neurophysiological testing and examination of the cerebrospinal fluid and confirmed by sural nerve biopsy as well as by exclusion of other causes of neuropathy. Seropositivity for human T lymphotropic virus type I (HTLV-I) was demonstrated. The clinical significance of this finding in an area with a high HTLV-I endemicity, as well as its possible aetiological relevance to the diagnosis of chronic inflammatory demyelinating polyneuropathy, is discussed.