Sample records for huntington beach ca

  1. 75 FR 11939 - Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation (United States)


    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-71,749] Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation Pursuant to Section 221 of the Trade Act of 1974, as amended, an investigation was initiated in response to a petition filed on July 21...

  2. Swash zone characteristics at Ocean Beach, San Francisco, CA (United States)

    Erikson, L.H.; Hanes, D.M.; Barnard, P.L.; Gibbs, A.E.


    Runup data collected during the summer of 2005 at Ocean Beach, San Francisco, CA are analyzed and considered to be typical summer swash characteristics at this site. Analysis shows that the beach was dissipative with Iribarren numbers between 0.05 and 0.4 and that infragravity energy dominated. Foreshore slopes were mild between 0.01 and 0.05 with swash periods on the order of a minute. Predicted runup heights obtained with six previously developed analytical runup formulae were compared to measured extreme runup statistics. Formulations dependent on offshore wave height, foreshore slope and deep water wavelength gave reasonable results.

  3. 78 FR 39599 - Safety Zone; Independence Day Fireworks, Kings Beach, CA (United States)


    ... Zone; Independence Day Fireworks, Kings Beach, CA AGENCY: Coast Guard, DHS. ACTION: Notice of... Fireworks, Kings Beach, CA in the Captain of the Port, San Francisco area of responsibility during the dates... from the hazards associated with the fireworks display. During the enforcement period, unauthorized...

  4. 77 FR 38005 - Safety Zone; Independence Day Fireworks, Kings Beach, CA (United States)


    ... Zone; Independence Day Fireworks, Kings Beach, CA AGENCY: Coast Guard, DHS. ACTION: Notice of... Independence Day Fireworks display from 7 a.m. until 10 p.m. on July 3, 2012. This action is necessary to protect life and property of the maritime public from the hazards associated with the fireworks display...

  5. Currents, temperature, conductivity, attenuation, and sigma-theta data from moorings deployed off the coast of Orange County, CA from platforms ROBERT GORDON SPROUL and YELLOWFIN from June 13, 2001 to January 22, 2003 (NODC Accession 0067572) (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — A large number of moorings were deployed in the summer of 2001 off Huntington Beach CA to monitor coastal ocean process that transport suspended material across the...

  6. Achieving Energy Savings in Municipal Construction in Long Beach, CA

    Energy Technology Data Exchange (ETDEWEB)

    Parrish, Kristen [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Regnier, Cindy [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)


    Long Beach Gas and Oil (LBGO), the public gas utility in Long Beach, California, partnered with the U.S. Department of Energy (DOE) to develop and implement solutions to build a new, low-energy modular office building that is at least 50% below requirements set by Energy Standard 90.1-2007 of the American Society of Heating, Refrigerating, and Air-conditioning Engineers (ASHRAE), the American National Standards Institute (ANSI), and the Illuminating Engineering Society of America (IESNA) as part of DOE’s Commercial Building Partnerships (CBP) program.3 The LBGO building, which demonstrates that modular construction can be very energy efficient, is expected to exceed the ASHRAE baseline by about 45%. The new 15,000-square foot (ft2) LBGO office building has two stories and houses private offices, open-plan cubicle offices, and a conference room and call center on the second floor. The building’s modular nature allowed LBGO to realize the cost benefits of fasttracked construction while saving substantial energy and reducing operational costs. The project was funded by the utility’s ratepayer revenue, which imposed a tight budget limit. The design process was a collaborative effort involving LBGO and its design-build team, Lawrence Berkeley National Laboratory (Berkeley Lab), and subcontractors Stantec (formerly Burt Hill) and LHB Inc. The team proposed efficiency measures based on computer modeling of the building in full compliance with ASHRAE 90.1-2007; in the modeled building, the lighting and cooling systems were the largest energy users, so increasing the efficiency of these systems was a top priority. Promising measures were modeled to estimate their energy performance, and each measure was evaluated for its feasibility within the budget.

  7. Coupling alongshore variations in wave energy to beach morphologic change using the SWAN wave model at Ocean Beach, San Francisco, CA (United States)

    Eshleman, Jodi L.; Barnard, Patrick L.; Erikson, Li H.; Hanes, Daniel M.


    Coastal managers have faced increasing pressure to manage their resources wisely over the last century as a result of heightened development and changing environmental forcing. It is crucial to understand seasonal changes in beach volume and shape in order to identify areas vulnerable to accelerated erosion. Shepard (1950) was among the first to quantify seasonal beach cycles. Sonu and Van Beek (1971) and Wright et al. (1985) described commonly occurring beach states. Most studies utilize widest spaced 2-D cross shore profiles or shorelines extracted from aerial photographs (e.g. Winant et al. 1975; Aubrey, 1979, Aubrey and Ross, 1985; Larson and Kraus, 1994; Jimenez et al., 1977; Lacey and Peck, 1998; Guillen et al., 1999; Norcorss et al., 2002) to analyzed systematic changes in beach evolution. But with the exception of established field stations, such as Duck, NC (Birkemeier and Mason, 1984), ans Hazaki Oceanographical Research Station (HORS) in Japan (Katoh, 1997), there are very few beach change data sets with high temporal and spatial resolutions (e.g. Dail et al., 2000; Ruggiero et al., 2005; Yates et al., in press). Comprehensive sets of nearshore morphological data and local in situ measurements outside of these field stations are very rare and virtually non-existent high-energy coasts. Studied that have attempted to relate wave statistics to beach morphology change require some knowledge of the nearshore wave climate, and have had limited success using offshore measurement (Sonu and Van Beek, 1971; Dail et al., 2000). The primary objective of this study is to qualitatively compare spatially variable nearshore wave predictions to beach change measurements in order to understand the processes responsible for a persistent erosion 'hotspot' at Ocean Beach, San Francisco, CA. Local wave measurements are used to calibrate and validate a wave model that provides nearshore wave prediction along the beach. The model is run for thousands of binned offshore wave

  8. Coastal processes study at Ocean Beach, San Francisco, CA: summary of data collection 2004-2006 (United States)

    Barnard, Patrick L.; Eshleman, Jodi; Erikson, Li H.; Hanes, Daniel M.


    Ocean Beach in San Francisco, California, contains a persistent erosional section in the shadow of the San Francisco ebb tidal delta and south of Sloat Boulevard that threatens valuable public infrastructure as well as the safe recreational use of the beach. Coastal managers have been discussing potential mediation measures for over a decade, with little scientific research available to aid in decision making. The United States Geological Survey (USGS) initiated the Ocean Beach Coastal Processes Study in April 2004 to provide the scientific knowledge necessary for coastal managers to make informed management decisions. This study integrates a wide range of field data collection and numerical modeling techniques to document nearshore sediment transport processes at the mouth of San Francisco Bay, with emphasis on how these processes relate to erosion at Ocean Beach. The Ocean Beach Coastal Processes Study is the first comprehensive study of coastal processes at the mouth of San Francisco Bay.

  9. Oxidative metabolism and Ca2+ handling in isolated brain mitochondria and striatal neurons from R6/2 mice, a model of Huntington's disease. (United States)

    Hamilton, James; Pellman, Jessica J; Brustovetsky, Tatiana; Harris, Robert A; Brustovetsky, Nickolay


    Alterations in oxidative metabolism and defects in mitochondrial Ca 2+ handling have been implicated in the pathology of Huntington's disease (HD), but existing data are contradictory. We investigated the effect of human mHtt fragments on oxidative metabolism and Ca 2+ handling in isolated brain mitochondria and cultured striatal neurons from the R6/2 mouse model of HD. Non-synaptic and synaptic mitochondria isolated from the brains of R6/2 mice had similar respiratory rates and Ca 2+ uptake capacity compared with mitochondria from wild-type (WT) mice. Respiratory activity of cultured striatal neurons measured with Seahorse XF24 flux analyzer revealed unaltered cellular respiration in neurons derived from R6/2 mice compared with neurons from WT animals. Consistent with the lack of respiratory dysfunction, ATP content of cultured striatal neurons from R6/2 and WT mice was similar. Mitochondrial Ca 2+ accumulation was also evaluated in cultured striatal neurons from R6/2 and WT animals. Our data obtained with striatal neurons derived from R6/2 and WT mice show that both glutamate-induced increases in cytosolic Ca 2+ and subsequent carbonilcyanide p-triflouromethoxyphenylhydrazone-induced increases in cytosolic Ca 2+ were similar between WT and R6/2, suggesting that mitochondria in neurons derived from both types of animals accumulated comparable amounts of Ca 2+ Overall, our data argue against respiratory deficiency and impaired Ca 2+ handling induced by human mHtt fragments in both isolated brain mitochondria and cultured striatal neurons from transgenic R6/2 mice. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail:

  10. Huntington's disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth; Law, Ian; Jønch, Aia


    In this open-label pilot study, the authors evaluated the effect of memantine on the distribution of brain glucose metabolism in four Huntington's disease (HD) patients as determined by serial 18-fluoro-deoxyglucose [F(18)]FDG-PET scans over a period of 3-4 months (90-129 days, with one patient...

  11. Can QMRA be used to Discount Pathogen Risk to Swimmers from Animal Fecal Contamination? Doheny Beach, CA Case Study (United States)

    Estimated health risks to swimmers from seagull and bather sources of fecal contamination at Doheny Beach, California were compared using quantitative microbial risk assessment (QMRA) with a view to aiding beach closure decisions. Surfzone pathogens from seagulls were thought to...

  12. Psychopathology in Huntington's disease

    NARCIS (Netherlands)

    Duijn, Erik van


    Dit proefschrift begint met een overzichtsartikel van oorspronkelijke onderzoek naar psychopathologie bij mutatiedragers voor de ziekte van Huntington. Aansluitend worden de resultaten van een cohortstudie naar de aanwezigheid en ernst van psychopathologie bij mensen met de ziekte van Huntington in

  13. Learning about Huntington's Disease (United States)

    Skip to main content Learning About Huntington's Disease Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research ...

  14. VT Data - Zoning 20120709, Huntington (United States)

    Vermont Center for Geographic Information — Zoning district data for the Town of Huntington, Vermont. For details regarding each zoning district refer to the current zoning regulations on town of Huntington's...

  15. Patterns of species richness in sandy beaches of South America ...

    African Journals Online (AJOL)

    The middle shore is primarily occupied by cirolanids and bivalves, and hippid crabs, bivalves and amphipods dominate the lower beach. Generally, species richness increases from upper to lower beach levels. Studies carried out on exposed sandy beaches of south-central Chile (ca. 40°S) show that different beach states ...

  16. Huntington\\'s disease: Genetic heterogeneity in black African patients

    African Journals Online (AJOL)

    Objective. Huntington's disease (HD) has been reported to occur rarely in black patients. A new genetic variant– Huntington's disease-like 2 (HDL2) – occurring more frequently in blacks, has recently been described. The absence of an expanded trinucleotide repeat at the chromosome 4 HD locus was previously regarded ...

  17. Clinical neurogenetics: huntington disease. (United States)

    Bordelon, Yvette M


    Huntington disease (HD) is an autosomal dominant, adult-onset, progressive neurodegenerative disease characterized by the triad of abnormal movements (typically chorea), cognitive impairment, and psychiatric problems. It is caused by an expanded CAG repeat in the gene encoding the protein huntingtin on chromosome 4 and causes progressive atrophy of the striatum as well as cortical and other extrastriatal structures. Genetic testing has been available since 1993 to confirm diagnosis in affected adults and for presymptomatic testing in at-risk individuals. This review covers HD signs, symptoms, and pathophysiology; current genetic testing issues; and current and future treatment strategies. Copyright © 2013 Elsevier Inc. All rights reserved.

  18. Huntington's disease presenting as amyotrophic lateral sclerosis.

    LENUS (Irish Health Repository)

    Phukan, Julie


    We present the clinical, electrophysiological and molecular genetic findings of a 58-year-old male with genetically confirmed Huntington\\'s disease (HD) and concurrent clinically definite ALS by El Escorial criteria. The patient presented with asymmetric upper limb amyotrophy and weakness, and subsequently developed chorea and cognitive change. Genetic testing confirmed the presence of expanded trinucleotide repeats in huntingtin, consistent with a diagnosis of Huntington\\'s disease. This case confirms the rare coexistence of Huntington\\'s disease and motor neuron degeneration.

  19. Biblioteca Publica en Huntington Beach (EE.UU.

    Directory of Open Access Journals (Sweden)

    Historico, Dion


    Full Text Available The first stage, which is the only one completed up to the present time, consists of a more extensive architectural complex conceived for housing different cultural functions besides the library: plastic arts, cinematography, scientific and informative activities, etc. The building is organized in two main floors in a rectangular shape, and in several mezzanines, reading rooms, book deposits, projection areas, music and painting departments and technical administration and maintenance offices, occupying a total of approximately 4,400 m2. The architectural design denotes the intention of integration in the treatment of the inside spaces, by the suppression of separate compartments, organizing the necessary distinction of functions in the reading rooms, by means of differences of floor levels and with the use of adequate decorative elements. In the same manner, the outside walls have been substituted by complete glass surfaces which allow full incorporation of the natural surroundings in the architecture.Constituye la primera fase, única realizada hasta el presente, de un complejo arquitectónico más amplio concebido para albergar distintas funciones culturales además de biblioteca: artes plásticas, cinematografía, actividades científicas e informativas, etc. El edificio se organiza en dos plantas principales, de forma rectangular, y en diversas entreplantas, salas de lectura, depósitos de libros, locales de proyección, departamentos de música y pintura, y oficinas técnicas de administración y mantenimiento, ocupando un total aproximado de 4.400 m2. El planteamiento arquitectónico denota un propósito integrador en el tratamiento de los espacios interiores, por la supresión de las compartimentaciones de fábrica, organizándose la necesaria distinción de ambientes en las salas de lectura, mediante diferencias de nivel del suelo y con el empleo de adecuados elementos decorativos. Del mismo modo, los muros de fachada se han sustituido por paramentos íntegramente acristalados, que permiten una plena incorporación del entorno natural en la arquitectura.

  20. Music therapy in Huntington's disease

    NARCIS (Netherlands)

    Bruggen-Rufi, van C.H.M.


    The thesis reports about the effects of music therapy with patients in the late stage of Huntington's disease. A literature review, a focus group study, a randomized controlled trial, an evaluation for complex interventions and a case report study are integrated in the thesis. The beneficial

  1. Human Health at the Beach (United States)

    ... and Research Centers Beaches Contact Us Share LEARN: Human Health at the Beach Swimming at beaches with pollution ... water pollution, there are other potential threats to human health at the beach to be aware of. The ...

  2. Beach Profile Locations (United States)

    California Natural Resource Agency — Beaches are commonly characterized by cross-shore surveys. The resulting profiles represent the elevation of the beach surface and nearshore seabed from the back of...

  3. Depth-Dependent Earthquake Properties Beneath Long-Beach, CA: Implications for the Rheology at the Brittle-Ductile Transition Zone (United States)

    Inbal, A.; Clayton, R. W.; Ampuero, J. P.


    Except for a few localities, seismicity along faults in southern California is generally confined to depths shallower than 15 km. Among faults hosting deep seismicity, the Newport-Inglewood Fault (NIF), which traverses the Los-Angeles basin, has an exceptionally mild surface expression and low deformation rates. Moreover, the NIF structure is not as well resolved as other, less well instrumented faults because of poor signal-to-noise ratio. Here we use data from three temporary dense seismic arrays, which were deployed for exploration purposes and contain up to several thousands of vertical geophones, to investigate the properties of deep seismicity beneath Long-Beach (LB), Compton and Santa-Fe Springs (SFS). The latter is located 15 km northeast of the NIF, presumably above a major detachment fault underthrusting the basin.Event detection is carried out using a new approach for microseismic multi-channel picking, in which downward-continued data are back-projected onto the volume beneath the arrays, and locations are derived from statistical analysis of back-projection images. Our technique reveals numerous, previously undetected events along the NIF, and confirms the presence of an active shallow structure gently dipping to the north beneath SFS. Seismicity characteristics vary along the NIF strike and dip. While LB seismicity is uncorrelated with the mapped trace of the NIF, Compton seismicity illuminates a sub-vertical fault that extends down to about 20 km. This result, along with the reported high flux of mantle Helium along the NIF (Boles et al., 2015), suggests that the NIF is deeply rooted and acts as a major conduit for mantle fluids. We find that the LB size distribution obeys the typical power-law at shallow depths, but falls off exponentially for events occurring below 20 km. Because deep seismicity occurs uniformly beneath LB, this transition is attributed to a reduction in seismic asperity density with increasing depth, consistent with a transition

  4. Virtual Beach Manager Toolset (United States)

    The Virtual Beach Manager Toolset (VB) is a set of decision support software tools developed to help local beach managers make decisions as to when beaches should be closed due to predicted high levels of water borne pathogens. The tools are being developed under the umbrella of...

  5. Huntington's disease: a perplexing neurological disease ...

    African Journals Online (AJOL)

    Huntington's disease is an inherited intricate brain illness. It is a neurodegenerative, insidious disorder; the onset of the disease is very late to diagnose. It is caused by an expanded CAG repeat in the Huntingtin gene, which encodes an abnormally long polyglutamine repeat in the Huntingtin protein. Huntington's disease ...

  6. Huntington's disease : Psychological aspects of predictive testing

    NARCIS (Netherlands)

    Timman, Reinier


    Predictive testing for Huntington's disease appears to have long lasting psychological effects. The predictive test for Huntington's disease (HD), a hereditary disease of the nervous system, was introduced in the Netherlands in the late eighties. As adverse consequences of the test were

  7. Psychiatric symptoms and CAG expansion in Huntington`s disease

    Energy Technology Data Exchange (ETDEWEB)

    Weber, M.W.; Schmid, W.; Spiegel, R. [Univ. of Zuerich (Switzerland)


    The mutation responsible for Huntington`s disease (HD) is an elongated CAG repeat in the coding region of the IT15 gene. A PCR-based test with high sensitivity and accuracy is now available to identify asymptomatic gene carriers and patients. An inverse correlation between CAG copy number and age at disease onset has been found in a large number of affected individuals. The influence of the CAG repeat expansion on other phenotypic manifestations, especially specific psychiatric symptoms has not been studied intensively. In order to elucidate this situation we investigated the relation between CAG copy number and distinct psychiatric phenotypes found in 79 HD-patients. None of the four differentiated categories (personality change, psychosis, depression, and nonspecific alterations) showed significant differences in respect to size of the CAG expansion. In addition, no influence of individual sex on psychiatric presentation could be found. On the other hand in patients with personality changes maternal transmission was significantly more frequent compared with all other groups. Therefore we suggest that clinical severity of psychiatric features in HD is not directly dependent on the size of the dynamic mutation involved. The complex pathogenetic mechanisms leading to psychiatric alterations are still unknown and thus genotyping does not provide information about expected psychiatric symptoms in HD gene carriers. 40 refs., 1 fig., 2 tabs.

  8. Louisiana's statewide beach cleanup (United States)

    Lindstedt, Dianne M.; Holmes, Joseph C.


    Litter along Lousiana's beaches has become a well-recognized problem. In September 1987, Louisiana's first statewide beach cleanup attracted about 3300 volunteers who filled 16,000 bags with trash collected along 15 beaches. An estimated 800,173 items were gathered. Forty percent of the items were made of plastic and 11% were of polystyrene. Of all the litter collected, 37% was beverage-related. Litter from the oil and gas, commercial fishing, and maritime shipping industries was found, as well as that left by recreational users. Although beach cleanups temporarily rid Louisiana beaches of litter, the real value of the effort is in public participation and education. Civic groups, school children, and individuals have benefited by increasing their awareness of the problems of trash disposal.

  9. Personality Traits in Huntington's Disease

    DEFF Research Database (Denmark)

    Larsen, Ida Unmack; Mortensen, Erik Lykke; Vinther-Jensen, Tua


    Huntington's disease (HD) is associated with risk for developing psychiatric symptoms. Vulnerability or resilience to psychiatric symptoms may be associated with personality traits. This exploratory study, aimed to investigate personality traits in a large cohort of HD carriers and at risk gene......-expansion negative individuals (HD non-carriers), exploring whether carrying the HD gene or growing up in an HD family influences personality traits. Forty-seven HD carriers, Thirty-nine HD non-carriers, and 121 healthy controls answered the Danish version of the revised NEO personality inventory. Comparisons...... symptoms. Our findings suggest that, there is no direct effect of the HD gene on personality traits, but that personality assessment may be relevant to use when identifying individuals from HD families who are vulnerable to develop psychiatric symptoms....

  10. Clinical presentation of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Ruocco Heloísa H.


    Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

  11. Huntington's disease: a clinical review

    Directory of Open Access Journals (Sweden)

    Roos Raymund AC


    Full Text Available Abstract Huntington disease (HD is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD. The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which

  12. Discrepancies in reporting the CAG repeat lengths for Huntington's disease

    DEFF Research Database (Denmark)

    Quarrell, Oliver W; Handley, Olivia; O'Donovan, Kirsty


    Huntington's disease results from a CAG repeat expansion within the Huntingtin gene; this is measured routinely in diagnostic laboratories. The European Huntington's Disease Network REGISTRY project centrally measures CAG repeat lengths on fresh samples; these were compared with the original...

  13. Beach Ball Coronagraph (United States)

    National Aeronautics and Space Administration — A precision formation flying coronagraph with an inflatable, passive ‘beach ball’ occulter has the chance to make possible the next generation of advances in coronal...

  14. National List of Beaches (United States)

    U.S. Environmental Protection Agency — EPA has published a list of coastal recreation waters adjacent to beaches (or similar points of access) used by the public in the U.S. The list, required by the...

  15. Perceptions of genetic discrimination among people at risk for Huntington?s disease: a cross sectional survey


    Bombard, Yvonne; Veenstra, Gerry; Friedman, Jan M; Creighton, Susan; Currie, Lauren; Paulsen, Jane S; Bottorff, Joan L; Hayden, Michael R


    Objective To assess the nature and prevalence of genetic discrimination experienced by people at risk for Huntington?s disease who had undergone genetic testing or remained untested. Design Cross sectional, self reported survey. Setting Seven genetics and movement disorders clinics servicing rural and urban communities in Canada. Participants 233 genetically tested and untested asymptomatic people at risk for Huntington?s disease (response rate 80%): 167 underwent testing (83 had the Huntingt...

  16. Morphodynamic characterization of the Spanish beaches of the Gulf of Cadiz

    Energy Technology Data Exchange (ETDEWEB)

    Benavente, J.; Gracia, F. J.; Rio, L. del; Anfuso, G.; Rodriguez-Ramirez, A.


    During the1980s several attempts were made to classify beaches according to their morphodynamic behav- iour. Published papers proposed classifications based mainly on wave incident energy and beach character- istics, such as foreshore slopes and sediment settling velocities. In the 1990s more complex classifications appeared, where the effect of tides on wave action was included, highlighting their relevance to the determi- nation of the morphodynamic state of the beach. In this paper we present a beach monitoring programme, in which more than 30 beaches located along the Spanish shores of the Gulf of Cadiz and the Strait of Gibraltar were surveyed for four years (2000-2004). The long study period allowed the monitoring of beach morphologies related both to fair weather (summer) and storm (winter) conditions. The coastal setting in the study area provided the opportunity for covering a wide range of tidal conditions, from high mesotidal (MSTR ca. 4 m) to microtidal (MSTR around 1 m). Furthermore, the dimensions of the study area permitted the mon- itoring of beaches linked to different boundary conditions, thus including both attached and detached beach- es located at varying distances from main sediment sources, and influenced by different wave regimes. The analysis of the beach morphologies related to such contrasting conditions allowed the identification of the real significance of the tidal effect on beach profile morphology and hence on beach morphodynamics. Finally, we conclude that the effect of tides on wave action is the main factor determining beach morphody- namic behaviour. (Author)

  17. Mapping energy poverty in Huntington, West Virginia (United States)

    Callicoat, Elizabeth Anne

    Energy poverty is a growing phenomenon culminating from the combination of low to mid household income, deteriorating housing structures and rising household energy costs. Energy prices are increasing for all households, but the burden is proportionally larger for those with low to mid income. These groups must sacrifice to afford energy, and are often unable or do not have the autonomy to make structural improvements, especially if they rent their home. Data on residential dwellings from the Cabell County Tax Assessor's Office was used within a geographic information system to map where energy poverty likely exists within the city limits of Huntington, WV. It was found that one fifth of Huntington households are at a high risk of energy poverty, primarily located across the northern section of the city and in the center, surrounding Marshall University, Downtown and Cabell Huntington Hospital.


    Safe beaches meet water quality standards and are valued for their aesthetics and the recreational opportunities that they afford. In the United States recreational water quality assessments and beach closure decisions are presently based on samples of enterococci or Escherichia ...

  19. Virtual Beach: Decision Support Tools for Beach Pathogen Prediction (United States)

    The Virtual Beach Managers Tool (VB) is decision-making software developed to help local beach managers make decisions as to when beaches should be closed due to predicted high levels of water borne pathogens. The tool is being developed under the umbrella of EPA's Advanced Monit...

  20. Summary of Annual Beach Notifications (United States)

    U.S. Environmental Protection Agency — To help beachgoers make informed decisions about swimming at U.S. beaches, EPA gathers state-by-state data about beach closings and advisories. Between 1999 and...

  1. Kas Huntington oli prohvet? / Priit Simson

    Index Scriptorium Estoniae

    Simson Priit, 1977-


    Autor käsitleb Samuel Huntingtoni teese ning leiab, et tegelikult Huntington ei pakkunud õigustust islamiriikide ründamisele, vaid pigem hoiatas tsivilisatsioonide siseasjusse sekkumise, tekkida võiva ahelreaktsiooni eest, kus üks tsivilisatsiooni liige tõmbab sõtta ka teise

  2. Destination and source memory in Huntington's disease

    NARCIS (Netherlands)

    El Haj, M.; Caillaud, M.; Verny, C.; Fasotti, L.; Allain, P.


    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults

  3. 20 CFR Appendix A to Subpart F of... - U.S. Seaports (United States)


    ... Port Costa, CA Port Hueneme, CA Port San Luis, CA Redwood City, CA Richmond, CA Sacramento, CA San... Sabine, TX Texas City, TX South Pacific Range Alameda, CA Antioch, CA Benicia, CA Carlsbad, CA Carpinteria, CA Crockett, CA El Segundo, CA Eureka, CA Estero Bay, CA Gaviota, CA Huntington Beach, CA Long...

  4. A Metabolic Study of Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Rajasree Nambron

    Full Text Available Huntington's disease patients have a number of peripheral manifestations suggestive of metabolic and endocrine abnormalities. We, therefore, investigated a number of metabolic factors in a 24-hour study of Huntington's disease gene carriers (premanifest and moderate stage II/III and controls.Control (n = 15, premanifest (n = 14 and stage II/III (n = 13 participants were studied with blood sampling over a 24-hour period. A battery of clinical tests including neurological rating and function scales were performed. Visceral and subcutaneous adipose distribution was measured using magnetic resonance imaging. We quantified fasting baseline concentrations of glucose, insulin, cholesterol, triglycerides, lipoprotein (a, fatty acids, amino acids, lactate and osteokines. Leptin and ghrelin were quantified in fasting samples and after a standardised meal. We assessed glucose, insulin, growth hormone and cortisol concentrations during a prolonged oral glucose tolerance test.We found no highly significant differences in carbohydrate, protein or lipid metabolism markers between healthy controls, premanifest and stage II/III Huntington's disease subjects. For some markers (osteoprotegerin, tyrosine, lysine, phenylalanine and arginine there is a suggestion (p values between 0.02 and 0.05 that levels are higher in patients with premanifest HD, but not moderate HD. However, given the large number of statistical tests performed interpretation of these findings must be cautious.Contrary to previous studies that showed altered levels of metabolic markers in patients with Huntington's disease, our study did not demonstrate convincing evidence of abnormalities in any of the markers examined. Our analyses were restricted to Huntington's disease patients not taking neuroleptics, anti-depressants or other medication affecting metabolic pathways. Even with the modest sample sizes studied, the lack of highly significant results, despite many being tested, suggests that

  5. Escherichia coli at Ohio Bathing Beaches--Distribution, Sources, Wastewater Indicators, and Predictive Modeling (United States)

    Francy, Donna S.; Gifford, Amie M.; Darner, Robert A.


    Results of studies during the recreational seasons of 2000 and 2001 strengthen the science that supports monitoring of our Nation?s beaches. Water and sediment samples were collected and analyzed for concentrations of Escherichia coli (E. coli). Ancillary water-quality and environmental data were collected or compiled to determine their relation to E. coli concentrations. Data were collected at three Lake Erie urban beaches (Edgewater, Villa Angela, and Huntington), two Lake Erie beaches in a less populated area (Mentor Headlands and Fairport Harbor), and one inland-lake beach (Mosquito Lake). The distribution of E. coli in water and sediments within the bathing area, outside the bathing area, and near the swash zone was investigated at the three Lake Erie urban beaches and at Mosquito Lake. (The swash zone is the zone that is alternately covered and exposed by waves.) Lake-bottom sediments from outside the bathing area were not significant deposition areas for E. coli. In contrast, interstitial water and subsurface sediments from near the swash zone were enriched with E. coli. For example, E. coli concentrations were as high as 100,000 colonies per 100 milliliters in some interstitial waters. Although there are no standards for E. coli in swash-zone materials, the high concentrations found at some locations warrant concern for public health. Studies were done at Mosquito Lake to identify sources of fecal contamination to the lake and bathing beach. Escherichia coli concentrations decreased with distance from a suspected source of fecal contamination that is north of the beach but increased at the bathing beach. This evidence indicated that elevated E. coli concentrations at the bathing beach are of local origin rather than from transport of bacteria from sites to the north. Samples collected from the three Lake Erie urban beaches and Mosquito Lake were analyzed to determine whether wastewater indicators could be used as surrogates for E. coli at bathing beaches

  6. Coastal Land Air Sea Interaction: "the" beach towers (United States)

    MacMahan, J. H.; Koscinski, J. S.; Ortiz-Suslow, D. G.; Haus, B. K.; Thornton, E. B.


    As part of the Coastal Land Air Sea Interaction (CLASI) experiment, an alongshore array of 6-m high towers instrumented with ultrasonic 3D anemometers and temperature-relative humidity sensors were deployed at five sandy beaches near the high-tide line in Monterey Bay, CA, in May-June 2016. A cross-shore array of towers was also deployed from within the active surfzone to the toe of the dune at one beach. In addition, waves and ocean temperature were obtained along the 10m isobath for each beach. The dissipative surfzone was O(80m) wide. The wave energy varies among the beaches owing to sheltering and refraction by the Monterey Canyon and headlands. The tides are semi-diurnal mixed, meso-tidal with a maximum tidal range of 2m. This results in a variable beach width from the tower to the tidal line. Footprint analysis for estimating the source region for the turbulent momentum fluxes, suggests that the observations represent three scenarios described as primarily ocean, mixed beach and ocean, and primarily beach. The direct-estimate of the atmospheric stability by the sonic anemometer suggest that all of the beaches are mostly unstable except for a few occurrences in the evening during low wind conditions. The onshore neutral drag coefficient (Cd) estimated at 10m heights is 3-5 times larger than open ocean estimates. Minimal variability was found in Cd based on the footprint analysis. Beach-specific spatial variability in Cd was found related to atmospheric stability and wave energy.

  7. The Wilson films--Huntington's chorea. (United States)

    Klein, Christine


    Wilson's Queen Square Case 9 with Huntington's chorea shows a 68-year-old man with mild to moderate generalized chorea, impaired fixation, and probable cognitive decline in keeping with a diagnosis of Huntington's disease (HD). An age of onset in the late sixties and a negative family history suggest a relatively small expanded trinucleotide repeat in the HTT gene in the patient and reduced penetrance of an even shorter repeat allele in one of his parents. A highly sensitive and specific gene test has been offered worldwide for diagnostic testing of HD for almost two decades. This test, obviously unavailable at Wilson's times, became the historic frontrunner for guidelines of symptomatic, presymptomatic, and prenatal testing for an adult-onset neurodegenerative disorder. Regarding treatment of HD, however, we are still awaiting the successful translation of research results into the development of effective cause-directed, neuropreventive and neurorestaurative therapies. Copyright © 2011 Movement Disorder Society.

  8. [Sporadic juvenile forms of Huntington's chorea]. (United States)

    Zinchenko, A P; Goncharov, V D; Burtianskii, D L; Zakhar'ev, Iu M


    Six patients with Huntington's chorea in the age of 15-24 years old, suffered from diffusive choreic hyperkynesis with slowly progressive dementia. The development of this disease in childhood and adolescence was atypical, as nobody in the family and in kin sufferred from it and it was difficult to diagnose the disease. Recognition of the disease was promoted by pneumoencephalography, electromyography and memory investigation.

  9. Earliest functional declines in Huntington disease (United States)

    Beglinger, Leigh J.; O'Rourke, Justin J.F.; Wang, Chiachi; Langbehn, Douglas R.; Duff, Kevin; Paulsen, Jane S.


    We examined the gold standard for Huntington disease (HD) functional assessment, the Unified Huntington's Disease Rating Scale (UHDRS), in a group of at-risk participants not yet diagnosed but who later phenoconverted to manifest HD. We also sought to determine which skill domains first weaken and the clinical correlates of declines. Using the UHDRS Total Functional Capacity (TFC) and Functional Assessment Scale (FAS), we examined participants from Huntington Study Group clinics who were not diagnosed at their baseline visit but were diagnosed at a later visit (N = 265). Occupational decline was the most common with 65.1% (TFC) and 55.6% (FAS) reporting some loss of ability to engage in their typical work. Inability to manage finances independently (TFC 49.2%, FAS 35.1%) and drive safely (FAS 33.5%) were also found. Functional decline was significantly predicted by motor, cognitive, and depressive symptoms. The UHDRS captured early functional losses in individuals with HD prior to formal diagnosis, however, fruitful areas for expanded assessment of early functional changes are performance at work, ability to manage finances, and driving. These are also important areas for clinical monitoring and treatment planning as up to 65% experienced loss in at least one area prior to diagnosis. PMID:20471695

  10. Clinical and genetic data of Huntington disease in Moroccan patients

    African Journals Online (AJOL)

    Background: Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10 /100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Methods: Clinical ...

  11. Dantrolene is neuroprotective in Huntington's disease transgenic mouse model

    Directory of Open Access Journals (Sweden)

    Chen Xi


    Full Text Available Abstract Background Huntington's disease (HD is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs. Our group has previously demonstrated that calcium (Ca2+ signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128. Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2 and spinocerebellar ataxia 3 (SCA3 mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. Results The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Conclusions Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that Ryan

  12. Dantrolene is neuroprotective in Huntington's disease transgenic mouse model. (United States)

    Chen, Xi; Wu, Jun; Lvovskaya, Svetlana; Herndon, Emily; Supnet, Charlene; Bezprozvanny, Ilya


    Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs). Our group has previously demonstrated that calcium (Ca2+) signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128). Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT) MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2) and spinocerebellar ataxia 3 (SCA3) mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg) twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that RyanR inhibitors and Ca2+ signaling stabilizers such as

  13. Mobilisation of toxic trace elements under various beach nourishments

    International Nuclear Information System (INIS)

    Pit, Iris R.; Dekker, Stefan C.; Kanters, Tobias J.; Wassen, Martin J.; Griffioen, Jasper


    mobilisation of trace elements. - Highlights: • Shoreface nourishments are recommended rather than beach nourishments. • Shoreface nourishments limit oxidation processes and retain seawater influences. • Toxic trace elements are mobilised in nourishments because of pyrite oxidation. • Low CaCO 3 content and rainwater influences can mobilise trace metals over time. - The mobility of trace elements at beach nourishments is caused by pyrite oxidation. Decalcification may occur in time. Shoreface nourishments are recommended over beach nourishments.

  14. Identification of genetic variants associated with Huntington's disease progression

    DEFF Research Database (Denmark)

    Hensman Moss, Davina J; Pardiñas, Antonio F; Langbehn, Douglas


    indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008-11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers...... in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression......BACKGROUND: Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate...

  15. Sediment supply to beaches

    DEFF Research Database (Denmark)

    Aagaard, Troels


    Many beaches have been built by an onshore supply of sand from the shoreface, and future long-term coastal evolution critically depends on cross-shore sediment exchange between the upper and the lower shorefaces. Even so, cross-shore sediment supply remains poorly known in quantitative terms...... and this reduces confidence in predictions of long-term shoreline change. In this paper, field measurements of suspended sediment load and cross-shore transport on the lower shoreface are used to derive a model for sediment supply from the lower to the upper shoreface at large spatial and temporal scales. Data...

  16. Isolation, identification, and pathological effects of beach sand bacterial extract on human skin keratinocytes in vitro

    Directory of Open Access Journals (Sweden)

    Fazli Subhan


    Full Text Available Background Beaches are recreational spots for people. However, beach sand contains harmful microbes that affect human health, and there are no established methods for either sampling and identifying beach-borne pathogens or managing the quality of beach sand. Method This study was conducted with the aim of improving human safety at beaches and augmenting the quality of the beach experience. Beach sand was used as a resource to isolate bacteria due to its distinctive features and the biodiversity of the beach sand biota. A selected bacterial isolate termed FSRS was identified as Pseudomonas stutzeri using 16S rRNA sequencing and phylogenetic analysis, and the sequence was deposited in the NCBI GenBank database under the accession number MF599548. The isolated P. stutzeri bacterium was cultured in Luria–Bertani growth medium, and a crude extract was prepared using ethyl acetate to examine the potential pathogenic effect of P. stutzeri on human skin. A human skin keratinocyte cell line (HaCaT was used to assess cell adhesion, cell viability, and cell proliferation using a morphological analysis and a WST-1 assay. Result The crude P. stutzeri extract inhibited cell adhesion and decreased cell viability in HaCaT cells. We concluded that the crude extract of P. stutzeri FSRS had a strong pathological effect on human skin cells. Discussion Beach visitors frequently get skin infections, but the exact cause of the infections is yet to be determined. The beach sand bacterium P. stutzeri may, therefore, be responsible for some of the dermatological problems experienced by people visiting the beach.

  17. Migrations of sandy beach meiofauna

    African Journals Online (AJOL)

    The meiofauna at higher tide levels on a sheltered beach has been found to .... temperature of the sea water was also measured in the shallows (about 0,5 m deep). For the ... Movement was monitored over a five-hour period on the rising tide (08h30-13h30) and a ...... Distribution of sand fauna in beaches at Miami, Florida.

  18. Spatial and temporal variations of carbonate content in the beach and nearshore environments off Goa, west coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Veerayya, M.

    -90%) in the foreshore regions of individual beaches whereas low CaCO sub(3) (less than 20%) in the dune and backshore areas of these beaches and adjoining nearshore environs up to 10 m isobath. The observed spatial distribution shows the occurrence of modern carbonate...

  19. Inventory and Evaluation of Cultural Resources, Bolsa Chica Mesa and Huntington Beach Mesa, Orange County, California (United States)


    Excelentisimo Conde de Monterey, Virrey Que Era dela Nueva Espana. In Monarchia Indiana, edited by J. de Torquemada, pp. 693-725. Madrid. 101 102 Baumhoff, M...biological bacterias , this includes the destruction of canyons, hills, mountains and the flora and fauna in these areas. Road construction, real

  20. High Protein Diet and Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Chiung-Mei Chen

    Full Text Available Huntington's disease (HD is a neurodegenerative disorder caused by the huntingtin (HTT gene with expanded CAG repeats. In addition to the apparent brain abnormalities, impairments also occur in peripheral tissues. We previously reported that mutant Huntingtin (mHTT exists in the liver and causes urea cycle deficiency. A low protein diet (17% restores urea cycle activity and ameliorates symptoms in HD model mice. It remains unknown whether the dietary protein content should be monitored closely in HD patients because the normal protein consumption is lower in humans (~15% of total calories than in mice (~22%. We assessed whether dietary protein content affects the urea cycle in HD patients. Thirty HD patients were hospitalized and received a standard protein diet (13.7% protein for 5 days, followed by a high protein diet (HPD, 26.3% protein for another 5 days. Urea cycle deficiency was monitored by the blood levels of citrulline and ammonia. HD progression was determined by the Unified Huntington's Disease Rating Scale (UHDRS. The HPD increased blood citrulline concentration from 15.19 μmol/l to 16.30 μmol/l (p = 0.0378 in HD patients but did not change blood ammonia concentration. A 2-year pilot study of 14 HD patients found no significant correlation between blood citrulline concentration and HD progression. Our results indicated a short period of the HPD did not markedly compromise urea cycle function. Blood citrulline concentration is not a reliable biomarker of HD progression.

  1. Huntington disease: Experimental models and therapeutic perspectives

    International Nuclear Information System (INIS)

    Serrano Sanchez, Teresa; Blanco Lezcano, Lisette; Garcia Minet, Rocio; Alberti Amador, Esteban; Diaz Armesto, Ivan and others


    Huntington's disease (HD) is a degenerative dysfunction of hereditary origin. Up to date there is not, an effective treatment to the disease which having lapsed 15 or 20 years advances inexorably, in a slow form, toward the total inability or death. This paper reviews the clinical and morphological characteristics of Huntington's disease as well as the experimental models more commonly used to study this disease, having as source the articles indexed in Medline data base, published in the last 20 years. Advantages and disadvantages of all experimental models to reproduce the disease as well as the perspectives to therapeutic assay have been also considered. the consent of outline reported about the toxic models, those induced by neurotoxins such as quinolinic acid, appears to be the most appropriate to reproduce the neuropathologic characteristic of the disease, an genetic models contributing with more evidence to the knowledge of the disease etiology. Numerous treatments ameliorate clinical manifestations, but none of them has been able to stop or diminish the affectations derived from neuronal loss. At present time it is possible to reproduce, at least partially, the characteristics of the disease in experimentation animals that allow therapy evaluation in HD. from the treatment view point, the more promissory seems to be transplantation of no neuronal cells, taking into account ethical issues and factibility. On the other hand the new technology of interference RNA emerges as a potential therapeutic tool for treatment in HD, and to respond basic questions on the development of the disease.

