Hemiballismus, Hyperphagia, and Behavioral Changes following Subthalamic Infarct

The function of subthalamic nucleus (STN) which is a part of the basal ganglia system is not clear, but it is hypothesized that this component might be involved in action selection. Unilateral damage to STN, which can commonly occur due to the small vessel stroke mainly, causes hemiballismus and sometimes hemichorea-hemiballismus. This paper deals with a 60-year-old patient with sudden onset of abnormal movements in his right limbs. He had increased appetite and hyperphagia and also developed mood and behavioral changes (aggressiveness, irritability, anxiety, and sometimes obscene speech). The magnetic resonance imaging revealed infarct area in left subthalamus. In our case, hemiballismus is caused by infarction in left subthalamic area. Occurrence of irritability, anxiety, and some behavioral changes such as aggressiveness and obscene speech can be explained by impairment of STN role in nonmotor behavior and cognitive function as a result of infarct

Urbach-Wiethe Syndrome and the Ophthalmologist: Review of the Literature and Introduction of the First Instance of Bilateral Uveitis

Patients suffering from Urbach-Wiethe syndrome (UWS), also known as lipoid proteinosis or hyalinosis cutis et mucosae, may have an ophthalmologist involved in the diagnosis and management of their disease. Along with moniliform blepharosis as a pathognomonic feature of the disease, an ophthalmologist may encounter other manifestations of UWS in any part of the eye such as cornea; conjunctiva; sclera; trabecular meshwork; iris/pupil; lens and zonular fibers; retina; nasolacrimal duct. This paper provides a review on the pathogenesis and the diverse ocular manifestations seen in UWS patients. Uncommon complications are discussed in this paper (glaucoma; dry eye and epiphora; complications of lens, retina, cornea; iris/pupil and conjunctiva). Moreover, a 27-year-old male UWS patient is described with bilateral diffuse anterior stromal iris atrophy, diffuse keratic precipitates; posterior subcapsular cataract; 1 + vitreous cell in anterior vitreous examination. This case was thought to be the first instance of bilateral uveitis associated with UWS. Overall, ophthalmologists may encounter diverse ocular complications accompanying this syndrome. They should be familiar with well-established ophthalmologic manifestations leading them to cooperate with other specialists in diagnosis and management of the disease

In Vitro Protective Effects of Aqueous and Alcoholic Extracts of Caryophyllus aromaticus on Reducing H2O2-Induced DNA Damage in Human Mononuclear White Blood Cells

Background and Aim: This study aimed to investigate the protective effects of aqueous and ethanolic extracts of Caryophyllus aromaticus on DNA damage in mononuclear white cells of human blood using the COMET method. Materials and Methods: Mononuclear white blood cells were isolated from blood samples taken from 15 healthy volunteers. Cells were treated with H2O2 (25, 50, 100, and 200 μM), as well as with aqueous and ethanolic extracts of aerial parts of cloves (0.05, 0.1, 0.5, 1, and 2.5 mg/ml). Finally, to induce DNA damage the cells were incubated in a combination of 100 μM H2O2 with each of the two aqueous and alcoholic extracts at a concentration of 2.5 mg/ml at 4°C for 30 minutes. The extent of DNA migration was measured using the alkaline single cell gel electrophoresis approach assay, and DNA damage was expressed as tail length (µm), percentage of tail DNA, and tail moment (µm). Results: DNA damage in the mononuclear white blood cells exposed to the combination of hydrogen peroxide with aqueous extract of clove plant was significantly less than that in the mononuclear white blood cells treated with H2O2 alone. Tail length (µm) was 5.30±1.21 versus 21.84±3.91, percentage of tail DNA (%) was 4.29±1.21 versus 16.21±4.18, and tail movement (µm) was 0.24±0.08 versus 2.35±0.72, respectively (p < 0.001). Conclusion: The results showed that the aqueous extract of clove plant (2.5 mg/ml) can prevent oxidative DNA damage in human mononuclear white blood cells, which is probably due to the presence of antioxidant compounds in the extract

Orbital invasion of ameloblastoma: A systematic review apropos of a rare entity

Purpose: Ameloblastoma is a non-encapsulated and slow-growing tumor with high recurrence rate. Orbital involvement by this neoplasm is an extremely rare entity. In this study, we present a systematic review on this situation along with clinical and paraclinical features of a case. Methods: An electronic search was conducted on major medical sources. Data of the cases in the literature in addition to our own case were extracted, summarized, and statistically analyzed. Results: A total of 36 other cases from 20 relevant studies were also reviewed. Review topics included epidemiology, clinical presentation, pathologic features, differential diagnosis, imaging, treatment, and prognosis. We provided a five-year history of a 50-year-old man with orbital/skull base invasion of plexiform maxillary ameloblastoma. Conclusions: Maxillary ameloblastoma is a locally aggressive neoplasm, and physicians must be alert to the biologic behavior of this tumor to detect any invasion to critical structures such as orbit and cranium. Orbital ameloblastoma causes significant morbidity and mortality. We advocate meticulous patient follow-up with regular clinical examinations and paraclinical work-up for timely detection of any invasion or recurrence. The best must be done to avoid extensions by aggressive removal of maxillary ameloblastoma