  2. 77 FR 50019 - Safety Zone; Cocoa Beach Air Show, Atlantic Ocean, Cocoa Beach, FL (United States)


    ... 1625-AA00 Safety Zone; Cocoa Beach Air Show, Atlantic Ocean, Cocoa Beach, FL AGENCY: Coast Guard, DHS... waters of the Atlantic Ocean located east of Cocoa Beach, Florida during the Cocoa Beach Air Show. The Cocoa Beach Air Show will include aircraft engaging in aerobatic maneuvers. The event is scheduled to...

  3. Beach Volume Change Using Uav Photogrammetry Songjung Beach, Korea (United States)

    Yoo, C. I.; Oh, T. S.


    Natural beach is controlled by many factors related to wave and tidal forces, wind, sediment, and initial topography. For this reason, if numerous topographic data of beach is accurately collected, coastal erosion/acceleration is able to be assessed and clarified. Generally, however, many studies on coastal erosion have limitation to analyse the whole beach, carried out of partial area as like shoreline (horizontal 2D) and beach profile (vertical 2D) on account of limitation of numerical simulation. This is an important application for prevention of coastal erosion, and UAV photogrammetry is also used to 3D topographic data. This paper analyses the use of unmanned aerial vehicles (UAV) to 3D map and beach volume change. UAV (Quadcopter) equipped with a non-metric camera was used to acquire images in Songjung beach which is located south-east Korea peninsula. The dynamics of beach topography, its geometric properties and estimates of eroded and deposited sand volumes were determined by combining elevation data with quarterly RTK-VRS measurements. To explore the new possibilities for assessment of coastal change we have developed a methodology for 3D analysis of coastal topography evolution based on existing high resolution elevation data combined with low coast, UAV and on-ground RTK-VRS surveys. DSMs were obtained by stereo-matching using Agisoft Photoscan. Using GCPs the vertical accuracy of the DSMs was found to be 10 cm or better. The resulting datasets were integrated in a local coordinates and the method proved to be a very useful fool for the detection of areas where coastal erosion occurs and for the quantification of beach change. The value of such analysis is illustrated by applications to coastal of South Korea sites that face significant management challenges.


    Directory of Open Access Journals (Sweden)

    C. I. Yoo


    Full Text Available Natural beach is controlled by many factors related to wave and tidal forces, wind, sediment, and initial topography. For this reason, if numerous topographic data of beach is accurately collected, coastal erosion/acceleration is able to be assessed and clarified. Generally, however, many studies on coastal erosion have limitation to analyse the whole beach, carried out of partial area as like shoreline (horizontal 2D and beach profile (vertical 2D on account of limitation of numerical simulation. This is an important application for prevention of coastal erosion, and UAV photogrammetry is also used to 3D topographic data. This paper analyses the use of unmanned aerial vehicles (UAV to 3D map and beach volume change. UAV (Quadcopter equipped with a non-metric camera was used to acquire images in Songjung beach which is located south-east Korea peninsula. The dynamics of beach topography, its geometric properties and estimates of eroded and deposited sand volumes were determined by combining elevation data with quarterly RTK-VRS measurements. To explore the new possibilities for assessment of coastal change we have developed a methodology for 3D analysis of coastal topography evolution based on existing high resolution elevation data combined with low coast, UAV and on-ground RTK-VRS surveys. DSMs were obtained by stereo-matching using Agisoft Photoscan. Using GCPs the vertical accuracy of the DSMs was found to be 10 cm or better. The resulting datasets were integrated in a local coordinates and the method proved to be a very useful fool for the detection of areas where coastal erosion occurs and for the quantification of beach change. The value of such analysis is illustrated by applications to coastal of South Korea sites that face significant management challenges.

  5. O desenvolvimento político em Huntington e Fukuyama Huntington and Fukuyama on political development

    Directory of Open Access Journals (Sweden)

    Natália Nóbrega de Mello


    Full Text Available O artigo contrasta as teses de Huntington e Fukuyama sobre desenvolvimento político. As obras analisadas, Ordem política nas sociedades em mudança e O fim da história, inscrevem-se entre duas conjunturas decisivas - 1968 e 1989. Huntington desmontou a equivalência entre desenvolvimento político e modernização e Fukuyama reafirmou a democracia como o destino de todos os países e, desse modo, como o fim da história. Nesta comparação, dois eixos se sobressaem: o contexto de produção das obras e a alternância entre os polos teóricos da democracia e da estabilidade. Procura-se demonstrar como, apesar de reinserir a democracia no desenvolvimento político, a instabilidade continua a ser um foco privilegiado de análise no pensamento de Fukuyama.The article contrasts the theories of Huntington and Fukuyama on political development. The analyzed works, Political order in changing societies and The end of history, fall between two decisive historical moments - in 1968 and 1989. Huntington disassembled the equivalence between political development and modernization; Fukuyama reaffirmed democracy as the destiny of all countries and, as such, it is the end of history. In this comparison, two axes call our attention: the production context of these works and the alternation between the theoreticals poles of democracy and stability. The article shows how, although reenters democracy in the political development theory, instablility remains a prime focus of analysis in Fukuyama's thought.

  6. Basic Remote Sensing Investigations for Beach Reconnaissance. (United States)

    Progress is reported on three tasks designed to develop remote sensing beach reconnaissance techniques applicable to the benthic, beach intertidal...and beach upland zones. Task 1 is designed to develop remote sensing indicators of important beach composition and physical parameters which will...ultimately prove useful in models to predict beach conditions. Task 2 is designed to develop remote sensing techniques for survey of bottom features in

  7. Américo Negrette and Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Moscovich


    Full Text Available The authors present a historical review of the seminal clinical contribution of Professor Américo Negrette, a Venezuelan neurologist, to the evolution of scientific knowledge about Huntington's disease.

  8. Unravelling and Exploiting Astrocyte Dysfunction in Huntington's Disease

    DEFF Research Database (Denmark)

    Khakh, Baljit S.; Beaumont, Vahri; Cachope, Roger


    Astrocytes are abundant within mature neural circuits and are involved in brain disorders. Here, we summarize our current understanding of astrocytes and Huntington's disease (HD), with a focus on correlative and causative dysfunctions of ion homeostasis, calcium signaling, and neurotransmitter...

  9. Beach rock from Goa Coast

    Digital Repository Service at National Institute of Oceanography (India)

    Setty, M.G.A.P.; Wagle, B.G.

    constituents of beach rock found along Goa coast is dealt with in detail. While discussing the various views on its origin, it is emphasized that the process of cementation is chiefly controlled by ground water evaporation, inorganic precipitation and optimum...

  10. Landing Techniques in Beach Volleyball (United States)

    Tilp, Markus; Rindler, Michael


    The aims of the present study were to establish a detailed and representative record of landing techniques (two-, left-, and right-footed landings) in professional beach volleyball and compare the data with those of indoor volleyball. Beach volleyball data was retrieved from videos taken at FIVB World Tour tournaments. Landing techniques were compared in the different beach and indoor volleyball skills serve, set, attack, and block with regard to sex, playing technique, and court position. Significant differences were observed between men and women in landings following block actions (χ2(2) = 18.19, p volleyball revealed overall differences both in men (χ2(2) = 161.4, p volleyball players land more often on both feet than indoor volleyball players. Besides the softer surface in beach volleyball, and therefore resulting lower loads, these results might be another reason for fewer injuries and overuse conditions compared to indoor volleyball. Key Points About 1/3 of all jumping actions in beach volleyball result in a landing on one foot. Especially following block situations men land on one foot more often than women. Landing techniques are related to different techniques and positions. Landings on one foot are less common in beach volleyball than indoor volleyball. This could be a reason for fewer injuries and overuse conditions. PMID:24149150

  11. Contribution of Neuroepigenetics to Huntington's Disease. (United States)

    Francelle, Laetitia; Lotz, Caroline; Outeiro, Tiago; Brouillet, Emmanuel; Merienne, Karine


    Unbalanced epigenetic regulation is thought to contribute to the progression of several neurodegenerative diseases, including Huntington's disease (HD), a genetic disorder considered as a paradigm of epigenetic dysregulation. In this review, we attempt to address open questions regarding the role of epigenetic changes in HD, in the light of recent advances in neuroepigenetics. We particularly discuss studies using genome-wide scale approaches that provide insights into the relationship between epigenetic regulations, gene expression and neuronal activity in normal and diseased neurons, including HD neurons. We propose that cell-type specific techniques and 3D-based methods will advance knowledge of epigenome in the context of brain region vulnerability in neurodegenerative diseases. A better understanding of the mechanisms underlying epigenetic changes and of their consequences in neurodegenerative diseases is required to design therapeutic strategies more effective than current strategies based on histone deacetylase (HDAC) inhibitors. Researches in HD may play a driving role in this process.

  12. Destination and source memory in Huntington's disease. (United States)

    El Haj, Mohamad; Caillaud, Marie; Verny, Christophe; Fasotti, Luciano; Allain, Philippe


    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults had to put 12 items in a black or a white box (destination task), and to extract another 12 items from a blue or a red box (source task). Afterwards, they had to decide in which box each item had previously been deposited (destination memory), and from which box each item had previously been extracted (source memory). HD participants showed poorer source as well as destination recall performance than healthy adults in the proposed tasks. Correlation analysis showed that destination recall was significantly correlated with episodic recall in HD participants. Destination memory impairment in HD participants seems to be considerably influenced by their episodic memory performance. © 2014 The British Psychological Society.

  13. Magnetic biomineralisation in Huntington's disease transgenic mice

    International Nuclear Information System (INIS)

    Beyhum, W; Hautot, D; Dobson, J; Pankhurst, Q A


    The concentration levels of biogenic magnetite nanoparticles in transgenic R6/2 Huntington's disease (HD) mice have been investigated, using seven control and seven HD mice each from an 8 week-old litter and from a 12 week-old litter. Hysteresis and isothermal remnant magnetisation data were collected on a SQUID magnetometer, and analysed using a model comprising dia/paramagnetic, ferrimagnetic and superparamagnetic contributions, to extract the magnetite and ferritin concentrations present. It was found that magnetite was present in both superparamagnetic and blocked states. A larger spread and higher concentration of magnetite levels was found in the diseased mice for both the 8 week-old and 12 week-old batches, compared to the controls

  14. Progressive Impairment of Lactate-based Gluconeogenesis in the Huntington?s Disease Mouse Model R6/2


    Nielsen, Signe Marie Borch; Hasholt, Lis; N?rrem?lle, Anne; Josefsen, Knud


    Huntington?s disease (HD) is a neurodegenerative illness, where selective neuronal loss in the brain caused by expression of mutant huntingtin protein leads to motor dysfunction and cognitive decline in addition to peripheral metabolic changes. In this study we confirm our previous observation of impairment of lactate-based hepatic gluconeogenesis in the transgenic HD mouse model R6/2 and determine that the defect manifests very early and progresses in severity with disease development, indic...

  15. Popham Beach, Maine: An example of engineering activity that saved beach property without harming the beach (United States)

    Kelley, Joseph T.


    Beach and property erosion on coasts is a widespread and chronic problem. Historical approaches to this issue, including seawalls and sand replenishment, are often inappropriate or too expensive. In Maine, seawalls were banned in 1983 and replenishment is too costly to employ. Replacement of storm-damaged buildings is also not allowed, and a precedent case on Popham Beach, Maine required that the owner remove an unpermitted building from a site where an earlier structure was damaged. When the most popular park in Maine, Popham Beach State Park, experienced inlet associated erosion that threatened park infrastructure (a bathhouse), temporary measures were all that the law allowed. Because it was clear that the inlet channel causing the erosion would eventually change course, the state opted to erect a temporary seawall with fallen trees at the site. This may or may not have slowed the erosion temporarily, but reassured the public that "something was being done". Once a storm cut a new tidal inlet channel and closed off the old one, tidal water still entered the former channel and continued to threaten the bathhouse. To ultimately save the property, beach scraping was employed. Sand was scraped from the lower beach to construct a sand berm that deflected the tidal current away from the endangered property. This action created enough time for natural processes to drive the remains of the former spit onto the beach and widen it significantly. Whereas many examples of engineering practices exist that endanger instead of saving beaches, this example is one of an appropriate engineering effort to rescue unwisely located beach-front property.

  16. Aeolian transport of seagrass ( Posidonia oceanica ) beach-cast to terrestrial systems

    KAUST Repository

    Jiménez, Maria A.


    The annual export of the Mediterranean seagrass (Posidonia oceanica) litter to adjacent beaches and coastal dunes was quantified by examining the fortnight evolution of seagrass beach-cast volume on two beaches in the NW Mediterranean (Son Real and Es Trenc, Mallorca Island, Spain) for two years and analyzing the wind speed and direction obtained from the closest Meteorological Spanish Agency surface weather stations. The decomposition stage of the deposits was examined by analyzing the total hydrolysable amino acids, its percentage distribution and derived degradation indexes. Prevalent winds exceeding 6 m s−1, the coastline morphology and type of terrestrial vegetation determine the annual dynamics of the seagrass beach-cast. In the most protected beach (Son Real) the seagrass beach-cast remained nearly stationary during the two studied years while it exhibited wide annual fluctuations in the less protected one (Es Trenc). The amounts of P. oceanica wrack washed on Son Real and Es Trenc beaches, respectively, were estimated at 309 kg DW m coastline−1 yr−1 and 1359 kg DW m coastline−1 yr−1. They supplied between 20 kg CaCO3 m coastline−1 yr−1 and 47 kg CaCO3 m coastline−1 yr−1. Between 54% (Son Real) and 70% (Es Trenc) of seagrass beach-cast, respectively accounting for 1.5 kg N m coastline−1 yr−1 and 8.6 kg N m coastline−1 yr−1, were annually exported from the beaches to adjacent dune systems. Our results reveal that Mediterranean seagrass meadows might be an important source of materials, including sand and nutrients, for adjacent terrestrial systems, able to support their functioning.

  17. Aeolian transport of seagrass (Posidonia oceanica) beach-cast to terrestrial systems (United States)

    Jiménez, Maria A.; Beltran, Rafel; Traveset, Anna; Calleja, Maria Ll; Delgado-Huertas, Antonio; Marbà, Núria


    The annual export of the Mediterranean seagrass (Posidonia oceanica) litter to adjacent beaches and coastal dunes was quantified by examining the fortnight evolution of seagrass beach-cast volume on two beaches in the NW Mediterranean (Son Real and Es Trenc, Mallorca Island, Spain) for two years and analyzing the wind speed and direction obtained from the closest Meteorological Spanish Agency surface weather stations. The decomposition stage of the deposits was examined by analyzing the total hydrolysable amino acids, its percentage distribution and derived degradation indexes. Prevalent winds exceeding 6 m s-1, the coastline morphology and type of terrestrial vegetation determine the annual dynamics of the seagrass beach-cast. In the most protected beach (Son Real) the seagrass beach-cast remained nearly stationary during the two studied years while it exhibited wide annual fluctuations in the less protected one (Es Trenc). The amounts of P. oceanica wrack washed on Son Real and Es Trenc beaches, respectively, were estimated at 309 kg DW m coastline-1 yr-1 and 1359 kg DW m coastline-1 yr-1. They supplied between 20 kg CaCO3 m coastline-1 yr-1 and 47 kg CaCO3 m coastline-1 yr-1. Between 54% (Son Real) and 70% (Es Trenc) of seagrass beach-cast, respectively accounting for 1.5 kg N m coastline-1 yr-1 and 8.6 kg N m coastline-1 yr-1, were annually exported from the beaches to adjacent dune systems. Our results reveal that Mediterranean seagrass meadows might be an important source of materials, including sand and nutrients, for adjacent terrestrial systems, able to support their functioning.

  18. Aeolian transport of seagrass ( Posidonia oceanica ) beach-cast to terrestrial systems

    KAUST Repository

    Jimé nez, Maria A.; Beltran, Rafel; Traveset, Anna; Calleja, Maria Ll; Delgado-Huertas, Antonio; Marbà , Nú ria


    The annual export of the Mediterranean seagrass (Posidonia oceanica) litter to adjacent beaches and coastal dunes was quantified by examining the fortnight evolution of seagrass beach-cast volume on two beaches in the NW Mediterranean (Son Real and Es Trenc, Mallorca Island, Spain) for two years and analyzing the wind speed and direction obtained from the closest Meteorological Spanish Agency surface weather stations. The decomposition stage of the deposits was examined by analyzing the total hydrolysable amino acids, its percentage distribution and derived degradation indexes. Prevalent winds exceeding 6 m s−1, the coastline morphology and type of terrestrial vegetation determine the annual dynamics of the seagrass beach-cast. In the most protected beach (Son Real) the seagrass beach-cast remained nearly stationary during the two studied years while it exhibited wide annual fluctuations in the less protected one (Es Trenc). The amounts of P. oceanica wrack washed on Son Real and Es Trenc beaches, respectively, were estimated at 309 kg DW m coastline−1 yr−1 and 1359 kg DW m coastline−1 yr−1. They supplied between 20 kg CaCO3 m coastline−1 yr−1 and 47 kg CaCO3 m coastline−1 yr−1. Between 54% (Son Real) and 70% (Es Trenc) of seagrass beach-cast, respectively accounting for 1.5 kg N m coastline−1 yr−1 and 8.6 kg N m coastline−1 yr−1, were annually exported from the beaches to adjacent dune systems. Our results reveal that Mediterranean seagrass meadows might be an important source of materials, including sand and nutrients, for adjacent terrestrial systems, able to support their functioning.

  19. Use of predictive models and rapid methods to nowcast bacteria levels at coastal beaches (United States)

    Francy, Donna S.


    The need for rapid assessments of recreational water quality to better protect public health is well accepted throughout the research and regulatory communities. Rapid analytical methods, such as quantitative polymerase chain reaction (qPCR) and immunomagnetic separation/adenosine triphosphate (ATP) analysis, are being tested but are not yet ready for widespread use.Another solution is the use of predictive models, wherein variable(s) that are easily and quickly measured are surrogates for concentrations of fecal-indicator bacteria. Rainfall-based alerts, the simplest type of model, have been used by several communities for a number of years. Deterministic models use mathematical representations of the processes that affect bacteria concentrations; this type of model is being used for beach-closure decisions at one location in the USA. Multivariable statistical models are being developed and tested in many areas of the USA; however, they are only used in three areas of the Great Lakes to aid in notifications of beach advisories or closings. These “operational” statistical models can result in more accurate assessments of recreational water quality than use of the previous day's Escherichia coli (E. coli)concentration as determined by traditional culture methods. The Ohio Nowcast, at Huntington Beach, Bay Village, Ohio, is described in this paper as an example of an operational statistical model. Because predictive modeling is a dynamic process, water-resource managers continue to collect additional data to improve the predictive ability of the nowcast and expand the nowcast to other Ohio beaches and a recreational river. Although predictive models have been shown to work well at some beaches and are becoming more widely accepted, implementation in many areas is limited by funding, lack of coordinated technical leadership, and lack of supporting epidemiological data.


    Directory of Open Access Journals (Sweden)

    Markus Tilp


    Full Text Available The aims of the present study were to establish a detailed and representative record of landing techniques (two-, left-, and right-footed landings in professional beach volleyball and compare the data with those of indoor volleyball. Beach volleyball data was retrieved from videos taken at FIVB World Tour tournaments. Landing techniques were compared in the different beach and indoor volleyball skills serve, set, attack, and block with regard to sex, playing technique, and court position. Significant differences were observed between men and women in landings following block actions (χ²(2 = 18.19, p < 0.01 but not following serve, set, and attack actions. Following blocking, men landed more often on one foot than women. Further differences in landings following serve and attack with regard to playing technique and position were mainly observed in men. The comparison with landing techniques in indoor volleyball revealed overall differences both in men (χ²(2 = 161.4, p < 0.01 and women (χ²(2 = 84.91, p < 0.01. Beach volleyball players land more often on both feet than indoor volleyball players. Besides the softer surface in beach volleyball, and therefore resulting lower loads, these results might be another reason for fewer injuries and overuse conditions compared to indoor volleyball

  1. Lake Beach Monitoring Locations in Iowa (United States)

    Iowa State University GIS Support and Research Facility — Monitored state lake beach locations in Iowa. The Watershed Monitoring & Assessment Section of the Iowa DNR takes regular water samples at these listed beaches...

  2. Heterotrophic bacterial populations in tropical sandy beaches

    Digital Repository Service at National Institute of Oceanography (India)

    Nair, S.; LokaBharathi, P.A.

    Distribution pattern of heterotrophic bacterial flora of three sandy beaches of the west coast of India was studied. The population in these beaches was microbiologically different. Population peaks of halotolerant and limnotolerant forms were...

  3. Studies on beach changes at Visakhapatnam

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Rao, T.V.N.; Rao, D.P.

    Various factors controlling the coastal processes at 7.5 km long Visakhapatnam Beach were investigated in detail. Studies reveal that the depositional and erosional phases differ from place to place along this coast. Major part of the beach...

  4. Santa Barbara Littoral Cell CRSMP Beaches 2009 (United States)

    California Natural Resource Agency — Several criteria were used for beach selection. BEACON 's Coastal Regional Sediment Management Plan included all of the most popular beaches in the two counties...

  5. 75 FR 13454 - Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY (United States)


    ...-AA08 Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY AGENCY: Coast... navigable waters of Huntington Bay, New York due to the annual Fran Schnarr Open Water Championships. The..., ``Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY'' (Docket number USCG...

  6. 75 FR 38710 - Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY (United States)


    ...-AA08 Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY AGENCY: Coast... Regulation on the navigable waters of Huntington Bay, New York due to the annual Fran Schnarr Open Water... ``Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY'' in the Federal...

  7. 78 FR 35596 - Special Local Regulation; Long Beach Regatta, Powerboat Race, Atlantic Ocean, Long Beach, NY (United States)


    ... 1625-AA08 Special Local Regulation; Long Beach Regatta, Powerboat Race, Atlantic Ocean, Long Beach, NY... temporary special local regulation on the navigable waters of the Atlantic Ocean off Long Beach, NY during... powerboat racing regatta. The event will be held on the Atlantic Ocean off Long Beach, NY and will feature...

  8. Mobilisation of toxic trace elements under various beach nourishments

    NARCIS (Netherlands)

    Pit, I.R.; Dekker, S.C.; Kanters, T.J.; Wassen, M.J.; Griffioen, J.


    To enhance protection and maintain wide beaches for recreation, beaches are replenished with sand: socalled beach nourishments. We compared four sites: two traditional beach nourishments, a mega beach nourishment and a reference without beach nourishment. Two sites contain calcareous-rich sand,

  9. Changes in mental state and behaviour in Huntington's disease. (United States)

    Eddy, Clare M; Parkinson, Ellice G; Rickards, Hugh E


    Changes in mental state and behaviour have been acknowledged in Huntington's disease since the original monograph in 1872 provided evidence of disinhibition and impaired social cognition. Behavioural problems can manifest before obvious motor symptoms and are frequently the most disabling part of the illness. Although pharmacological treatments are used routinely for psychiatric difficulties in Huntington's disease, the scientific evidence base for their use is somewhat sparse. Moreover, effective treatments for apathy and cognitive decline do not currently exist. Understanding the social cognitive impairments associated with Huntington's disease can assist management, but related therapeutic interventions are needed. Future research should aim to design rating scales for behaviour and mental state in Huntington's disease that can detect change in clinical trials. Generally, communication and understanding of behaviour and mental state in Huntington's would be enhanced by a clear conceptual framework that unifies ideas around movement, cognition, emotion, behaviour, and mental state, reflecting both the experience of the patient and their underlying neuropathology. Copyright © 2016 Elsevier Ltd. All rights reserved.

  10. The ecology of sandy beaches in Transkei

    African Journals Online (AJOL)

    Data from an ecological survey of three sandy beaches in. Transkei and from Gulu beach on the eastern Cape coast,. South Africa, are presented. Physical parameters such as beach profile, sand particle size, Eh and carbonate content, as well as abundance, composition, biomass and distribution of the macrofauna and ...

  11. Differentiating Experts' Anticipatory Skills in Beach Volleyball (United States)

    Canal-Bruland, Rouwen; Mooren, Merel; Savelsbergh, Geert J. P.


    In this study, we examined how perceptual-motor expertise and watching experience contribute to anticipating the outcome of opponents' attacking actions in beach volleyball. To this end, we invited 8 expert beach volleyball players, 8 expert coaches, 8 expert referees, and 8 control participants with no beach volleyball experience to watch videos…

  12. The beach ridges of India: A review

    Digital Repository Service at National Institute of Oceanography (India)

    Kunte, P.D.; Wagle, B.G.

    , and is presented in a consolidated form. Beach ridges of the east and west coast of India are grouped in thirteen-beach ridge complexes based on their association. Review indicates that the beach ridges of India are not older than the Holocene age...

  13. The ecology of sandy beaches in Natal

    African Journals Online (AJOL)

    The ecology of sandy beaches in Natal. A.H. Dye, A. Mclachlan and T. Wooldridge. Department of Zoology, University of Port Elizabeth, Port Elizabeth. Data from an ecological survey of four sandy beaches on the. Natal coast of South Africa are presented. Physical para· meters such as beach profile, particle size, moisture, ...

  14. Haplotype-based stratification of Huntington's disease. (United States)

    Chao, Michael J; Gillis, Tammy; Atwal, Ranjit S; Mysore, Jayalakshmi Srinidhi; Arjomand, Jamshid; Harold, Denise; Holmans, Peter; Jones, Lesley; Orth, Michael; Myers, Richard H; Kwak, Seung; Wheeler, Vanessa C; MacDonald, Marcy E; Gusella, James F; Lee, Jong-Min


    Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by expansion of a CAG trinucleotide repeat in HTT, resulting in an extended polyglutamine tract in huntingtin. We and others have previously determined that the HD-causing expansion occurs on multiple different haplotype backbones, reflecting more than one ancestral origin of the same type of mutation. In view of the therapeutic potential of mutant allele-specific gene silencing, we have compared and integrated two major systems of HTT haplotype definition, combining data from 74 sequence variants to identify the most frequent disease-associated and control chromosome backbones and revealing that there is potential for additional resolution of HD haplotypes. We have used the large collection of 4078 heterozygous HD subjects analyzed in our recent genome-wide association study of HD age at onset to estimate the frequency of these haplotypes in European subjects, finding that common genetic variation at HTT can distinguish the normal and CAG-expanded chromosomes for more than 95% of European HD individuals. As a resource for the HD research community, we have also determined the haplotypes present in a series of publicly available HD subject-derived fibroblasts, induced pluripotent cells, and embryonic stem cells in order to facilitate efforts to develop inclusive methods of allele-specific HTT silencing applicable to most HD patients. Our data providing genetic guidance for therapeutic gene-based targeting will significantly contribute to the developments of rational treatments and implementation of precision medicine in HD.

  15. Bali beach conservation project and issues related to beach maintenance after completion of project


    Onaka, S.; Endo, S.; Uda, T.


    Bali Island in Indonesia is a world-famous resort area, and the beaches composed of coral sand are one of the most important resources for tourism. However, serious beach erosion has occurred since the 1970s owing to the tourism development along the coastal areas. To recover previous natural sandy beaches, Bali Beach Conservation Project was undertaken by the Indonesian Government as the ODA project financed by Japan. Three seriously eroded beaches (Sanur, Nusa Dua and Kuta) with a total len...

  16. Calcium Handling by Endoplasmic Reticulum and Mitochondria in a Cell Model of Huntington's Disease. (United States)

    De Mario, Agnese; Scarlatti, Chiara; Costiniti, Veronica; Primerano, Simona; Lopreiato, Raffaele; Calì, Tito; Brini, Marisa; Giacomello, Marta; Carafoli, Ernesto


    Huntington disease (HD) is caused by the CAG (Q) expansion in exon 1 of the IT15 gene encoding a polyglutamine (poly-Q) stretch of the Huntingtin protein (Htt). In the wild type protein, the repeats specify a stretch of up 34 Q in the N-terminal portion of Htt. In the pathological protein (mHtt) the poly-Q tract is longer. Proteolytic cleavage of the protein liberates an N-terminal fragment containing the expanded poly-Q tract becomes harmful to cells, in particular to striatal neurons. The fragments cause the transcriptional dysfunction of genes that are essential for neuronal survival. Htt, however, could also have non-transcriptional effects, e.g. it could directly alter Ca2+ homeostasis and/or mitochondrial morphology and function. Ca2+ dyshomeostasis and mitochondrial dysfunction are considered important in the molecular aetiology of the disease. Here we have analyzed the effect of the overexpression of Htt fragments (18Q, wild type form, wtHtt and 150Q mutated form, mHtt) on Ca2+ homeostasis in striatal neuronal precursor cells (Q7/7). We have found that the transient overexpression of the Htt fragments increases Ca2+ transients in the mitochondria of cells stimulated with Ca2+-mobilizing agonists. The bulk Ca2+ transients in the cytosol were unaffected, but the Ca2+ content of the endoplasmic reticulum was significantly decreased in the case of mHtt expression. To rule out possible transcriptional effects due to the presence of mHtt, we have measured the mRNA level of a subunit of the respiratory chain complex II, whose expression is commonly altered in many HD models. No effects on the mRNA level was found suggesting that, in our experimental condition, transcriptional action of Htt is not occurring and that the effects on Ca2+ homeostasis were dependent to non-transcriptional mechanisms.

  17. Virtual Beach 3: user's guide (United States)

    Cyterski, Mike; Brooks, Wesley; Galvin, Mike; Wolfe, Kurt; Carvin, Rebecca; Roddick, Tonia; Fienen, Mike; Corsi, Steve


    Virtual Beach version 3 (VB3) is a decision support tool that constructs site-specific statistical models to predict fecal indicator bacteria (FIB) concentrations at recreational beaches. VB3 is primarily designed for beach managers responsible for making decisions regarding beach closures or the issuance of swimming advisories due to pathogen contamination. However, researchers, scientists, engineers, and students interested in studying relationships between water quality indicators and ambient environmental conditions will find VB3 useful. VB3 reads input data from a text file or Excel document, assists the user in preparing the data for analysis, enables automated model selection using a wide array of possible model evaluation criteria, and provides predictions using a chosen model parameterized with new data. With an integrated mapping component to determine the geographic orientation of the beach, the software can automatically decompose wind/current/wave speed and magnitude information into along-shore and onshore/offshore components for use in subsequent analyses. Data can be examined using simple scatter plots to evaluate relationships between the response and independent variables (IVs). VB3 can produce interaction terms between the primary IVs, and it can also test an array of transformations to maximize the linearity of the relationship The software includes search routines for finding the "best" models from an array of possible choices. Automated censoring of statistical models with highly correlated IVs occurs during the selection process. Models can be constructed either using previously collected data or forecasted environmental information. VB3 has residual diagnostics for regression models, including automated outlier identification and removal using DFFITs or Cook's Distances.

  18. Embodied emotion impairment in Huntington's Disease. (United States)

    Trinkler, Iris; Devignevielle, Sévérine; Achaibou, Amal; Ligneul, Romain V; Brugières, Pierre; Cleret de Langavant, Laurent; De Gelder, Beatrice; Scahill, Rachael; Schwartz, Sophie; Bachoud-Lévi, Anne-Catherine


    Theories of embodied cognition suggest that perceiving an emotion involves somatovisceral and motoric re-experiencing. Here we suggest taking such an embodied stance when looking at emotion processing deficits in patients with Huntington's Disease (HD), a neurodegenerative motor disorder. The literature on these patients' emotion recognition deficit has recently been enriched by some reports of impaired emotion expression. The goal of the study was to find out if expression deficits might be linked to a more motoric level of impairment. We used electromyography (EMG) to compare voluntary emotion expression from words to emotion imitation from static face images, and spontaneous emotion mimicry in 28 HD patients and 24 matched controls. For the latter two imitation conditions, an underlying emotion understanding is not imperative (even though performance might be helped by it). EMG measures were compared to emotion recognition and to the capacity to identify and describe emotions using alexithymia questionnaires. Alexithymia questionnaires tap into the more somato-visceral or interoceptive aspects of emotion perception. Furthermore, we correlated patients' expression and recognition scores to cerebral grey matter volume using voxel-based morphometry (VBM). EMG results replicated impaired voluntary emotion expression in HD. Critically, voluntary imitation and spontaneous mimicry were equally impaired and correlated with impaired recognition. By contrast, alexithymia scores were normal, suggesting that emotion representations on the level of internal experience might be spared. Recognition correlated with brain volume in the caudate as well as in areas previously associated with shared action representations, namely somatosensory, posterior parietal, posterior superior temporal sulcus (pSTS) and subcentral sulcus. Together, these findings indicate that in these patients emotion deficits might be tied to the "motoric level" of emotion expression. Such a double

  19. An improved assay for the determination of Huntington`s disease allele size

    Energy Technology Data Exchange (ETDEWEB)

    Reeves, C.; Klinger, K.; Miller, G. [Intergrated Genetics, Framingham, MA (United States)


    The hallmark of Huntington`s disease (HD) is the expansion of a polymorphic (CAG)n repeat. Several methods have been published describing PCR amplification of this region. Most of these assays require a complex PCR reaction mixture to amplify this GC-rich region. A consistent problem with trinucleotide repeat PCR amplification is the presence of a number of {open_quotes}stutter bands{close_quotes} which may be caused by primer or amplicon slippage during amplification or insufficient polymerase processivity. Most assays for HD arbitrarily select a particular band for diagnostic purposes. Without a clear choice for band selection such an arbitrary selection may result in inconsistent intra- or inter-laboratory findings. We present an improved protocol for the amplification of the HD trinucleotide repeat region. This method simplifies the PCR reaction buffer and results in a set of easily identifiable bands from which to determine allele size. HD alleles were identified by selecting bands of clearly greater signal intensity. Stutter banding was much reduced thus permitting easy identification of the most relevant PCR product. A second set of primers internal to the CCG polymorphism was used in selected samples to confirm allele size. The mechanism of action of N,N,N trimethylglycine in the PCR reaction is not clear. It may be possible that the minimal isostabilizing effect of N,N,N trimethylglycine at 2.5 M is significant enough to affect primer specificity. The use of N,N,N trimethylglycine in the PCR reaction facilitated identification of HD alleles and may be appropriate for use in other assays of this type.

  20. Remifentanil in a patient with Huntington's chorea - case report ...

    African Journals Online (AJOL)

    Relatively few published case reports related to the anaesthetic management of Huntington's chorea (HC) exist. At the time of surgery no publications were found related to remifentanil's use in patients with HC. This case report describes the management of a confirmed HC patient requiring urgent decompression of a spinal ...


    Directory of Open Access Journals (Sweden)

    Mirela Batta


    Full Text Available Background. Huntington disease occurrs rarely, it can be encountered not only by neurologists and psychiatrists but also by other medical practitioners. Its characteristic features are involuntary movements, cognitive disorders and gradual development of dementia. Diagnosis is given on the basis of these clinical features, positive familial anamnesis, with the laboratory exclusion of other neuropsychiatric diseases and with the help of neuroimaging methods (in particular NMR. The disease can be only confirmed by means of genetic analysis.Patients and methods. In this article, four cases of patients with Huntington disease and diverse psychiatric disorders that were hospitalised at the psychiatric department of the Maribor General Hospital between October 2002 and March 2003 are described. All the patients fulfilled the valid criteria for the diagnosis of Huntington disease. However, they differed according to their accompanying psychiatric psychopathology, age and social problems.Conclusions. The purpose of this article is to draw attention to different psychiatric symptoms and clinical manifestations of Huntington disease that are often misleading in the diagnostic process. In addition, exigency of early diagnostics, guidelines for referrals to genetic testing and psychiatric monitoring of these patients are emphasised.

  2. Ethical issues and Huntington's disease | Kromberg | South African ...

    African Journals Online (AJOL)

    The practice of genetic counselling gives rise to many ethical dilemmas, and counsellors need to be familiar with the principles of biomedical ethics. The primary principles include respect for autonomy, beneficence, non-maleficence and justice. A case of identical twins at 50% risk for Huntington's disease, in which only one ...

  3. The emotional experiences of family carers in Huntington disease. (United States)

    Williams, Janet K; Skirton, Heather; Paulsen, Jane S; Tripp-Reimer, Toni; Jarmon, Lori; McGonigal Kenney, Meghan; Birrer, Emily; Hennig, Bonnie L; Honeyford, Joann


    This paper is a report of a study conducted to examine the emotional experience of caregiving by family carers of people with Huntington disease and to describe strategies they used to deal with that experience. Huntington disease, commonly diagnosed in young to middle adulthood, is an inherited single gene disorder involving loss of cognitive, motor and neuropsychiatric function. Many family members become caregivers as well as continuing as parents and wage earners. The emotional aspects of caregiving contribute to mental health risks for family members. Focus groups were conducted with 42 adult carers of people with Huntington disease in four United States and two Canadian Huntington disease centers between 2001 and 2005. Data were analyzed through descriptive coding and thematic analysis. All participants reported multiple aspects of emotional distress. Being a carer was described as experiencing disintegration of one's life. Carers attempted to cope by seeking comfort from selected family members, anticipating the time when the care recipient had died and/or using prescription medications. Spousal carers were distressed by the loss of their relationship with their spouse and dealt with this by no longer regarding the person as an intimate partner. Carers were concerned about the disease risk for children in their families and hoped for a cure. Emotional distress can compromise the well-being of family carers, who attempt to maintain multiple roles. Nurses should monitor carer mental health, identify sources of emotional distress and support effective strategies used by carers to mediate distress.

  4. Semantic, phonologic, and verb fluency in Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Jardim Azambuja

    Full Text Available Abstract Verbal fluency tasks have been identified as important indicators of executive functioning impairment in patients with frontal lobe dysfunction. Although the usual evaluation of this ability considers phonologic and semantic criteria, there is some evidence that fluency of verbs would be more sensitive in disclosing frontostriatal physiopathology since frontal regions primarily mediate retrieval of verbs. Huntington's disease usually affects these circuitries. Objective: To compare three types of verbal fluency task in the assessment of frontal-striatal dysfunction in HD subjects. Methods: We studied 26 Huntington's disease subjects, divided into two subgroups: mild (11 and moderate (15 along with 26 normal volunteers matched for age, gender and schooling, for three types of verbal fluency: phonologic fluency (F-A-S, semantic fluency and fluency of verbs. Results: Huntington's disease subjects showed a significant reduction in the number of words correctly generated in the three tasks when compared to the normal group. Both controls and Huntington's disease subjects showed a similar pattern of decreasing task performance with the greatest number of words being generated by semantic elicitation followed by verbs and lastly phonologic criteria. We did not find greater production of verbs compared with F-A-S and semantic conditions. Moreover, the fluency of verbs distinguished only the moderate group from controls. Conclusion: Our results indicated that phonologic and semantic fluency can be used to evaluate executive functioning, proving more sensitive than verb fluency. However, it is important to point out that the diverse presentations of Huntington's disease means that an extended sample is necessary for more consistent analysis of this issue.

  5. Major Superficial White Matter Abnormalities in Huntington's Disease (United States)

    Phillips, Owen R.; Joshi, Shantanu H.; Squitieri, Ferdinando; Sanchez-Castaneda, Cristina; Narr, Katherine; Shattuck, David W.; Caltagirone, Carlo; Sabatini, Umberto; Di Paola, Margherita


    Background: The late myelinating superficial white matter at the juncture of the cortical gray and white matter comprising the intracortical myelin and short-range association fibers has not received attention in Huntington's disease. It is an area of the brain that is late myelinating and is sensitive to both normal aging and neurodegenerative disease effects. Therefore, it may be sensitive to Huntington's disease processes. Methods: Structural MRI data from 25 Pre-symptomatic subjects, 24 Huntington's disease patients and 49 healthy controls was run through a cortical pattern-matching program. The surface corresponding to the white matter directly below the cortical gray matter was then extracted. Individual subject's Diffusion Tensor Imaging (DTI) data was aligned to their structural MRI data. Diffusivity values along the white matter surface were then sampled at each vertex point. DTI measures with high spatial resolution across the superficial white matter surface were then analyzed with the General Linear Model to test for the effects of disease. Results: There was an overall increase in the axial and radial diffusivity across much of the superficial white matter (p < 0.001) in Pre-symptomatic subjects compared to controls. In Huntington's disease patients increased diffusivity covered essentially the whole brain (p < 0.001). Changes are correlated with genotype (CAG repeat number) and disease burden (p < 0.001). Conclusions: This study showed broad abnormalities in superficial white matter even before symptoms are present in Huntington's disease. Since, the superficial white matter has a unique microstructure and function these abnormalities suggest it plays an important role in the disease. PMID:27242403

  6. Evaluating Recall and Recognition Memory Using the Montreal Cognitive Assessment: Applicability for Alzheimer's and Huntington's Diseases. (United States)

    Van Liew, Charles; Santoro, Maya S; Goldstein, Jody; Gluhm, Shea; Gilbert, Paul E; Corey-Bloom, Jody


    We sought to investigate whether the Montreal Cognitive Assessment (MoCA) could provide a brief assessment of recall and recognition using Huntington disease (HD) and Alzheimer disease (AD) as disorders characterized by different memory deficits. This study included 80 participants with HD, 64 participants with AD, and 183 community-dwelling control participants. Random-effects hierarchical logistic regressions were performed to assess the relative performance of the normal control (NC), participants with HD, and participants with AD on verbal free recall, cued recall, and multiple-choice recognition on the MoCA. The NC participants performed significantly better than participants with AD at all the 3 levels of assessment. No difference existed between participants with HD and NC for cued recall, but NC participants performed significantly better than participants with HD on free recall and recognition. The participants with HD performed significantly better than participants with AD at all the 3 levels of assessment. The MoCA appears to be a valuable, brief cognitive assessment capable of identifying specific memory deficits consistent with known differences in memory profiles. © The Author(s) 2016.

  7. Beach and Morphology Change Using Lidar (United States)


    of Expertise. Beach profile surveys were provided by USACE Jacksonville District (SAJ), University of South Florida (USF), and Coastal Planning ...the Gulf of Mexico from Clearwater Beach in Pinellas County, FL, to Venice Beach in Sarasota County, FL (Figure 1). Active Federal projects existing...since the early 1900s. At present, most of the shoreline is considered to be urban . The coastal area is directly under the influence of past and present

  8. Beach Observations using Quadcopter Imagery (United States)

    Yang, Yi-Chung; Wang, Hsing-Yu; Fang, Hui-Ming; Hsiao, Sung-Shan; Tsai, Cheng-Han


    Beaches are the places where the interaction of the land and sea takes place, and it is under the influence of many environmental factors, including meteorological and oceanic ones. To understand the evolution or changes of beaches, it may require constant monitoring. One way to monitor the beach changes is to use optical cameras. With careful placements of ground control points, land-based optical cameras, which are inexpensive compared to other remote sensing apparatuses, can be used to survey a relatively large area in a short time. For example, we have used terrestrial optical cameras incorporated with ground control points to monitor beaches. The images from the cameras were calibrated by applying the direct linear transformation, projective transformation, and Sobel edge detector to locate the shoreline. The terrestrial optical cameras can record the beach images continuous, and the shorelines can be satisfactorily identified. However, the terrestrial cameras have some limitations. First, the camera system set a sufficiently high level so that the camera can cover the whole area that is of interest; such a location may not be available. The second limitation is that objects in the image have a different resolution, depending on the distance of objects from the cameras. To overcome these limitations, the present study tested a quadcopter equipped with a down-looking camera to record video and still images of a beach. The quadcopter can be controlled to hover at one location. However, the hovering of the quadcopter can be affected by the wind, since it is not positively anchored to a structure. Although the quadcopter has a gimbal mechanism to damp out tiny shakings of the copter, it will not completely counter movements due to the wind. In our preliminary tests, we have flown the quadcopter up to 500 m high to record 10-minnte video. We then took a 10-minute average of the video data. The averaged image of the coast was blurred because of the time duration of

  9. Factor analysis of the hospital anxiety and depression scale among a Huntington's disease population

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Martucci, Rossana


    INTRODUCTION: Depression and anxiety are common in Huntington's disease, a genetic neurodegenerative disorder. There is a need for measurement tools of mood to be validated within a Huntington's disease population. The current study aimed to analyze the factor structure of the Hospital Anxiety...... and Depression Scale in Huntington's disease. METHODS: Data from the European Huntington's Disease Network study REGISTRY 3 were used to undertake a factor analysis of the scale among a sample of 492 Huntington's disease mutation carriers. The sample was randomly divided into two equal subsamples...... support for an eight-item version of the scale to be used as a measure of general distress within Huntington's disease populations. © 2015 International Parkinson and Movement Disorder Society....

  10. Disease stage, but not sex, predicts depression and psychological distress in Huntington's disease

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Shimozaki, Steve


    OBJECTIVE: Depression and anxiety significantly affect morbidity in Huntington's disease. Mice. models of Huntington's disease have identified sex differences in mood-like behaviours that vary across disease lifespan, but this interaction has not previously been explored in humans with Huntington......'s disease. However, among certain medical populations, evidence of sex differences in mood across various disease stages has been found, reflecting trends among the general population that women tend to experience anxiety and depression 1.5 to 2 times more than men. The current study examined whether...... disease stage and sex, either separately or as an interaction term, predicted anxiety and depression in Huntington's disease. METHODS: A cross-sectional study of REGISTRY data involving 453 Huntington's disease participants from 12 European countries was undertaken using the Hospital Anxiety...

  11. Topographic changes of the beach at Valiathura, Kerala

    Digital Repository Service at National Institute of Oceanography (India)

    Murty, C.S.; Varadachari, V.V.R.

    Studies on the topography of the beach and the inshore reaches at Valiathura, near Trivandrum reveal that the beach is in a stable equilibrium and presents well defined cyclicity in accretion and erosion every year. During 1 year, the beach...

  12. Studies on Thiobacilli spp. isolated from sandy beaches of Kerala

    Digital Repository Service at National Institute of Oceanography (India)

    Gore, P.S.; Raveendran, O.; Unnithan, R.V.

    Occurrence, isolation and oxidative activity of Thiobacilli spp. from some sandy beaches of Kerala are reported. These organisms were encountered in polluted beaches and were dominant during monsoon in all the beaches...

  13. Huntington disease: a case study of early onset presenting as depression. (United States)

    Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael


    Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and rigidity, is reported. Meanwhile, the father developed the adult variant of Huntington disease. The boy's diagnosis was confirmed by molecular genetic analysis and magnetic resonance imaging. It is important to be aware of hereditary conditions such as Huntington disease and to provide family counseling before genetic testing and after the diagnosis is confirmed.

  14. The Huntington disease locus is most likely within 325 kilobases of the chromosome 4p telomere

    International Nuclear Information System (INIS)

    Doggett, N.A.; Cheng, J.F.; Smith, C.L.; Cantor, C.R.


    The genetic defect responsible for Huntington disease was originally localized near the tip of the short arm of chromosome 4 by genetic linkage to the locus D4S10. Several markers closer to Huntington disease have since been isolated, but these all appear to be proximal to the defect. A physical map that extends from the most distal of these loci, D4S90, to the telomere of chromosome 4 was constructed. This map identifies at least two CpG islands as markers for Huntington disease candidate genes and places the most likely location of the Huntington disease defect remarkably close (within 325 kilobases) to the telomere

  15. Five years of beach drainage survey on a macrotidal beach (Quend-Plage, northern France) (United States)

    Bain, Olivier; Toulec, Renaud; Combaud, Anne; Villemagne, Guillaume; Barrier, Pascal


    A drainage system was installed in 2008 on the macrotidal beach of Quend-Plage, close to Abbeville (Somme, northern France), following a period of significant erosion of recreational areas. The "Direction départementale des territoires et de la mer" (French Coastal Department Authority) has requested a biannual survey in order to validate the beach drainage setup and its efficiency. This paper presents the methodology used for this survey, and the response of the coastal system to this soft engineering method for preventing erosion. These five years of drainage operation have strongly modified the morphology of the beach. Three main modifications occurred: (i) accretion of the upper beach and foredune, (ii) erosion of the lower and middle beach and (iii) a slight shift in directions of the beach bars and troughs. These morphological changes finally led to the stabilization of the beach.

  16. Psychodynamic theory and counseling in predictive testing for Huntington's disease. (United States)

    Tassicker, Roslyn J


    This paper revisits psychodynamic theory, which can be applied in predictive testing counseling for Huntington's Disease (HD). Psychodynamic theory has developed from the work of Freud and places importance on early parent-child experiences. The nature of these relationships, or attachments are reflected in adult expectations and relationships. Two significant concepts, identification and fear of abandonment, have been developed and expounded by the psychodynamic theorist, Melanie Klein. The processes of identification and fear of abandonment can become evident in predictive testing counseling and are colored by the client's experience of growing up with a parent affected by Huntington's Disease. In reflecting on family-of-origin experiences, clients can also express implied expectations of the future, and future relationships. Case examples are given to illustrate the dynamic processes of identification and fear of abandonment which may present in the clinical setting. Counselor recognition of these processes can illuminate and inform counseling practice.

  17. Family caregivers' views on coordination of care in Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C


    BACKGROUND: Collaboration between family caregivers and health professionals in specialised hospitals or community-based primary healthcare systems can be challenging. During the course of severe chronic disease, several health professionals might be involved at a given time, and the patient......'s illness may be unpredictable or not well understood by some of those involved in the treatment and care. AIM: The aim of this study was to explore the experiences and expectations of family caregivers for persons with Huntington's disease concerning collaboration with healthcare professionals. METHODS......: To shed light on collaboration from the perspectives of family caregivers, we conducted an explorative, qualitative interview study with 15 adult participants experienced from caring for family members in all stages of Huntington's disease. Data were analysed with systematic text condensation, a cross...

  18. Caregiver roles in families affected by Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C


    AIM: The objective of this study was to explore family caregivers' experiences with the impact of Huntington's disease (HD) on the family structure and roles in the family. METHODOLOGY: We interviewed 15 family caregivers in families affected by HD, based on a semi-structured interview guide...... for impairments by taking on adult responsibilities, and in some families, a child had the role as main caregiver. The increasing need for care could cause conflicts between the role as family member and family caregiver. The burden of care within the family could fragment and isolate the family. CONCLUSIONS......: Huntington's disease has a major impact on family systems. Caregiver roles are shaped by impairments in the affected family member and corresponding dynamic adoption and change in roles within the family. Making assessments of the family structure and roles, professionals may understand more about how...

  19. Tidal flow separation at protruding beach nourishments

    NARCIS (Netherlands)

    Radermacher, M.; de Schipper, M.A.; Swinkels, Cilia M.; MacMahan, Jamie; Reniers, A.J.H.M.


    In recent years, the application of large-scale beach nourishments has been discussed, with the Sand Motor in the Netherlands as the first real-world example. Such protruding beach nourishments have an impact on tidal currents, potentially leading to tidal flow separation and the generation of tidal

  20. Morphological changes of the beaches of Goa

    Digital Repository Service at National Institute of Oceanography (India)

    Murty, C.S.; Veerayya, M.; Varadachari, V.V.R.

    Morphological variations of 2 of the major beaches of Goa have been found to be cyclic over a period of approximately 1 yr. These beaches attain their maximum sediment storage around April/May. They are then subjected to rapid rates of erosion...

  1. Differentiating experts’ anticipatory skills in beach volleyball

    NARCIS (Netherlands)

    Canal Bruland, R.; Mooren, M.; Savelsbergh, G.J.P.


    In this study, we examined how perceptual-motor expertise and watching experience contribute to anticipating the outcome of opponents' attacking actions in beach volleyball. To this end, we invited 8 expert beach volleyball players, 8 expert coaches, 8 expert referees, and 8 control participants

  2. Stability and safety of Anjuna beach, Goa

    Digital Repository Service at National Institute of Oceanography (India)

    Swamy, G.N.; Varadachari, V.V.R.

    are generally weak and the rip currents are very rare. The sweep zone is around 1.5 m in the foreshore of the beach. Investigations on beach volume indicate that sand bypasses Baga promontory and moves northward and gets locked up in the southern part of Anjuna...

  3. Striatal grafts in a rat model of Huntington's disease

    DEFF Research Database (Denmark)

    Guzman, R; Meyer, M; Lövblad, K O


    Survival and integration into the host brain of grafted tissue are crucial factors in neurotransplantation approaches. The present study explored the feasibility of using a clinical MR scanner to study striatal graft development in a rat model of Huntington's disease. Rat fetal lateral ganglionic...... time-points graft location could not be further verified. Measures for graft size and ventricle size obtained from MR images highly correlated with measures obtained from histologically processed sections (R = 0.8, P fetal rat lateral ganglionic...

  4. Challenges of Huntington's disease and quest for therapeutic biomarkers

    Czech Academy of Sciences Publication Activity Database

    Kotrčová, Eva; Jarkovská, Karla; Valeková, Ivona; Žižková, Martina; Motlík, Jan; Gadher, S. J.; Kovářová, Hana


    Roč. 9, 1-2 (2015), s. 147-158 ISSN 1862-8346 R&D Projects: GA MŠk ED2.1.00/03.0124; GA TA ČR(CZ) TA01011466 Institutional support: RVO:67985904 Keywords : HD biomarkers * Huntington´s disease * Huntingtin neurotoxicity * Huntingtin pathogenesis Subject RIV: FH - Neurology Impact factor: 2.959, year: 2015

  5. Effects of beach cast cleaning on beach quality, microbial food web, and littoral macrofaunal biodiversity (United States)

    Malm, Torleif; Råberg, Sonja; Fell, Sabine; Carlsson, Per


    At the end of the summer, drifting filamentous red algae cover shallow bottoms and accumulate in huge cast walls on the open shores of the non-tidal central Baltic Sea. The hypotheses that beach cleaning increases water clarity, decreases the organic content of the sand, and increases the species diversity in the shallow zone closest to the shore, were tested through field investigations and experiments. Cleaned shorelines were compared with un-cleaned shorelines at two sites with different intensity of beach cleaning in a rural area of SE Sweden. The results show that water clarity was significantly increased off the intensively cleaned beach but not off the moderately cleaned one. Similarly, the total leakage of nitrogenous compounds decreased off the intensively cleaned beach, but not off the moderately cleaned. The organic content of the sand was lower on both cleaned beaches compared with nearby un-cleaned beaches. The total animal biomass was significantly lower on the intensively cleaned beach compared with the un-cleaned beach, but the moderately cleaned beach gave no such effect. The difference in biodiversity and community structure between cleaned and un-cleaned beaches was insignificant. The most obvious difference in species composition was a much higher number of planktivore opossum shrimps of the genus Mysis and Praunus on the un-cleaned beaches. The bacterial production and the amount of ciliates larger than 20 mm were also higher on un-cleaned beaches, indicating that the microbial food web off the un-cleaned beaches is stimulated by the discharge of decomposing algal material. The conclusion of the study is that mechanical cleaning reduces the organic content of the beach sand and may change the water quality and microbial production, but the effect on the macrofaunal biodiversity is insignificant.

  6. Monitoring beach changes using GPS surveying techniques (United States)

    Morton, Robert; Leach, Mark P.; Paine, Jeffrey G.; Cardoza, Michael A.


    A need exists for frequent and prompt updating of shoreline positions, rates of shoreline movement, and volumetric nearshore changes. To effectively monitor and predict these beach changes, accurate measurements of beach morphology incorporating both shore-parallel and shore-normal transects are required. Although it is possible to monitor beach dynamics using land-based surveying methods, it is generally not practical to collect data of sufficient density and resolution to satisfy a three-dimensional beach-change model of long segments of the coast. The challenge to coastal scientists is to devise new beach monitoring methods that address these needs and are rapid, reliable, relatively inexpensive, and maintain or improve measurement accuracy.

  7. Extreme beach retreat history inferred from cut-and-fill beach deposits at Moruya, SE Australia (United States)

    Tamura, T.; Woodroffe, C. D.; Oliver, T.; Cunningham, A. C.


    A sequence of beach ridges often records a `cut and fill', where the fair-weather swash accretion of beach sand is punctuated by storm erosion. The detailed chronology of the sequence is thus a clue to decipher past storm events and associated beach erosion, but has not been explored much. Here we explore the potential of such a sequence to detect past extreme retreats in Bengello Beach at Moruya, southeastern Australia. Beach monitoring since 1972 reveals that Bengello beach has shown a typical cut and fill, in which the beach retreats several tens of meters in relation to storms and recovers within a following few years. A storm event caused extreme retreat up to 50 m in 1974. Since then, no retreat exceeded 30 m. The beach monitoring highlights the sporadic nature of the prograded beach deposits; they can only be preserved as stratigraphic records during rapid beach recovery following a large retreat deeper than the beach profile envelope. Thus, ages of the preserved beach deposits roughly correspond to timings of large retreat. Optically-stimulated luminescence (OSL) ages were determined for beach deposits at 5-10 m intervals along a shore-normal transect from the modern foredune to beach ridge 120 m inland. The most landward sample was dated as 510 yr, indicating that the net progradation rate is 0.24 m/yr, concordant with both the long- and short-term rates since the mid Holocene and 1972, respectively. Other ages show four events of retreat around 350, 180, 130 and 90 yr, and also reflect the beach scarp resulting from the 1974 event. The retreat of each event is given by the distance between the shoreline position prior to storm erosion and relevant gap in OSL age. The position of the pre-storm shoreline is estimated by assuming a constant rate of the net progradation of 0.24 m/yr, as with long- and short-term rates. The retreat of the four events is then determined as 45-55 m, similar to the 1974 event. In summary, extreme beach retreats, including that in

  8. aliving with Huntington´s disease in the Czech Republic

    Czech Academy of Sciences Publication Activity Database

    Baxa, Monika


    Roč. 78, Suppl 2 (2015), s. 6-6 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] Institutional support: RVO:67985904 Keywords : Czech Huntington Association * life with Huntington ´ disease Subject RIV: FH - Neurology

  9. 77 FR 71636 - Huntington Foam LLC, Fort Smith, AR; Notice of Revised Determination on Reconsideration (United States)


    ... Smith, AR; Notice of Revised Determination on Reconsideration On August 8, 2012, the Department of Labor... workers and former workers of Huntington Foam LLC, Fort Smith, Arkansas (subject firm). The workers are... reconsideration investigation, I determine that workers of Huntington Foam LLC, Fort Smith, Arkansas, who were...

  10. Beach Advisory and Closing Online Notification (BEACON) system (United States)

    Beach Advisory and Closing Online Notification system (BEACON) is a colletion of state and local data reported to EPA about beach closings and advisories. BEACON is the public-facing query of the Program tracking, Beach Advisories, Water quality standards, and Nutrients database (PRAWN) which tracks beach closing and advisory information.

  11. Tar loads on Omani beaches

    International Nuclear Information System (INIS)

    Badawy, M.I.; Al-Harthy, F.T.


    Owing to Oman's geographic position and long coastal line, the coastal areas of Oman are particularly vulnerable to oil pollution from normal tanker operations, illegal discharges, and accidental spills as well as local sources of oil input. UNEP carried out a survey on the coasts of Oman to determine the major sources of oil pollution and concluded that the major shoreline pollution problems in Oman arose from operational discharges of oil from passing vessels traffic. The oil, because of the high sea and air temperatures in the area, was subjected to relatively high rates of evaporation and photo-oxidation and tended to arrive at the coast as heavy petroleum particulate residues (tar balls). The aim of the present study was to measure the loads of tar balls in Omani coastal areas and to identify the source of oil pollutants on beaches

  12. Did the "Woman in the Attic" in Jane Eyre Have Huntington Disease? (United States)

    Coon, Elizabeth A; Hassan, Anhar


    References to neurologic disorders are frequently found in fictional literature and may precede description in the medical literature. Our aim was to compare Charlotte Brontë's depiction of Bertha Mason in Jane Eyre to the tenets set forth in George Huntington's original essay "On chorea" with the hypothesis that Mason was displaying features of Huntington disease. Charlotte Brontë's 1847 Victorian novel Jane Eyre features the character Bertha Mason, who is portrayed with a progressive psychiatric illness, violent movements, and possible cognitive decline. Similar to Huntington's tenets, Mason has a disorder with a strong family history suggestive of autosomal dominant inheritance with onset in adulthood, and culminating in suicide. Brontë's character had features of Huntington disease as originally described by Huntington. Brontë's keen characterization may have increased awareness of treatment of neuropsychiatric patients in the Victorian era.

  13. Morphodynamics of a mesotidal rocky beach: Palmeras beach, Gorgona Island National Natural Park, Colombia (United States)

    Gómez-García, A. M.; Bernal, G. R.; Osorio, A. F.; Botero, V.


    The response of a rocky beach to different possible combinations of hydrodynamic conditions (tides, waves, oceanic currents) has been little studied. In this work, the morphodynamic response to different hydrodynamic forcing is evaluated from sedimentological and geomorphological analysis in seasonal and medium term (19 years) scale in Palmeras beach, located in the southwest of Gorgona Island National Natural Park (NNP), a mesotidal rocky island on the Colombian Pacific continental shelf. Palmeras is an important nesting area of two types of marine turtles, with no anthropogenic stress. In the last years, coastal erosion has reduced the beach width, restricting the safe areas for nesting and conservation of these species. Until now, the sinks, sources, reservoirs, rates, and paths of sediments were unknown, as well as their hydrodynamic forcing. The beach seasonal variability, from October 2010 to August 2012, was analyzed based on biweekly or monthly measurements of five beach profiles distributed every 200 m along the 1.2 km of beach length. The main paths for sediment transport were defined from the modeling of wave currents with the SMC model (Coastal Modeling System), as well as the oceanic currents, simulated for the dry and wet seasons of 2011 using the ELCOM model (Estuary and Lake COmputer Model). Extreme morphologic variations over a time span of 19 years were analyzed with the Hsu and Evans beach static equilibrium parabolic model, from one wave diffraction point which dominates the general beach plan shape. The beach lost 672 m3/m during the measuring period, and erosional processes were intensified during the wet season. The beach trends responded directly to a wave mean energy flux change, resulting in an increase of up to 14 m in the width northward and loss of sediments in the beach southward. This study showed that to obtain the integral morphodynamic behavior of a rocky beach it is necessary to combine information of hydrodynamic, sedimentology

  14. Gone to the Beach — Using GIS to infer how people value different beaches for salt water recreation. (United States)

    Estimating the non-market value of beaches for saltwater recreation is complex. An individual’s preference for a beach depends on their perception of beach characteristics. When choosing one beach over another, an individual balances these personal preferences with any addi...

  15. Beach Nourishment History (1920s to 2000) (United States)

    California Natural Resource Agency — This is a dataset of beach nourishment history for the California Coastline from the 1920s to 2000. The original data was in tabular form (an Excel spreadsheet) and...

  16. Measurement of biological oxygen demand sandy beaches

    African Journals Online (AJOL)

    Measurements of biological oxygen demand in a sandy beach using conventional .... counting the cells present in a sample of aged seawater and comparing this with .... This activity peaked at 71 % above the undisturbed level after 16 hours.

  17. March 1933 Long Beach, USA Images (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — 5 kilometers southwest of Newport Beach. Seriously affected area: 1,200 square kilometers. Damage: $40 million. Schools were among the buildings most severely...

  18. Plastics and beaches: A degrading relationship

    International Nuclear Information System (INIS)

    Corcoran, Patricia L.; Biesinger, Mark C.; Grifi, Meriem


    Plastic debris in Earth's oceans presents a serious environmental issue because breakdown by chemical weathering and mechanical erosion is minimal at sea. Following deposition on beaches, plastic materials are exposed to UV radiation and physical processes controlled by wind, current, wave and tide action. Plastic particles from Kauai's beaches were sampled to determine relationships between composition, surface textures, and plastics degradation. SEM images indicated that beach plastics feature both mechanically eroded and chemically weathered surface textures. Granular oxidation textures were concentrated along mechanically weakened fractures and along the margins of the more rounded plastic particles. Particles with oxidation textures also produced the most intense peaks in the lower wavenumber region of FTIR spectra. The textural results suggest that plastic debris is particularly conducive to both chemical and mechanical breakdown in beach environments, which cannot be said for plastics in other natural settings on Earth

  19. Macrodebris and microplastics from beaches in Slovenia. (United States)

    Laglbauer, Betty J L; Franco-Santos, Rita Melo; Andreu-Cazenave, Miguel; Brunelli, Lisa; Papadatou, Maria; Palatinus, Andreja; Grego, Mateja; Deprez, Tim


    The amount of marine debris in the environment is increasing worldwide, which results in an array of negative effects to biota. This study provides the first account of macrodebris on the beach and microplastics in the sediment (shoreline and infralittoral) in relation to tourism activities in Slovenia. The study assessed the quality and quantity of macrodebris and the quality, size and quantity of microplastics at six beaches, contrasting those under the influences of tourism and those that were not. Beach cleanliness was estimated using the Clean Coast Index. Tourism did not seem to have an effect on macrodebris or microplastic quantity at beaches. Over 64% of macrodebris was plastic, and microplastics were ubiquitous, which calls for classification of plastics as hazardous materials. Standard measures for marine debris assessment are needed, especially in the form of an all-encompassing debris index. Recommendations for future assessments are provided for the Adriatic region. Copyright © 2014 Elsevier Ltd. All rights reserved.

  20. Cerebral oxygen desaturation during beach chair position

    NARCIS (Netherlands)

    Moerman, Annelies T.; de Hert, Stefan G.; Jacobs, Tom F.; de Wilde, Lieven F.; Wouters, Patrick F.


    Cases of ischaemic brain damage have been reported in relatively healthy patients undergoing shoulder surgery in the beach chair position. Unrecognised cerebral hypoperfusion may have contributed to these catastrophic events, indicating that routine anaesthesia monitoring may not suffice.

  1. Radiosensitivity in Huntington's disease: implications for pathogenesis and presymptomatic diagnosis

    International Nuclear Information System (INIS)

    Moshell, A.N.; Tarone, R.E.; Barrett, S.F.; Robbins, J.H.


    Huntington's disease (HD) is a dominantly inherited fatal disorder characterised by premature death of nerve cells. Cultured lymphocyte lines from four patients with HD were abnormally sensitive to the lethal effects of X rays, as were lines from two of five subjects at risk for HD. The hypersensitivity is specific for ionising radiation, since HD lines had normal survival after exposure to ultraviolet radiation. The hypersensitivity, which may reflect an inherited defect in DNA repair, provides the basis for a presymptomatic diagnostic test for the disease. (author)

  2. 75 FR 4344 - Foreign-Trade Zone 50-Long Beach, California Application for Subzone Allegro Mfg. Inc. (Cosmetic... (United States)


    ... DEPARTMENT OF COMMERCE Foreign-Trade Zones Board [Docket 6-2010] Foreign-Trade Zone 50--Long Beach, California Application for Subzone Allegro Mfg. Inc. (Cosmetic, Organizer and Electronic Bags and Accessories) Commerce, CA An application has been submitted to the Foreign-Trade Zones Board (the Board) by the Port of...

  3. Moving sands along a headland-embayed beach system (Algarve, Southern Portugal) (United States)

    Oliveira, Sónia; Horta, João; Nascimento, Ana; Gomes, Ana; Veiga-Pires, Cristina; Moura, Delminda


    Resilience of embayed and pocket beaches located at the southernmost coast of Portugal is currently a major question to coastal management of this region. In fact, several among those beaches have been artificially fed aiming to increase the width of the beach allowing people to maintain a safe distance to the unstable rocky cliffs. The sand is dredged from the offshore (ca. 2 miles from the shoreline) representing high costs for the Portuguese government. For how long will the artificial feeding solve the problem? Which beaches are worth being nourished taking into account the morphosedimentary processes? The present work is the result of a field experiment aiming to study the efficiency of the alongshore sedimentary transport between successive embayed beaches. The experiment was performed in the very indented rocky coast of the Algarve region (Southern Portugal) and comprised two field campaigns, both in 2014, during spring tides in March and November. The Algarve coast experiences a semi-diurnal meso-tidal regime ranging from 1.3 m during neap tides to 3.5 m at spring tides and the waves approach from WSW (232°) during 72% of observations along the year, almost normal to the study area shoreline. The wave and current characteristics (significant height-Hs and Period-T for waves, velocity and direction for currents) were measured during three and six tidal cycles respectively for the first and second campaign, using two pressure transducers and one electromagnetic current meter. We used sand painted with orange fluorescent dye (100 kg in March and 200 kg in November) as tracer to track the movement of the sand along the coast. The marked sand was placed on the beach face of the westernmost beach of the study area during the first low tide of each campaign. Following, hundreds of sediment samples were collected during low tide, through the monitored period, in the nodes of a georeferenced square mesh of 10 x 20 m covering three embayed beaches. Later in the

  4. Motor outcome measures in Huntington disease clinical trials. (United States)

    Reilmann, Ralf; Schubert, Robin


    Deficits in motor function are a hallmark of Huntington disease (HD). The Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS) is a categoric clinical rating scale assessing multiple domains of motor disability in HD. The UHDRS-TMS or subsets of its items have served as primary or secondary endpoints in numerous clinical trials. In spite of a well-established video-based annual online certification system, intra- and interrater variability, subjective error, and rater-induced placebo effects remain a concern. In addition, the UHDRS-TMS was designed to primarily assess motor symptoms in manifest HD. Recently, advancement of technology resulted in the introduction of the objective Q-Motor (i.e., Quantitative-Motor) assessments in biomarker studies and clinical trials in HD. Q-Motor measures detected motor signs in blinded cross-sectional and longitudinal analyses of manifest, prodromal, and premanifest HD cohorts up to two decades before clinical diagnosis. In a multicenter clinical trial in HD, Q-Motor measures were more sensitive than the UHDRS-TMS and exhibited no placebo effects. Thus, Q-Motor measures are currently explored in several multicenter trials targeting both symptomatic and disease-modifying mechanisms. They may supplement the UHDRS-TMS, increase the sensitivity and reliability in proof-of-concept studies, and open the door for phenotype assessments in clinical trials in prodromal and premanifest HD. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Huntington's Disease in a Patient Misdiagnosed as Conversion Disorder. (United States)

    Nogueira, João Machado; Franco, Ana Margarida; Mendes, Susana; Valadas, Anabela; Semedo, Cristina; Jesus, Gustavo


    Huntington's disease (HD) is an inherited, progressive, and neurodegenerative neuropsychiatric disorder caused by the expansion of cytosine-adenine-guanine (CAG) trinucleotide in Interested Transcript (IT) 15 gene on chromosome 4. This pathology typically presents in individuals aged between 30 and 50 years and the age of onset is inversely correlated with the length of the CAG repeat expansion. It is characterized by chorea, cognitive deficits, and psychiatric symptoms. Usually the psychiatric disorders precede motor and cognitive impairment, Major Depressive Disorder and anxiety disorders being the most common presentations. We present a clinical case of a 65-year-old woman admitted to our Psychiatric Acute Unit. During the 6 years preceding the admission, the patient had clinical assessments made several times by different specialties that focused only on isolated symptoms, disregarding the syndrome as a whole. In the course of her last admission, the patient was referred to our Neuropsychiatric Team, which made the provisional diagnosis of late-onset Huntington's disease, later confirmed by genetic testing. This clinical vignette highlights the importance of a multidisciplinary approach to atypical clinical presentations and raises awareness for the relevance of investigating carefully motor symptoms in psychiatric patients.

  6. Apolipoprotein E and presenilin-1 genotypes in Huntington's disease. (United States)

    Panas, M; Avramopoulos, D; Karadima, G; Petersen, M B; Vassilopoulos, D


    Huntington's disease (HD) is an autosomal dominant degenerative disease of the central nervous system manifested by involuntary movements (chorea), psychiatric manifestations, and cognitive impairment with a variable age at onset. This variability is mainly attributed to genetic factors. The so-called aging genes [e.g., those for apolipoprotein E (APOE) and presenilin-1 (PS-1) have been implicated in determining the age at onset of Alzheimer's disease, a disease sharing common clinical features with HD. In 60 unrelated patients suffering from HD (mean age at onset 40.1 years, range 20-65) we determined number of CAG repeats and the distribution of the APOE alleles (epsilon2, epsilon3, epsilon4) and PS-1 alleles. The results showed that: (a) The age at onset was higher in the group of patients with the epsilon4 allele (51.6 vs. 38.0 P<0.002), (b) The correlation between the age at onset and the number of CAG repeats was strong in patients with the epsilon3/epsilon3 genotype while it was not detected in patients with epsilon3/epsilon4 genotype. (c) No correlation was found between age at onset and PS-1 alleles. In conclusion, APOE seems to be a significant factor influencing the age at onset of Huntington's disease.

  7. Sedimentology, geochemistry and rock magnetic properties of beach sands in Galapagos Islands - implications for nesting marine turtles (United States)

    Perez-Cruz, L.; Urrutia-Fucugauchi, J.; Vazquez-Gutierrez, F.; Carranza-Edwards, A.


    Marine turtles are well known for their navigation ability in the open ocean and fidelity to nesting beaches. Green turtle adult females migrate from foraging areas to island nesting beaches, traveling hundreds or thousands of kilometers each way. The marine turtle breeding in the Galapagos Islands is the Green Sea Turtle (Chelonia mydas agassisi); fairly common throughout the islands but with nesting sites located at Las Bachas (Santa Cruz), Barahona and Quinta Playa (Isabela), Salinas (Baltra), Gardner Bay (Española) and Bartolomé Islet. In order to characterize and to identify the geochemical signature of nesting marine turtle beaches in Galapagos Islands, sedimentological, geochemical and rock magnetic parameters are used. A total of one hundred and twenty sand samples were collected in four beaches to relate compositional characteristics between equivalent areas, these are: Las Bachas, Salinas, Barahona and Quinta Playa. Grain size is evaluated using laser particle analysis (Model Coulter LS 230). Bulk ICP-MS geochemical analysis is performed, following trace elements are analyzed: Al, V, Cr, Co, Ni, Cu, Zn, Cd, Ba, Pb, Fe, Mn, K, Na, Mg, Sr, Ca and Hg; and low-field magnetic susceptibility is measured in all samples at low and high frequencies. Granulometric analysis showed that Barahona and Quinta Playa are characterized for fine grained sands. In contrast, Salinas and Las Bachas exhibit medium to coarse sands. Trace metals concentrations and magnetic susceptibility show different distribution patterns in the beach sands. Calcium is the most abundant element in the samples. In particular, Co, K, and Na show similar concentrations in the four beaches. Las Bachas beach shows highest concentrations of Pb and Hg (maximum values 101.1 and 118.5 mg/kg, respectively), we suggest that the enrichment corresponds to an anthropogenic signal. Salinas beach samples show high concentrations of Fe, V, Cr, Zn, Mn and the highest values of magnetic susceptibility (maximum

  8. Xeroderma pigmentosum is a definite cause of Huntington's disease-like syndrome. (United States)

    Garcia-Moreno, Hector; Fassihi, Hiva; Sarkany, Robert P E; Phukan, Julie; Warner, Thomas; Lehmann, Alan R; Giunti, Paola


    Xeroderma pigmentosum is characterized by cutaneous, ophthalmological, and neurological features. Although it is typical of childhood, late presentations can mimic different neurodegenerative conditions. We report two families presenting as Huntington's disease-like syndromes. The first case (group G) presented with neuropsychiatric features, cognitive decline and chorea. Typical lentigines were only noticed after the neurological disease started. The second case (group B) presented adult-onset chorea and neuropsychiatric symptoms after an aggressive ocular melanoma. Xeroderma pigmentosum can manifest as a Huntington's Disease-like syndrome. Classic dermatological and oncological features have to be investigated in choreic patients with negative genetic tests for Huntington's disease-like phenotypes.

  9. Sunburn Protection by Sunscreen Sprays at Beach

    Directory of Open Access Journals (Sweden)

    Hao Ou-Yang


    Full Text Available Background: The efficacy of sunscreen is evaluated by SPF values, which are quantitatively determined in laboratories on the backs of human subjects according to a standardized procedure. However, SPF cannot be directly translated to sunburn protection under real-life situations because actual efficacy depends on various factors related to human behaviors and environmental conditions. This study clinically evaluated the efficacy of two sunscreen sprays (SPF 30 and SPF 70 under natural sunlight exposure on healthy subjects at the beach. Methods: Twenty subjects were divided into two cells for the two sunscreen sprays (SPF 70 and SPF 30 in a single-center, actual usage test. The primary endpoint of the study was sunburn protection on the dorsal arms and the secondary endpoint was protection on the face and neck. Subjects stayed at the beach for 4 h after application of the sunscreens with normal beach activities. Subjects’ behavior at the beach, the amounts of sunscreen applied and reapplied, and environmental conditions were all recorded. Results: There was no significant sunburn for a majority of the subjects in either cell. However, neither sunscreen completely blocked the sunburn, especially the face/neck area. We found that the SPF 70 sunscreen was more effective than the SPF 30 sunscreen. Conclusion: Modern sunscreen sprays, applied liberally, are effective in providing sunburn protection for the body in a beach setting.

  10. Beach Management & Analysis of Visitors’ Remarks

    Directory of Open Access Journals (Sweden)

    Perihan Paksoy


    Full Text Available User perceptions can become vital especially at beach preferences as cleanliness, safety and amenities are some of the apparent factors that will affect. With the awareness of probable adaptation of beach users’ demands into policy recommendations, a case study has been carried out at Black Sea Coast of İstanbul at Şile beaches. Şile has been chosen in this study purposefully as it is a touristic district of İstanbul which has aimed to earn Blue Flag award previously. Secondly, it receives high amount of visitors especially during the peak periods in weekends; as it has a very close location to the city, people are choosing here most of the time just for the day. In this research with factors about human use of beach and impacts like cleanliness and sufficiency of amenities (showers, toilets, changing cubicles, parks etc. and the number of lifeguards are studied. Regarding the findings, the researchers consequently highlight recommendations for Şile beach management which could enhance the visitor experience.

  11. Internal structure of a barrier beach as revealed by ground penetrating radar (GPR): Chesil beach, UK (United States)

    Bennett, Matthew R.; Cassidy, Nigel J.; Pile, Jeremy


    Chesil Beach (Dorset) is one of the most famous coastal landforms on the British coast. The gravel beach is over 18 km long and is separated for much of its length from land by a tidal lagoon known as The Fleet. The beach links the Isle of Portland in the east to the mainland in the west. Despite its iconic status there is little available information on its internal geometry and evolutionary history. Here we present a three-fold model for the evolution of Chesil Beach based on a series of nine ground penetrating radar (GPR) traverses located at three sites along its length at Abbotsbury, Langton Herring and at Ferry Bridge. The GPR traverses reveal a remarkably consistent picture of the internal structure of this barrier beach. The first phase of evolution involves the landward transgression of a small sand and gravel beach which closed upon the coast leading to deposition of freshwater peat between 5 and 7 k yr BP. The second evolutionary phase involves the 'bulking-out' of the beach during continued sea level rise, but in the presence of abundant gravel supplied by down-drift erosion of periglacial slope deposits. This episode of growth was associated with a series of washover fans which accumulated on the landward flank of the barrier increasing its breadth and height but without significant landward transgression of the barrier as a whole. The final phase in the evolution of Chesil Beach involves the seaward progradation of the beach crest and upper beach face associated with continued sediment abundance, but during a still-stand or slight fall in relative sea level. This phase may provide further evidence of a slight fall in relative sea level noted elsewhere along the South Coast of Britain and dated to between 1.2 and 2.4 k yr BP. Subsequently the barrier appears to have become largely inactive, except for the reworking of sediment on the beach face during storm events. The case study not only refines the evolutionary picture of Chesil Beach, but

  12. The plight of the beaches of Greece (United States)

    Ewing, L.; Foteinis, S.; Kalligeris, N.; Palaiologou, A.; Synolakis, C. E.


    The coastlines of the Greece are rapidly retreating at a rate that has increased substantially in the past decade. We describe here specific examples of rapid erosion and we speculate as to the causes. In some instances, erosion is advancing at a rate of 1m/year. As in other parts of the Mediterranean, the causes are anthropogenic and include sand mining from the beaches and rivers, poor design of coastal structures that create reflection patterns that focus waves on vulnerable areas, removal of sand dunes to build roads, and coastal construction too close to shoreline. The underlying problem is the complete lack of any semblance of coastal zone management in Greece and antiquated legislation. We conclude that unless urgent salvage measures to protect the beaches and end sand mining and dune removal, several beaches will disappear within the next decade.

  13. Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles, Vermont (United States)

    Vermont Center for Geographic Information — Digital Data from VG95-9A Thompson, PJ�and Thompson, TB, 1995, Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles,...

  14. The role of tau in the pathological process and clinical expression of Huntington's disease

    DEFF Research Database (Denmark)

    Vuono, Romina; Winder-Rhodes, Sophie; de Silva, Rohan


    and progression of Huntington's disease, the exact molecular mechanisms driving its pathogenic cascade and clinical features, especially the dementia, are not fully understood. Recently the microtubule associated protein tau, MAPT, which is associated with several neurodegenerative disorders, has been implicated......-mortem brain samples from patients with Huntington's disease (n = 16) compared to cases with a known tauopathy and healthy controls. Next, we undertook a genotype-phenotype analysis of a large cohort of patients with Huntington's disease (n = 960) with a particular focus on cognitive decline. We report...... not only on the tau pathology in the Huntington's disease brain but also the association between genetic variation in tau gene and the clinical expression and progression of the disease. We found extensive pathological inclusions containing abnormally phosphorylated tau protein that co-localized in some...

  15. Huntington's disease does not appear to increase the risk of diabetes mellitus

    DEFF Research Database (Denmark)

    Boesgaard, T W; Nielsen, Troels Tolstrup; Josefsen, Knud Elnegaard


    Huntington's disease (HD) is an autosomal, dominantly inherited, neurodegenerative disorder characterised by neurological, cognitive and psychiatric symptoms. HD has been associated with diabetes mellitus, which is, to some extent, supported by studies in transgenic HD mice. In transgenic mice...

  16. Transgenic miniature pig as a model for the study of Huntington´s Disease

    Czech Academy of Sciences Publication Activity Database

    Baxa, Monika


    Roč. 22, č. 2 (2012), s. 23-25 ISSN 1210-1737 Institutional support: RVO:67985904 Keywords : transgenic pig * Huntington ´s disease * large animal model * neurodegenerative disease Subject RIV: EB - Genetics ; Molecular Biology

  17. Huntington Revisited: Is Conservative Realism Still Essential for the Military Ethic

    National Research Council Canada - National Science Library

    Mahoney-Norris, Kathleen A


    ...). Furthermore, Huntington has developed what appears to be a powerful argument as to why conservative realism should be considered a fundamental component of the professional ethic of the military officer...

  18. Beach Advisory and Closing Online Notification (BEACON) system (United States)

    U.S. Environmental Protection Agency — Beach Advisory and Closing Online Notification system (BEACON) is a colletion of state and local data reported to EPA about beach closings and advisories. BEACON is...

  19. Climate induced changes in beach morphology and sediment dynamics, Machilipatnam

    Digital Repository Service at National Institute of Oceanography (India)

    Chauhan, O.S.

    The wave climate, littoral current patterns, monthly and seasonal longshore drift rates, beach profile changes, and sediment budget of the beach sediments were determined along Machilipatnam, Andhra Pradesh (India) for the NE, SW monsoons...

  20. Beach processes between Mulgund and Shiroda, west coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Nayak, B.U.; Sanilkumar, V.; Pathak, K.C.

    Study on beach processes for an year shows seasonal changes without annual net erosion. The beaches are stable and regain the maximum profiles during February to April. Distribution of longshore current direction is not uniform along the study...

  1. The ecology of sandy beaches in southern Africa

    African Journals Online (AJOL)

    diversity and abundance of macrofauna, beach slope and par- ticle size are analysed in ... Particular attention will be given to the distribution .of physical beach types .... hibited some evidence of phytoplankton blooms which are an important ...

  2. EPA Office of Water (OW): Beaches NHDPlus Indexed Dataset (United States)

    U.S. Environmental Protection Agency — The Beaches Environmental Assessment and Coastal Health (BEACH) Program focuses on the following five areas to meet the goals of improving public health and...