Myasthenia Gravis during the Course of Neuromyelitis Optica

Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder of the central nervous system that has been thought to be a severe subtype of multiple sclerosis for a long time. The discovery of aquaporin-4 (AQP4) antibody as a highly specific marker responsible for the pathogenesis of NMO, not only has made a revolutionary pace in establishing a serologic distinction between the two diseases, but it has also classified NMO as an antibody-mediated disorder. Similarly, myasthenia gravis (MG) is a well-known antibody-mediated disorder. In this report, we describe the case of a middle-aged female patient who experienced definite MG with an unclear clinical picture of chronic demyelinating disease that initially reflected the diagnosis of MS, but further imaging and paraclinical workup (e.g. positive AQP4 antibody test) revealed NMO. The coexistence of NMO and MG is previously described. However, this is the first case with NMO symptoms preceding the onset of MG. Of note, the development of MG occurred after a 2-year period of interferon β-1b (IFN β-1b) administration. This calls the question to mind of whether in our case MG is induced by the administration of interferon, instead of an original pathogenic link between MG and NMO. In other words, immunomodulatory treatments can slip the immunity towards T-helper II predominant pathways that can trigger MG. However, if we assume that such an explanation (i.e. increased susceptibility to autoantibody-mediated disorders) is true, our case can be considered the first case of NMO who developed MG following IFN β-1b treatment

25-hydroxyvitamin D Concentrations in Patients with Optic Neuritis as a Clinically Isolated Syndrome and Healthy Controls

Objectives: The onset of multiple sclerosis in the majority of the cases occurs as a clinically isolated syndrome (CIS). We sought to assess serum levels of 25-hydroxyvitamin D (25-OHD) in CIS patients and healthy controls. Methods: In this cross-sectional study 40 patients (36 women and 4 men) with CIS manifesting as a single isolated optic neuritis and 40 Age- and sex-matched healthy controls (35 women and 5 men) were enrolled between late October 2010 and early March 2011. General vitamin D deficiency was defined as serum 25-OHD levels of lower than 20 ng/ml and was classified as mild (15 < 25-OHD <20 ng/ml), moderate (8 < 25-OHD <15 ng/ml), and severe (25-OHD <8 ng/ml). Results: We found no difference in the median interquartile range [IQR] between CIS patients and controls (17.95 [10.40-29.13] vs. 17.00 [12.25-31.00]; P=0.57). However, when stratified by the levels of deficiency, among CIS patients a significantly higher proportion had severe vitamin D deficiency in comparison to healthy controls (20% vs. 2.5%; P=0.034). Nevertheless, the frequency of general (62.5% vs. 60%, P=0.82), mild (25% vs. 30%, P=0.80), and moderate (17.5% vs. 27.5%, P=0.42) vitamin D deficiency were not different between the two groups. Conclusions: Our findings do not indicate any significant difference of serum 25-OHD between CIS patients and healthy controls. However, in our series severe vitamin D deficiency was more frequent among CIS patients

Linkage of Multiple Sclerosis and Guillain-Barre Syndrome: A Population-Based Survey in Isfahan, Iran

Background. Multiple Sclerosis (MS) and Guillain Barre Syndrome (GBS) are autoimmune demyelinating disorders of Central and Peripheral Nervous system, respectively. The coexistence of these two syndromes in an individual's life span is rare. Objectives. To inspect throughout Isfahan MS society (IMSS) records for MS cases who had history of documented GBS whether before the onset of MS or after it. Methods. This retrospective survey was carried out by analyzing the clinical records of 3,522 MS patients who were registered with IMSS, from April 2003 to July 2010. Eligible cases were requested to attend to IMSS for final clinical/paraclinical examinations. Results. Among 3,522 (2,716 women and 806 men) MS subjects, we could identify seven patients (six females and one male) with documented diagnosis of GBS. Six patients (five women and one man) had developed MS within 6.5±7.0 (range: 1–16) years after being diagnosed with GBS and one (a woman) had developed GBS three years after the diagnosis of MS. Conclusion. It seems that the development of MS in individuals with history of GBS is more than a simple incidental event

Global fertility in 204 countries and territories, 1950–2021, with forecasts to 2100: a comprehensive demographic analysis for the Global Burden of Disease Study 2021