  3. Through the sands of time: Beach litter trends from nine cleaned north cornish beaches. (United States)

    Watts, Andrew J R; Porter, Adam; Hembrow, Neil; Sharpe, Jolyon; Galloway, Tamara S; Lewis, Ceri


    Marine litter and its accumulation on beaches is an issue of major current concern due to its significant environmental and economic impacts. Yet our understanding of spatio-temporal trends in beach litter and the drivers of these trends are currently limited by the availability of robust long term data sets. Here we present a unique data set collected systematically once a month, every month over a six year period for nine beaches along the North Coast of Cornwall, U.K. to investigate the key drivers of beach litter in the Bude, Padstow and Porthcothan areas. Overall, an average of 0.02 litter items m -2 per month were collected during the six year study, with Bude beaches (Summerleaze, Crooklets and Widemouth) the most impacted (0.03 ± 0.004 litter items m -2 per month). The amount of litter collected each month decreased by 18% and 71% respectively for Padstow (Polzeath, Trevone and Harlyn) and Bude areas over the 6 years, possibly related to the regular cleaning, however litter increased by 120% despite this monthly cleaning effort on the Padstow area beaches. Importantly, at all nine beaches the litter was dominated by small, fragmented plastic pieces and rope fibres, which account for 32% and 17% of all litter items collected, respectively. The weathered nature of these plastics indicates they have been in the marine environment for an extended period of time. So, whilst classifying the original source of these plastics is not possible, it can be concluded they are not the result of recent public littering. This data highlights both the extent of the marine litter problem and that current efforts to reduce littering by beach users will only tackle a fraction of this litter. Such information is vital for developing effective management strategies for beach and marine litter at both regional and global levels. Copyright © 2017 Elsevier Ltd. All rights reserved.

  4. Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study


    Hensman Moss, Davina J; Pardinas, Antonio; Langbehn, Douglas; Lo, Kitty; Leavitt, Blair R; Roos, Raymund; Durr, Alexandra; Mead, Simon; Holmans, Peter; Jones, Lesley; Tabrizi, Sarah J; Coleman, A; Santos, R Dar; Decolongon, J; Sturrock, A


    Background\\ud \\ud Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure.\\ud \\ud Methods\\ud \\ud We generated a progression score on the basis of principal ...

  5. Reduction in mitochondrial DNA copy number in peripheral leukocytes after onset of Huntington's disease

    DEFF Research Database (Denmark)

    Petersen, Maria Hvidberg; Budtz-Jørgensen, Esben; Sørensen, Sven Asger


    Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by movement disorder, cognitive symptoms and psychiatric symptoms with predominantly adult-onset. The mutant huntingtin protein leads to mitochondrial dysfunction in blood leukocytes. This discovery led to the inve......Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by movement disorder, cognitive symptoms and psychiatric symptoms with predominantly adult-onset. The mutant huntingtin protein leads to mitochondrial dysfunction in blood leukocytes. This discovery led...

  6. [The life as a caregiver of a person affected by Chorea Huntington: multiple case study]. (United States)

    Winkler, Evi; Ausserhofer, Dietmar; Mantovan, Franco


    Chorea Huntington is an autosomal dominantly inherited, neurodegenerative brain disorder that leads to involuntary hyperkinesia, psychotic symptoms and dementia. The illness not only changes the life of the person itself but also the world of the caregivers. The challenges in the care of a person which is affected by Chorea Huntington have an effect on the daily living as an assemblage of natural and social conditions. a multiple case study was conducted. It included semi-structured interviews with three caregivers of people with Chorea Huntington in South Tyrol. The qualitative data was analyzed using the qualitative structured analysis of Mayring (2007). The objective of this study was to describe the phenomenon of change of life from family members that care people affected by Chorea Huntington in a specific cultural setting (South Tyrol, Italy). The caregivers reported that the diagnosis of Chorea Huntington leads to negative changes in "relationship and family". Particularly, frustration, aggression, impatience and apathy were perceived as stressful. At the same time they highlight the positive changes through home care. They report that the relationship became more intimate and integral and it was characterized by more cohesion. Family caregivers get valuable support from the home care service, however, they complain that there is no facility in South Tyrol, which is specialized to care people with Chorea Huntington. Therefore, the caregivers have to "give up a lot" and don't have any personal desires, dreams and expectations for the future. The caregivers have learned independently to deal with their changed life step by step, and to see also the positive effects of the caring role. The life of family caregivers of a person which is affected by Chorea Huntington is characterized by abandonment. A continuous and professional care would be important for the affected and his caregiver. A continuous and professional care is important for both, addressing the

  7. Burrowing inhibition by fine textured beach fill: Implications for recovery of beach ecosystems (United States)

    Viola, Sloane M.; Hubbard, David M.; Dugan, Jenifer E.; Schooler, Nicholas K.


    Beach nourishment is often considered the most environmentally sound method of maintaining eroding shorelines. However, the ecological consequences are poorly understood. Fill activities cause intense disturbance and high mortality and have the potential to alter the diversity, abundance, and distribution of intertidal macroinvertebrates for months to years. Ecological recovery following fill activities depends on successful recolonization and recruitment of the entire sandy intertidal community. The use of incompatible sediments as fill material can strongly affect ecosystem recovery. We hypothesized that burrowing inhibition of intertidal animals by incompatible fine fill sediments contributes to ecological impacts and limits recovery in beach ecosystems. We experimentally investigated the influence of intertidal zone and burrowing mode on responses of beach invertebrates to altered sediment texture (28-38% fines), and ultimately the potential for colonization and recovery of beaches disturbed by beach filling. Using experimental trials in fill material and natural beach sand, we found that the mismatched fine fill sediments significantly inhibited burrowing of characteristic species from all intertidal zones, including sand crabs, clams, polychaetes, isopods, and talitrid amphipods. Burrowing performance of all five species we tested was consistently reduced in the fill material and burrowing was completely inhibited for several species. The threshold for burrowing inhibition by fine sediment content in middle and lower beach macroinvertebrates varied by species, with highest sensitivity for the polychaete (4% fines, below the USA regulatory limit of 10% fines), followed by sand crabs and clams (20% fines). These results suggest broader investigation of thresholds for burrowing inhibition in fine fill material is needed for beach animals. Burrowing inhibition caused by mismatched fill sediments exposes beach macroinvertebrates to stresses, which could depress

  8. Through the sands of time: Beach litter trends from nine cleaned north cornish beaches

    International Nuclear Information System (INIS)

    Watts, Andrew J.R.; Porter, Adam; Hembrow, Neil; Sharpe, Jolyon; Galloway, Tamara S.; Lewis, Ceri


    Marine litter and its accumulation on beaches is an issue of major current concern due to its significant environmental and economic impacts. Yet our understanding of spatio-temporal trends in beach litter and the drivers of these trends are currently limited by the availability of robust long term data sets. Here we present a unique data set collected systematically once a month, every month over a six year period for nine beaches along the North Coast of Cornwall, U.K. to investigate the key drivers of beach litter in the Bude, Padstow and Porthcothan areas. Overall, an average of 0.02 litter items m −2 per month were collected during the six year study, with Bude beaches (Summerleaze, Crooklets and Widemouth) the most impacted (0.03 ± 0.004 litter items m −2 per month). The amount of litter collected each month decreased by 18% and 71% respectively for Padstow (Polzeath, Trevone and Harlyn) and Bude areas over the 6 years, possibly related to the regular cleaning, however litter increased by 120% despite this monthly cleaning effort on the Padstow area beaches. Importantly, at all nine beaches the litter was dominated by small, fragmented plastic pieces and rope fibres, which account for 32% and 17% of all litter items collected, respectively. The weathered nature of these plastics indicates they have been in the marine environment for an extended period of time. So, whilst classifying the original source of these plastics is not possible, it can be concluded they are not the result of recent public littering. This data highlights both the extent of the marine litter problem and that current efforts to reduce littering by beach users will only tackle a fraction of this litter. Such information is vital for developing effective management strategies for beach and marine litter at both regional and global levels. - Highlights: • Unique and systemically collected beach clean data set from 9 beaches over 6 years. • The most abundant litter items were

  9. Patterns of species richness in sandy beaches of South America

    African Journals Online (AJOL)

    beaches with rdkctive and dissip:1tive characteristics (sensu. R eprodu ced by Sabin et G atew ay u n der licen ce gran ted by th e P u blish er (dated 2009). ... beach intertidal communities WaS reviewed, (b) location of len sam.!y beaches studied in south-central Chile, imd (c) location of two sandy beaches studied on the ...

  10. Sodium phenylbutyrate in Huntington's disease: a dose-finding study. (United States)

    Hogarth, Penelope; Lovrecic, Luca; Krainc, Dimitri


    Transcriptional dysregulation in Huntington's disease (HD) is mediated in part by aberrant patterns of histone acetylation. We performed a dose-finding study in human HD of sodium phenylbutyrate (SPB), a histone deacetylase inhibitor that ameliorates the HD phenotype in animal models. We used a dose-escalation/de-escalation design, using prespecified toxicity criteria and standard clinical and laboratory safety measures. The maximum tolerated dose was 15 g/day. At higher doses, toxicity included vomiting, lightheadedness, confusion, and gait instability. We saw no significant laboratory or electrocardiographic abnormalities. Gene expression changes in blood suggested an inverse dose-response. In conclusion, SPB at 12 to 15 g/day appears to be safe and well-tolerated in human HD. 2007 Movement Disorder Society

  11. Making a measurable difference in advanced Huntington disease care. (United States)

    Moskowitz, Carol Brown; Rao, Ashwini K


    Neurologists' role in the care of people with advanced Huntington disease (HD) (total functional capacity speech and language pathology), behavioral and psychiatric professionals for problem-solving strategies, which must be reviewed with direct care staff before implementation; (3) encourage and support qualitative and quantitative interdisciplinary research studies, and randomized controlled studies of nonpharmacologic interventions; and (4) assist in the development of meaningful measures to further document what works to provide a good quality of life for the patient and family and a comfortable thoughtful approach to a good death. Collaborative models of care depend on: (1) clear communication; (2) ongoing education and support programs; with (3) pharmacologic and rehabilitation interventions, always in the context of respect for the person with HD, a preservation of the individuals' dignity, autonomy, and individual preferences. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Swallowing endoscopy findings in Huntington's disease: a case report. (United States)

    Alves, Thaís Coelho; Cola, Paula Cristina; Santos, Rarissa Rúbia Dallaqua Dos; Motonaga, Suely Mayumi; Silva, Roberta Gonçalves da


    Huntington's disease (HD) is a degenerative genetic disorder with autosomal-dominant transmission. The triad of symptoms of this disease consists of psychiatric disorders, jerky movements, and dementia. Oropharyngeal dysphagia, which is more evident with disease progression, is also present. Few studies have addressed the swallowing characteristics using objective analysis in this population. The purpose of this research was to describe the swallowing endoscopic findings of the pharyngeal phase in HD. This is a cross-sectional study addressing a clinical case which included two individuals of the same family, male, 32 and 63 years old, designated as individual A and individual B, with progression of the disease for five and 13 years, respectively. Consistent liquid, nectar, and puree were offered during the evaluation. There was presence of posterior oral spillage in liquid and nectar, small amount of pharyngeal residues, and no laryngeal penetration or aspiration in the individuals with HD in this study.

  13. Structural imaging in premanifest and manifest Huntington disease. (United States)

    Scahill, Rachael I; Andre, Ralph; Tabrizi, Sarah J; Aylward, Elizabeth H


    Huntington disease (HD) neuropathology has a devastating effect on brain structure and consequently brain function; neuroimaging provides a means to assess these effects in gene carriers. In this chapter we first outline the unique utility of structural imaging in understanding HD and discuss some of the acquisition and analysis techniques currently available. We review the existing literature to summarize what we know so far about structural brain changes across the spectrum of disease from premanifest through to manifest disease. We then consider how these neuroimaging findings relate to patient function and nonimaging biomarkers, and can be used to predict disease onset. Finally we review the utility of imaging measures for assessment of treatment efficacy in clinical trials. Copyright © 2017 Elsevier B.V. All rights reserved.

  14. [Speed of ocular saccades in Huntington disease. Prospective study]. (United States)

    García Ruiz, P J; Cenjor, C; Ulmer, E; Hernández, J; Cantarero, S; Fanjul, S; García de Yébenes, J


    Oculomotor abnormalities, especially slow saccades, have long been recognized in Huntington's disease (HD). To study prospectively horizontal saccade velocity by videonystagmography in 21 patients with genetically confirmed HD. The study included a baseline analysis and a second evaluation after 18.8 +/- 7.1 months. We included a control group of 15 subjects. HD group exhibited decreased saccade velocity when compared with that from a control group (for predictive and unpredictive target). HD patients showed decreased saccade velocity with the passage of time (for predictive target, p < 0.01). Finally we found statistical significant correlation between saccade velocity and triplet length. The measurement of saccade velocity might be an objective method to study the natural evolution of HD, and thus evaluate the effectiveness of future therapies.

  15. Brain atrophy in Huntington's disease: A CT-scan study

    International Nuclear Information System (INIS)

    Starkstein, S.E.; Folstein, S.E.; Brandt, J.; McDonnell, A.; Folstein, M.


    CT-scan measurements of cortical and subcortical atrophy were carried out in 34 patients with Huntington's disease (HD). While a significant correlation was observed between parameters of subcortical atrophy (bicaudate ratio, bifrontal ratio and third ventricular ratio) and duration of the disease, there was no significant correlation between these parameters and age. On the other hand, measurements of cortical atrophy (frontal fissure ratio and cortical sulci ratio) correlated significantly with age but not with duration of the disease. When a group of 24 HD patients were compared on CT-scan measurements with a group of 24 age-matched normal controls, significant differences were obtained for all the variables examined, but the bicaudate ratio showed the highest sensitivity and specificity. Even mildly affected patients, with duration of motor symptoms less than 3 years had higher bicaudate ratios than age-matched controls. (orig.)

  16. Nucleic Acid-Based Therapy Approaches for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Tatyana Vagner


    Full Text Available Huntington's disease (HD is caused by a dominant mutation that results in an unstable expansion of a CAG repeat in the huntingtin gene leading to a toxic gain of function in huntingtin protein which causes massive neurodegeneration mainly in the striatum and clinical symptoms associated with the disease. Since the mutation has multiple effects in the cell and the precise mechanism of the disease remains to be elucidated, gene therapy approaches have been developed that intervene in different aspects of the condition. These approaches include increasing expression of growth factors, decreasing levels of mutant huntingtin, and restoring cell metabolism and transcriptional balance. The aim of this paper is to outline the nucleic acid-based therapeutic strategies that have been tested to date.

  17. Reduced gluconeogenesis and lactate clearance in Huntington's disease

    DEFF Research Database (Denmark)

    Josefsen, Knud; Nielsen, Signe M B; Campos, André


    We studied systemic and brain glucose and lactate metabolism in Huntington's disease (HD) patients in response to ergometer cycling. Following termination of exercise, blood glucose increased abruptly in control subjects, but no peak was seen in any of the HD patients (2.0 ± 0.5 vs. 0.0 ± 0.2mM, P...... HD mouse model R6/2 following a lactate challenge, combined with reduced phosphoenolpyruvate carboxykinase and increased pyruvate kinase activity in the mouse liver suggest a reduced capacity...... for gluconeogenesis in HD, possibly contributing to the clinical symptoms of HD. We propose that blood glucose concentration in the recovery from exercise can be applied as a liver function test in HD patients....

  18. Exploring Genetic Factors Involved in Huntington Disease Age of Onset

    DEFF Research Database (Denmark)

    Valcárcel-Ocete, Leire; Alkorta-Aranburu, Gorka; Iriondo, Mikel


    age (motor AO or mAO). Multiple linear regression analyses were performed between genetic variation within 20 candidate genes and eAO or mAO, using DNA and clinical information of 253 HD patients from REGISTRY project. Gene expression analyses were carried out by RT-qPCR with an independent sample......Age of onset (AO) of Huntington disease (HD) is mainly determined by the length of the CAG repeat expansion (CAGexp) in exon 1 of the HTT gene. Additional genetic variation has been suggested to contribute to AO, although the mechanism by which it could affect AO is presently unknown. The aim...... of this study is to explore the contribution of candidate genetic factors to HD AO in order to gain insight into the pathogenic mechanisms underlying this disorder. For that purpose, two AO definitions were used: the earliest age with unequivocal signs of HD (earliest AO or eAO), and the first motor symptoms...

  19. Daily beach profiles and littoral environmental observations off Baga, Calangute and Miramar beaches during November-December 1999

    Digital Repository Service at National Institute of Oceanography (India)

    Jayakumar, S.; Raju, N.S.N.; Gowthaman, R.; AshokKumar, K.; Anand, N.M.

    16th November-15th December 1999, are as follows: (1) daily beach profiles, (2) daily littoral environmental observations and (3) beach sediment samples for grain size distribution. Longshore sediment transport rate is estimated theoretically based...

  20. Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey. (United States)

    Bombard, Yvonne; Veenstra, Gerry; Friedman, Jan M; Creighton, Susan; Currie, Lauren; Paulsen, Jane S; Bottorff, Joan L; Hayden, Michael R


    To assess the nature and prevalence of genetic discrimination experienced by people at risk for Huntington's disease who had undergone genetic testing or remained untested. Cross sectional, self reported survey. Seven genetics and movement disorders clinics servicing rural and urban communities in Canada. 233 genetically tested and untested asymptomatic people at risk for Huntington's disease (response rate 80%): 167 underwent testing (83 had the Huntington's disease mutation, 84 did not) and 66 chose not to be tested. Self reported experiences of genetic discrimination and related psychological distress based on family history or genetic test results. Discrimination was reported by 93 respondents (39.9%). Reported experiences occurred most often in insurance (29.2%), family (15.5%), and social (12.4%) settings. There were few reports of discrimination in employment (6.9%), health care (8.6%), or public sector settings (3.9%). Although respondents who were aware that they carried the Huntington's disease mutation reported the highest levels of discrimination, participation in genetic testing was not associated with increased levels of genetic discrimination. Family history of Huntington's disease, rather than the result of genetic testing, was the main reason given for experiences of genetic discrimination. Psychological distress was associated with genetic discrimination (PGenetic discrimination was commonly reported by people at risk for Huntington's disease and was a source of psychological distress. Family history, and not genetic testing, was the major reason for genetic discrimination.

  1. The Addenbrooke's Cognitive Examination-Revised accurately detects cognitive decline in Huntington's disease. (United States)

    Begeti, Faye; Tan, Adrian Y K; Cummins, Gemma A; Collins, Lucy M; Guzman, Natalie Valle; Mason, Sarah L; Barker, Roger A


    Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's disease varied according to disease severity with extensive cognitive decline observed in early-stage Huntington's disease patients, indicating that this would be an optimal stage for interventions designed to halt cognitive decline, and lesser changes in the advanced cases. We next examined cognitive performance in patients prescribed antidopaminergic drugs as these drugs are known to decrease cognition when administered to healthy volunteers. We paradoxically found that these drugs may be beneficial, as early-stage Huntington's disease participants in receipt of them had improved attention and Mini-Mental State Examination scores. In conclusion, this is the first study to test the usefulness of the ACE-R in a Huntington's disease population and demonstrates that this is a brief, inexpensive and practical way to measure global cognitive performance in clinical practice with potential use in clinical trials.

  2. The environmental impacts of beach sport tourism events: a case ...

    African Journals Online (AJOL)

    Durban has several established beach sport events. One of the many events is the Mr Price Pro, an internationally recognised surfi ng event, which takes place during the Vodacom Beach Africa festival, held annually during the July holiday season. This article examines the environmental impact of beach tourism events by ...

  3. Seasonal impact on beach morphology and the status of heavy ...

    Indian Academy of Sciences (India)

    the status of heavy mineral distribution along central Tamil Nadu coast, India. ... the seasonal changes in beach morphology and it does not affect the heavy ... of beach dynamics and depositional environment. ...... erates beach ridges and inland sediment deposits ... Waterways, Harbors and Coastal Engineering Division;.

  4. Fine particle deposition at Vainguinim tourist beach, Goa, India

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Jayakumar, S.; SanilKumar, V.; Ilangovan, D.

    Vainguinim Beach is a small and narrow pocket beach located on the rocky coast of Dona Paula Bay, at the estuarine front of the Zuari River in Goa, India. The beach has been widely used for recreation and swimming by a large number of tourists...

  5. Beach Sand Analysis for Indicators of Microbial Contamination (United States)

    Traditional beach monitoring has focused on water quality, with little attention paid to health risks associated with beach sand. Recent research has reported that fecal indicator bacteria, as well as human pathogens can be found in beach sand and may constitute a risk to human h...

  6. Post tsunami rebuilding of beaches and the texture of sediments

    Digital Repository Service at National Institute of Oceanography (India)

    Loveson, V.J.; Gujar, A.R.; Rajamanickam, G.V.; Chandrasekar, N.; Manickaraj, D.S.; Chandrasekaran, R.; Chaturvedi, S.K.; Mahesh, R.; Josephine, P.J.; Deepa, V.; Sudha, V.; Sunderasen, D.

    and textural statistic studies. In view of the presence tsunami in between, the beach sand composition and texture have been drastically changed, the studies on beach re-building effort has been initiated in continuing the beach sand sample collection to 2006...

  7. Landscape Visual Quality and Meiofauna Biodiversity on Sandy Beaches (United States)

    Felix, Gabriela; Marenzi, Rosemeri C.; Polette, Marcos; Netto, Sérgio A.


    Sandy beaches are central economic assets, attracting more recreational users than other coastal ecosystems. However, urbanization and landscape modification can compromise both the functional integrity and the attractiveness of beach ecosystems. Our study aimed at investigating the relationship between sandy beach artificialization and the landscape perception by the users, and between sandy beach visual attractiveness and biodiversity. We conducted visual and biodiversity assessments of urbanized and semiurbanized sandy beaches in Brazil and Uruguay. We specifically examined meiofauna as an indicator of biodiversity. We hypothesized that urbanization of sandy beaches results in a higher number of landscape detractors that negatively affect user evaluation, and that lower-rated beach units support lower levels of biodiversity. We found that urbanized beach units were rated lower than semiurbanized units, indicating that visual quality was sensitive to human interventions. Our expectations regarding the relationship between landscape perception and biodiversity were only partially met; only few structural and functional descriptors of meiofauna assemblages differed among classes of visual quality. However, lower-rated beach units exhibited signs of lower environmental quality, indicated by higher oligochaete densities and significant differences in meiofauna structure. We conclude that managing sandy beaches needs to advance beyond assessment of aesthetic parameters to also include the structure and function of beach ecosystems. Use of such supporting tools for managing sandy beaches is particularly important in view of sea level rise and increasing coastal development.

  8. Intertidal beach slope predictions compared to field data

    NARCIS (Netherlands)

    Madsen, A.J.; Plant, N.G.


    This paper presents a test of a very simple model for predicting beach slope changes. The model assumes that these changes are a function of both the incident wave conditions and the beach slope itself. Following other studies, we hypothesized that the beach slope evolves towards an equilibrium

  9. Measured spatial variability of beach erosion due to aeolian processes.

    NARCIS (Netherlands)

    de Vries, S.; Verheijen, A.H.; Hoonhout, B.M.; Vos, S.E.; Cohn, Nicholas; Ruggiero, P; Aagaard, T.; Deigaard, R.; Fuhrman, D.


    This paper shows the first results of measured spatial variability of beach erosion due to aeolian processes during the recently conducted SEDEX2 field experiment at Long Beach, Washington, U.S.A.. Beach erosion and sedimentation were derived using series of detailed terrestrial LIDAR measurements

  10. Orphan drugs in development for Huntington's disease: challenges and progress

    Directory of Open Access Journals (Sweden)

    Burgunder JM


    advanced strategies to develop novel treatments in Huntington's disease are examined. Keywords: Huntington's disease, symptomatic treatment, disease-modifying therapy

  11. Bibliography of sandy beaches and sandy beach organisms on the African continent

    CSIR Research Space (South Africa)

    Bally, R


    Full Text Available This bibliography covers the literature relating to sandy beaches on the African continent and outlying islands. The bibliography lists biological, chemical, geographical and geological references and covers shallow marine sediments, surf zones off...

  12. 75 FR 16201 - FPL Energy Point Beach, LLC; Point Beach Nuclear Plant, Units 1 and 2; Exemption (United States)


    ... NUCLEAR REGULATORY COMMISSION [Docket Nos. 50-266 and 50-301; NRC-2010-0123] FPL Energy Point Beach, LLC; Point Beach Nuclear Plant, Units 1 and 2; Exemption 1.0 Background FPL Energy Point Beach.... Borchardt (NRC) to M. S. Fertel (Nuclear Energy Institute) dated June 4, 2009. The licensee's request for an...

  13. Alongshore variability of nourished and natural beaches

    NARCIS (Netherlands)

    De Schipper, M.A.


    Alongshore variability in topography (i.e. height differences in bed level along the coast) can exist on both natural and nourished beaches. An important question prior to implementation of a nourishment project is how alongshore variability is going to evolve and, related to this variability, the

  14. Erosion in the Beaches of Crete (United States)

    Synolakis, C. E.; Foteinis, S.; Voukouvalas, V.; Kalligeris, N.


    In the past decade, erosion rates for the coastlines of Greece are rapidly increasing. Many beaches on the northern coast of the island have substantially retreated, while others have disappeared or will disappear within the present or the following decade if no action is taken. For the better understanding and visualization of the current situation, specific examples of rapid erosion are described and afterwards we speculate as to the causes. We infer that, as in other parts of the Mediterranean, the causes are anthropogenic and include removal of sand dunes to build roads, sand mining from beaches and rivers, permanent building construction within the active coastal zone, on or too close to shoreline, and poor design of coastal structures. The reason behind the rapid erosion of Greece coastlines is the complete lack of any semblance of coastal zone management and antiquated legislation. We conclude that unless urgent measures for the protection and even salvation of the beaches are taken and if the sand mining and dune removal does not stop, then several beaches will disappear within the present and the following decade.

  15. Spectral signatures for swash on reflective, intermediate and dissipative beaches

    DEFF Research Database (Denmark)

    Hughes, Michael G; Aagaard, Troels; Baldock, Tom E


    (reflective, intermediate and dissipative), with beach gradients ranging from approximately 1:6 to 1:60 exposed to offshore significant wave heights of 0.5–3.0 m. The ratio of swash energy in the short-wave (f > 0.05 Hz) to long-wave (f ... the three beach types. Swash energy at short-wave frequencies is dominant on reflective and intermediate beaches and swash at long-wave frequencies is dominant on dissipative beaches; consistent with previously reported spectral signatures for the surf zone on these beach types. The available swash spectra...

  16. Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease. (United States)

    Novak, Marianne J U; Warren, Jason D; Henley, Susie M D; Draganski, Bogdan; Frackowiak, Richard S; Tabrizi, Sarah J


    Huntington's disease is an inherited neurodegenerative disease that causes motor, cognitive and psychiatric impairment, including an early decline in ability to recognize emotional states in others. The pathophysiology underlying the earliest manifestations of the disease is not fully understood; the objective of our study was to clarify this. We used functional magnetic resonance imaging to investigate changes in brain mechanisms of emotion recognition in pre-manifest carriers of the abnormal Huntington's disease gene (subjects with pre-manifest Huntington's disease): 16 subjects with pre-manifest Huntington's disease and 14 control subjects underwent 1.5 tesla magnetic resonance scanning while viewing pictures of facial expressions from the Ekman and Friesen series. Disgust, anger and happiness were chosen as emotions of interest. Disgust is the emotion in which recognition deficits have most commonly been detected in Huntington's disease; anger is the emotion in which impaired recognition was detected in the largest behavioural study of emotion recognition in pre-manifest Huntington's disease to date; and happiness is a positive emotion to contrast with disgust and anger. Ekman facial expressions were also used to quantify emotion recognition accuracy outside the scanner and structural magnetic resonance imaging with voxel-based morphometry was used to assess the relationship between emotion recognition accuracy and regional grey matter volume. Emotion processing in pre-manifest Huntington's disease was associated with reduced neural activity for all three emotions in partially separable functional networks. Furthermore, the Huntington's disease-associated modulation of disgust and happiness processing was negatively correlated with genetic markers of pre-manifest disease progression in distributed, largely extrastriatal networks. The modulated disgust network included insulae, cingulate cortices, pre- and postcentral gyri, precunei, cunei, bilateral putamena

  17. Trophic niche shifts driven by phytoplankton in sandy beach ecosystems (United States)

    Bergamino, Leandro; Martínez, Ana; Han, Eunah; Lercari, Diego; Defeo, Omar


    Stable isotopes (δ13C and δ15N) together with chlorophyll a and densities of surf diatoms were used to analyze changes in trophic niches of species in two sandy beaches of Uruguay with contrasting morphodynamics (i.e. dissipative vs. reflective). Consumers and food sources were collected over four seasons, including sediment organic matter (SOM), suspended particulate organic matter (POM) and the surf zone diatom Asterionellopsis guyunusae. Circular statistics and a Bayesian isotope mixing model were used to quantify food web differences between beaches. Consumers changed their trophic niche between beaches in the same direction of the food web space towards higher reliance on surf diatoms in the dissipative beach. Mixing models indicated that A. guyunusae was the primary nutrition source for suspension feeders in the dissipative beach, explaining their change in dietary niche compared to the reflective beach where the proportional contribution of surf diatoms was low. The high C/N ratios in A. guyunusae indicated its high nutritional value and N content, and may help to explain the high assimilation by suspension feeders at the dissipative beach. Furthermore, density of A. guyunusae was higher in the dissipative than in the reflective beach, and cell density was positively correlated with chlorophyll a only in the dissipative beach. Therefore, surf diatoms are important drivers in the dynamics of sandy beach food webs, determining the trophic niche space and productivity. Our study provides valuable insights on shifting foraging behavior by beach fauna in response to changes in resource availability.

  18. Cycling of acid and base cations in deciduous stands of Huntington Forest, New York, and Turkey Lakes, Ontario

    Energy Technology Data Exchange (ETDEWEB)

    Foster, N W; Morrison, I K [Forestry Canada, Sault Ste. Marie, ON (Canada); Mitchell, M J [State Univ. of New York, Syracuse, NY (USA); Shepard, J P [National Council of the Paper Industry for Air and Stream Improvement, Gainesville, FL (USA)


    Annual nutrient fluxes within two forests exposed to acidic deposition were compared for a 1-year period. Calcium (Ca{sup 2+}) was the dominant cation in throughfall and soil solutions from tolerant hardwood dominated Spodosols (Podzols) at both Huntington Forest (HF), New York, and the Turkey Lakes watershed (TLW), Ontario. There was a net annual export of Ca{sup 2+} and Mg{sup 2+} from the TLW soil, whereas base cation inputs in precipitation equalled outputs at HF. In 1986, leaching losses of base cations were five times greater at TLW than at HF. A higher percentage of the base cation reserves was leached from the soil at TLW (5%) than at HF (1%). Relative to throughfall, aluminum concentrations increased in forest-floor and mineral-soil solutions, especially at HF. The TLW soil appears more sensitive to soil acidification. Deposited atmospheric acidity, however, was small in comparison with native soil acidity (total and exchangeable) and the reserves of base cations in each soil. Soil acidity and base saturation, therefore, are likely only to change slowly. 57 refs., 1 fig., 5 tabs.

  19. Effect of beach management policies on recreational water quality. (United States)

    Kelly, Elizabeth A; Feng, Zhixuan; Gidley, Maribeth L; Sinigalliano, Christopher D; Kumar, Naresh; Donahue, Allison G; Reniers, Adrianus J H M; Solo-Gabriele, Helena M


    When beach water monitoring programs identify poor water quality, the causes are frequently unknown. We hypothesize that management policies play an important role in the frequency of fecal indicator bacteria (FIB) exceedances (enterococci and fecal coliform) at recreational beaches. To test this hypothesis we implemented an innovative approach utilizing large amounts of monitoring data (n > 150,000 measurements per FIB) to determine associations between the frequency of contaminant exceedances and beach management practices. The large FIB database was augmented with results from a survey designed to assess management policies for 316 beaches throughout the state of Florida. The FIB and survey data were analyzed using t-tests, ANOVA, factor analysis, and linear regression. Results show that beach geomorphology (beach type) was highly associated with exceedance of regulatory standards. Low enterococci exceedances were associated with open coast beaches (n = 211) that have sparse human densities, no homeless populations, low densities of dogs and birds, bird management policies, low densities of seaweed, beach renourishment, charge access fees, employ lifeguards, without nearby marinas, and those that manage storm water. Factor analysis and a linear regression confirmed beach type as the predominant factor with secondary influences from grooming activities (including seaweed densities and beach renourishment) and beach access (including charging fees, employing lifeguards, and without nearby marinas). Our results were observable primarily because of the very large public FIB database available for analyses; similar approaches can be adopted at other beaches. The findings of this research have important policy implications because the selected beach management practices that were associated with low levels of FIB can be implemented in other parts of the US and around the world to improve recreational beach water quality. Copyright © 2018 Elsevier Ltd. All

  20. Relationships between sand and water quality at recreational beaches. (United States)

    Phillips, Matthew C; Solo-Gabriele, Helena M; Piggot, Alan M; Klaus, James S; Zhang, Yifan


    Enterococci are used to assess the risk of negative human health impacts from recreational waters. Studies have shown sustained populations of enterococci within sediments of beaches but comprehensive surveys of multiple tidal zones on beaches in a regional area and their relationship to beach management decisions are limited. We sampled three tidal zones on eight South Florida beaches in Miami-Dade and Broward counties and found that enterococci were ubiquitous within South Florida beach sands although their levels varied greatly both among the beaches and between the supratidal, intertidal and subtidal zones. The supratidal sands consistently had significantly higher (p sand) than the other two zones. Levels of enterococci within the subtidal sand correlated with the average level of enterococci in the water (CFU/100mL) for the season during which samples were collected (r(s) = 0.73). The average sand enterococci content over all the zones on each beach correlated with the average water enterococci levels of the year prior to sand samplings (r(s) = 0.64) as well as the average water enterococci levels for the month after sand samplings (r(s) = 0.54). Results indicate a connection between levels of enterococci in beach water and sands throughout South Florida's beaches and suggest that the sands are one of the predominant reservoirs of enterococci impacting beach water quality. As a result, beaches with lower levels of enterococci in the sand had fewer exceedences relative to beaches with higher levels of sand enterococci. More research should focus on evaluating beach sand quality as a means to predict and regulate marine recreational water quality. Copyright © 2011 Elsevier Ltd. All rights reserved.

  1. Integrated protecting plan for beach erosion. A case study in Plaka beach, E. Crete, Greece (United States)

    Petrakis, Stelios; Alexandrakis, George; Kozyrakis, George; Hatziyanni, Eleni; Kampanis, Nikolaos


    Coastal zones are among the most active areas on Earth, being subjected to extreme wind / wave conditions, thus vulnerable to erosion. In Greece and Crete in particular, beach zones are extremely important for the welfare of the inhabitants, since, apart for the important biological and archaeological value of the beach zones, the socio-economic value is critical since a great number of human activities are concentrated in such areas (touristic facilities, fishing harbors etc.). The present study investigates the erosional procedures observed in Plaka beach, E. Crete, Greece, a highly touristic developed area with great archaeological interest and proposes a cost-effective solution. The factors taken into consideration for the proposed solution in reducing the erosion of the beach were the study of the climatological, geological and geomorphological regime of the area, the recent (~70 years) shifting of the coastline through the study of topographic maps, aerial photographs and satellite images, the creation of detailed bathymetric and seabed classification maps of the area and finally, a risk analysis in terms of erosional phenomena. On the basis of the above, it is concluded that the area under investigation is subjected to an erosional rate of about 1 m/10 years and the total land-loss for the past 70 years is about 4600 m2. Through the simulation of the wave regime we studied 3 possible scenarios, the "do-nothing" scenario, the construction of a detached submerged breakwater at the depth of 3 meters and, finally, the armoring of the existing beach-wall through the placement of appropriate size and material boulders, forming an artificial slope for the reducing of the wave breaking energy and a small scale nourishment plan. As a result, through the modeling of the above, the most appropriate and cost-effective solution was found to be the third, armoring of the existing coastal wall and nourishment of the beach periodically, thus the further undermining of the

  2. Field Evaluation/Demonstration of a Multisegmented Dewatering System for Accreting Beach Sand in a High-Wave-Energy Environment

    National Research Council Canada - National Science Library

    Curtis, William


    This study documents the use of beach dewatering systems to accrete beach sand and minimize erosion, and to develop quantitative guidance for constructing and operating beach dewatering installations...

  3. Mauritius: A journey from beach to laboratory

    Digital Repository Service at National Institute of Oceanography (India)

    Mukhopadhyay, R.

    and fun-filled sunny golden beach-centered tourism. However, despite having an Exclusive Economic Zone (EEZ) thousand times its landmass, the ocean based economic activities contributing to the Gross National Product of Mauritius have been... and necessary legislation for conservation and sustainable use of marine biodiversity and its Exclusive Economic Zone (in yellow) of the Republic of Mauritius 79 components. Setting up of an integrated coastal zone management framework involving impact...

  4. Huntington Disease - principles and practice of nutritional management. (United States)

    Zukiewicz-Sobczak, Wioletta; Król, Renata; Wróblewska, Paula; Piątek, Jacek; Gibas-Dorna, Magdalena


    Huntington disease (HD) is a degenerative brain disease clinically manifested by the characteristic triad: physical symptoms including involuntary movements and poor coordination, cognitive changes with less ability to organize routine tasks, and some emotional and behavioral disturbances. For patients with HD, feeding is one of the problems they have to face. People with HD often have lower than average body weight and struggle with malnutrition. As a part of therapy, good nutrition is an intervention maintaining health and functional ability for maximally prolonged time. In the early stages of HD, small amounts of blenderized foods given orally are recommended. In more advanced stages, enteral nutrition is essential using gastric, or jejunal tubes for short term. Most severe cases require gastrostomy or gastrojejunostomy. Although enteral feeding is well tolerated by most of the patients, a number of complications may occur, including damage to the nose, pharynx, or esophagus, aspiration pneumonia, sinusitis, metabolic imbalances due to improper nutrient and fluid supply, adverse effects affecting gastrointestinal system, and refeeding syndrome. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  5. The story of George Huntington and his disease

    Directory of Open Access Journals (Sweden)

    Kalyan B Bhattacharyya


    Full Text Available George Huntington described some families with choreiform movements in 1872 in the United States of America and since then many such families have been described in other parts of the world and works on the genetics of the disease have brought new vistas in the understanding of the disease. In 1958, Americo Negrette, a young Venezuelan physician observed similar subjects in the vicinity of Lake Maracaibo which was presented by his co-worker, Ramon Avilla Giron at New York in 1972 when United States of America had been commemorating the centenary year of Huntington′s disease. Nancy Wexler, a psychoanalyst, whose mother had been suffering from the disease attended the meeting and organized a research team to Venezuela and they systematically studied more than 18,000 individuals in order to work out a common pedigree. They identified the genetic locus of the disease in the short arm of chromosome 4 and observed that it was a trinucleotide repeat disorder.

  6. Bradykinesia in Huntington's disease. A prospective, follow-up study. (United States)

    García Ruiz, Pedro J; Hernández, Jaime; Cantarero, Susana; Bartolomé, Manuel; Sánchez Bernardos, Vicenta; García de Yébenez, Justo


    Bradykinesia is a frequent finding in Huntington's disease (HD), but some aspects are presently unknown; including the natural evolution of bradykinesia over time and the correlation between bradykinesia and functional capacity. We studied the motor performance of 20 genetically confirmed patients with HD (age: 40+/-10.8 years; age at onset 33.6+/-11 years; total functional capacity (TFC): 9.57+/-3; UHDRS total motor scale: 31.4+/-13, triplet length (CAG)n: 46.7+/-4 triplets). These patients were studied in baseline conditions and after 18.7+/-6 months of follow-up. In addition, HD patients were compared with 20 age-matched normal controls. Motor study included the four CAPIT timed tests commonly used for Parkinson's disease: pronation-supination (PS), finger dexterity (FD), movement between two points (MTP) and walking test (WT). HD patients were significantly slower than controls in all motor tasks. A significant deterioration occurred over time in three of the four motor tasks (especially FD and WT). A significant correlation between timed tests and TFC score was found (for MTP, r: -0.845; p < 0,0001). In addition a significant correlation between timed tests and the UHDRDS total motor scale was also found (for MTP, r: 0.864; p < 0.0001). In conclusion, simple timed motor tests can detect a deterioration of motor activity over time in HD. Timed tests might be useful to follow the natural evolution of HD and to assess the efficacy of new therapies.

  7. Medical management of motor manifestations of Huntington disease. (United States)

    McCusker, Elizabeth A; Loy, Clement T


    The motor and movement disorders of Huntington disease (HD) are managed in the context of the other disease features. Chorea and dystonia are the most common HD-associated movement disorders, and they can be assessed on research rating scales. However other motor manifestations have a significant impact. In particular, dysphagia influences choice and tolerance of treatment for the movement disorder, as will comorbidities, patient awareness, and distress related to the motor feature or movement. Treatment for other disease features may aggravate the motor disorder, e.g., increased swallowing difficulty associated with antipsychotic agents. Basic principles in deciding to institute a treatment are outlined as well as treatment of specific motor manifestations and movements. There is a paucity of evidence to support the treatments available for the motor disorder, with only one agent with class 1 evidence, tetrabenazine, for chorea. There are, however, treatments informed by expert opinion which reflect the management of a wider HD phenotype than that represented in clinical trials. Some treatments are based on evidence from use in other conditions. Medical management is usually undertaken later in the disease with concurrent nonmedical interventions after multidisciplinary assessments. Medication review with HD progression is essential. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. Pitfalls in the detection of cholesterol in Huntington's disease models. (United States)

    Marullo, Manuela; Valenza, Marta; Leoni, Valerio; Caccia, Claudio; Scarlatti, Chiara; De Mario, Agnese; Zuccato, Chiara; Di Donato, Stefano; Carafoli, Ernesto; Cattaneo, Elena


    Background Abnormalities in brain cholesterol homeostasis have been reported in Huntington's disease (HD), an adult-onset neurodegenerative disorder caused by an expansion in the number of CAG repeats in the huntingtin (HTT) gene. However, the results have been contradictory with respect to whether cholesterol levels increase or decrease in HD models. Biochemical and mass spectrometry methods show reduced levels of cholesterol precursors and cholesterol in HD cells and in the brains of several HD animal models. Abnormal brain cholesterol homeostasis was also inferred from studies in HD patients. In contrast, colorimetric and enzymatic methods indicate cholesterol accumulation in HD cells and tissues. Here we used several methods to investigate cholesterol levels in cultured cells in the presence or absence of mutant HTT protein. Results Colorimetric and enzymatic methods with low sensitivity gave variable results, whereas results from a sensitive analytical method, gas chromatography-mass spectrometry, were more reliable. Sample preparation, high cell density and cell clonality also influenced the detection of intracellular cholesterol. Conclusions Detection of cholesterol in HD samples by colorimetric and enzymatic assays should be supplemented by detection using more sensitive analytical methods. Care must be taken to prepare the sample appropriately. By evaluating lathosterol levels using isotopic dilution mass spectrometry, we confirmed reduced cholesterol biosynthesis in knock-in cells expressing the polyQ mutation in a constitutive or inducible manner. *Correspondence should be addressed to Elena Cattaneo:

  9. Mitochondrial DNA levels in Huntington disease leukocytes and dermal fibroblasts. (United States)

    Jędrak, Paulina; Krygier, Magdalena; Tońska, Katarzyna; Drozd, Małgorzata; Kaliszewska, Magdalena; Bartnik, Ewa; Sołtan, Witold; Sitek, Emilia J; Stanisławska-Sachadyn, Anna; Limon, Janusz; Sławek, Jarosław; Węgrzyn, Grzegorz; Barańska, Sylwia


    Huntington disease (HD) is an inherited neurodegenerative disorder caused by mutations in the huntingtin gene. Involvement of mitochondrial dysfunctions in, and especially influence of the level of mitochondrial DNA (mtDNA) on, development of this disease is unclear. Here, samples of blood from 84 HD patients and 79 controls, and dermal fibroblasts from 10 HD patients and 9 controls were analysed for mtDNA levels. Although the type of mitochondrial haplogroup had no influence on the mtDNA level, and there was no correlation between mtDNA level in leukocytes in HD patients and various parameters of HD severity, some considerable differences between HD patients and controls were identified. The average mtDNA/nDNA relative copy number was significantly higher in leukocytes, but lower in fibroblasts, of symptomatic HD patients relative to the control group. Moreover, HD women displayed higher mtDNA levels in leukocytes than HD men. Because this is the largest population analysed to date, these results might contribute to explanation of discrepancies between previously published studies concerning levels of mtDNA in cells of HD patients. We suggest that the size of the investigated population and type of cells from which DNA is isolated could significantly affect results of mtDNA copy number estimation in HD. Hence, these parameters should be taken into consideration in studies on mtDNA in HD, and perhaps also in other diseases where mitochondrial dysfunction occurs.

  10. Cognitive and behavioral changes in Huntington disease before diagnosis. (United States)

    Paulsen, Jane S; Miller, Amanda C; Hayes, Terry; Shaw, Emily


    Phenotypic manifestations of Huntington disease (HD) can be detected at least 15 years prior to the time when a motor diagnosis is given. Advances in clinical care and future research will require consistent use of HD definitions and HD premanifest (prodromal) stages being used across clinics, sites, and countries. Cognitive and behavioral (psychiatric) changes in HD are summarized and implications for ongoing advancement in our knowledge of prodromal HD are suggested. The earliest detected cognitive changes are observed in the Symbol Digit Modalities Test, Stroop Interference, Stroop Color and Word Test-interference condition, and Trail Making Test. Cognitive changes in the middle and near motor diagnostic stages of prodromal HD involve nearly every cognitive test administered and the greatest changes over time (i.e., slopes) are found in those prodromal HD participants who are nearest to motor diagnosis. Psychiatric changes demonstrate significant worsening over time and remain elevated compared with healthy controls throughout the prodromal disease course. Psychiatric and behavior changes in prodromal HD are much lower than that obtained using cognitive assessment, although the psychiatric and behavioral changes represent symptoms most debilitating to independent capacity and wellness. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Controlled clinical trial of cannabidiol in Huntington's disease. (United States)

    Consroe, P; Laguna, J; Allender, J; Snider, S; Stern, L; Sandyk, R; Kennedy, K; Schram, K


    Based on encouraging preliminary findings, cannabidiol (CBD), a major nonpsychotropic constituent of Cannabis, was evaluated for symptomatic efficacy and safety in 15 neuroleptic-free patients with Huntington's Disease (HD). The effects of oral CBD (10 mg/kg/day for 6 weeks) and placebo (sesame oil for 6 weeks) were ascertained weekly under a double-blind, randomized cross-over design. A comparison of the effects of CBD and placebo on chorea severity and other therapeutic outcome variables, and on a Cannabis side effect inventory, clinical lab tests and other safety outcome variables, indicated no significant (p greater than 0.05) or clinically important differences. Correspondingly, plasma levels of CBD were assayed by GC/MS, and the weekly levels (mean range of 5.9 to 11.2 ng/ml) did not differ significantly over the 6 weeks of CBD administration. In summary, CBD, at an average daily dose of about 700 mg/day for 6 weeks, was neither symptomatically effective nor toxic, relative to placebo, in neuroleptic-free patients with HD.

  12. Total recognition discriminability in Huntington's and Alzheimer's disease. (United States)

    Graves, Lisa V; Holden, Heather M; Delano-Wood, Lisa; Bondi, Mark W; Woods, Steven Paul; Corey-Bloom, Jody; Salmon, David P; Delis, Dean C; Gilbert, Paul E


    Both the original and second editions of the California Verbal Learning Test (CVLT) provide an index of total recognition discriminability (TRD) but respectively utilize nonparametric and parametric formulas to compute the index. However, the degree to which population differences in TRD may vary across applications of these nonparametric and parametric formulas has not been explored. We evaluated individuals with Huntington's disease (HD), individuals with Alzheimer's disease (AD), healthy middle-aged adults, and healthy older adults who were administered the CVLT-II. Yes/no recognition memory indices were generated, including raw nonparametric TRD scores (as used in CVLT-I) and raw and standardized parametric TRD scores (as used in CVLT-II), as well as false positive (FP) rates. Overall, the patient groups had significantly lower TRD scores than their comparison groups. The application of nonparametric and parametric formulas resulted in comparable effect sizes for all group comparisons on raw TRD scores. Relative to the HD group, the AD group showed comparable standardized parametric TRD scores (despite lower raw nonparametric and parametric TRD scores), whereas the previous CVLT literature has shown that standardized TRD scores are lower in AD than in HD. Possible explanations for the similarity in standardized parametric TRD scores in the HD and AD groups in the present study are discussed, with an emphasis on the importance of evaluating TRD scores in the context of other indices such as FP rates in an effort to fully capture recognition memory function using the CVLT-II.

  13. Therapeutic approaches to preventing cell death in Huntington disease. (United States)

    Kaplan, Anna; Stockwell, Brent R


    Neurodegenerative diseases affect the lives of millions of patients and their families. Due to the complexity of these diseases and our limited understanding of their pathogenesis, the design of therapeutic agents that can effectively treat these diseases has been challenging. Huntington disease (HD) is one of several neurological disorders with few therapeutic options. HD, like numerous other neurodegenerative diseases, involves extensive neuronal cell loss. One potential strategy to combat HD and other neurodegenerative disorders is to intervene in the execution of neuronal cell death. Inhibiting neuronal cell death pathways may slow the development of neurodegeneration. However, discovering small molecule inhibitors of neuronal cell death remains a significant challenge. Here, we review candidate therapeutic targets controlling cell death mechanisms that have been the focus of research in HD, as well as an emerging strategy that has been applied to developing small molecule inhibitors-fragment-based drug discovery (FBDD). FBDD has been successfully used in both industry and academia to identify selective and potent small molecule inhibitors, with a focus on challenging proteins that are not amenable to traditional high-throughput screening approaches. FBDD has been used to generate potent leads, pre-clinical candidates, and has led to the development of an FDA approved drug. This approach can be valuable for identifying modulators of cell-death-regulating proteins; such compounds may prove to be the key to halting the progression of HD and other neurodegenerative disorders. Copyright © 2012 Elsevier Ltd. All rights reserved.

  14. Predictive gene testing for Huntington disease and other neurodegenerative disorders. (United States)

    Wedderburn, S; Panegyres, P K; Andrew, S; Goldblatt, J; Liebeck, T; McGrath, F; Wiltshire, M; Pestell, C; Lee, J; Beilby, J


    Controversies exist around predictive testing (PT) programmes in neurodegenerative disorders. This study sets out to answer the following questions relating to Huntington disease (HD) and other neurodegenerative disorders: differences between these patients in their PT journeys, why and when individuals withdraw from PT, and decision-making processes regarding reproductive genetic testing. A case series analysis of patients having PT from the multidisciplinary Western Australian centre for PT over the past 20 years was performed using internationally recognised guidelines for predictive gene testing in neurodegenerative disorders. Of 740 at-risk patients, 518 applied for PT: 466 at risk of HD, 52 at risk of other neurodegenerative disorders - spinocerebellar ataxias, hereditary prion disease and familial Alzheimer disease. Thirteen percent withdrew from PT - 80.32% of withdrawals occurred during counselling stages. Major withdrawal reasons related to timing in the patients' lives or unknown as the patient did not disclose the reason. Thirty-eight HD individuals had reproductive genetic testing: 34 initiated prenatal testing (of which eight withdrew from the process) and four initiated pre-implantation genetic diagnosis. There was no recorded or other evidence of major psychological reactions or suicides during PT. People withdrew from PT in relation to life stages and reasons that are unknown. Our findings emphasise the importance of: (i) adherence to internationally recommended guidelines for PT; (ii) the role of the multidisciplinary team in risk minimisation; and (iii) patient selection. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

  15. Constitutive upregulation of chaperone-mediated autophagy in Huntington's disease. (United States)

    Koga, Hiroshi; Martinez-Vicente, Marta; Arias, Esperanza; Kaushik, Susmita; Sulzer, David; Cuervo, Ana Maria


    Autophagy contributes to the removal of prone-to-aggregate proteins, but in several instances these pathogenic proteins have been shown to interfere with autophagic activity. In the case of Huntington's disease (HD), a congenital neurodegenerative disorder resulting from mutation in the huntingtin protein, we have previously described that the mutant protein interferes with the ability of autophagic vacuoles to recognize cytosolic cargo. Growing evidence supports the existence of cross talk among autophagic pathways, suggesting the possibility of functional compensation when one of them is compromised. In this study, we have identified a compensatory upregulation of chaperone-mediated autophagy (CMA) in different cellular and mouse models of HD. Components of CMA, namely the lysosome-associated membrane protein type 2A (LAMP-2A) and lysosomal-hsc70, are markedly increased in HD models. The increase in LAMP-2A is achieved through both an increase in the stability of this protein at the lysosomal membrane and transcriptional upregulation of this splice variant of the lamp-2 gene. We propose that CMA activity increases in response to macroautophagic dysfunction in the early stages of HD, but that the efficiency of this compensatory mechanism may decrease with age and so contribute to cellular failure and the onset of pathological manifestations.

  16. Huntington's disease in Greece: the experience of 14 years. (United States)

    Panas, M; Karadima, G; Vassos, E; Kalfakis, N; Kladi, A; Christodoulou, K; Vassilopoulos, D


    A large scale genetic and epidemiological study of Huntington's disease (HD) was carried out in Greece from January 1995 to December 2008. Diagnostic testing was carried out in 461 symptomatic individuals, while 256 were tested for presymptomatic purposes. The diagnosis of HD with a CAG expansion ≥ 36 was confirmed in 278 symptomatic individuals. The prevalence of HD in Greece was estimated at approximately 2.5 to 5.4:100,000, while the mean minimum incidence was estimated at 2.2 to 4.4 per million per year. The molecular diagnosis of HD was confirmed in the majority of patients (84.4%) sent for confirmation. The false-positive cases 15.6% were characterized by the absence of a family history of HD and the presence of an atypical clinical picture. The uptake of predictive testing for HD was 8.6%. A prenatal test was requested in six pregnancies. The findings of our study do not differ significantly from those of similar studies from other European countries despite the relative genetic isolation of Greece. Of interest is the identification of clusters of HD in Greece. The presence or absence of a family history of HD should be interpreted cautiously, during the diagnostic process. © 2010 John Wiley & Sons A/S.

  17. Plasma homovanillic acid and prolactin in Huntington's disease. (United States)

    Markianos, Manolis; Panas, Marios; Kalfakis, Nikos; Vassilopoulos, Dimitrios


    Dopaminergic activity is expected to be altered in patients with Huntington's disease (HD) and be related to factors like duration and severity of illness or patients' specific symptomatology like dementia, depression, or psychotic features. We assessed plasma homovanillic acid (pHVA) and plasma prolactin (pPRL), two correlates of dopaminergic activity, in 116 subjects with CAG repeats expansion in the HD gene, 26 presymptomatic (18 females) and 90 with overt symptomatology (43 females). Patients were evaluated using the Unified HD Rating Scale and the Total Functional Capacity Scale. Presence of dementia, depression, and psychotic features were also assessed. The age range of the patients was 22-83 years, duration of illness from 0.5 to 27 years, and CAG repeat number from 34 to 66. A group of 60 age and sex matched healthy subjects served as control group. Plasma PRL in subjects at risk and in neuroleptic-free patients, evaluated separately for males and females, did not differ from controls. Plasma HVA levels did not differ from controls in the group of presymptomatic subjects, but were significantly higher in the patients group. This increase was positively associated mainly with severity of illness and functional capacity of the patients, and not with presence of depression or dementia. Plasma HVA levels may be proven to be a peripheral index of disease progression. Reducing dopaminergic activity may have not only symptomatic, but also neuroprotective effects in HD.

  18. Great auricular neuropraxia with beach chair position

    Directory of Open Access Journals (Sweden)

    Joshi M


    Full Text Available Minal Joshi,1 Ruth Cheng,2 Hattiyangadi Kamath,1 Joel Yarmush1 1Department of Anesthesiology, New York Methodist Hospital, New York, NY, USA; 2School of Medicine, St. George’s University, Grenada, West Indies Abstract: Shoulder arthroscopy has been shown to be the procedure of choice for many diagnostic and therapeutic interventions. Neuropraxia of the great auricular nerve (GAN is an uncommon complication of shoulder surgery, with the patient in the beach chair position. We report a case of great auricular neuropraxia associated with direct compression by a horseshoe headrest, used in routine positioning for uncomplicated shoulder surgery. In this case, an arthroscopic approach was taken, under regional anesthesia with sedation in the beach chair position. The GAN, a superficial branch of the cervical plexus, is vulnerable to neuropraxia due to its superficial anatomical location. We recommend that for the procedures of the beach chair position, the auricle be protected and covered with cotton and gauze to avoid direct compression and the position of the head and neck be checked and corrected frequently. Keywords: neuropraxia, anesthesia, arthroscopy, great auricular nerve

  19. Intensified coastal development behind nourished beaches (United States)

    Armstrong, Scott; Lazarus, Eli; Limber, Patrick; Goldstein, Evan; Thorpe, Curtis; Ballinger, Rhoda


    Population density, housing development, and property values in coastal counties along the U.S. Atlantic and Gulf Coasts continue to rise despite increasing hazard from storm impacts. Since the 1970s, beach nourishment, which involves importing sand to deliberately widen an eroding beach, has been the main strategy in the U.S. for protecting coastal properties from erosion and flooding hazards. Paradoxically, investment in hazard protection may intensify development. Here, we examine the housing stock of all existing shorefront single-family homes in Florida - a microcosm of U.S. coastal hazards and development - to quantitatively compare development in nourishing and non-nourishing towns. We find that nourishing towns now account for more than half of Florida's coastline, and that houses in nourishing towns are larger and more numerous. Even as the mean size of single-family homes nationwide has grown steadily since 1970, Florida's shorefront stock has exceeded the national average by 34%, and in nourishing towns by 45%. This emergent disparity between nourishing and non-nourishing towns in Florida demonstrates a pattern of intensifying coastal risk, and is likely representative of a dominant trend in coastal development more generally. These data lend empirical support to the hypothesis that US coastal development and hazard mitigation through beach nourishment have become dynamically coupled.

  20. Internal wave turbulence near a Texel beach.

    Directory of Open Access Journals (Sweden)

    Hans van Haren

    Full Text Available A summer bather entering a calm sea from the beach may sense alternating warm and cold water. This can be felt when moving forward into the sea ('vertically homogeneous' and 'horizontally different', but also when standing still between one's feet and body ('vertically different'. On a calm summer-day, an array of high-precision sensors has measured fast temperature-changes up to 1 °C near a Texel-island (NL beach. The measurements show that sensed variations are in fact internal waves, fronts and turbulence, supported in part by vertical stable stratification in density (temperature. Such motions are common in the deep ocean, but generally not in shallow seas where turbulent mixing is expected strong enough to homogenize. The internal beach-waves have amplitudes ten-times larger than those of the small surface wind waves. Quantifying their turbulent mixing gives diffusivity estimates of 10(-4-10(-3 m(2 s(-1, which are larger than found in open-ocean but smaller than wave breaking above deep sloping topography.

  1. Recreational impacts on Colorado River beaches in Glen Canyon, Arizona (United States)

    Carothers, Steven W.; Johnson, Robert A.; Dolan, Robert


    Recreational impact was measured on eight beaches in Glen Canyon National Recreation Area and 15 beaches in Grand Canyon National Park using permanently located transects and plots. Recreational impact indices included densities of human trash and charcoal and a measure of sand discoloration due to charcoal. Significant increases in the indices occurred on several Glen Canyon beaches over a seven-month period. Sand discoloration became significantly higher over all Glen Canyon beaches during the same time period. All indices were significantly higher in Glen Canyon than on similar Grand Canyon beaches. These differences are probably due to differences in: (a) level of impacts tolerated by the respective management regimes and, (b) in the number of user days among the two National Park Service administrative units. Management alternatives are presented for reversing the present trends of recreational impact on Glen Canyon beaches.

  2. Significance of beach geomorphology on fecal indicator bacteria levels. (United States)

    Donahue, Allison; Feng, Zhixuan; Kelly, Elizabeth; Reniers, Ad; Solo-Gabriele, Helena M


    Large databases of fecal indicator bacteria (FIB) measurements are available for coastal waters. With the assistance of satellite imagery, we illustrated the power of assessing data for many sites by evaluating beach features such as geomorphology, distance from rivers and canals, presence of piers and causeways, and degree of urbanization coupled with the enterococci FIB database for the state of Florida. We found that beach geomorphology was the primary characteristic associated with enterococci levels that exceeded regulatory guidelines. Beaches in close proximity to marshes or within bays had higher enterococci exceedances in comparison to open coast beaches. For open coast beaches, greater enterococci exceedances were associated with nearby rivers and higher levels of urbanization. Piers and causeways had a minimal contribution, as their effect was often overwhelmed by beach geomorphology. Results can be used to understand the potential causes of elevated enterococci levels and to promote public health. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. Shifts in the microbial community composition of Gulf Coast beaches following beach oiling.

    Directory of Open Access Journals (Sweden)

    Ryan J Newton

    Full Text Available Microorganisms associated with coastal sands serve as a natural biofilter, providing essential nutrient recycling in nearshore environments and acting to maintain coastal ecosystem health. Anthropogenic stressors often impact these ecosystems, but little is known about whether these disturbances can be identified through microbial community change. The blowout of the Macondo Prospect reservoir on April 20, 2010, which released oil hydrocarbons into the Gulf of Mexico, presented an opportunity to examine whether microbial community composition might provide a sensitive measure of ecosystem disturbance. Samples were collected on four occasions, beginning in mid-June, during initial beach oiling, until mid-November from surface sand and surf zone waters at seven beaches stretching from Bay St. Louis, MS to St. George Island, FL USA. Oil hydrocarbon measurements and NOAA shoreline assessments indicated little to no impact on the two most eastern beaches (controls. Sequence comparisons of bacterial ribosomal RNA gene hypervariable regions isolated from beach sands located to the east and west of Mobile Bay in Alabama demonstrated that regional drivers account for markedly different bacterial communities. Individual beaches had unique community signatures that persisted over time and exhibited spatial relationships, where community similarity decreased as horizontal distance between samples increased from one to hundreds of meters. In contrast, sequence analyses detected larger temporal and less spatial variation among the water samples. Superimposed upon these beach community distance and time relationships, was increased variability in bacterial community composition from oil hydrocarbon contaminated sands. The increased variability was observed among the core, resident, and transient community members, indicating the occurrence of community-wide impacts rather than solely an overprinting of oil hydrocarbon-degrading bacteria onto otherwise

  4. Dune recovery after storm erosion on a high-energy beach: Vougot Beach, Brittany (France) (United States)

    Suanez, Serge; Cariolet, Jean-Marie; Cancouët, Romain; Ardhuin, Fabrice; Delacourt, Christophe


    On 10th March 2008, the high energy storm Johanna hit the French Atlantic coast, generating severe dune erosion on Vougot Beach (Brittany, France). In this paper, the recovery of the dune of Vougot Beach is analysed through a survey of morphological changes and hydrodynamic conditions. Data collection focused on the period immediately following storm Johanna until July 2010, i.e. over two and a half years. Results showed that the dune retreated by a maximum of almost 6 m where storm surge and wave attack were the most energetic. Dune retreat led to the creation of accommodation space for the storage of sediment by widening and elevating space between the pre- and post-storm dune toe, and reducing impacts of the storm surge. Dune recovery started in the month following the storm event and is still ongoing. It is characterised by the construction of "secondary" embryo dunes, which recovered at an average rate of 4-4.5 cm per month, although average monthly volume changes varied from - 1 to 2 m 3.m - 1 . These embryo dunes accreted due to a large aeolian sand supply from the upper tidal beach to the existing foredune. These dune-construction processes were facilitated by growth of vegetation on low-profile embryo dunes promoting backshore accretion. After more than two years of survey, the sediment budget of the beach/dune system showed that more than 10,000 m 3 has been lost by the upper tidal beach. We suggest that seaward return currents generated during the storm of 10th March 2008 are responsible for offshore sediment transport. Reconstitution of the equilibrium beach profile following the storm event may therefore have generated cross-shore sediment redistribution inducing net erosion in the tidal zone.

  5. HARDNESS PHENOMENON IN BEACH PEA (Lethyrus maritimus L.)


    U.D. Chavan; R. Amarowicz; F. Shahidi


    Beach pea is mostly grown on seashores and it contains higher amount of protein than other legumes. However, the pea has several undesirable  attributes, such as long cooking time and hard to germinate (imbibitions) that limited its use as food. The present investigation aimed to study the physico-chemical properties, cooking characteristics and hull crude fibre structure of beach pea as compare to other similar legumes. Standard methods of processing pulses were used for present study. Beach...

  6. Storm Impact Assessment for Beaches at Panama City, Florida (United States)


    Panama City Beaches, and they contain a wide variety of beach homes, condominiums, hotels, small commercial tourism - based enterprises, and resorts. The...exam Mexico Beach T O2.5 miles MaVO Ma KLLT GUL F OF MEXI CO Erosion Area No. 5I C EWoM Crooked Island 4.2 miles ECT Erosion Area No. 4 BAY Lwcmca.n

  7. A 24-Hour Study of the Hypothalamo-Pituitary Axes in Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Eirini Kalliolia

    Full Text Available Huntington's disease is an inherited neurodegenerative disorder characterised by motor, cognitive and psychiatric disturbances. Patients exhibit other symptoms including sleep and mood disturbances, muscle atrophy and weight loss which may be linked to hypothalamic pathology and dysfunction of hypothalamo-pituitary axes.We studied neuroendocrine profiles of corticotropic, somatotropic and gonadotropic hypothalamo-pituitary axes hormones over a 24-hour period in controlled environment in 15 healthy controls, 14 premanifest and 13 stage II/III Huntington's disease subjects. We also quantified fasting levels of vasopressin, oestradiol, testosterone, dehydroepiandrosterone sulphate, thyroid stimulating hormone, free triiodothyronine, free total thyroxine, prolactin, adrenaline and noradrenaline. Somatotropic axis hormones, growth hormone releasing hormone, insulin-like growth factor-1 and insulin-like factor binding protein-3 were quantified at 06:00 (fasting, 15:00 and 23:00. A battery of clinical tests, including neurological rating and function scales were performed.24-hour concentrations of adrenocorticotropic hormone, cortisol, luteinizing hormone and follicle-stimulating hormone did not differ significantly between the Huntington's disease group and controls. Daytime growth hormone secretion was similar in control and Huntington's disease subjects. Stage II/III Huntington's disease subjects had lower concentration of post-sleep growth hormone pulse and higher insulin-like growth factor-1:growth hormone ratio which did not reach significance. In Huntington's disease subjects, baseline levels of hypothalamo-pituitary axis hormones measured did not significantly differ from those of healthy controls.The relatively small subject group means that the study may not detect subtle perturbations in hormone concentrations. A targeted study of the somatotropic axis in larger cohorts may be warranted. However, the lack of significant results despite many

  8. Music therapy in Huntington's disease: a protocol for a multi-center randomized controlled trial. (United States)

    van Bruggen-Rufi, Monique; Vink, Annemieke; Achterberg, Wilco; Roos, Raymund


    Huntington's disease is a progressive, neurodegenerative disease with autosomal dominant inheritance, characterized by motor disturbances, cognitive decline and behavioral and psychological symptoms. Since there is no cure, all treatment is aimed at improving quality of life. Music therapy is a non-pharmacological intervention, aiming to improve the quality of life, but its use and efficacy in patients with Huntington's disease has hardly been studied. In this article, a protocol is described to study the effects of music therapy in comparison with a control intervention to improve quality of life through stimulating expressive and communicative skills. By targeting these skills we assume that the social-cognitive functioning will improve, leading to a reduction in behavioral problems, resulting in an overall improvement of the quality of life in patients with Huntington's disease. The study is designed as a multi-center single-blind randomised controlled intervention trial. Sixty patients will be randomised using centre-stratified block-permuted randomisation. Patients will be recruited from four long-term care facilities specialized in Huntington's disease-care in The Netherlands. The outcome measure to assess changes in expressive and communication skills is the Behaviour Observation Scale Huntington and changes in behavior will be assessed by the Problem Behaviour Assesment-short version and by the BOSH. Measurements take place at baseline, then 8, 16 (end of intervention) and 12 weeks after the last intervention (follow-up). This randomized controlled study will provide greater insight into the effectiveness of music therapy on activities of daily living, social-cognitive functioning and behavior problems by improving expressive and communication skills, thus leading to a better quality of life for patients with Huntington's disease. Netherlands Trial Register: NTR4904 , registration date Nov. 15, 2014.

  9. Characterisation of aggression in Huntington's disease: rates, types and antecedents in an inpatient rehabilitation setting. (United States)

    Brown, Anahita; Sewell, Katherine; Fisher, Caroline A


    To systematically review aggression in an inpatient Huntington's cohort examining rates, types and antecedents. Although the prevalence of aggression in Huntington's disease is high, research into this problematic behaviour has been limited. Few studies have investigated the nature of aggressive behaviour in Huntington's disease or antecedents that contribute to its occurrence. A systematic, double-coded, electronic medical file audit. The electronic hospital medical records of 10 people with Huntington's disease admitted to a brain disorders unit were audited for a 90-day period using the Overt Aggression Scale-Modified for Neurorehabilitation framework, yielding 900 days of clinical data. Nine of 10 clients exhibited aggression during the audit period. Both verbal (37·1%) aggression and physical aggression were common (33·8%), along with episodes of mixed verbal and physical aggression (15·2%), while aggression to objects/furniture was less prevalent (5·5%). The most common antecedent was physical guidance with personal care, far exceeding any other documented antecedents, and acting as the most common trigger for four of the nine clients who exhibited aggression. For the remaining five clients, there was intraindividual heterogeneity in susceptibility to specific antecedents. In Huntington's sufferers at mid- to late stages following disease onset, particular care should be made with personal care assistance due to the propensity for these procedures to elicit an episode of aggression. However, given the degree of intraindividual heterogeneity in susceptibility to specific antecedents observed in the present study, individualised behaviour support plans and sensory modulation interventions may be the most useful in identifying triggers and managing aggressive episodes. Rates of aggression in Huntington's disease inpatients can be high. Knowledge of potential triggers, such as personal care, is important for nursing and care staff, so that attempts can be

  10. Chenang Beach and its Crowding Capacity: A Malaysian Perspective

    Directory of Open Access Journals (Sweden)

    Mohamad Diana


    Full Text Available This working paper focuses in enjoyment factors, specifically: number of beach users, perceived maximum number of beach users accepted, perceived maximum number of beach users that affects the tourism experience and perceived maximum number of beach users that affects the beach quality. At a deeper extent, the evaluation is categorized by number of visitation, visitation motivations, and Chenang Island’s push and pull factors. Relationships between variables were assessed using a two-phase evaluation framework where interestingly, only one demographic factor works with all the studied independent variables. It is also learned that the density of an area number of people seen is considered as a n accepted crowding factor, as opposed to this working paper scope experienced crowding . A unique relationship was observed for crowding level, and visitation satisfaction level and overall evaluation of Chenang beach quality. This working paper further supports the previous literature on the significance of beach carrying capacity management and it is learned that the idea of crowding standard is interlinks with ‘gender, ‘time spend’ and ‘number of boaters’. From findings, this working paper envisages the preferences polar exchange where this should be of interest to tourism-related personnel. It is within this working paper interest to highlight the pressing need in brandishing the image of Chenang Beach. This is to ensure that Chenang Beach, as a field, is maintaining its importance and popularity.

  11. Exploring the social dimension of sandy beaches through predictive modelling. (United States)

    Domínguez-Tejo, Elianny; Metternicht, Graciela; Johnston, Emma L; Hedge, Luke


    Sandy beaches are unique ecosystems increasingly exposed to human-induced pressures. Consistent with emerging frameworks promoting this holistic approach towards beach management, is the need to improve the integration of social data into management practices. This paper aims to increase understanding of links between demographics and community values and preferred beach activities, as key components of the social dimension of the beach environment. A mixed method approach was adopted to elucidate users' opinions on beach preferences and community values through a survey carried out in Manly Local Government Area in Sydney Harbour, Australia. A proposed conceptual model was used to frame demographic models (using age, education, employment, household income and residence status) as predictors of these two community responses. All possible regression-model combinations were compared using Akaike's information criterion. Best models were then used to calculate quantitative likelihoods of the responses, presented as heat maps. Findings concur with international research indicating the relevance of social and restful activities as important social links between the community and the beach environment. Participant's age was a significant variable in the four predictive models. The use of predictive models informed by demographics could potentially increase our understanding of interactions between the social and ecological systems of the beach environment, as a prelude to integrated beach management approaches. The research represents a practical demonstration of how demographic predictive models could support proactive approaches to beach management. Copyright © 2018 Elsevier Ltd. All rights reserved.

  12. Hyperspectral image classifier based on beach spectral feature

    International Nuclear Information System (INIS)

    Liang, Zhang; Lianru, Gao; Bing, Zhang


    The seashore, especially coral bank, is sensitive to human activities and environmental changes. A multispectral image, with coarse spectral resolution, is inadaptable for identify subtle spectral distinctions between various beaches. To the contrary, hyperspectral image with narrow and consecutive channels increases our capability to retrieve minor spectral features which is suit for identification and classification of surface materials on the shore. Herein, this paper used airborne hyperspectral data, in addition to ground spectral data to study the beaches in Qingdao. The image data first went through image pretreatment to deal with the disturbance of noise, radiation inconsistence and distortion. In succession, the reflection spectrum, the derivative spectrum and the spectral absorption features of the beach surface were inspected in search of diagnostic features. Hence, spectra indices specific for the unique environment of seashore were developed. According to expert decisions based on image spectrums, the beaches are ultimately classified into sand beach, rock beach, vegetation beach, mud beach, bare land and water. In situ surveying reflection spectrum from GER1500 field spectrometer validated the classification production. In conclusion, the classification approach under expert decision based on feature spectrum is proved to be feasible for beaches

  13. Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease

    DEFF Research Database (Denmark)

    Aziz, N A; Jurgens, C K; Landwehrmeyer, G B


    OBJECTIVE: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in the HD gene (HTT). We aimed to assess whether interaction between CAG repeat sizes in the mutant and normal allele could affect disease severity and progression. METHODS: Using...... with less severe symptoms and pathology. CONCLUSIONS: Increasing CAG repeat size in normal HTT diminishes the association between mutant CAG repeat size and disease severity and progression in Huntington disease. The underlying mechanism may involve interaction of the polyglutamine domains of normal...

  14. Mental Symptoms in Huntington's Disease and a Possible Primary Aminergic Neuron Lesion (United States)

    Mann, J. John; Stanley, Michael; Gershon, Samuel; Rossor, M.


    Monoamine oxidase activity was higher in the cerebral cortex and basal ganglia of patients dying from Huntington's disease than in controls. Enzyme kinetics and multiple substrate studies indicated that the increased activity was due to elevated concentrations of monoamine oxidase type B. Concentrations of homovanillic acid were increased in the cerebral cortex but not in the basal ganglia of brains of patients with Huntington's disease. These changes may represent a primary aminergic lesion that could underlie some of the mental symptoms of this disease.

  15. Predicting Fecal Indicator Bacteria Concentrations in the South Fork Broad River Watershed Using Virtual Beach (United States)

    Virtual Beach (VB) is a decision support tool that constructs site-specific statistical models to predict fecal indicator bacteria (FIB) at recreational beaches. Although primarily designed for making decisions regarding beach closures or issuance of swimming advisories based on...

  16. Beach changes at Visakhapatnam due to the cyclone of May 1979

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Rao, B.P.

    The impact of the May, 1979 cyclonic storm on Visakhapatnam beach, India and the observations made on beach profiles, waves and littoral currents prior to and during the storm are discussed. In general, at Visakhapatnam beach accretion trend starts...

  17. Wave refraction in relation to beach stability along the coast from Cape Ramas to Karwar

    Digital Repository Service at National Institute of Oceanography (India)

    Gouveia, A.D.; Joseph, P.S.; Kurup, P.G.

    Results of wave refraction and beach profile studies are presented for a stretch of 35 km shore line comprising of Loliem Beach, Karwar, Karnataka, India which is separated by rock promontories from comparatively stable beaches on either side of it...

  18. Tremor in neurodegenerative ataxias, Huntington disease and tic disorder. (United States)

    Rudzińska, M; Krawczyk, M; Wójcik-Pędziwiatr, M; Szczudlik, A; Tomaszewski, T


    Tremor is the most prevalent movement disorder, defined as rhythmic oscillations of a body part, caused by alternating or synchronic contractions of agonistic or antagonistic muscles. The aim of the study was to assess prevalence and to characterize parameters of tremor accompanying de-generative ataxias, Huntington disease (HD) and tic disorders in comparison with a control group. Forty-three patients with degenerative ataxias, 28 with HD and 26 with tic disorders together with 51 healthy controls were included in the study. For each participant, clinical and instrumental assessment (accelerometer, electromyography [EMG], graphic tablet) of hand tremor was performed. Frequency and severity of tremor were assessed in three positions: at rest (rest tremor), with hands extended (postural tremor), during the 'finger-to-nose' test and during Archimedes spiral drawing (kinetic tremor). Based on the mass load test, the type of tremor was determined as essential tremor type or enhanced physiological tremor type. The incidence of tremor in the accelerometry in patients with degenerative ataxia (50%) significantly differs from controls (10%) (p = 0.001). The dominant tremor was postural, low-intense, with 7-Hz frequency, essential tremor (23%) or other tremor type (23%), while enhanced physiological tremor was the least frequent (2%). Tremor in patients with HD and tic disorders was found in 10% and 20% of patients, respectively, similarly to the control group. Tremor was mild, postural and of essential tremor type, less frequently of enhanced physiological tremor type. No correlation between severity of tremor and severity of disease was found. The prevalence of tremor is considerably higher among patients with degenerative ataxias compared with HD, tic disorder and the control group. The most common type of tremor accompanying ataxias, HD and tic disorders is essential tremor type.

  19. Predictors of Workplace Disability in a Premanifest Huntington's Disease Cohort. (United States)

    Goh, Anita M Y; You, Emily; Perin, Stephanie; Clay, Fiona J; Loi, Samantha; Ellis, Kathryn; Chong, Terence; Ames, David; Lautenschlager, Nicola


    Huntington's disease (HD) is an inherited neurodegenerative disease involving motor, cognitive, and psychiatric/behavioral impairments that will eventually affect work role functioning. Few objective data exist regarding predictors of workplace disability in HD. The authors explored the predictors of work impairment and disability in a cross-sectional cohort of 656 employed, premanifest HD (preHD) individuals. In this cohort-the majority of whom were female, urban-dwelling, married/partnered, and working full-time, with minimal cognitive impairment, good function, minimal motor abnormality, and no indication of significant mental health issues-the number of participants who reported that they had missed work due to HD was low (2.4%). However, 12% of the study sample reported experiencing impairment while working due to preHD, 12.2% reported work-related activity impairment due to preHD, and 12.7% reported impairment in their overall work ability. Higher numbers of CAG repeats on the mutant allele and having more motor symptoms were associated with significantly higher odds of experiencing workplace impairment. Importantly, several modifiable factors were also found to predict workplace disability. Specifically, higher levels of anxiety symptoms were associated with significantly higher odds of experiencing workplace impairment. Good mental and physical health served as protective factors, where good physical health was associated with 6% lower odds of experiencing impairment or missing work time and good mental health was associated with of 10%-12% lower. The results provide important new knowledge for the development of future targeted intervention trials to support preHD individuals in maintaining their work roles as long as possible.

  20. Patterns of False Memory in Patients with Huntington's Disease. (United States)

    Chen, I-Wen; Chen, Chiung-Mei; Wu, Yih-Ru; Hua, Mau-Sun


    Increased false memory recognition in patients with Huntington's disease (HD) has been widely reported; however, the underlying memory constructive processes remain unclear. The present study explored gist memory, item-specific memory, and monitoring ability in patients with HD. Twenty-five patients (including 13 patients with mild HD and 12 patients with moderate-to-severe HD) and 30 healthy comparison participants (HC) were recruited. We used the Deese-Roediger-McDermott (DRM) paradigm to investigate participants' false recognition patterns, along with neuropsychological tests to assess general cognitive function. Both mild and moderate-to-severe patients with HD showed significant executive functioning and episodic memory impairment. On the DRM tasks, both HD patient groups showed significantly impaired performance in tasks assessing unrelated false recognition and item-specific memory as compared to the HC group; moderate-to-severe patients performed more poorly than mild patients did. Only moderate-severe patients exhibited significantly poorer related false recognition index scores than HCs in the verbal DRM task; performance of HD patient groups was comparable to the HC group on the pictorial DRM task. It appears that diminished verbatim memory and monitoring ability are early signs of cognitive decline during the HD course. Conversely, gist memory is relatively robust, with only partial decline during advanced-stage HD. Our findings suggest that medial temporal lobe function is relatively preserved compared to that of frontal-related structures in early HD. Thus, gist-based memory rehabilitation programs might be beneficial for patients with HD. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail:

  1. An electrophysiological analysis of altered cognitive functions in Huntington disease. (United States)

    Münte, T F; Ridao-Alonso, M E; Preinfalk, J; Jung, A; Wieringa, B M; Matzke, M; Dengler, R; Johannes, S


    Neuropsychological deficits are a main feature of Huntington disease (HD) with previous data suggesting involvement of memory functions and visual processing. To increase the knowledge about cognitive malfunction in HD in the domains of visual processing and memory by the use of modern electrophysiological techniques (event-related potentials [ERPs]). A case-control design was used. Three ERP paradigms were used; a parallel visual search paradigm allowed for the simultaneous processing of a multi-element visual array in search of a target stimulus, while a serial search paradigm with varied numbers of distractor items necessitated a serial one by one scanning of the arrays. The third experiment was a word-recognition memory task. The measurements were obtained in a neurophysiological laboratory of a university hospital. Nine patients with HD and 9 control subjects matched for age, sex, and education were studied. Components of averaged ERPs were quantified by latency and amplitude measures and subjected to statistical analysis. Behavioral measures (search time, hit rate, and recognition accuracy) were assessed as well. The early visual components showed a significant latency shift (delay of about 50 milliseconds) in HD. In the search paradigms the P3 components differentiating target and standard stimuli were virtually absent in HD as was the ERP effect indexing word recognition. This was accompanied by a marked delay in search times and lower hit rates in the search tasks and a grossly reduced recognition accuracy in the memory task. The results suggest marked impairments of patients with HD in early visual sensory processing (early components). Deficits in visual search might be attributed to an impairment to deploy attentional resources across the visual field and/or an inability to control eye movements. The ERPs in the memory task differed grossly from similar data obtained by others in patients with Alzheimer disease, suggesting a different neural basis for

  2. Identification of extreme motor phenotypes in Huntington's disease. (United States)

    Braisch, Ulrike; Hay, Birgit; Muche, Rainer; Rothenbacher, Dietrich; Landwehrmeyer, G Bernhard; Long, Jeffrey D; Orth, Michael


    The manifestation of motor signs in Huntington's disease (HD) has a well-known inverse relationship with HTT CAG repeat length, but the prediction is far from perfect. The probability of finding disease modifiers is enhanced in individuals with extreme HD phenotypes. We aimed to identify extreme HD motor phenotypes conditional on CAG and age, such as patients with very early or very late onset of motor manifestation. Retrospective data were available from 1,218 healthy controls and 9,743 HD participants with CAG repeats ≥40, and a total of about 30,000 visits. Boundaries (2.5% and 97.5% quantiles) for extreme motor phenotypes (UHDRS total motor score (TMS) and motor age-at-onset) were estimated using quantile regression for longitudinal data. More than 15% of HD participants had an extreme TMS phenotype for at least one visit. In contrast, only about 4% of participants were consistent TMS extremes at two or more visits. Data from healthy controls revealed an upper cut-off of 13 for the TMS representing the extreme of motor ratings for a normal aging population. In HD, boundaries of motor age-at-onset based on diagnostic confidence or derived from the TMS data cut-off in controls were similar. In summary, a UHDRS TMS of more than 13 in an individual carrying the HD mutation indicates a high likelihood of motor manifestations of HD irrespective of CAG repeat length or age. The identification of motor phenotype extremes can be useful in the search for disease modifiers, for example, genetic or environmental such as medication. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  3. Drosophila eye color mutants as therapeutic tools for Huntington disease. (United States)

    Green, Edward W; Campesan, Susanna; Breda, Carlo; Sathyasaikumar, Korrapati V; Muchowski, Paul J; Schwarcz, Robert; Kyriacou, Charalambos P; Giorgini, Flaviano


    Huntington disease (HD) is a fatal inherited neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin protein (htt). A pathological hallmark of the disease is the loss of a specific population of striatal neurons, and considerable attention has been paid to the role of the kynurenine pathway (KP) of tryptophan (TRP) degradation in this process. The KP contains three neuroactive metabolites: 3-hydroxykynurenine (3-HK), quinolinic acid (QUIN), and kynurenic acid (KYNA). 3-HK and QUIN are neurotoxic, and are increased in the brains of early stage HD patients, as well as in yeast and mouse models of HD. Conversely, KYNA is neuroprotective and has been shown to be decreased in HD patient brains. We recently used a Drosophila model of HD to measure the neuroprotective effect of genetic and pharmacological inhibition of kynurenine monoxygenase (KMO)-the enzyme catalyzing the formation of 3-HK at a pivotal branch point in the KP. We found that KMO inhibition in Drosophila robustly attenuated neurodegeneration, and that this neuroprotection was correlated with reduced levels of 3-HK relative to KYNA. Importantly, we showed that KP metabolites are causative in this process, as 3-HK and KYNA feeding experiments modulated neurodegeneration. We also found that genetic inhibition of the upstream KP enzyme tryptophan-2,3-dioxygenase (TDO) was neuroprotective in flies. Here, we extend these results by reporting that genetic impairment of KMO or TDO is protective against the eclosion defect in HD model fruit flies. Our results provide further support for the possibility of therapeutic KP interventions in HD.