BackgroundAccurate assessments of current and future fertility—including overall trends and changing population age structures across countries and regions—are essential to help plan for the profound social, economic, environmental, and geopolitical challenges that these changes will bring. Estimates and projections of fertility are necessary to inform policies involving resource and health-care needs, labour supply, education, gender equality, and family planning and support. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021 produced up-to-date and comprehensive demographic assessments of key fertility indicators at global, regional, and national levels from 1950 to 2021 and forecast fertility metrics to 2100 based on a reference scenario and key policy-dependent alternative scenarios. MethodsTo estimate fertility indicators from 1950 to 2021, mixed-effects regression models and spatiotemporal Gaussian process regression were used to synthesise data from 8709 country-years of vital and sample registrations, 1455 surveys and censuses, and 150 other sources, and to generate age-specific fertility rates (ASFRs) for 5-year age groups from age 10 years to 54 years. ASFRs were summed across age groups to produce estimates of total fertility rate (TFR). Livebirths were calculated by multiplying ASFR and age-specific female population, then summing across ages 10–54 years. To forecast future fertility up to 2100, our Institute for Health Metrics and Evaluation (IHME) forecasting model was based on projections of completed cohort fertility at age 50 years (CCF50; the average number of children born over time to females from a specified birth cohort), which yields more stable and accurate measures of fertility than directly modelling TFR. CCF50 was modelled using an ensemble approach in which three sub-models (with two, three, and four covariates variously consisting of female educational attainment, contraceptive met need, population density in habitable areas, and under-5 mortality) were given equal weights, and analyses were conducted utilising the MR-BRT (meta-regression—Bayesian, regularised, trimmed) tool. To capture time-series trends in CCF50 not explained by these covariates, we used a first-order autoregressive model on the residual term. CCF50 as a proportion of each 5-year ASFR was predicted using a linear mixed-effects model with fixed-effects covariates (female educational attainment and contraceptive met need) and random intercepts for geographical regions. Projected TFRs were then computed for each calendar year as the sum of single-year ASFRs across age groups. The reference forecast is our estimate of the most likely fertility future given the model, past fertility, forecasts of covariates, and historical relationships between covariates and fertility. We additionally produced forecasts for multiple alternative scenarios in each location: the UN Sustainable Development Goal (SDG) for education is achieved by 2030; the contraceptive met need SDG is achieved by 2030; pro-natal policies are enacted to create supportive environments for those who give birth; and the previous three scenarios combined. Uncertainty from past data inputs and model estimation was propagated throughout analyses by taking 1000 draws for past and present fertility estimates and 500 draws for future forecasts from the estimated distribution for each metric, with 95% uncertainty intervals (UIs) given as the 2·5 and 97·5 percentiles of the draws. To evaluate the forecasting performance of our model and others, we computed skill values—a metric assessing gain in forecasting accuracy—by comparing predicted versus observed ASFRs from the past 15 years (2007–21). A positive skill metric indicates that the model being evaluated performs better than the baseline model (here, a simplified model holding 2007 values constant in the future), and a negative metric indicates that the evaluated model performs worse than baseline. FindingsDuring the period from 1950 to 2021, global TFR more than halved, from 4·84 (95% UI 4·63–5·06) to 2·23 (2·09–2·38). Global annual livebirths peaked in 2016 at 142 million (95% UI 137–147), declining to 129 million (121–138) in 2021. Fertility rates declined in all countries and territories since 1950, with TFR remaining above 2·1—canonically considered replacement-level fertility—in 94 (46·1%) countries and territories in 2021. This included 44 of 46 countries in sub-Saharan Africa, which was the super-region with the largest share of livebirths in 2021 (29·2% [28·7–29·6]). 47 countries and territories in which lowest estimated fertility between 1950 and 2021 was below replacement experienced one or more subsequent years with higher fertility; only three of these locations rebounded above replacement levels. Future fertility rates were projected to continue to decline worldwide, reaching a global TFR of 1·83 (1·59–2·08) in 2050 and 1·59 (1·25–1·96) in 2100 under the reference scenario. The number of countries and territories with fertility rates remaining above replacement was forecast to be 49 (24·0%) in 2050 and only six (2·9%) in 2100, with three of these six countries included in the 2021 World Bank-defined low-income group, all located in the GBD super-region of sub-Saharan Africa. The proportion of livebirths occurring in sub-Saharan Africa was forecast to increase to more than half of the world's livebirths in 2100, to 41·3% (39·6–43·1) in 2050 and 54·3% (47·1–59·5) in 2100. The share of livebirths was projected to decline between 2021 and 2100 in most of the six other super-regions—decreasing, for example, in south Asia from 24·8% (23·7–25·8) in 2021 to 16·7% (14·3–19·1) in 2050 and 7·1% (4·4–10·1) in 2100—but was forecast to increase modestly in the north Africa and Middle East and high-income super-regions. Forecast estimates for the alternative combined scenario suggest that meeting SDG targets for education and contraceptive met need, as well as implementing pro-natal policies, would result in global TFRs of 1·65 (1·40–1·92) in 2050 and 1·62 (1·35–1·95) in 2100. The forecasting skill metric values for the IHME model were positive across all age groups, indicating that the model is better than the constant prediction. InterpretationFertility is declining globally, with rates in more than half of all countries and territories in 2021 below replacement level. Trends since 2000 show considerable heterogeneity in the steepness of declines, and only a small number of countries experienced even a slight fertility rebound after their lowest observed rate, with none reaching replacement level. Additionally, the distribution of livebirths across the globe is shifting, with a greater proportion occurring in the lowest-income countries. Future fertility rates will continue to decline worldwide and will remain low even under successful implementation of pro-natal policies. These changes will have far-reaching economic and societal consequences due to ageing populations and declining workforces in higher-income countries, combined with an increasing share of livebirths among the already poorest regions of the world. FundingBill & Melinda Gates Foundation