  4. Brain imaging and cognitive dysfunctions in Huntington's disease (United States)

    Montoya, Alonso; Price, Bruce H.; Menear, Matthew; Lepage, Martin


    Recent decades have seen tremendous growth in our understanding of the cognitive dysfunctions observed in Huntington's disease (HD). Advances in neuroimaging have contributed greatly to this growth. We reviewed the role that structural and functional neuroimaging techniques have played in elucidating the cerebral bases of the cognitive deficits associated with HD. We conducted a computer-based search using PubMed and PsycINFO databases to retrieve studies of patients with HD published between 1965 and December 2004 that reported measures on cognitive tasks and used neuroimaging techniques. Structural neuroimaging has provided important evidence of morphological brain changes in HD. Striatal and cortical atrophy are the most common findings, and they correlate with cognitive deficits in attention, working memory and executive functions. Functional studies have also demonstrated correlations between striatal dysfunction and cognitive performance. Striatal hypoperfusion and decreased glucose utilization correlate with executive dysfunction. Hypometabolism also occurs throughout the cerebral cortex and correlates with performance on recognition memory, language and perceptual tests. Measures of presynaptic and postsynaptic dopamine biochemistry have also correlated with measurements of episodic memory, speed of processing and executive functioning. Aided by the results of numerous neuroimaging studies, it is becoming increasingly clear that cognitive deficits in HD involve abnormal connectivity between the basal ganglia and cortical areas. In the future, neuroimaging techniques may shed the most light on the pathophysiology of HD by defining neurodegenerative disease phenotypes as a valuable tool for knowing when patients become “symptomatic,” having been in a gene-positive presymptomatic state, and as a biomarker in following the disease, thereby providing a prospect for improved patient care. PMID:16496032

  5. Progressive microstructural changes of the occipital cortex in Huntington's disease. (United States)

    Odish, Omar F F; Reijntjes, Robert H A M; van den Bogaard, Simon J A; Roos, Raymund A C; Leemans, Alexander


    In this study we longitudinally investigated the rate of microstructural alterations in the occipital cortex in different stages of Huntington's disease (HD) by applying an automated atlas-based approach to diffusion MRI data. Twenty-two premanifest (preHD), 10 early manifest HD (early HD) and 24 healthy control subjects completed baseline and two year follow-up scans. The preHD group was stratified based on the predicted years to disease onset into a far (preHD-A) and near (preHD-B) to disease onset group. Clinical and behavioral measures were collected per assessment time point. An automated atlas-based DTI analysis approach was used to obtain the mean, axial and radial diffusivities of the occipital cortex. We found that the longitudinal rate of diffusivity change in the superior occipital gyrus (SOG), middle occipital gyrus (MOG), and inferior occipital gyrus (IOG) was significantly higher in early HD compared to both preHD and controls (all p's ≤ 0.005), which can be interpreted as an increased rate of microstructural degeneration. Furthermore, the change rate in the diffusivity of the MOG could significantly discriminate between preHD-B compared to preHD-A and the other groups (all p's ≤ 0.04). Finally, we found an inverse correlation between the Stroop Word Reading task and diffusivities in the SOG and MOG (all p's ≤ 0.01). These findings suggest that measures obtained from the occipital cortex can serve as sensitive longitudinal biomarkers for disease progression in preHD-B and early HD. These could in turn be used to assess potential effects of proposed disease modifying therapies.

  6. Late-onset Huntington's disease: diagnostic and prognostic considerations. (United States)

    Koutsis, Georgios; Karadima, Georgia; Kladi, Athina; Panas, Marios


    To address diagnostic and prognostic issues in patients with late-onset Huntington's disease (HD). We analyzed a cohort of 41 late-onset (≥60 years) HD patients and compared them to 39 late-onset patients referred for HD testing that were negative for the HD-expansion and to 290 usual-onset (20-59 years) HD patients. Disease severity was assessed by the Total Functional Capacity Scale. Late-onset HD comprised 11.5% of our HD cohort. In total, 70.7% of late-onset HD patients had positive family history compared to 15.4% of late-onset expansion-negative patients (p < 0.001). Clinical features at onset or presentation could not usefully distinguish between late-onset expansion-positive and negative patients, excepting hemichorea, which was absent from the HD group (p = 0.024). Chorea was the first clinical feature in 53.7% and a presenting feature in 90.2% of late-onset HD. The mutation hit rate for late-onset patients was 51.3%, lower than in usual-onset patients (p = 0.04). Frequencies of chorea, cognitive impairment and psychiatric manifestations at onset or presentation were not significantly different between late-onset and usual-onset HD patients. Gait unsteadiness however was more common at presentation in late-onset HD (p = 0.007). Late-onset HD patients reached a severe stage of illness on average 2.8 years earlier than usual-onset HD patients (p = 0.046). A positive family history suggestive of HD, although absent in a third of patients, remains a helpful clue in diagnosing late-onset HD. Prognosis of late-onset HD in terms of Total Functional Capacity appears no better and shows a trend of being somewhat less favorable compared to usual-onset HD. Copyright © 2014 Elsevier Ltd. All rights reserved.


    Krause, A; Mitchell, CL; Essop, F; Tager, S; Temlett, J; Stevanin, G; Ross, CA; Rudnicki, DD; Margolis, RL


    Huntington disease (HD) is a progressive autosomal dominant neurodegenerative disorder, characterized by abnormal movements, cognitive decline and psychiatric symptoms, caused by a CAG repeat expansion in the huntingtin (HTT) gene on chromosome 4p. A CAG/CTG repeat expansion in the junctophilin-3 (JPH3) gene on chromosome 16q24.2 causes a Huntington disease-like phenotype (HDL2). All patients to date with HDL2 have some African ancestry. The present study aimed to characterize the genetic basis of the Huntington disease phenotype in South Africans and to investigate the possible origin of the JPH3 mutation. In a sample of unrelated South African individuals referred for diagnostic HD testing, 62% (106/171) of white patients compared to only 36% (47/130) of black patients had an expansion in HTT. However, 15% (20/130) of black South African patients and no white patients (0/171) had an expansion in JPH3, confirming the diagnosis of Huntington disease like 2 (HDL2). Individuals with HDL2 share many clinical features with individuals with HD and are clinically indistinguishable in many cases, although the average age of onset and diagnosis in HDL2 is 5 years later than HD and individual clinical features may be more prominent. HDL2 mutations contribute significantly to the HD phenotype in South Africans with African ancestry. JPH3 haplotype studies in 31 families, mainly from South Africa and North America, provide evidence for a founder mutation and support a common African origin for all HDL2 patients. Molecular testing in individuals with an HD phenotype and African ancestry should include testing routinely for JPH3 mutations. PMID:26079385

  8. Mixed oceanic and freshwater depositional conditions for beach rocks of NE Brazil: Evidence from C and O isotopes

    International Nuclear Information System (INIS)

    Chaves, Nubia S.; Kiang, Chang H.


    Full text: Beach rocks, a common feature of northeastern coastline of Brazil are formed in the inter tidal zone, considered as ancient coastal line, cemented by CaCO 3 which have variable extension. They occur parallel to the coastline as linear ridges. Beach rocks are sub horizontally disposed and surfaces are irregular, displacing potholes due to differential erosion, perforations by organisms, diaclasis, cross stratification and rare laminations. The dominant detrital components are quartz and minor fractions of feldspars and rock fragments. Zircon, epi dote, hornblende, garnet, muscovite, rutile, opaque and sillimanite are present in trace quantity. Bivalves, mollusks, gastropods, halimeda, corals, pelecipods and equinoids, constitute the biotic components. Beach rocks cement vary from aragonite to Mg-calcite. The dominant micro facies, consists of isopach crystals of aragonite, enclosing bioclastic and/or clastic grains forming uniform fringe formed in the marine phreatic zone. The second is represented by cryptocrystalline inter-granulate cement like micritic envelop formed in meteoric phreatic environment (Moore 1971). The third is formed by inter granulate cryptocrystalline cement, filling the pores. Beach rock samples locate in the coastal zone show an interval with depleted C and O ratios (average δ 13 C = -1.3%0, δ 18 O = -2.1%0) and an interval of enriched isotopic ratios (average δ 13 C = +3.5%0, δ 18 O +1.2%0). Depleted oxygen isotope values considered to be indicative of meteoric diagenesis with minor freshwater influx (Allan and Mattew 1982). (author)

  9. Radiogenic heavy minerals in Brazilian beach sand

    International Nuclear Information System (INIS)

    Malanca, A.


    Sand samples collected on the beaches of the 'radioactive' Brazilian town of Guarapari were first separated by flotation in bromoform and successively divided into various magnetic fractions with a Franz isodynamic separator. concentrations of background radionuclides in samples of monazite, ilmenite, and zircon were determined by a γ-ray spectrometer. Chemical composition of monazite, ilmenite and magnetite were assessed by means of an electron microprobe. Monazite resulted to be relatively rich in ThO 2 whose abundance ranged from 5.3 to 7.7 (wt%). (author)

  10. Evaluation of potential sources and transport mechanisms of fecal indicator bacteria to beach water, Murphy Park Beach, Door County, Wisconsin (United States)

    Juckem, Paul F.; Corsi, Steven R.; McDermott, Colleen; Kleinheinz, Gregory; Fogarty, Lisa R.; Haack, Sheridan K.; Johnson, Heather E.


    Fecal Indicator Bacteria (FIB) concentrations in beach water have been used for many years as a criterion for closing beaches due to potential health concerns. Yet, current understanding of sources and transport mechanisms that drive FIB occurrence remains insufficient for accurate prediction of closures at many beaches. Murphy Park Beach, a relatively pristine beach on Green Bay in Door County, Wis., was selected for a study to evaluate FIB sources and transport mechanisms. Although the relatively pristine nature of the beach yielded no detection of pathogenic bacterial genes and relatively low FIB concentrations during the study period compared with other Great Lakes Beaches, its selection limited the number of confounding FIB sources and associated transport mechanisms. The primary sources of FIB appear to be internal to the beach rather than external sources such as rivers, storm sewer outfalls, and industrial discharges. Three potential FIB sources were identified: sand, swash-zone groundwater, and Cladophora mats. Modest correlations between FIB concentrations in these potential source reservoirs and FIB concentrations at the beach from the same day illustrate the importance of understanding transport mechanisms between FIB sources and the water column. One likely mechanism for transport and dispersion of FIB from sand and Cladophora sources appears to be agitation of Cladophora mats and erosion of beach sand due to storm activity, as inferred from storm indicators including turbidity, wave height, current speed, wind speed, sky visibility, 24-hour precipitation, and suspended particulate concentration. FIB concentrations in beach water had a statistically significant relation (p-value ‹0.05) with the magnitude of these storm indicators. In addition, transport of FIB in swash-zone groundwater into beach water appears to be driven by groundwater recharge associated with multiday precipitation and corresponding increased swash-zone groundwater discharge at

  11. Bodies that Matter: Performing White Possession on the Beach (United States)

    Moreton-Robinson, Aileen


    Beaches remain important places within indigenous coastal peoples' territories, although the silence about our ownership is deafening. Many authors have argued that within Australian popular culture the beach is a key site where racialized and gendered transgressions, fantasies, and desires are played out, but none have elucidated how these…

  12. Sand transport in urbanized beaches - models and reality

    International Nuclear Information System (INIS)

    Pineiro, G.; Norbis, W.; Panario, D.


    The general objective is to quantify the wind transport of sand in the urbanized beaches. The specific objectives include testing and calibration of the wind velocity as well as the classification of the beaches according to the magnitude and the direction of sand transport


    A biological and inorganic assessment of five beaches on Lake Texoma was conducted from September 1999 through July 2001. Water samples for each beach site were divided into two groups, a swimming season and non-swimming season. Water properties such as temperature, alkalinity,...

  14. Modelling wind forced bedforms on a sandy beach

    NARCIS (Netherlands)

    de Vries, S.; Van Thiel de Vries, J.; Ruessink, B.G.


    This paper aims to conceptually simulate observed spatial and temporal variability in aeolian sediment transport rates, erosion and deposition on a beach. Traditional strategies of modeling aeolian sediment transport rates do not account for supply limitations that are common on natural beaches. A

  15. Behavioral testing of minipigs transgenic for the Huntington gene-A three-year observational study

    Czech Academy of Sciences Publication Activity Database

    Schuldenzucker, V.; Schubert, R.; Muratori, L. M.; Freisfeld, F.; Rieke, L.; Matheis, T.; Schramke, S.; Motlík, Jan; Kemper, N.; Radespiel, U.; Reilmann, R.


    Roč. 12, č. 10 (2017), č. článku e0185970. E-ISSN 1932-6203 Institutional support: RVO:67985904 Keywords : Huntington´s disease * minipigs Subject RIV: EG - Zoology OBOR OECD: Behavioral sciences biology Impact factor: 2.806, year: 2016

  16. Motor, emotional and cognitive deficits in adult BACHD mice : A model for Huntington's disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Schreiber, Rudy; Ellenbroek, Bart


    Rationale: Huntington's disease (HD) is characterized by progressive motor dysfunction, emotional disturbances and cognitive deficits. It is a genetic disease caused by an elongation of the polyglutamine repeats in the huntingtin gene. Whereas HD is a complex disorder, previous studies in mice

  17. Vertraagde diagnose van de ziekte van Huntington in een psychiatrische setting

    NARCIS (Netherlands)

    Tak, L M; Sizoo, B; de Stegge, B M Aan; Adema, S; van Duijn, E; Kremer, B


    Huntington's disease (hd) is characterised by a triad of neuropsychiatric symptoms, motor disturbances and cognitive decline. If initial symptoms are of neuropsychiatric nature they maybe misinterpreted, which can lead to delayed diagnosis. Three examples of delayed hd diagnosis in a psychiatric

  18. CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion

    DEFF Research Database (Denmark)

    Lee, J-M; Ramos, E M; Lee, J-H


    Age at onset of diagnostic motor manifestations in Huntington disease (HD) is strongly correlated with an expanded CAG trinucleotide repeat. The length of the normal CAG repeat allele has been reported also to influence age at onset, in interaction with the expanded allele. Due to profound...

  19. Striatal dopamine D2 receptors, metabolism, and volume in preclinical Huntington disease

    NARCIS (Netherlands)

    van Oostrom, JCH; Maguire, RP; Verschuuren-Bemelmans, CC; van der Duin, LV; Pruim, J; Roos, RAC; Leenders, KL


    Among 27 preclinical carriers of the Huntington disease mutation (PMC), the authors found normal striatal values for MRI volumetry in 88% and for fluorodesoxyglucose PET metabolic index in 67%. Raclopride PET binding potential (RAC-BP) was decreased in 50% and correlated with increases in the

  20. Hypocretin and melanin-concentrating hormone in patients with Huntington disease.

    NARCIS (Netherlands)

    Aziz, A.; Fronczek, R.; Maat-Schieman, M.L.; Unmehopa, U.A.; Roelandse, F.W.; Overeem, S.; Duinen, S.G. van; Lammers, G.J.; Swaab, D.F.; Roos, R.A.C.


    To evaluate whether hypocretin-1 (orexin-A) and melanin-concentrating hormone (MCH) neurotransmission are affected in patients with Huntington disease (HD), we immunohistochemically stained hypocretin and MCH neurons and estimated their total numbers in the lateral hypothalamus of both HD patients

  1. Functional Compensation of Motor Function in Pre-Symptomatic Huntington's Disease (United States)

    Kloppel, Stefan; Draganski, Bogdan; Siebner, Hartwig R.; Tabrizi, Sarah J.; Weiller, Cornelius; Frackowiak, Richard S. J.


    Involuntary choreiform movements are a clinical hallmark of Huntington's disease. Studies in clinically affected patients suggest a shift of motor activations to parietal cortices in response to progressive neurodegeneration. Here, we studied pre-symptomatic gene carriers to examine the compensatory mechanisms that underlie the phenomenon of…

  2. 4p16.3 haplotype modifying age at onset of Huntington disease

    DEFF Research Database (Denmark)

    Nørremølle, A; Budtz-Jørgensen, E; Fenger, K


    Huntington disease (HD) is caused by an expanded CAG repeat sequence in the HD gene. Although the age at onset is correlated to the CAG repeat length, this correlation only explains approximately half of the variation in onset age. Less variation between siblings indicates that the variation is, ...

  3. The use of stem cells in regenerative medicine for Parkinson's and Huntington's Diseases. (United States)

    Lescaudron, L; Naveilhan, P; Neveu, I


    Cell transplantation has been proposed as a means of replacing specific cell populations lost through neurodegenerative processes such as that seen in Parkinson's or Huntington's diseases. Improvement of the clinical symptoms has been observed in a number of Parkinson and Huntington's patients transplanted with freshly isolated fetal brain tissue but such restorative approach is greatly hampered by logistic and ethical concerns relative to the use of fetal tissue, in addition to potential side effects that remain to be controlled. In this context, stem cells that are capable of self-renewal and can differentiate into neurons, have received a great deal of interest, as demonstrated by the numerous studies based on the transplantation of neural stem/progenitor cells, embryonic stem cells or mesenchymal stem cells into animal models of Parkinson's or Huntington's diseases. More recently, the induction of pluripotent stem cells from somatic adult cells has raised a new hope for the treatment of neurodegenerative diseases. In the present article, we review the main experimental approaches to assess the efficiency of cell-based therapy for Parkinson's or Huntington's diseases, and discuss the recent advances in using stem cells to replace lost dopaminergic mesencephalic or striatal neurons. Characteristics of the different stem cells are extensively examined with a special attention to their ability of producing neurotrophic or immunosuppressive factors, as these may provide a favourable environment for brain tissue repair and long-term survival of transplanted cells in the central nervous system. Thus, stem cell therapy can be a valuable tool in regenerative medicine.

  4. Selected CSF biomarkers indicate no evidence of early neuroinflammation in Huntington disease

    DEFF Research Database (Denmark)

    Vinther-Jensen, Tua; Börnsen, Lars Svend; Budtz-Jorgensen, Esben


    Objective: To investigate CSF biomarkers of neuroinflammation and neurodegeneration in Huntington disease (HD) gene-expansion carriers compared to controls and to investigate these biomarkers in association with clinical HD rating scales and disease burden score. Methods: We collected CSF from 32...

  5. Beautiful Science: The Public and Private History of Astronomy at the Huntington Library (United States)

    Lewis, Daniel


    The history of astronomy has a long tradition within research libraries. The rare collections at the Huntington Library (encompassing American and British history from around 1000 CE to the present, in many different subject areas) are among the most heavily-used in the United States, The history of astronomy holdings are a cornerstone within the library's history of science holdings. This talk will present the two faces of the history of astronomy holdings at the Huntington Library. The first of these is the research end of operations: what the collections consist of, how the scholarly public uses the collections, and what the implications are for modern astronomical practice. The second element concerns the public exhibit face of the history of astronomy holdings at The Huntington. Of the 600,000 people who visit the Huntington each year, the majority visit public displays and rare book and manuscript exhibits. "Beautiful Science: Ideas That Changed the World” is a new permanent history of science exhibit. One quarter of the exhibit relates to the history of astronomy. Public exhibits require a particular kind of planning and bring a specific set of values to the history of astronomy. Public exhibits also have their own concerns, and this talk will cover a number of those issues as well as the research issues.

  6. 77 FR 22616 - Huntington Asset Advisors, Inc., et al.; Notice of Application (United States)


    ...''), Huntington Strategy Shares (``Trust''), and SEI Investments Distribution Co. Summary of Application.... \\14\\ If Shares are listed on The NASDAQ Stock Market LLC (``Nasdaq'') or a similar electronic Listing... SECURITIES AND EXCHANGE COMMISSION [Investment Company Act Release No. 30032; 812-13785...

  7. 77 FR 51064 - Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application... (United States)


    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-81,475] Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application for Reconsideration By application dated May 21, 2012, the State Workforce Office requested administrative reconsideration of the negative...

  8. The use of olanzapine in Huntington disease accompanied by psychotic symptoms

    Directory of Open Access Journals (Sweden)

    Cafer Alhan


    Full Text Available Huntington's disease is an autosomal dominant neurodegenerative disease. The disease begins between the ages of 30-50, including motor symptoms, psychiatric symptoms and is characterized by progressive dementia. Common psychiatric disorders of Huntington’s disease include mood and anxiety disorders, behavior and personality changes. Psychosis is relatively rare. Here, a patient is present, who has Huntington’s disease, which is associated with psychotic symptoms. 61-year-old male patient who were followed for Huntington disease for 25 years was admitted for complaints of thinking of poisoning and refuse to eat something. Patient was started on olanzapine at dose of 5 mg/day. In follow up psychotic symptoms disappeared. Emerging psychotic symptoms in Huntington disease is created a need for antipsychotic treatment. Atypical antipsychotic agents should be preferred in the treatment and as in the case olanzapine may be used as a treatment option should be kept in mind to control both involuntary movements and psychotic symptoms in Huntington's disease with psychotic features. J Clin Exp Invest 2014; 5 (2: 326-328

  9. 3-NP-induced neurodegeneration studies in experimental models of Huntington's disease.

    NARCIS (Netherlands)

    Vis, J.C.


    This thesis investigates the possible role of apoptosis, or programmed cell death, in Huntington's disease (HD). HD is caused by an expanded CAG repeat in the N-terminal region of the huntingtin protein leading to specific neostriatal neurodegeneration. The sequence of events that leads to this

  10. Evidence for Deficits on Different Components of Theory of Mind in Huntington's Disease

    NARCIS (Netherlands)

    Allain, P.; Havel-Thomassin, V.; Verny, C.; Gohier, B.; Lancelot, C.; Besnard, J.; Fasotti, L.; Le Gall, D.


    Objective: The main aim of this study was to investigate the effects of Huntington's disease (HD) on cognitive and affective Theory of Mind (ToM) abilities. The relation of ToM performance and executive functions was also examined. Method: Eighteen HD patients, early in the course of the disease,

  11. A new mutation for Huntington disease following maternal transmission of an intermediate allele

    NARCIS (Netherlands)

    Semaka, Alicia; Kay, Chris; Belfroid, René D. M.; Bijlsma, Emilia K.; Losekoot, Monique; van Langen, Irene M.; van Maarle, Merel C.; Oosterloo, Mayke; Hayden, Michael R.; van Belzen, Martine J.


    New mutations for Huntington disease (HD) originate from CAG repeat expansion of intermediate alleles (27-35 CAG). Expansions of such alleles into the pathological range (≥ 36 CAG) have been exclusively observed in paternal transmission. We report the occurrence of a new mutation that defies the

  12. Reversal learning and associative memory impairments in a BACHD rat model for Huntington disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Nguyen, Huu Phuc; Ellenbroek, Bart; Schreiber, Rudy


    Chorea and psychiatric symptoms are hallmarks of Huntington disease (HD), a neurodegenerative disorder, genetically characterized by the presence of expanded CAG repeats (>35) in the HUNTINGTIN (HTT) gene. HD patients present psychiatric symptoms prior to the onset of motor symptoms and we recently

  13. Motor cortex synchronization influences the rhythm of motor performance in premanifest huntington's disease. (United States)

    Casula, Elias P; Mayer, Isabella M S; Desikan, Mahalekshmi; Tabrizi, Sarah J; Rothwell, John C; Orth, Michael


    In Huntington's disease there is evidence of structural damage in the motor system, but it is still unclear how to link this to the behavioral disorder of movement. One feature of choreic movement is variable timing and coordination between sequences of actions. We postulate this results from desynchronization of neural activity in cortical motor areas. The objective of this study was to explore the ability to synchronize activity in a motor network using transcranial magnetic stimulation and to relate this to timing of motor performance. We examined synchronization in oscillatory activity of cortical motor areas in response to an external input produced by a pulse of transcranial magnetic stimulation. We combined this with EEG to compare the response of 16 presymptomatic Huntington's disease participants with 16 age-matched healthy volunteers to test whether the strength of synchronization relates to the variability of motor performance at the following 2 tasks: a grip force task and a speeded-tapping task. Phase synchronization in response to M1 stimulation was lower in Huntington's disease than healthy volunteers (P synchronization (r = -0.356; P synchronization and desynchronization could be a physiological basis for some key clinical features of Huntington's disease. © 2018 International Parkinson and Movement Disorder Society. © 2018 International Parkinson and Movement Disorder Society.

  14. Application of GIS in Beach Placer Exploration

    International Nuclear Information System (INIS)

    Babu, M.N.


    Application of Geographic Information System (GIS) in beach placer exploration combines the activities of data collection, organisation, visualisation, query, analysis and prediction. GIS has the potential to enhance the capability for creative data analysis, modelling and interpretation. Exploration software and GIS are essential tools for geoscientists searching for mineral deposits. GIS technology has come a long way in the past fifteen years, especially in data handling, analysis, visualization etc. Modern technology and solutions are now evolving to allow geoscientists to share data easily between mapping platforms and enterprise level GIS environments. In beach placer exploration programme, data handling and processing are the main challenging tasks due to generation and processing of large volume of field and laboratory data of the areas under investigation. Though there are limitations in visualising map data as a single map on a screen, due to its non-proportionate lengths and widths, a GIS can very well handle all these varied datasets to demarcate highly potential zones within a narrow coastal strip. Generally these datasets contain information from thousands of drillholes about their location co-ordinates, depth, height, description and thickness of lithounits, water table level, radioactivity and other pertinent subsurface properties. Field data collection can be efficiently done using a hand held global positioning system (GPS) installed with mobile-GIS application and data handling software

  15. Ilmenite Mineral's Recovery from Beach Sand Tailings

    International Nuclear Information System (INIS)

    Mulaba-Bafubiandi, Antoine F.; Mukendi-Ngalula, David; Waanders, Frans B.


    The mineral ilmenite is the major source of rutile for industrial use and is of interest to paint and fertiliser industries. Enormous unutilised tailing dams lie on the eastern coast of the South Africa. Although covered by a simulation of the original indigenous vegetation, these tailings are still ilmenite bearing and of economic value. Tailings emanating from beach sand mineral slimes dams of the Kwazulu-Natal area (South Africa) have been processed. Screening, flotation, spiral concentration and magnetic separation methods were used either separately or successively. The present work sheds light on alternative routes for the extraction of the ilmenite, from these tailings. It moreover points out the usefulness of the Moessbauer spectroscopy in the mineral processing product monitoring. Tailings from the beach sands were used in the present study after the economic industrial minerals zirconia, ilmenite and rutile had been extracted in previous mining operations. About 61% natural ilmenite recovery was observed in the flotation concentrate of a Humphrey Spiral concentrate while a 62% recovery of hematite was found in the flotation tailings. The combination of screening, spiral concentration and magnetic separation, and flotation yielded a product with the highest ilmenite and hematite concentration being 71% and 19%, respectively. A natural ilmenite mineral, containing 87% ilmenite and 13% hematite, could be produced and extracted from the tailings of the flotation process, collected subsequently to the spiral concentration and the initial screening.

  16. Gleaning and Dreaming on Car Park Beach

    Directory of Open Access Journals (Sweden)

    Jo Croft


    Full Text Available This article explores beachcombing and gleaning as practices that combine mobility with daydreaming and which allow us to experience our environment with the perception of ‘tactile nearness’ (Benjamin. Through eco-poetics shaped by ‘inconceivable analogies and connections’ (Benjamin, the author re-imagines a neglected space used as a short-cut on the way to work—the Liverpool Adelphi car park in Liverpool—as “Car Park Beach”. Inspired by the situationists’ slogan ‘Sous les pavés, la plage’, the author argues that Car Park Beach opens up imaginative possibilities for a different form of ecological encounter with our own precarity, one ushered in by a ‘close-up’ awareness of how waste transforms our world. Car Park Beach is a site that the author associates with the drift-like, distracted movements of both people and matter, and this article therefore attempts to deploy an equivalent method of analysis. Drawing on her own practice of gleaning photos and objects on the way to work, the author places a vocabulary of flotsam and jetsam at the axis of her discussion. Allusive, often layered, connections are followed between a diverse range of sources including beachcombing guides, literary memoirs, documentary films, eco-criticism, and auto-ethnography.

  17. Activation of IGF-1 and insulin signaling pathways ameliorate mitochondrial function and energy metabolism in Huntington's Disease human lymphoblasts. (United States)

    Naia, Luana; Ferreira, I Luísa; Cunha-Oliveira, Teresa; Duarte, Ana I; Ribeiro, Márcio; Rosenstock, Tatiana R; Laço, Mário N; Ribeiro, Maria J; Oliveira, Catarina R; Saudou, Frédéric; Humbert, Sandrine; Rego, A Cristina


    Huntington's disease (HD) is an inherited neurodegenerative disease caused by a polyglutamine repeat expansion in the huntingtin protein. Mitochondrial dysfunction associated with energy failure plays an important role in this untreated pathology. In the present work, we used lymphoblasts obtained from HD patients or unaffected parentally related individuals to study the protective role of insulin-like growth factor 1 (IGF-1) versus insulin (at low nM) on signaling and metabolic and mitochondrial functions. Deregulation of intracellular signaling pathways linked to activation of insulin and IGF-1 receptors (IR,IGF-1R), Akt, and ERK was largely restored by IGF-1 and, at a less extent, by insulin in HD human lymphoblasts. Importantly, both neurotrophic factors stimulated huntingtin phosphorylation at Ser421 in HD cells. IGF-1 and insulin also rescued energy levels in HD peripheral cells, as evaluated by increased ATP and phosphocreatine, and decreased lactate levels. Moreover, IGF-1 effectively ameliorated O2 consumption and mitochondrial membrane potential (Δψm) in HD lymphoblasts, which occurred concomitantly with increased levels of cytochrome c. Indeed, constitutive phosphorylation of huntingtin was able to restore the Δψm in lymphoblasts expressing an abnormal expansion of polyglutamines. HD lymphoblasts further exhibited increased intracellular Ca(2+) levels before and after exposure to hydrogen peroxide (H2O2), and decreased mitochondrial Ca(2+) accumulation, being the later recovered by IGF-1 and insulin in HD lymphoblasts pre-exposed to H2O2. In summary, the data support an important role for IR/IGF-1R mediated activation of signaling pathways and improved mitochondrial and metabolic function in HD human lymphoblasts.

  18. An image-based model of brain volume biomarker changes in Huntington's disease. (United States)

    Wijeratne, Peter A; Young, Alexandra L; Oxtoby, Neil P; Marinescu, Razvan V; Firth, Nicholas C; Johnson, Eileanoir B; Mohan, Amrita; Sampaio, Cristina; Scahill, Rachael I; Tabrizi, Sarah J; Alexander, Daniel C


    Determining the sequence in which Huntington's disease biomarkers become abnormal can provide important insights into the disease progression and a quantitative tool for patient stratification. Here, we construct and present a uniquely fine-grained model of temporal progression of Huntington's disease from premanifest through to manifest stages. We employ a probabilistic event-based model to determine the sequence of appearance of atrophy in brain volumes, learned from structural MRI in the Track-HD study, as well as to estimate the uncertainty in the ordering. We use longitudinal and phenotypic data to demonstrate the utility of the patient staging system that the resulting model provides. The model recovers the following order of detectable changes in brain region volumes: putamen, caudate, pallidum, insula white matter, nonventricular cerebrospinal fluid, amygdala, optic chiasm, third ventricle, posterior insula, and basal forebrain. This ordering is mostly preserved even under cross-validation of the uncertainty in the event sequence. Longitudinal analysis performed using 6 years of follow-up data from baseline confirms efficacy of the model, as subjects consistently move to later stages with time, and significant correlations are observed between the estimated stages and nonimaging phenotypic markers. We used a data-driven method to provide new insight into Huntington's disease progression as well as new power to stage and predict conversion. Our results highlight the potential of disease progression models, such as the event-based model, to provide new insight into Huntington's disease progression and to support fine-grained patient stratification for future precision medicine in Huntington's disease.

  19. 75 FR 14206 - FPL Energy Point Beach, LLC; Point Beach Nuclear Plant, Units 1 and 2; Environmental Assessment... (United States)


    ... NUCLEAR REGULATORY COMMISSION [Docket Nos. 50-266 And 50-301; NRC-2010-0123 FPL Energy Point Beach, LLC; Point Beach Nuclear Plant, Units 1 and 2; Environmental Assessment and Finding of No Significant Impact The U.S. Nuclear Regulatory Commission (NRC) is considering issuance of an Exemption, pursuant to...

  20. Striatal pre-enkephalin overexpression improves Huntington's disease symptoms in the R6/2 mouse model of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    Stéphanie Bissonnette

    Full Text Available The reduction of pre-enkephalin (pENK mRNA expression might be an early sign of striatal neuronal dysfunction in Huntington's disease (HD, due to mutated huntingtin protein. Indeed, striatopallidal (pENK-containing neurodegeneration occurs at earlier stage of the disease, compare to the loss of striatonigral neurons. However, no data are available about the functional role of striatal pENK in HD. According to the neuroprotective properties of opioids that have been recognized recently, the objective of this study was to investigate whether striatal overexpression of pENK at early stage of HD can improve motor dysfunction, and/or reduce striatal neuronal loss in the R6/2 transgenic mouse model of HD. To achieve this goal recombinant adeno-associated-virus (rAAV2-containing green fluorescence protein (GFP-pENK was injected bilaterally in the striatum of R6/2 mice at 5 weeks old to overexpress opioid peptide pENK. Striatal injection of rAAV2-GFP was used as a control. Different behavioral tests were carried out before and/or after striatal injections of rAAV2. The animals were euthanized at 10 weeks old. Our results demonstrate that striatal overexpression of pENK had beneficial effects on behavioral symptoms of HD in R6/2 by: delaying the onset of decline in muscular force; reduction of clasping; improvement of fast motor activity, short-term memory and recognition; as well as normalization of anxiety-like behavior. The improvement of behavioral dysfunction in R6/2 mice having received rAAV2-GFP-pENK associated with upregulation of striatal pENK mRNA; the increased level of enkephalin peptide in the striatum, globus pallidus and substantia nigra; as well as the slight increase in the number of striatal neurons compared with other groups of R6/2. Accordingly, we suggest that at early stage of HD upregulation of striatal enkephalin might play a key role at attenuating illness symptoms.

  1. Environmental contaminants in the food chain, NWS Seal Beach and Seal Beach NWR

    Energy Technology Data Exchange (ETDEWEB)

    Ohlendorf, H.M.; Byron, E.R. [CH2M Hill, Sacramento, CA (United States); Freas, K.E. [CH2M Hill, San Jose, CA (United States); Casados, E.M.; Kidwell, J.J. [Naval Facilities Engineering Command, San Diego, CA (United States). SW Division


    The authors conducted a study to determine whether environmental contaminants occurred in fish and invertebrates at concentrations that could be harmful to birds feeding in the estuarine salt marsh at Seal Beach National Wildlife Refuge (NWR), which is part of Naval Weapons Station (NWS) Seal Beach. Management of the refuge is focused primarily on endangered species, especially the light-footed clapper rail and the California least tern. Important food-chain organisms taken by rails (e.g., crabs and snails) and least terns (small fish) were sampled and analyzed for inorganic and organic contaminants that might be related to Navy activities at the Station. Results indicated that those contaminants are not likely to have lethal effects on rails or terns, although some chemicals (including cadmium, chromium, copper, lead, zinc and DDE) occurred at elevated concentrations in portions of the marsh. Possible sublethal effects also were evaluated and will be discussed.

  2. Enhanced Store-Operated Calcium Entry Leads to Striatal Synaptic Loss in a Huntington's Disease Mouse Model. (United States)

    Wu, Jun; Ryskamp, Daniel A; Liang, Xia; Egorova, Polina; Zakharova, Olga; Hung, Gene; Bezprozvanny, Ilya


    In Huntington's disease (HD), mutant Huntingtin (mHtt) protein causes striatal neuron dysfunction, synaptic loss, and eventual neurodegeneration. To understand the mechanisms responsible for synaptic loss in HD, we developed a corticostriatal coculture model that features age-dependent dendritic spine loss in striatal medium spiny neurons (MSNs) from YAC128 transgenic HD mice. Age-dependent spine loss was also observed in vivo in YAC128 MSNs. To understand the causes of spine loss in YAC128 MSNs, we performed a series of mechanistic studies. We previously discovered that mHtt protein binds to type 1 inositol (1,4,5)-trisphosphate receptor (InsP3R1) and increases its sensitivity to activation by InsP3. We now report that the resulting increase in steady-state InsP3R1 activity reduces endoplasmic reticulum (ER) Ca(2+) levels. Depletion of ER Ca(2+) leads to overactivation of the neuronal store-operated Ca(2+) entry (nSOC) pathway in YAC128 MSN spines. The synaptic nSOC pathway is controlled by the ER resident protein STIM2. We discovered that STIM2 expression is elevated in aged YAC128 striatal cultures and in YAC128 mouse striatum. Knock-down of InsP3R1 expression by antisense oligonucleotides or knock-down or knock-out of STIM2 resulted in normalization of nSOC and rescue of spine loss in YAC128 MSNs. The selective nSOC inhibitor EVP4593 was identified in our previous studies. We now demonstrate that EVP4593 reduces synaptic nSOC and rescues spine loss in YAC128 MSNs. Intraventricular delivery of EVP4593 in YAC128 mice rescued age-dependent striatal spine loss in vivo. Our results suggest EVP4593 and other inhibitors of the STIM2-dependent nSOC pathway as promising leads for HD therapeutic development. In Huntington's disease (HD) mutant Huntingtin (mHtt) causes early corticostriatal synaptic dysfunction and eventual neurodegeneration of medium spine neurons (MSNs) through poorly understood mechanisms. We report here that corticostriatal cocultures prepared from

  3. Brazilian sandy beaches: characteristics, ecosystem services, impacts, knowledge and priorities

    Directory of Open Access Journals (Sweden)

    Antonia Cecília Zacagnini Amaral

    Full Text Available ABSTRACT Sandy beaches constitute a key ecosystem and provide socioeconomic goods and services, thereby playing an important role in the maintenance of human populations and in biodiversity conservation. Despite the ecological and social importance of these ecosytems, Brazilian sandy beaches are significantly impacted by human interference, chemical and organic pollution and tourism, as well as global climate change. These factors drive the need to better understand the environmental change and its consequences for biota. To promote the implementation of integrated studies to detect the effects of regional and global environmental change on beaches and on other benthic habitats of the Brazilian coast, Brazilian marine researchers have established The Coastal Benthic Habitats Monitoring Network (ReBentos. In order to provide input for sample planning by ReBentos, we have conducted an intensive review of the studies conducted on Brazilian beaches and summarized the current knowledge about this environment. In this paper, we present the results of this review and describe the physical, biological and socioeconomics features of Brazilian beaches. We have used these results, our personal experience and worldwide literature to identify research projects that should be prioritized in the assessment of regional and global change on Brazilian sandy beaches. We trust that this paper will provide insights for future studies and represent a significant step towards the conservation of Brazilian beaches and their biodiversity.

  4. Presence of Campylobacter and Salmonella in sand from bathing beaches. (United States)

    Bolton, F J; Surman, S B; Martin, K; Wareing, D R; Humphrey, T J


    The purpose of this study was to determine the presence of thermophilic Campylobacter spp. and Salmonella spp. in sand from non-EEC standard and EEC standard designated beaches in different locations in the UK and to assess if potentially pathogenic strains were present. Campylobacter spp. were detected in 82/182 (45%) of sand samples and Salmonella spp. in 10/182 (6%). Campylobacter spp. were isolated from 46/92 (50%) of samples from non-EEC standard beaches and 36/90 (40%) from EEC standard beaches. The prevalence of Campylobacter spp. was greater in wet sand from both types of beaches but, surprisingly, more than 30% of samples from dry sand also contained these organisms. The major pathogenic species C. jejuni and C. coli were more prevalent in sand from non-EEC standard beaches. In contrast, C. lari and urease positive thermophilic campylobacters, which are associated with seagulls and other migratory birds, were more prevalent in sand from EEC standard beaches. Campylobacter isolates were further characterized by biotyping and serotyping, which confirmed that strains known to be of types associated with human infections were frequently found in sand on bathing beaches.

  5. Synthesis study of an erosion hot spot, Ocean Beach, California (United States)

    Barnard, Patrick L.; Hansen, Jeff E.; Erikson, Li H.


    A synthesis of multiple coastal morphodynamic research efforts is presented to identify the processes responsible for persistent erosion along a 1-km segment of 7-km-long Ocean Beach in San Francisco, California. The beach is situated adjacent to a major tidal inlet and in the shadow of the ebb-tidal delta at the mouth of San Francisco Bay. Ocean Beach is exposed to a high-energy wave climate and significant alongshore variability in forcing introduced by varying nearshore bathymetry, tidal forcing, and beach morphology (e.g., beach variably backed by seawall, dunes, and bluffs). In addition, significant regional anthropogenic factors have influenced sediment supply and tidal current strength. A variety of techniques were employed to investigate the erosion at Ocean Beach, including historical shoreline and bathymetric analysis, monthly beach topographic surveys, nearshore and regional bathymetric surveys, beach and nearshore grain size analysis, two surf-zone hydrodynamic experiments, four sets of nearshore wave and current experiments, and several numerical modeling approaches. Here, we synthesize the results of 7 years of data collection to lay out the causes of persistent erosion, demonstrating the effectiveness of integrating an array of data sets covering a huge range of spatial scales. The key findings are as follows: anthropogenic influences have reduced sediment supply from San Francisco Bay, leading to pervasive contraction (i.e., both volume and area loss) of the ebb-tidal delta, which in turn reduced the regional grain size and modified wave focusing patterns along Ocean Beach, altering nearshore circulation and sediment transport patterns. In addition, scour associated with an exposed sewage outfall pipe causes a local depression in wave heights, significantly modifying nearshore circulation patterns that have been shown through modeling to be key drivers of persistent erosion in that area.

  6. Application of geostatistics in Beach Placer

    International Nuclear Information System (INIS)

    Sundar, G.


    The goal of Geostatistics is in the prediction of possible spatial distribution of a property. Application of Geostatistics has gained significance in the field of exploration, evaluation and mining. In the case of beach and inland placer sands exploration, geostatistics can be used in optimising the drill hole spacing, estimate resources of the total heavy minerals (THM), estimation on different grid pattern and grade - tonnage curves. Steps involved in a geostatistical study are exploratory data analysis, creation of experimental variogram, variogram model fitting, kriging and cross validation. Basic tools in geostatistics are variogram and kriging. Characteristics of a variogram are sill, range and nugget. There is a necessity for variogram model fitting prior to kriging. Commonly used variogram models are spherical, exponential and gaussian

  7. Renewable Energy Development in Hermosa Beach, California (United States)

    Morris, K.


    The City of Hermosa Beach, California, with the support of the AGU's TEX program, is exploring the potential for renewable energy generation inside the City, as part of the implementation of the City's 2015 Municipal Carbon Neutral Plan. Task 1: Estimate the technical potential of existing and future technologies Given the City's characteristics, this task will identify feasible technologies: wind, solar, tidal/wave, wastewater biogas, landfill biogas, microscale anaerobic digestion (AD), and complementary energy storage. Some options may be open to the City acting alone, but others will require working with municipal partners and private entities that provide services to Hermosa Beach (e.g., wastewater treatment). Energy storage is a means to integrate intermittent renewable energy output. Task 2: Review transaction types and pathways In this task, feasible technologies will be further examined in terms of municipal ordinances and contractual paths: (a) power purchase agreements (PPAs) with developers, under which the City would purchase energy or storage services directly; (b) leases with developers, under which the City would rent sites (e.g., municipal rooftops) to developers; (c) ordinances related to permitting, under which the City would reduce regulatory barriers to entry for developers; (d) pilot projects, under which the City would engage with developers to test new technologies such as wind/wave/microscale AD (pursuant to PPAs and/or leases); and (e) existing projects, under which the City would work with current wastewater and landfill contractors to understand (i) current plans to develop renewable energy, and (ii) opportunities for the City to work with such contractors to promote renewable energy. Task 3: Estimate costs by technology Finally, the last task will gather existing information about the costs, both current and projected, of the feasible technologies, including (i) overnight construction cost (capital); (ii) integration costs (e

  8. Brazilian sandy beach macrofauna production: a review

    Directory of Open Access Journals (Sweden)

    Marcelo Petracco


    Full Text Available The state of the art of the studies on the production of Brazilian sandy beach macrofauna was analyzed on the basis of the data available in the literature. For this purpose, the representativeness of the production dataset was examined by latitudinal distribution, degree of exposure and morphodynamic state of beaches, taxonomic groups, and methods employed. A descriptive analysis was, further, made to investigate the trends in production of the more representative taxonomic groups and species of sandy beach macrofauna. A total of 69 macrofauna annual production estimates were obtained for 38 populations from 25 studies carried out between 22º56'S and 32º20'S. Production estimates were restricted to populations on beaches located on the southern and southeastern Brazilian coast. Most of the populations in the dataset inhabit exposed dissipative sandy beaches and are mainly represented by mollusks and crustaceans, with a smaller number of polychaetes. The trends in production among taxonomic groups follow a similar pattern to that observed on beaches throughout the world, with high values for bivalves and decapods. The high turnover rate (P/B ratio of the latter was due to the presence of several populations of the mole crab Emerita brasiliensis, which can attain high values of productivity, in the dataset. Most of the studies focus on the comparison of production and, especially, of P/B ratio according to life history traits in populations of the same species/taxonomic group. Despite the importance of life history-production studies, other approaches, such as the effect of man-induce disturbances on the macrofauna, should be undertaken in these threatened environments.O estado da arte dos estudos de produção da macrofauna de praias arenosas brasileiras foi analisado a partir de informações disponíveis na literatura. Para essa finalidade, a representatividade dos dados de produção foi examinada de acordo com a distribuição latitudinal

  9. Cranial nerves neuropraxia after shoulder arthroscopy in beach chair position. (United States)

    Cogan, A; Boyer, P; Soubeyrand, M; Hamida, F Ben; Vannier, J-L; Massin, P


    We report a case of neuropraxia of the 9th, 10th and 12th cranial nerve pairs after arthroscopic rotator cuff repair in the beach chair position. The elements in the medical file seem to exclude an intracranial cause of the lesions and support a mechanical, extracranial cause due to intubation and/or the beach chair position. This clinical case report shows the neurological risks of the beach chair position during arthroscopic shoulder surgery and presents the essential safety measures to prevent these risks. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  10. Clinical and genetic study of a juvenile-onset Huntington disease

    Directory of Open Access Journals (Sweden)

    HAO Ying


    Full Text Available Background Huntington's disease (HD is an autosomal dominant hereditary progressive neurodegenerative disorder with a distinct phenotype characterized by chorea, dementia, cognitive and affective impairment. There are selective neural cell loss and atrophy in the caudate and putamen. Dr. George Huntington firstly described the disease accurately and insightfully, which led to a widespread recognition of the inherited chorea that now bears his name. Huntington disease gene (IT15 locus on chromosome 4p16.3, and encompasses 67 exons with a trinucleotide repeat (CAG in the first exon. The CAG repeat length is highly polymorphic in the population and expanded on at least one chromosome of individuals with HD. Clinically, patient with HD are often onset in adulthood. Juvenile-onset HD is relatively rare. Adult-onset HD patients usually have a CAG expansion from 40 to 55 whereas those with juvenile-onset greater than 60 which are often inherited from the father. We investigated the clinical features of a juvenile-onset case with Huntington disease and dynamic mutation of his family. Methods The CAG repeats of IT15 gene were detected using polymerase chain reaction and capillary electrophoresis in 115 individuals with preliminary diagnosis as Huntington disease. The repeat numbers of some samples carried expanded or intermediate alleles were verified by the pMD18-T vector clone sequencing. Results Fragment analysis showed that one juvenile-onset case presenting with cognitive dysfunction and hypokinesis carried 15/68 CAG repeats of IT15. His father carried 17/37 and mother carried 15/17. Conclusion 1 The juvenile-onset case of HD presented with different clinical features compared with adult-onset cases. The typical signs of adult-onset cases include progressive chorea, rigidity and dementia. The most common sign of juvenile-onset Huntington disease is cognitive decline. 2 The dynamic mutation of IT15 gene expansion of the CAG repeats in the

  11. Presence of tau pathology within foetal neural allografts in patients with Huntington's and Parkinson's disease. (United States)

    Cisbani, Giulia; Maxan, Alexander; Kordower, Jeffrey H; Planel, Emmanuel; Freeman, Thomas B; Cicchetti, Francesca


    Cell replacement has been explored as a therapeutic strategy to repair the brain in patients with Huntington's and Parkinson's disease. Post-mortem evaluations of healthy grafted tissue in such cases have revealed the development of Huntington- or Parkinson-like pathology including mutant huntingtin aggregates and Lewy bodies. An outstanding question remains if tau pathology can also be seen in patients with Huntington's and Parkinson's disease who had received foetal neural allografts. This was addressed by immunohistochemical/immunofluorescent stainings performed on grafted tissue of two Huntington's disease patients, who came to autopsy 9 and 12 years post-transplantation, and two patients with Parkinson's disease who came to autopsy 18 months and 16 years post-transplantation. We show that grafts also contain tau pathology in both types of transplanted patients. In two patients with Huntington's disease, the grafted tissue showed the presence of hyperphosphorylated tau [both AT8 (phospho-tau Ser202 and Thr205) and CP13 (pSer202) immunohistochemical stainings] pathological inclusions, neurofibrillary tangles and neuropil threads. In patients with Parkinson's disease, the grafted tissue was characterized by hyperphosphorylated tau (AT8; immunofluorescent staining) pathological inclusions, neurofibrillary tangles and neuropil threads but only in the patient who came to autopsy 16 years post-transplantation. Abundant tau-related pathology was observed in the cortex and striatum of all cases studied. While the striatum of the grafted Huntington's disease patient revealed an equal amount of 3-repeat and 4-repeat isoforms of tau, the grafted tissue showed elevated 4-repeat isoforms by western blot. This suggests that transplants may have acquired tau pathology from the host brain, although another possibility is that this was due to acceleration of ageing. This finding not only adds to the recent reports that tau pathology is a feature of these neurodegenerative

  12. Solid Waste Transportation through Ocean Currents: Marine Debris Sightings and their Waste Quantification at Port Dickson Beaches, Peninsular Malaysia


    Chong Jing Yi; Narayanan Kannan


    Four beaches at Port Dickson, Peninsular Malaysia, namely Saujana Beach, Nelayan Beach, Bagan Pinang Beach and Cermin beach have been sampled for marine debris from 7th June 2014 until 26th July 2014, on every Saturday. These beaches face the Strait of Malacca with a coastline stretching 18 km each. Our observations revealed a total debris items of 13193 in those beaches. The top three items of highest frequency were cigarette butts, foamed fragments and food wrappers. Plastic debris scaled h...

  13. The influence of anthropic actions on the evolution of an urban beach: Case study of Marineta Cassiana beach, Spain. (United States)

    Pagán, J I; Aragonés, L; Tenza-Abril, A J; Pallarés, P


    Coastal areas have been historically characterized as being a source of wealth. Nowadays, beaches have become more relevant as a place for rest and leisure. This had led to a very high population pressure due to rapid urbanisation processes. The impacts associated with coastal tourism, demand the development of anthropic actions to protect the shoreline. This paper has studied the impacts of these actions on the Marineta Cassiana beach, in Denia, Spain. This particular Mediterranean beach has traditionally suffered a major shoreline regression, and the beach nourishments carried out in the 1980s would not have achieved the reliability desired. This research has analysed the historic evolution of the beach and its environment for a period of 65years (1950-2015). A Geographic Information System (GIS) has been used to integrate and perform a spatial analysis of urban development, soil erosion, stream flow, swell, longshore transport, submerged vegetation species and shoreline evolution. The results show how the anthropic actions have affected the shoreline. After the excessive urban development of the catchments, there is no natural sediment supply to the beach. The change in the typology of the sediment, from pebbles to sand, during the beach nourishments has led to a crucial imbalance in the studied area. Moreover, the beach area gained has disappeared, affecting the Posidonia oceanica meadow, and incrementing the erosion rates. The findings obtained are relevant, not only in the management and maintenance of the beaches, but also, in the decision-making for future nourishments. Copyright © 2016 Elsevier B.V. All rights reserved.

  14. Mechanical grooming and beach award status are associated with low strandline biodiversity in Scotland (United States)

    Gilburn, Andre S.


    Beach grooming and beach award status are both shown to be associated with low macroinvertebrate taxon richness in Scotland. Previous studies in California have revealed that mechanical raking to remove wrack from sandy beaches has negative ecological consequences for coastal ecosystems. In the current study the presence and absence of eight common taxa that inhabit beached wrack on sandy beaches in Scotland was assessed at 60 sites, 24 of which were groomed and 29 of which were in receipt of a beach award. On average 4.86 of the eight taxa were found to be present on ungroomed beaches, whereas only 1.13 taxa were present on groomed beaches. Thus, beach grooming seems to be having a major effect on the biodiversity of beach macroinvertebrates in Scotland. Fewer macroinvertebrate taxa were also found on award (1.5) compared to non-award (4.38) beaches. It was also revealed that award beaches were much more likely to be groomed than non-award beaches, with 69% of award beaches surveyed being groomed compared to only 6% of non-award beaches. This pattern is surprising as the awarding bodies discourage the removal of seaweed and regulations state that beached wrack should only be removed if it constitutes a nuisance. It is concluded that award status, not nuisance level, has the main factor driving most beach grooming and that this has resulted in the substantial loss of macroinvertebrate biodiversity from award beaches in Scotland. In conclusion it is shown that beach grooming has a substantial negative impact upon strandline macroinvertebrate biodiversity in Scotland and that grooming is much more likely to occur on award beaches.

  15. Evaluating the radiological health compliance of some beach ...

    African Journals Online (AJOL)

    Journal of Applied Sciences and Environmental Management ... Evaluating the radiological health compliance of some beach environments in Delta State ... as well as specified members of the public (customers) are not at radiological risk.

  16. Type and Quantity of Shipborne Garbage at Selected Tropical Beaches

    Directory of Open Access Journals (Sweden)

    Julyus-Melvin Mobilik


    Full Text Available Marine debris is widely distributed at the coastal area of the global oceans; however, shipborne garbage source studies are still lacking to document the pollution in Malaysia Territorial Water. Thus, this study has adopted a standard method of beach marine debris survey at five beaches and inspected 115 vessels to assess the type and amount of debris from shipping source stranded on the beach. This study found that vessel visiting Malaysian ports observed the MARPOL 73/78 Annex V requirements; however, identified objects from shipping activity (1.3%; 2 items/km found on the beaches indicate that there are vessels disposing of garbage illegally at sea. Therefore, there is a need to promote the use of biodegradable material and introduce environmental education to increase awareness on the vessel.

  17. Plastic pellets on the Caranzalem beach sands, Goa, India

    Digital Repository Service at National Institute of Oceanography (India)

    Nigam, R.

    Postmonsoonal survey of Caranzalem beach, Goa, India indicated the presence of plastic pellets. These pellets varied in shape, size and number, and are considered to be contaminants of marine environment...

  18. Daytona Beach, Florida Tsunami Forecast Grids for MOST Model (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Daytona Beach, Florida Forecast Model Grids provides bathymetric data strictly for tsunami inundation modeling with the Method of Splitting Tsunami (MOST) model....

  19. Macrofauna and meiofauna of two sandy beaches at Mombasa, Kenya

    Digital Repository Service at National Institute of Oceanography (India)

    Ansari, Z.A.; Ingole, B.S.; Parulekar, A.H.

    Macrofauna and meiofauna of 2 sandy beaches having medium and fine sand particles, respectively, were investigated, quantitatively Macrofauna density was highest around high water mark and progressively decreased towards low water mark Meiofauna...

  20. Miramar (Goa) Beach Management Project: An Oceanographic Evaluation

    Digital Repository Service at National Institute of Oceanography (India)

    Mascarenhas, A.; Ingole, B.S.

    implications of sand dunes, significance of benthic biodiversity, knowledge of coastal geological processes and restoration of degraded sand dunes should form an integral part of any beach management strategy....

  1. Source identification of a tar residue from Mumbai Beach

    Digital Repository Service at National Institute of Oceanography (India)

    Kadam, A; Rokade, M.A

    A tar residue from Mumbai Beach, Maharashtra, India was matched with the suspected source sample from a tanker using UV, IR and GLC techniques. Negligible differences in several ratios of UV absorbances and ratios of infrared transmittances...

  2. Myrtle Beach, South Carolina Tsunami Forecast Grids for MOST Model (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Myrtle Beach, South Carolina Forecast Model Grids provides bathymetric data strictly for tsunami inundation modeling with the Method of Splitting Tsunami (MOST)...

  3. Beach morphological variations over micro-time scales

    Digital Repository Service at National Institute of Oceanography (India)

    Murty, C.S.; Veerayya, M.; Sastry, J.S.; Varadachari, V.V.R.

    formation, variations in the profiles show anomalous behaviour. The differences in grain-size distribution of the sediments of these 2 beaches are attributed to the available wave energies at these 2 locations...

  4. Virginia Beach Tsunami Forecast Grids for MOST Model (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Virginia Beach, Virginia Forecast Model Grids provides bathymetric data strictly for tsunami inundation modeling with the Method of Splitting Tsunami (MOST)...

  5. Type and Quantity of Shipborne Garbage at Selected Tropical Beaches (United States)

    Husain, Mohd-Lokman


    Marine debris is widely distributed at the coastal area of the global oceans; however, shipborne garbage source studies are still lacking to document the pollution in Malaysia Territorial Water. Thus, this study has adopted a standard method of beach marine debris survey at five beaches and inspected 115 vessels to assess the type and amount of debris from shipping source stranded on the beach. This study found that vessel visiting Malaysian ports observed the MARPOL 73/78 Annex V requirements; however, identified objects from shipping activity (1.3%; 2 items/km) found on the beaches indicate that there are vessels disposing of garbage illegally at sea. Therefore, there is a need to promote the use of biodegradable material and introduce environmental education to increase awareness on the vessel. PMID:27819020

  6. Study of longshore current equations for currents in Visakhapatnam beach

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Rao, T.V.N.

    Longshore currents were measured along the Visakhapatnam Beach, Andhra Pradesh, India at weekly intervals from March 1978 to March 1979. Visual observations on breaker characteristics were also made during this period. Using modified Longuet...

  7. Palm Beach, Florida Tsunami Forecast Grids for MOST Model (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Palm Beach, Florida Forecast Model Grids provides bathymetric data strictly for tsunami inundation modeling with the Method of Splitting Tsunami (MOST) model....

  8. Wave refraction and littoral currents off Colva Beach, Goa

    Digital Repository Service at National Institute of Oceanography (India)

    Veerayya, M.; Murty, C.S.; Varadachari, V.V.R.

    Wave refraction studies have been carried out for waves of different periods approaching the coast at Colva, with directions of approach lying between180 degrees and 340 degrees, to obtain a qualitative picture of littoral flows along the beach...

  9. Geochemistry of dark coastal heavy-mineral beaches sand (Annaba ...

    African Journals Online (AJOL)


    3 Institute of Earth and Environmental Science, University of Potsdam, ... Some beaches are characterized by a red-brownish sand colour, the Ain Achir and the ... The occurrence of clays has been determined using the methyl-blue method.

  10. Radiation sensitivity of fibroblast strains from patients with Usher's syndrome, Duchenne muscular dystrophy, and Huntington's disease

    International Nuclear Information System (INIS)

    Nove, J.; Little, J.B.; Tarone, R.E.; Robbins, J.H.


    The colony-forming ability of 10 normal human fibroblast cell strains and of 10 strains representing 3 degenerative diseases of either nerve or muscle cells was determined after exposure of the cells to X-rays or β-particles from tritiated water. Both methods of irradiation yielded similar comparative results. The fibroblast strains from the 5 Usher's syndrome patients and from 1 of the 2 Huntington's disease patients were hypersensitive to radiation, while those from the 3 Duchenne muscular dystrophy patients and the second Huntington's disease patient had normal sensitivity to radiation. These results indicate both disease-specific and strain-specific differences in the survival of fibroblasts after exposure to ionizing radiation. 38 refs.; 2 figs.; 3 tabs

  11. Huntington's disease predictive testing: the case for an assessment approach to requests from adolescents. (United States)

    Binedell, J; Soldan, J R; Scourfield, J; Harper, P S


    Adolescents who are actively requesting Huntington's predictive testing of their own accord pose a dilemma to those providing testing. In the absence of empirical evidence as regards the impact of genetic testing on minors, current policy and guidelines, based on the ethical principles of non-maleficence and respect for individual autonomy and confidentiality, generally exclude the testing of minors. It is argued that adherence to an age based exclusion criterion in Huntington's disease predictive testing protocols is out of step with trends in UK case law concerning minors' consent to medical treatment. Furthermore, contributions from developmental psychology and research into adolescents' decision making competence suggest that adolescents can make informed choices about their health and personal lives. Criteria for developing an assessment approach to such requests are put forward and the implications of a case by case evaluation of competence to consent in terms of clinicians' tolerance for uncertainty are discussed. PMID:8950670

  12. A proxy late Holocene climatic record deduced from northwest Alaskan beach ridges

    International Nuclear Information System (INIS)

    Mason, O.K.; Jordan, J.W.


    A climatically-sensitive, oscillatory pattern of progradation and erosion is revealed in late Holocene accretionary sand ridge and barrier island complexes of Seward Peninsula, northwest Alaska. Archaeological and geological radiocarbon dates constrain the authors chronology for the Cape Espenberg beach ridge plain and the Shishmaref barrier islands, 50 km to the southwest. Cape Espenberg, acts as the depositional sink for the northeastward longshore transport system and contains the oldest sedimentary deposits: based on 3700±90 B.P. (β-23170) old grass from a paleosol capping a low dune facies. The oldest date on the Shishmaref barrier islands is 1550±70 B.P. (β-23183) and implies that the modem barrier is a comparatively recent phenomenon. Late Holocene sedimentation varies between intervals of erosion and rapid progradation. During erosional periods higher dunes are built atop beach ridges: as between 3000-2000 yrs. BP and intermittently from 1000 BP to the present. At other times, rapid progradation predominated, generating wide swales and low beach ridges without dunes. Tentatively, dune formation is correlative with the Neo-glacial and Little Ice Age glacial advances and increased alluviation in north Alaska. Rapid progradation is contemporaneous with warmer intervals of soil and peat formation atop alluvial terraces, dated to ca. 4000-3500 and 2000-1000 yrs. B.P. In the record of the last 1000 years, dune building is correlative with heightened storminess, as reflected in northwest Alaska tree-ring chronologies and weather anomalies such as spring dust storms and winter thunderstorms in East Asian locations

  13. Integrated Co-management of Lakes through Beach Management Units


    Goverment of Uganda; Department for International Development (DFID) of the UK Government


    Metadata only record In 1999, the Integrated Co-management of Lakes through Beach Management Units project was started in an effort to implement a new approach to the management of lake resources in Uganda. The main components of this plan involved decentralization, local community management, and improving the livelihood of the poor. In order to finance the management of these areas, the Beach Management Units (BMU's) are charging user fees to those individuals who obtain benefit from the...

  14. Marine pollution: Let us not forget beach sand


    Galgani, Francois; Ellerbrake, Katrin; Fries, Elke; Goreux, Chantal


    Background: Assessing the chemical or bacterial contamination in marine waters and sediments is a very common approach to evaluate marine pollution and associated risks. However, toxicity and organic pollution of beach sands have not yet been considered, except in adjacent waters. In the present study, the toxicity and the chemical contamination of natural beach sands collected 20 m from the shoreline at two sites located on the Mediterranean Sea (Marseille and La Marana, Corsica) were studie...

  15. Stability of the beaches in Nagapattinam District, Tamilnadu, India

    Digital Repository Service at National Institute of Oceanography (India)

    Manickaraj, D.S.; Chandrasekaran, R.; Gujar, A.R.; Loveson, V.J.; Angusamy, N.; Chandrasekar, N.; Rajamanickam, G.V.

    in Nagapattinam District, Tamilnadu, India Soosai Manickaraj, D 1 ., Chandrasekaran, R 1 ., Gujar, A.R 2 ., Loveson, V.J 3 ., Angusamy, N 4 ., Chandrasekar, N 5 ., and Victor Rajamanickam, G 1 1 Disaster Management, SASTRA Deemed University, Thanjavur... measurements are necessary. By comparing measurements taken at different times, a beach's stability is determined. Shoreline change data has many uses. This information is needed to evaluate the carrying capacity of the beach. It is also used to set...

  16. Mobilisation of toxic trace elements under various beach nourishments. (United States)

    Pit, Iris R; Dekker, Stefan C; Kanters, Tobias J; Wassen, Martin J; Griffioen, Jasper


    To enhance protection and maintain wide beaches for recreation, beaches are replenished with sand: so-called beach nourishments. We compared four sites: two traditional beach nourishments, a mega beach nourishment and a reference without beach nourishment. Two sites contain calcareous-rich sand, whereas the other two sites have calcareous-poor sand. We aimed to understand hydrogeochemical processes to indicate factors critical for the mobility of trace elements at nourishments. We therefore analysed the chemical characteristics of sediment and pore water to ascertain the main drivers that mobilise toxic trace elements. With Dutch Quality Standards for soil and groundwater, the characteristics of sediment and pore water were compared to Target Values (the values at which there is a sustainable soil quality) and Intervention Values (the threshold above which the soil's functions are at risk). The pore water characteristics revealed that Target Values were regularly exceeded, especially for the nourishment sites and mainly for Mo (78%), Ni (24%), Cr (55%), and As (21%); Intervention Values for shallow groundwater were occasionally exceeded for As (2%), Cr (2%) and Zn (2%). The sediment characteristics did not exceed the Target Values and showed that trace elements were mainly present in the fine fraction of <150 μm. The oxidation of sulphide minerals such as pyrite resulted into the elevated concentration for all nourishment sites, especially when an unsaturated zone was present and influence of rainwater was apparent. To prevent trace metal mobility at a mega beach nourishment it is important to retain seawater influences and limit oxidation processes. In this respect, a shoreface nourishment is recommended rather than a mega beach nourishment with a thick unsaturated zone. Consequently, we conclude that whether a site is carbonate-rich or carbonate-poor is unimportant, as the influence of seawater will prevent decalcification, creating a low risk of mobilisation

  17. Estuarine beaches of the Amazon coast: environmental and recreational characterization


    de Sousa, Rosigleyse C.; Pereira, Luci Cajueiro Carneiro; Jiménez Quintana, José Antonio


    The Amazon coast is rich in natural resources, with highly valued natural landscapes and ecological systems. These environments include estuarine beaches, which are important areas for recreational activities. The present study provides an environmental and recreational diagnosis of three of these estuarine beaches on the Amazon coast (Colares, Maruda, and Murubira). The study was conducted in July, 2012, 2013 and 2015. An set of variables was assessed: (i) physical variables (hydrodynamics),...

  18. Medium-term dynamics of a middle Adriatic barred beach

    Directory of Open Access Journals (Sweden)

    M. Postacchini


    Full Text Available In recent years, attention has been paid to beach protection by means of soft and hard defenses. Along the Italian coast of the Adriatic Sea, sandy beaches are the most common landscape feature and around 70 % of the Marche region's coast (central Adriatic is protected by defense structures. The longest free-from-obstacle nearshore area in the region includes the beach of Senigallia, frequently monitored in the last decades and characterized by a multiple bar system, which represents a natural beach defense. The bathymetries surveyed in 2006, 2010, 2011, 2012 and 2013 show long-term stability, confirmed by a good adaptation of an analyzed stretch of the beach to the Dean-type equilibrium profile, though a strong short- to medium-term variability of the wave climate has been observed during the monitored periods. The medium-term dynamics of the beach, which deal with the evolution of submerged bars and are of the order of years or seasons, have been related to the wave climate collected, during the analyzed temporal windows, by a wave buoy located about 40 km off Senigallia. An overall interpretation of the hydrodynamics, sediment characteristics and seabed morphology suggests that the wave climate is fundamental for the morphodynamic changes of the beach in the medium term. These medium-term time ranges during which waves mainly come from NNE/ESE are characterized by a larger/smaller steepness and by a larger/smaller relative wave height, and seem to induce seaward/shoreward bar migration as well as bar smoothing/steepening. Moving southeastward, the bar dimension increases, while the equilibrium profile shape suggests the adaptation to a decreasing sediment size in the submerged beach. This is probably due to the presence of both the harbor jetty and river mouth north of the investigated area.

  19. Sediment Transport and erosion modeling at Heaundae Beach in Korea. (United States)

    Do, K.; Yoo, J.; McCall, R. T.


    The sand pocket beaches with two headlands are global features, but it's not easy to predict berm and dune erosion due to alongshore variation of water depth. This study investigates the sediment transport and morphological change using available wave and beach profile data, as well as to assess the applicability of the XBeach morphological model (Roelvink et al., 2009). The Haeundae is small pocket beach, 1.4 km long, located in the southern corner of the Korean Peninsula. The Korea Institute of Ocean Science and Technology (KIOST) measured beach profile along 27 survey lines. The beach profiles were surveyed five times from 17 June 2014 to 10 October 2014. For this duration, a wave gauge (AWAC) was installed at a depth about 23 m off the coast of Haeundae Beach. Severe four storms attacked Haeundae Beach for this duration and these storms lasted about 1 2 days with a peak significant wave height of 2.5 4.0 m. The placed sand is fairly sorted and its median diameter is 0.23 mm. 2DH coastal morphological model, XBeach developed to simulate dune erosion due to storm impacts. The model is based on the nonlinear shallow water equation and resolves nearshore hydrodynamics by employing a 2DH description of wave groups and infragravity motions. In this study, the numerical model XBeach was compared with the field data and used to estimate the sediment transport pattern on the sand pocket beach. The numerical model resulted in a comparable prediction in the west-part, but the east-part cannot reproduce the erosion and accretion of the sand, partly due to complex bathymetry and the lack of sediment. This limitation needs to be improved to use measured sand thickness data in future study

  20. A holistic evaluation of a typical beach nourishment

    DEFF Research Database (Denmark)

    Margheritini, Lucia; Frigaard, Peter; Wahl, Niels Arne


    is the primary method used by the Danish Coastal Authority for coastal protection and represents a management tool which serves a dual purpose. Beach Nourishment is protecting coastal lands as well as backshore properties (infrastructures, buildings etc.) and preserving natural heritages. Nevertheless, more...... an example of a holistic evaluation of a 721.000 m3  (155 m3 /m) Beach Nourishment done at the Danish West Coast in 2005....

  1. Grunting in genetically modified minipig animal model for Huntington ´s disease - a pilot experiment

    Czech Academy of Sciences Publication Activity Database

    Tykalová, T.; Hlavnička, J.; Mačáková, Monika; Baxa, Monika; Cmejla, R.; Motlík, Jan; Klempíř, J.; Rusz, J.


    Roč. 78, Suppl 2 (2015), s. 12-13 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * mitochondria * DNA damage Subject RIV: FH - Neurology

  2. Double strand DNA breaks response in Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Šolc, Petr; Valášek, Jan; Rausová, Petra; Juhásová, Jana; Juhás, Štefan; Motlík, Jan


    Roč. 78, Suppl 2 (2015), s. 15-15 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * DNA damage * double strand DNA breaks Subject RIV: FH - Neurology

  3. Dynamics of the connectome in Huntington's disease: A longitudinal diffusion MRI study


    Odish, Omar F.F.; Caeyenberghs, Karen; Hosseini, Hadi; van den Bogaard, Simon J.A.; Roos, Raymund A.C.; Leemans, Alexander


    Abstract Objectives To longitudinally investigate the connectome in different stages of Huntington's disease (HD) by applying graph theoretical analysis to diffusion MRI data. Experimental design We constructed weighted structural networks and calculated their topological properties. Twenty-two premanifest (preHD), 10 early manifest HD and 24 healthy controls completed baseline and 2 year follow-up scans. We stratified the preHD group based on their predicted years to disease onset into a far...

  4. Minipig Model of Huntington's Disease: H-1 Magnetic Resonance Spectroscopy of the Brain

    Czech Academy of Sciences Publication Activity Database

    Jozefovičová, M.; Herynek, V.; Jírů, F.; Dezortová, M.; Juhásová, Jana; Juhás, Štefan; Motlík, Jan; Hájek, M.


    Roč. 65, č. 1 (2016), s. 155-163 ISSN 0862-8408 R&D Projects: GA TA ČR(CZ) TA01011466; GA MŠk(CZ) 7F14308; GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington´s disease * minipigs * magnetic resonance spectroscopy Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 1.461, year: 2016

  5. Human glia can both induce and rescue aspects of disease phenotype in Huntington disease

    DEFF Research Database (Denmark)

    Benraiss, Abdellatif; Wang, Su; Herrlinger, Stephanie


    The causal contribution of glial pathology to Huntington disease (HD) has not been heavily explored. To define the contribution of glia to HD, we established human HD glial chimeras by neonatally engrafting immunodeficient mice with mutant huntingtin (mHTT)-expressing human glial progenitor cells...... chimeras are hyperexcitable. Conversely, normal glia can ameliorate disease phenotype in transgenic HD mice, as striatal transplantation of normal glia rescues aspects of electrophysiological and behavioural phenotype, restores interstitial potassium homeostasis, slows disease progression and extends...

  6. The Effect of Music Therapy in Patients with Huntington's Disease: A Randomized Controlled Trial. (United States)

    van Bruggen-Rufi, Monique C H; Vink, Annemieke C; Wolterbeek, Ron; Achterberg, Wilco P; Roos, Raymund A C


    Music therapy may have beneficial effects on improving communication and expressive skills in patients with Huntington's disease (HD). Most studies are, however, small observational studies and methodologically limited. Therefore we conducted a multi-center randomized controlled trial. To determine the efficacy of music therapy in comparison with recreational therapy in improving quality of life of patients with advanced Huntington's disease by means of improving communication. Sixty-three HD-patients with a Total Functional Capacity (TFC) score of ≤7, admitted to four long-term care facilities in The Netherlands, were randomized to receive either group music therapy or group recreational therapy in 16 weekly sessions. They were assessed at baseline, after 8, 16 and 28 weeks using the Behaviour Observation Scale for Huntington (BOSH) and the Problem Behaviour Assessment-short version (PBA-s). A linear mixed model with repeated measures was used to compare the scores between the two groups. Group music therapy offered once weekly for 16 weeks to patients with Huntington's disease had no additional beneficial effect on communication or behavior compared to group recreational therapy. This was the first study to assess the effect of group music therapy on HD patients in the advanced stages of the disease. The beneficial effects of music therapy, recorded in many, mainly qualitative case reports and studies, could not be confirmed with the design (i.e. group therapy vs individual therapy) and outcome measures that have been used in the present study. A comprehensive process-evaluation alongside the present effect evaluation is therefore performed.

  7. EMQN/CMGS best practice guidelines for the molecular genetic testing of Huntington disease


    Losekoot, Monique; van Belzen, Martine J; Seneca, Sara; Bauer, Peter; Stenhouse, Susan A R; Barton, David E


    Huntington disease (HD) is caused by the expansion of an unstable polymorphic trinucleotide (CAG)n repeat in exon 1 of the HTT gene, which translates into an extended polyglutamine tract in the protein. Laboratory diagnosis of HD involves estimation of the number of CAG repeats. Molecular genetic testing for HD is offered in a wide range of laboratories both within and outside the European community. In order to measure the quality and raise the standard of molecular genetic testing in these ...

  8. Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease


    Mealer, Robert G.; Subramaniam, Srinivasa; Snyder, Solomon H.


    Although the mutated protein causing Huntington's disease (HD) is expressed throughout the body, the major pathology of HD is localized to the striatum of the brain. We previously reported that the striatal-enriched protein Rhes binds the mutated huntingtin protein and enhances its cytotoxicity. We now demonstrate that Rhes-deleted mice are dramatically protected from neurotoxicity and motor dysfunction in a striatal-specific model of HD elicited by 3-nitropropionic acid. This finding suggest...

  9. Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study. (United States)


    To determine whether ethyl-eicosapentaenoic acid (ethyl-EPA), an omega-3 fatty acid, improves the motor features of Huntington disease. Six-month multicenter, randomized, double-blind, placebo-controlled trial followed by a 6-month open-label phase without disclosing initial treatment assignments. Forty-one research sites in the United States and Canada. Three hundred sixteen adults with Huntington disease, enriched for a population with shorter trinucleotide (cytosine-adenine-guanine) repeat length expansions. Random assignment to placebo or ethyl-EPA, 1 g twice a day, followed by open-label treatment with ethyl-EPA. Six-month change in the Total Motor Score 4 component of the Unified Huntington's Disease Rating Scale analyzed for all research participants and those with shorter cytosine-adenine-guanine repeat length expansions (<45). At 6 months, the Total Motor Score 4 point change for patients receiving ethyl-EPA did not differ from that for those receiving placebo. No differences were found in measures of function, cognition, or global impression. Before public disclosure of the 6-month placebo-controlled results, 192 individuals completed the open-label phase. The Total Motor Score 4 change did not worsen for those who received active treatment for 12 continuous months compared with those who received active treatment for only 6 months (2.0-point worsening; P=.02). Ethyl-EPA was not beneficial in patients with Huntington disease during 6 months of placebo-controlled evaluation. Clinical Trial Registry Identifier: NCT00146211.

  10. Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease


    Keene, C. Dirk; Rodrigues, Cecilia M. P.; Eich, Tacjana; Chhabra, Manik S.; Steer, Clifford J.; Low, Walter C.


    Huntington's disease (HD) is an untreatable neurological disorder caused by selective and progressive degeneration of the caudate nucleus and putamen of the basal ganglia. Although the etiology of HD pathology is not fully understood, the observed loss of neuronal cells is thought to occur primarily through apoptosis. Furthermore, there is evidence in HD that cell death is mediated through mitochondrial pathways, and mitochondrial deficits are commonly associated with HD. We have previously r...

  11. Prevalence of Huntington's disease gene CAG trinucleotide repeat alleles in patients with bipolar disorder. (United States)

    Ramos, Eliana Marisa; Gillis, Tammy; Mysore, Jayalakshmi S; Lee, Jong-Min; Alonso, Isabel; Gusella, James F; Smoller, Jordan W; Sklar, Pamela; MacDonald, Marcy E; Perlis, Roy H


    Huntington's disease is a neurodegenerative disorder characterized by motor, cognitive, and psychiatric symptoms that are caused by huntingtin gene (HTT) CAG trinucleotide repeat alleles of 36 or more units. A greater than expected prevalence of incompletely penetrant HTT CAG repeat alleles observed among individuals diagnosed with major depressive disorder raises the possibility that another mood disorder, bipolar disorder, could likewise be associated with Huntington's disease. We assessed the distribution of HTT CAG repeat alleles in a cohort of individuals with bipolar disorder. HTT CAG allele sizes from 2,229 Caucasian individuals diagnosed with DSM-IV bipolar disorder were compared to allele sizes in 1,828 control individuals from multiple cohorts. We found that HTT CAG repeat alleles > 35 units were observed in only one of 4,458 chromosomes from individuals with bipolar disorder, compared to three of 3,656 chromosomes from control subjects. These findings do not support an association between bipolar disorder and Huntington's disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Palliative Care in Huntington Disease: Personal Reflections and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Christopher G. Tarolli


    Full Text Available Background Huntington disease is a fatal, autosomal dominant, neurodegenerative disorder manifest by the triad of a movement disorder, behavioral disturbances, and dementia. At present, no curative or disease modifying therapies exist for the condition and current treatments are symptomatic. Palliative care is an approach to care that focuses on symptom relief, patient and caregiver support, and end of life care. There is increasing evidence of the benefit of palliative care throughout the course of neurodegenerative conditions including Parkinson disease and amyotrophic lateral sclerosis. However, beyond its application at the end of life, little is known about the role of palliative care in Huntington disease.Methods In this article, we discuss what is known about palliative care in Huntington disease, specifically related to early disease burden, caregiver burnout, advance care planning, and end of life care.Results We provide a review of the current literature and discuss our own care practices.Discussion We conclude by discussing questions that remain unanswered and positing ideas for future work in the field.

  13. Did the "Woman in the Attic" in Jane Eyre Have Huntington Disease?

    Directory of Open Access Journals (Sweden)

    Elizabeth A. Coon


    Full Text Available Background: References to neurologic disorders are frequently found in fictional literature and may precede description in the medical literature. Aim: Our aim was to compare Charlotte Brontë’s depiction of Bertha Mason in Jane Eyre to the tenets set forth in George Huntington’s original essay “On chorea” with the hypothesis that Mason was displaying features of Huntington disease. Results: Charlotte Brontë’s 1847 Victorian novel Jane Eyre features the character Bertha Mason, who is portrayed with a progressive psychiatric illness, violent movements, and possible cognitive decline. Similar to Huntington’s tenets, Mason has a disorder with a strong family history suggestive of autosomal dominant inheritance with onset in adulthood, and culminating in suicide. Conclusion: Brontë’s character had features of Huntington disease as originally described by Huntington. Brontë’s keen characterization may have increased awareness of treatment of neuropsychiatric patients in the Victorian era.

  14. Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

    Directory of Open Access Journals (Sweden)



    Full Text Available Early-onset Huntington's disease (HD occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.Doença de Huntington (DH de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.

  15. Mineral legislations applicable to beach sand industry

    International Nuclear Information System (INIS)

    D'Cruz, Eric


    India has got a wealth of natural resources in different geological environs and shoreline placers form an important constituent of the natural resources. Large reserves of beach sand minerals, viz. imenite, rutile, leucoxene, zircon, sillimanite, garnet and monazite are the economic minerals in the coastal and inland placer sands. In the federal structure of India, the State Governments are the owners of minerals located within their respective boundaries. The State Governments grant the mineral concessions for all the minerals located within the boundary of the State, under the provisions of the Acts and Rules framed for the purpose. Though the mineral wealth is under the control of the State, the power for framing the rules for the grant of mineral concessions vastly rest with the Central Government. Since mineral concessions are often granted for a longer duration of thirty to fifty years or more, a historical perspective of these rules are imperative in understanding the issues involved with BSM mining industry. Under the Govt. of India Act, 1935, Regulation of Mines and Oilfields and Mineral Development was kept under Federal control, declared by Federal Law. The word 'Federal' was substituted by the word 'Dominion' by the India (Provincial Constitution) Order, 1947. No legislation was, however, enacted in pursuance of above power until after Independence. However, the Govt. on India made the Mining Concession (Central) Rules, 1939 for regulating grants of prospecting license

  16. Slurry discharge management-beach profile prediction

    Energy Technology Data Exchange (ETDEWEB)

    Bravo, R.; Nawrot, J.R. [Southern Illinois University at Carbondale, Carbondale, IL (United States). Dept. of Civil Engineering


    Mine tailings dams are embankments used by the mining industry to retain the tailings products after the mineral preparation process. Based on the acid-waste stereotype that all coal slurry is acid producing, current reclamation requires a four foot soil cover for inactive slurry disposal areas. Compliance with this requirement is both difficult and costly and in some case unnecessary, as not all the slurry, or portions of slurry impoundments are acid producing. Reduced costs and recent popularity of wetland development has prompted many operators to request reclamation variances for slurry impoundments. Waiting to address slurry reclamation until after the impoundment is full, limits the flexibility of reclamation opportunities. This paper outlines a general methodology to predict the formation of the beach profile for mine tailings dams, by the discharge volume and location of the slurry into the impoundment. The review is presented under the perspective of geotechnical engineering and waste disposal management emphasizing the importance of pre-planning slurry disposal land reclamation. 4 refs., 5 figs.

  17. Understanding beach health throughout the Great Lakes-Entering a new era of investigations (United States)



    For over a decade, the U.S. Geological Survey (USGS) has been a leader in the science of beach health. The overall mission of this work is to provide science-based information and methods that will allow beach managers to more accurately make beach closure and advisory decisions, understand the sources and physical processes affecting beach contaminants, and understand how science-based information can be used to mitigate and restore beaches and protect the public. The work consists of four science elements-real-time assessments; pathogens and microbial source tracking; coastal processes; and data analysis, interpretation, and communication - which are described in this fact sheet. Some of the key questions for USGS beach research are the following: Are there better ways to inform the public whether they can use a beach without risking their health? How do new rapid analytical methods compare to traditional methods for determining concentrations of fecal-indicator bacteria at beaches? Are pathogens present at beaches and, if so, how do they get to the beach, and what is their source? How do sand movement and wave action on the beach affect fecal-indicator-bacteria and pathogen concentrations in the lake water? What are the best indicators of pathogenic microorganisms? With so many potential sources of fecal contamination at a beach, what methods can be used to distinguish the contributions from humans? What characteristics of beaches contribute most to influencing bacterial indicator and pathogen concentrations in beach sands and groundwater?

  18. Users' Perception as a Tool to Improve Urban Beach Planning and Management (United States)

    Cervantes, Omar; Espejel, Ileana; Arellano, Evarista; Delhumeau, Sheila


    Four beaches that share physiographic characteristics (sandy, wide, and long) but differ in socioeconomic and cultural terms (three are located in northwestern Mexico and one in California, USA) were evaluated by beach users. Surveys (565) composed of 36 questions were handed out to beach users on weekends and holidays in 2005. The 25 questions that revealed the most information were selected by factor analysis and classified by cluster analysis. Beach users’ preferences were assigned a value by comparing the present survey results with the characteristics of an “ideal” recreational urban beach. Cluster analysis separated three groups of questions: (a) services and infrastructure, (b) recreational activities, and (c) beach conditions. Cluster linkage distance ( r = 0.82, r = 0.78, r = 0.67) was used as a weight and multiplied by the value of beach descriptive factors. Mazatlán and Oceanside obtained the highest values because there are enough infrastructure and services; on the contrary, Ensenada and Rosarito were rated medium and low because infrastructure and services are lacking. The presently proposed method can contribute to improving current beach evaluations because the final score represents the beach users’ evaluation of the quality of the beach. The weight considered in the present study marks the beach users’ preferences among the studied beaches. Adding this weight to beach evaluation will contribute to more specific beach planning in which users’ perception is considered.

  19. Survival End Points for Huntington Disease Trials Prior to a Motor Diagnosis. (United States)

    Long, Jeffrey D; Mills, James A; Leavitt, Blair R; Durr, Alexandra; Roos, Raymund A; Stout, Julie C; Reilmann, Ralf; Landwehrmeyer, Bernhard; Gregory, Sarah; Scahill, Rachael I; Langbehn, Douglas R; Tabrizi, Sarah J


    Predictive genetic testing in Huntington disease (HD) enables therapeutic trials in HTT gene expansion mutation carriers prior to a motor diagnosis. Progression-free survival (PFS) is the composite of a motor diagnosis or a progression event, whichever comes first. To determine if PFS provides feasible sample sizes for trials with mutation carriers who have not yet received a motor diagnosis. This study uses data from the 2-phase, longitudinal cohort studies called Track and from a longitudinal cohort study called the Cooperative Huntington Observational Research Trial (COHORT). Track had 167 prediagnosis mutation carriers and 156 noncarriers, whereas COHORT had 366 prediagnosis mutation carriers and noncarriers. Track studies were conducted at 4 sites in 4 countries (Canada, France, England, and the Netherlands) from which data were collected from January 17, 2008, through November 17, 2014. The COHORT was conducted at 38 sites in 3 countries (Australia, Canada, and the United States) from which data were collected from February 14, 2006, through December 31, 2009. Results from the Track data were externally validated with data from the COHORT. The required sample size was estimated for a 2-arm prediagnosis clinical trial. Data analysis took place from May 1, 2016, to June 10, 2017. The primary end point is PFS. Huntington disease progression events are defined for the Unified Huntington's Disease Rating Scale total motor score, total functional capacity, symbol digit modalities test, and Stroop word test. Of Track's 167 prediagnosis mutation carriers, 93 (55.6%) were women, and the mean (SD) age was 40.06 (8.92) years; of the 156 noncarriers, 87 (55.7%) were women, and the mean (SD) age was 45.58 (10.30) years. Of the 366 COHORT participants, 229 (62.5%) were women and the mean (SD) age was 42.21 (12.48) years. The PFS curves of the Track mutation carriers showed good external validity with the COHORT mutation carriers after adjusting for initial progression. For

  20. Network topology and functional connectivity disturbances precede the onset of Huntington's disease. (United States)

    Harrington, Deborah L; Rubinov, Mikail; Durgerian, Sally; Mourany, Lyla; Reece, Christine; Koenig, Katherine; Bullmore, Ed; Long, Jeffrey D; Paulsen, Jane S; Rao, Stephen M


    Cognitive, motor and psychiatric changes in prodromal Huntington's disease have nurtured the emergent need for early interventions. Preventive clinical trials for Huntington's disease, however, are limited by a shortage of suitable measures that could serve as surrogate outcomes. Measures of intrinsic functional connectivity from resting-state functional magnetic resonance imaging are of keen interest. Yet recent studies suggest circumscribed abnormalities in resting-state functional magnetic resonance imaging connectivity in prodromal Huntington's disease, despite the spectrum of behavioural changes preceding a manifest diagnosis. The present study used two complementary analytical approaches to examine whole-brain resting-state functional magnetic resonance imaging connectivity in prodromal Huntington's disease. Network topology was studied using graph theory and simple functional connectivity amongst brain regions was explored using the network-based statistic. Participants consisted of gene-negative controls (n = 16) and prodromal Huntington's disease individuals (n = 48) with various stages of disease progression to examine the influence of disease burden on intrinsic connectivity. Graph theory analyses showed that global network interconnectivity approximated a random network topology as proximity to diagnosis neared and this was associated with decreased connectivity amongst highly-connected rich-club network hubs, which integrate processing from diverse brain regions. However, functional segregation within the global network (average clustering) was preserved. Functional segregation was also largely maintained at the local level, except for the notable decrease in the diversity of anterior insula intermodular-interconnections (participation coefficient), irrespective of disease burden. In contrast, network-based statistic analyses revealed patterns of weakened frontostriatal connections and strengthened frontal-posterior connections that evolved as disease

  1. Feeding ecology of the beach silverside Atherinella blackburni (Atherinopsidae in a tropical sandy beach, Southeastern Brazil

    Directory of Open Access Journals (Sweden)

    Júlio Guazzelli Gonzalez

    Full Text Available Abstract The feeding ecology of the beach silverside (Atherinella blackburni in the surf zone of a tropical sand beach, located in the southeastern Brazil, was accessed through the gut content analysis of 198 fish. Factors such as fish's size, season and day period were analysed to understand how these variables affect the diet composition of the species. Results show that A. blackburni is a coastal neritic fish with a broad feeding niche. Most recurrent prey were zooplanktonic crustaceans, insects and benthic molluscs, in which Copepoda crustaceans were the dominant dietary item in occurrence and abundance. A. blackburni appears to have a slight ontogenetic shift in its diet, changing from benthic molluscs to crustaceans and insects along its life cycle. The diel activity also reveals to be an important factor to the A. blackburni feeding ecology. The predominant occurrence of small fish during the morning, along with the main preys for this size class, suggests that small individuals use shallower waters as feeding grounds during the morning and, during the night, they move to deeper waters for protection against predators.

  2. Experimental Study of Irregular Waves on a Gravel Beach (United States)

    Hu, Nai-Ren; Wu, Yun-Ta; Hwung, Hwung-Hweng; Yang, Ray-Yeng


    In the east coast of Taiwan, the sort grain size more belongs to cobble or gravel, which is physically distinct compared to the sandy beach in the west coast of Taiwan. Although gravel beaches can dissipate more of wave energy, gravel beaches were eroded and coastal road were damaged especially during typhoons. The purpose of this study is to investigate the geomorphological response of gravel beach due to irregular waves. This experiment was carry out in a 21m long, 50 cm wide, 70 cm high wave tank at Tainan Hydraulics Laboratory, National Cheng-Kung University, Taiwan. To simulate of the geometry in the east coast of Taiwan, a physical model with 1/36 scale-down was used, in which the seawall was 10cm built upon a 1:10 slope and gravel grains with D50 being 3.87 mm was nourished in front of the seawall. In terms of typhoon-scale wave condition, irregular waves with scale-down conditions were generated for 600 s for each scenarios and, three different water levels with respect to the gravel beach are designed. Application of laser combined with image processing to produce 3D topographic map, the erosion zone and accretion zone would be found. The resulting morphological change of gravel beach will be measured using an integrated laser and image processing tool to have 3D topographic maps. It is expected to have more understanding about under what conditions the gravel coasts suffer the least damage. In particular, the relation between erosion rates of gravel beach, the angle of gravel slope and the length of the plane on the gravel slope will be achieved

  3. Effects of beach morphology and waves on onshore larval transport (United States)

    Fujimura, A.; Reniers, A.; Paris, C. B.; Shanks, A.; MacMahan, J.; Morgan, S.


    Larvae of intertidal species grow offshore, and migrate back to the shore when they are ready to settle on their adult substrates. In order to reach the habitat, they must cross the surf zone, which is characterized as a semi-permeable barrier. This is accomplished through physical forcing (i.e., waves and current) as well as their own behavior. Two possible scenarios of onshore larval transport are proposed: Negatively buoyant larvae stay in the bottom boundary layer because of turbulence-dependent sinking behavior, and are carried toward the shore by streaming of the bottom boundary layer; positively buoyant larvae move to the shore during onshore wind events, and sink to the bottom once they encounter high turbulence (i.e., surf zone edge), where they are carried by the bottom current toward the shore (Fujimura et al. 2014). Our biophysical Lagrangian particle tracking model helps to explain how beach morphology and wave conditions affect larval distribution patterns and abundance. Model results and field observations show that larval abundance in the surf zone is higher at mildly sloped, rip-channeled beaches than at steep pocket beaches. Beach attributes are broken up to examine which and how beach configuration factors affect larval abundance. Modeling with alongshore uniform beaches with variable slopes reveal that larval populations in the surf zone are negatively correlated with beach steepness. Alongshore variability enhances onshore larval transport because of increased cross-shore water exchange by rip currents. Wave groups produce transient rip currents and enhance cross-shore exchange. Effects of other wave components, such as wave height and breaking wave rollers are also considered.

  4. A randomized, double-blind, placebo-controlled study of latrepirdine in patients with mild to moderate Huntington disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth


    BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect of latrepir......BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect...... of latrepirdine on cognition and global function in patients with mild to moderate Huntington disease. DESIGN Randomized, double-blind, placebo-controlled study. SETTING Sixty-four research centers in Australia, Europe, and North America. PATIENTS Four hundred three patients with mild to moderate Huntington...... between those randomized to latrepirdine (68.5%) and placebo (68.0%). CONCLUSION In patients with mild to moderate Huntington disease and cognitive impairment, treatment with latrepirdine for 6 months was safe and well tolerated but did not improve cognition or global function relative to placebo. TRIAL...

  5. Swashed away? Storm impacts on sandy beach macrofaunal communities (United States)

    Harris, Linda; Nel, Ronel; Smale, Malcolm; Schoeman, David


    Storms can have a large impact on sandy shores, with powerful waves eroding large volumes of sand off the beach. Resulting damage to the physical environment has been well-studied but the ecological implications of these natural phenomena are less known. Since climate change predictions suggest an increase in storminess in the near future, understanding these ecological implications is vital if sandy shores are to be proactively managed for resilience. Here, we report on an opportunistic experiment that tests the a priori expectation that storms impact beach macrofaunal communities by modifying natural patterns of beach morphodynamics. Two sites at Sardinia Bay, South Africa, were sampled for macrofauna and physical descriptors following standard sampling methods. This sampling took place five times at three- to four-month intervals between April 2008 and August 2009. The second and last sampling events were undertaken after unusually large storms, the first of which was sufficiently large to transform one site from a sandy beach into a mixed shore for the first time in living memory. A range of univariate (linear mixed-effects models) and multivariate (e.g. non-metric multidimensional scaling, PERMANOVA) methods were employed to describe trends in the time series, and to explore the likelihood of possible explanatory mechanisms. Macrofaunal communities at the dune-backed beach (Site 2) withstood the effects of the first storm but were altered significantly by the second storm. In contrast, macrofauna communities at Site 1, where the supralittoral had been anthropogenically modified so that exchange of sediments with the beach was limited, were strongly affected by the first storm and showed little recovery over the study period. In line with predictions from ecological theory, beach morphodynamics was found to be a strong driver of temporal patterns in the macrofaunal community structure, with the storm events also identified as a significant factor, likely

  6. Behavioural adaptations in talitrids from two Atlantic beaches (United States)

    Rossano, Claudia; Gambineri, Simone; Fanini, Lucia; Durier, Virginie; Rivault, Colette; Scapini, Felicita


    The aim of the present study was to test sun orientation and rhythmic activity of two sandhopper populations from two Atlantic macro-tidal beaches. A population from Le Verger beach (orientated to 346°, Ille et Vilaine, Brittany, France) and a population from Damgan (orientated to 195°, Morbihan, Brittany, France), were tested on the beach under clear sky discriminating for landscape vision. For both populations locomotor activity rhythm was recorded in the laboratory. The two beaches differed for climatic features, tidal range and for human use. Both talitrid populations resulted very well orientated toward the shoreline, and both used solar position and landscape vision to orient. However the multiple regression analysis of orientation with climatic features showed a different use of local cues by the two populations and a slight influence of tidal regime (ebbing and rising tide), in spite of the supralittoral zonation of sandhoppers. In the laboratory they showed a well defined rhythmic behaviour as well as a bimodal rhythmicity, explained as a tidal one. These results are a new brick in the complex picture of orientation and rhythm studies on sandy beach invertebrates.

  7. Evaluation of beach cleanup effects using linear system analysis. (United States)

    Kataoka, Tomoya; Hinata, Hirofumi


    We established a method for evaluating beach cleanup effects (BCEs) based on a linear system analysis, and investigated factors determining BCEs. Here we focus on two BCEs: decreasing the total mass of toxic metals that could leach into a beach from marine plastics and preventing the fragmentation of marine plastics on the beach. Both BCEs depend strongly on the average residence time of marine plastics on the beach (τ(r)) and the period of temporal variability of the input flux of marine plastics (T). Cleanups on the beach where τ(r) is longer than T are more effective than those where τ(r) is shorter than T. In addition, both BCEs are the highest near the time when the remnants of plastics reach the local maximum (peak time). Therefore, it is crucial to understand the following three factors for effective cleanups: the average residence time, the plastic input period and the peak time. Copyright © 2014 Elsevier Ltd. All rights reserved.

  8. Prevalence and correlates of lifetime disordered gambling in Cambodian refugees residing in Long Beach, CA. (United States)

    Marshall, Grant N; Elliott, Marc N; Schell, Terry L


    Research has suggested that disordered gambling is endemic to Cambodian refugees. Whereas earlier study of the prevalence and correlates of disordered gambling has relied on convenience sampling, this investigation used a subset of a sample representative of the largest Cambodian refugee community in the US. Face-to-face interviews assessing gambling disorder were conducted with a subsample of persons (N = 127) participating in a broader study of the mental health of this community. 13.9% of participants met screening criteria for lifetime disordered gambling, in contrast to previous research suggesting that prevalence rates may exceed 70%. After adjusting for a range of covariates, breadth of trauma exposure and marital status emerged as significant predictors of disordered gambling. Given the myriad mental health challenges facing the Cambodian refugee community, these data indicate that scarce prevention and treatment resources may be more productively channeled toward addressing other mental health and social service needs.

  9. H12618: NOS Hydrographic Survey , Approaches to LA Long Beach, CA, 2013-11-04 (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The National Oceanic and Atmospheric Administration (NOAA) has the statutory mandate to collect hydrographic data in support of nautical chart compilation for safe...

  10. 76 FR 16297 - Drawbridge Operation Regulation; Cerritos Channel, Long Beach, CA (United States)


    ... Federal holidays. FOR FURTHER INFORMATION CONTACT: If you have questions on this rule, call or e-mail... [email protected] . If you have questions on viewing the docket, call Renee V. Wright, Program...

  11. H12619: NOS Hydrographic Survey , Approaches to LA Long Beach, CA, 2013-11-03 (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The National Oceanic and Atmospheric Administration (NOAA) has the statutory mandate to collect hydrographic data in support of nautical chart compilation for safe...

  12. Establish nesting beach origins for all turtles in CA via genetic techniques (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — To examine the stock origin and evaluate current life-history hypotheses of green turtles foraging in southern California, 780 bp of the mitochondrial (mtDNA)...

  13. 78 FR 68858 - Seal Beach National Wildlife Refuge, Orange County, CA; Final Comprehensive Conservation Plan and... (United States)


    ... provide refuge managers with a 15-year plan for achieving refuge purposes and contributing toward the... photography, environmental education and interpretation. We intend to review and update the CCP at least every... for new research projects that would benefit Refuge resources and Refuge management. Alternative C...

  14. H12617: NOS Hydrographic Survey , Approaches to LA Long Beach, CA, 2013-11-03 (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The National Oceanic and Atmospheric Administration (NOAA) has the statutory mandate to collect hydrographic data in support of nautical chart compilation for safe...

  15. Response of intertidal sandy-beach macrofauna to human trampling: An urban vs. natural beach system approach. (United States)

    Reyes-Martínez, Ma José; Ruíz-Delgado, Ma Carmen; Sánchez-Moyano, Juan Emilio; García-García, Francisco José


    Sandy beaches are subjected to intense stressors, which are mainly derived from the increasing pattern of beach urbanization. These ecosystems are also a magnet for tourists, who prefer these locations as leisure and holiday destinations, and such activity further increases the factors that have an adverse effect on beaches. In the study reported here the effect of human trampling on macrofauna assemblages that inhabit intertidal areas of sandy beaches was assessed using a BACI design. For this purpose, three contrasting sectors of the same beach were investigated: an urban area with a high level of visitors, a protected sector with a low density of users, and a transitional area with a high level of human occupancy. The physical variables were constant over time in each sector, whereas differences were found in the intensity of human use between sectors. Density variations and changes in the taxonomic structure of the macrofauna with time were shown by PERMANOVA analysis in the urban and transitional locations whereas the protected sector remained constant throughout the study period. The amphipod Bathyporeia pelagica appears sensitive to human trampling pressure and the use of this species as a bioindicator for these types of impact is recommended. Copyright © 2014 Elsevier Ltd. All rights reserved.

  16. A method for determining average beach slope and beach slope variability for U.S. sandy coastlines (United States)

    Doran, Kara S.; Long, Joseph W.; Overbeck, Jacquelyn R.


    The U.S. Geological Survey (USGS) National Assessment of Hurricane-Induced Coastal Erosion Hazards compares measurements of beach morphology with storm-induced total water levels to produce forecasts of coastal change for storms impacting the Gulf of Mexico and Atlantic coastlines of the United States. The wave-induced water level component (wave setup and swash) is estimated by using modeled offshore wave height and period and measured beach slope (from dune toe to shoreline) through the empirical parameterization of Stockdon and others (2006). Spatial and temporal variability in beach slope leads to corresponding variability in predicted wave setup and swash. For instance, seasonal and storm-induced changes in beach slope can lead to differences on the order of 1 meter (m) in wave-induced water level elevation, making accurate specification of this parameter and its associated uncertainty essential to skillful forecasts of coastal change. A method for calculating spatially and temporally averaged beach slopes is presented here along with a method for determining total uncertainty for each 200-m alongshore section of coastline.

  17. Use of unmanned aerial vehicles for efficient beach litter monitoring

    KAUST Repository

    Martin, Cecilia


    A global beach litter assessment is challenged by use of low-efficiency methodologies and incomparable protocols that impede data integration and acquisition at a national scale. The implementation of an objective, reproducible and efficient approach is therefore required. Here we show the application of a remote sensing based methodology using a test beach located on the Saudi Arabian Red Sea coastline. Litter was recorded via image acquisition from an Unmanned Aerial Vehicle, while an automatic processing of the high volume of imagery was developed through machine learning, employed for debris detection and classification in three categories. Application of the method resulted in an almost 40 times faster beach coverage when compared to a standard visual-census approach. While the machine learning tool faced some challenges in correctly detecting objects of interest, first classification results are promising and motivate efforts to further develop the technique and implement it at much larger scales.

  18. Sediment Transport Study in Haeundae Beach using Radioisotope Labelled Compound

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jin Seop; Kim, Jong Bum; Jung, Sung Hee [Korea Atomic Energy Research Institute, Taejon (Korea, Republic of); Lee, Jong Sup [Pukyong National Univ., Busan (Korea, Republic of)


    Haeundae beach is one of the most famous resorts in Korea and plays an important role as a special tourism district. However, the length and width of the beach are being reduced continuously, which would have bad influence on the regional economy and be the financial burden to the local authority considering that a large amount of budget is spent in the beach nourishment annually. Hence, it is necessary to understand the dynamic behavior of sediments in the coast for the systematic preservation plan of coastal environment. Lately a monitoring system using radioactive isotope as tracers is considered as a novel technique in understanding the dynamic transport of sediments. The objective of this study is to investigate the possible variations in sedimentary distribution and quantify the characteristics of sediments using radiotracer.

  19. Sediment Transport Study in Haeundae Beach using Radioisotope Labelled Compound

    International Nuclear Information System (INIS)

    Kim, Jin Seop; Kim, Jong Bum; Jung, Sung Hee; Lee, Jong Sup


    Haeundae beach is one of the most famous resorts in Korea and plays an important role as a special tourism district. However, the length and width of the beach are being reduced continuously, which would have bad influence on the regional economy and be the financial burden to the local authority considering that a large amount of budget is spent in the beach nourishment annually. Hence, it is necessary to understand the dynamic behavior of sediments in the coast for the systematic preservation plan of coastal environment. Lately a monitoring system using radioactive isotope as tracers is considered as a novel technique in understanding the dynamic transport of sediments. The objective of this study is to investigate the possible variations in sedimentary distribution and quantify the characteristics of sediments using radiotracer

  20. Plastic pollution on the Baltic beaches of Kaliningrad region, Russia. (United States)

    Esiukova, Elena


    Contamination of sandy beaches of the Baltic Sea in Kaliningrad region is evaluated on the base of surveys carried out from June 2015 to January 2016. Quantity of macro/meso/microplastic objects in the upper 2cm of the sandy sediments of the wrack zone at 13 sampling sites all along the Russian coast is reported. Occurrence of paraffin and amber pieces at the same sites is pointed out. Special attention is paid to microplastics (range 0.5-5mm): its content ranges between 1.3 and 36.3 items per kg dry sediment. The prevailing found type is foamed plastic. No sound differences in contamination are discovered between beaches with high and low anthropogenic load. Mean level of contamination is of the same order of magnitude as has been reported by other authors for the Baltic Sea beaches. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Use of unmanned aerial vehicles for efficient beach litter monitoring

    KAUST Repository

    Martin, Cecilia; Parkes, Stephen; Zhang, Qiannan; Zhang, Xiangliang; McCabe, Matthew; Duarte, Carlos M.


    A global beach litter assessment is challenged by use of low-efficiency methodologies and incomparable protocols that impede data integration and acquisition at a national scale. The implementation of an objective, reproducible and efficient approach is therefore required. Here we show the application of a remote sensing based methodology using a test beach located on the Saudi Arabian Red Sea coastline. Litter was recorded via image acquisition from an Unmanned Aerial Vehicle, while an automatic processing of the high volume of imagery was developed through machine learning, employed for debris detection and classification in three categories. Application of the method resulted in an almost 40 times faster beach coverage when compared to a standard visual-census approach. While the machine learning tool faced some challenges in correctly detecting objects of interest, first classification results are promising and motivate efforts to further develop the technique and implement it at much larger scales.

  2. Textural studies of beach sediments from Sadashivagad and Karwar, Central west coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Mislankar, P.G.; Antao, F.B.

    . Frequency distribution curves for both the beach sediments differ in their modal class showing unimodal to weakly bimodal trend for the Sadashivagad and strongly bimodal to unimodal trend for the Karwar beach sediments. The plots of mean grain size vs...

  3. Wave energy level and geographic setting correlate with Florida beach water quality. (United States)

    Feng, Zhixuan; Reniers, Ad; Haus, Brian K; Solo-Gabriele, Helena M; Kelly, Elizabeth A


    Many recreational beaches suffer from elevated levels of microorganisms, resulting in beach advisories and closures due to lack of compliance with Environmental Protection Agency guidelines. We conducted the first statewide beach water quality assessment by analyzing decadal records of fecal indicator bacteria (enterococci and fecal coliform) levels at 262 Florida beaches. The objectives were to depict synoptic patterns of beach water quality exceedance along the entire Florida shoreline and to evaluate their relationships with wave condition and geographic location. Percent exceedances based on enterococci and fecal coliform were negatively correlated with both long-term mean wave energy and beach slope. Also, Gulf of Mexico beaches exceeded the thresholds significantly more than Atlantic Ocean ones, perhaps partially due to the lower wave energy. A possible linkage between wave energy level and water quality is beach sand, a pervasive nonpoint source that tends to harbor more bacteria in the low-wave-energy environment. Copyright © 2016 Elsevier Ltd. All rights reserved.

  4. Observations on the ecology of some sandy beaches of the southwest coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Panikkar, B.M.; Rajan, S.

    The seasonal cycles of organic matter and chlorophyll at some beaches of the Kerala Coast were studied in relation to the abundance of the interstitial fauna at one of the beaches The faunal abundance showed no definite correlation either...

  5. Valuing Coastal Beaches and Closures Using Benefit Transfer: An Application to Barnstable, Massachusetts (United States)

    Each year, millions of Americans visit beaches for recreation and contribute significantly to local coastal economies as a result. Considering the frequency that coastal beaches are used for recreation, closures due to bacterial contamination have the potential to negatively impa...

  6. Short-term observation of beach dynamics using cross-shore profiles and foreshore sediment

    Digital Repository Service at National Institute of Oceanography (India)

    Dora, G.U.; SanilKumar, V.; Johnson, G.; Philip, C.S.; Vinayaraj, P.

    Cross-shore beach profiles and textural characteristics of foreshore sediment were analyzed for understanding an annual cycle of intertidal beach dynamics at Devbag, an Island sheltered estuarine coast. Cross-shore transects were monitored in a...

  7. A simple model to estimate the impact of sea-level rise on platform beaches (United States)

    Taborda, Rui; Ribeiro, Mónica Afonso


    Estimates of future beach evolution in response to sea-level rise are needed to assess coastal vulnerability. A research gap is identified in providing adequate predictive methods to use for platform beaches. This work describes a simple model to evaluate the effects of sea-level rise on platform beaches that relies on the conservation of beach sand volume and assumes an invariant beach profile shape. In closed systems, when compared with the Inundation Model, results show larger retreats; the differences are higher for beaches with wide berms and when the shore platform develops at shallow depths. The application of the proposed model to Cascais (Portugal) beaches, using 21st century sea-level rise scenarios, shows that there will be a significant reduction in beach width.

  8. Dose rates of beach sands along the Enshu-nada coast

    International Nuclear Information System (INIS)

    Minato, Susumu; Shimo, Michikuni


    In order to understand the mechanism of beach-to-beach variations in dose rates, measurements at 35 sand beaches were carried out along the Enshu-nada coast (a total of 140km). The sand samples were collected at 10 beaches to obtain the concentrations of potassium, uranium and thorium by means of Ge(Li) spectroscopy. Factors affecting the variation in dose rates were discussed, incorporating these data with data of coastal sea-bottom sediments taken by the Geological Survey of Japan. Two-dimensional measurements were performed at 50 locations each in two beaches near the Tenryu river mouth to know within-beach variations in detail. Contour maps of the data revealed a considerable regularity. It was found from simple analyses of the data collected in this work that the dose rate levels at beaches are affected by the distance from the river mouth, erosion or sedimentation of beach, and depth distribution of seawater. (author)

  9. 77 FR 51475 - Safety Zone; Apache Pier Labor Day Fireworks; Myrtle Beach, SC (United States)


    ...-AA00 Safety Zone; Apache Pier Labor Day Fireworks; Myrtle Beach, SC AGENCY: Coast Guard, DHS. ACTION... Atlantic Ocean in the vicinity of Apache Pier in Myrtle Beach, SC, during the Labor Day fireworks... [[Page 51476

  10. Studies on nearshore processes at Yarada beach (South of Visakhapatnam harbour) east coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Rao, T.V.N.; Rao, D.P.; Rao, B.P.

    Influence of breakwaters on Yarada Beach, Andhra Pradesh, India (3.5 km length) stability and distribution on wave induced longshore currents in this region were studied. Monthly observations on variation in beach levels, distribution of wave...

  11. Land use and beach closure 2004-2013 in the United States (United States)

    U.S. Environmental Protection Agency — The dataset contains the beach closure data and land use information around each beach in 2006 and 2011 in the United States. The original data are created by EPA...

  12. Wave Runup on a Frozen Beach Under High Energy Conditions (United States)

    Didier, D.; Bernatchez, P.; Dumont, D.; Corriveau, M.


    High and mid-latitude beaches have typical morphological characteristics influenced by nearshore processes prevailing under ice conditions during cold season. Nearshore ice complexes (NIC) offer a natural coastal protection by covering beach sediments, while offshore ice-infested waters dissipate incoming waves. Climate change contributes to sea ice shrinking therefore reducing its protection against erosion and flooding. In the Estuary and Gulf of the St. Lawrence (ESL, GSL) (eastern Canada), sea ice cover undergoes an overall shrinking and simulated future projections tend toward a negligible effect on wave climate by 2100. Quantifying the effect of nearshore dynamics on frozen beaches is therefore imperative for coastal management as more wave energy at the coast is expected in the future. To measure the effect of a frozen beach on wave runup elevations, this study employs a continuous video recording of the swash motion at 4Hz. Video-derived wave runup statistics have been extracted during a tidal cycle on a frozen beach, using the Pointe-Lebel beach (ESL) as a test case. Timestack analysis was combined with offshore water levels and wave measurements. A comparison of runup under icy conditions (Dec. 30 2016) with a runup distribution during summer was made under similar high energy wave conditions. Results indicate high runup excursions potentially caused by lowered sediment permeability due to high pore-ice saturation in the swash zone, accentuating the overwash of the eroding coastline and thus the risk of flooding. With projected reduction in coastal sea ice cover and thus higher wave energy, this study suggests that episodes of degradation and weakening could influence the coastal flood risk in mid- and high-latitude cold environments.

  13. Sports injuries and illnesses during the second Asian Beach Games. (United States)

    Al-Shaqsi, Sultan; Al-Kashmiri, Ammar; Al-Risi, Ahmed; Al-Mawali, Suleiman


    Prevention of sport injuries and illnesses is a focus for epidemiological surveillance. To record and analyse all sports injuries and illnesses registered during the second Asian Beach Games. A descriptive epidemiological study using the International Olympic Committee Surveillance system to register injuries and illnesses during the second Asian Beach Games. The second Asian Beach Games hosted 1132 athletes from 43 countries competing in 14 beach sports. All National Olympic Committees' physicians of the participating teams were invited to report all injuries and illnesses. In addition, medical officers at the different Olympic venues and the main Olympic village reported injuries and illnesses treated at the clinics on a daily basis. A total of 177 injuries were reported equating to an incidence rate of 156.4 per 1000 registered athletes. Tent pegging recorded the highest incidence of injuries with 357 per 1000 registered athletes. The most prevalent injuries were in the foot/toe with 14.1% of all reported injuries. The majority of injuries were incurred during competition (75.4%). In addition, the most common mechanism of injury was contact with another athlete (n=42, 23.7%) and combined sudden and gradual overuse contributed to 30% of the total injury burden. Furthermore, 118 illnesses were reported resulting in an incidence rate of 104.2 illnesses per 1000 registered athletes. The most affected system was the respiratory tract (39.1%) with infection being the most common cause (n=33, 38.0%). The incidence of injury and illness differed significantly among the 14 sports. The data indicate that the risk of injury from beach games is sport dependant. This means that any preventive measures have to be tailored for each discipline. Furthermore, the study showed that respiratory infections are the commonest illness in beach sports and therefore, event organisers should focus improving public health measures and hygiene awareness.

  14. Predaceous ants, beach replenishment, and nest placement by sea turtles. (United States)

    Wetterer, James K; Wood, Lawrence D; Johnson, Chris; Krahe, Holly; Fitchett, Stephanie


    Ants known for attacking and killing hatchling birds and reptiles include the red imported fire ant (Solenopsis invicta Buren), tropical fire ant [Solenopsis geminata (Fabr.)], and little fire ant [Wasmannia auropunctata (Roger)]. We tested whether sea turtle nest placement influenced exposure to predaceous ants. In 2000 and 2001, we surveyed ants along a Florida beach where green turtles (Chelonia mydas L.), leatherbacks (Dermochelys coriacea Vandelli), and loggerheads (Caretta caretta L.) nest. Part of the beach was artificially replenished between our two surveys. As a result, mean beach width experienced by nesting turtles differed greatly between the two nesting seasons. We surveyed 1,548 sea turtle nests (2000: 909 nests; 2001: 639 nests) and found 22 ant species. S. invicta was by far the most common species (on 431 nests); S. geminata and W. auropunctata were uncommon (on 3 and 16 nests, respectively). In 2000, 62.5% of nests had ants present (35.9% with S. invicta), but in 2001, only 30.5% of the nests had ants present (16.4% with S. invicta). Turtle nests closer to dune vegetation had significantly greater exposure to ants. Differences in ant presence on turtle nests between years and among turtle species were closely related to differences in nest placement relative to dune vegetation. Beach replenishment significantly lowered exposure of nests to ants because on the wider beaches turtles nested farther from the dune vegetation. Selective pressures on nesting sea turtles are altered both by the presence of predaceous ants and the practice of beach replenishment.

  15. The Virginia Beach shallow ground-water study (United States)

    Johnson, Henry M.


    IntroductionVirginia Beach is a rapidly growing city of more than 425,000 people. Sources of fresh water within the city, however, are limited. Prior to 1998, the Virginia Beach Public Utilities Department met the city's water needs by purchasing treated drinking water from the City of Norfolk. Because Norfolk had to meet its own requirements, the amount of water available to Virginia Beach was limited to about 30 million gallons per day (mgd) and even less during droughts. This water supply was supplemented with ground water from city-owned, community, and private wells. In many parts of the city, however, ground water cannot be used because of high concentrations of chloride, iron, and (or) sulfur, which give the water an unpleasant taste.In early 1998, a pipeline came on-line that can carry up to 45 mgd of water from Lake Gaston to Virginia Beach. The Gaston pipeline has alleviated concerns about water supply and quality for most residents living north of the "Green Line." These residents primarily use ground water only for small-scale domestic activities such as watering lawns, filling ponds and pools, and washing cars. City water and sewer services have been extended beyond the Green Line into the "Transition Area." Residents and businesses south of the Transition Area, however, continue to rely on ground water to meet most of their needs for potable and non-potable water. To help assure a continued, reliable supply of ground water, the U.S. Geological Survey (USGS), in cooperation with the City of Virginia Beach Public Utilities Department, has begun an assessment of the shallow ground-water resources underlying the City of Virginia Beach.

  16. Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study (United States)

    Piano, Carla; Losurdo, Anna; Della Marca, Giacomo; Solito, Marcella; Calandra-Buonaura, Giovanna; Provini, Federica; Bentivoglio, Anna Rita; Cortelli, Pietro


    Study Objectives: To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD). Design: Cross-sectional cohort study. Setting: Sleep laboratory. Patients: Thirty HD patients, 16 women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y). Interventions: Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale [UHDMRS]), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG). Measurements and Results: The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m2. No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P Marca G, Solito M, Calandra-Buonaura G, Provini F, Bentivoglio AR, Cortelli P. Polysomnographic findings and clinical correlates in Huntington disease: a cross-sectional cohort study. SLEEP 2015;38(9):1489–1495. PMID:25845698

  17. Nonparametric estimation for censored mixture data with application to the Cooperative Huntington's Observational Research Trial. (United States)

    Wang, Yuanjia; Garcia, Tanya P; Ma, Yanyuan


    This work presents methods for estimating genotype-specific distributions from genetic epidemiology studies where the event times are subject to right censoring, the genotypes are not directly observed, and the data arise from a mixture of scientifically meaningful subpopulations. Examples of such studies include kin-cohort studies and quantitative trait locus (QTL) studies. Current methods for analyzing censored mixture data include two types of nonparametric maximum likelihood estimators (NPMLEs) which do not make parametric assumptions on the genotype-specific density functions. Although both NPMLEs are commonly used, we show that one is inefficient and the other inconsistent. To overcome these deficiencies, we propose three classes of consistent nonparametric estimators which do not assume parametric density models and are easy to implement. They are based on the inverse probability weighting (IPW), augmented IPW (AIPW), and nonparametric imputation (IMP). The AIPW achieves the efficiency bound without additional modeling assumptions. Extensive simulation experiments demonstrate satisfactory performance of these estimators even when the data are heavily censored. We apply these estimators to the Cooperative Huntington's Observational Research Trial (COHORT), and provide age-specific estimates of the effect of mutation in the Huntington gene on mortality using a sample of family members. The close approximation of the estimated non-carrier survival rates to that of the U.S. population indicates small ascertainment bias in the COHORT family sample. Our analyses underscore an elevated risk of death in Huntington gene mutation carriers compared to non-carriers for a wide age range, and suggest that the mutation equally affects survival rates in both genders. The estimated survival rates are useful in genetic counseling for providing guidelines on interpreting the risk of death associated with a positive genetic testing, and in facilitating future subjects at risk

  18. Current status of PET imaging in Huntington's disease

    International Nuclear Information System (INIS)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios


    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading ''Huntington Disease'' combined with text and key words ''Huntington Disease'', ''Neuroimaging'' and ''PET''. Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([ 18 F]FDG and [ 15 O]H 2 O), presynaptic ([ 18 F]fluorodopa, [ 11 C]β-CIT and [ 11 C]DTBZ) and postsynaptic ([ 11 C]SCH22390, [ 11 C]FLB457 and [ 11 C]raclopride) dopaminergic function, phosphodiesterases ([ 18 F]JNJ42259152, [ 18 F]MNI-659 and [ 11 C]IMA107), and adenosine ([ 18 F]CPFPX), cannabinoid ([ 18 F]MK-9470), opioid ([ 11 C]diprenorphine) and GABA ([ 11 C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed for monitoring potential neuroprotective and preventive treatment

  19. Short G-rich oligonucleotides as a potential therapeutic for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Parekh-Olmedo Hetal


    Full Text Available Abstract Background Huntington's Disease (HD is an inherited autosomal dominant genetic disorder in which neuronal tissue degenerates. The pathogenesis of the disease appears to center on the development of protein aggregates that arise initially from the misfolding of the mutant HD protein. Mutant huntingtin (Htt is produced by HD genes that contain an increased number of glutamine codons within the first exon and this expansion leads to the production of a protein that misfolds. Recent studies suggest that mutant Htt can nucleate protein aggregation and interfere with a multitude of normal cellular functions. Results As such, efforts to find a therapy for HD have focused on agents that disrupt or block the mutant Htt aggregation pathway. Here, we report that short guanosine monotonic oligonucleotides capable of adopting a G-quartet structure, are effective inhibitors of aggregation. By utilizing a biochemical/immunoblotting assay as an initial screen, we identified a 20-mer, all G-oligonucleotide (HDG as an active molecule. Subsequent testing in a cell-based assay revealed that HDG was an effective inhibitor of aggregation of a fusion protein, comprised of a mutant Htt fragment and green fluorescent protein (eGFP. Taken together, our results suggest that a monotonic G-oligonucleotide, capable of adopting a G-quartet conformation is an effective inhibitor of aggregation. This oligonucleotide can also enable cell survival in PC12 cells overexpressing a mutant Htt fragment fusion gene. Conclusion Single-stranded DNA oligonucleotides capable of forming stable G-quartets can inhibit aggregation of the mutant Htt fragment protein. This activity maybe an important part of the pathogenecity of Huntington's Disease. Our results reveal a new class of agents that could be developed as a therapeutic approach for Huntington's Disease.

  20. Morphosedimentary evolution of carbonate sandy beaches at decadal scale : case study in Reunion Island , Indian Ocean (United States)

    Mahabot, Marie-Myriam; Pennober, Gwenaelle; Suanez, Serge; Troadec, Roland; Delacourt, Christophe


    Global change introduce a lot of uncertainties concerning future trajectory of beaches by directly or indirectly modifying major driving factors. An improved understanding of the past shoreline evolution may help for anticipate future coastline response. However, in tropical environment, studies concerning carbonate beaches dynamics are scarce compared to open sandy beaches. Consequently, coral reef protected beaches morphological adjustment is still poorly understood and long-term evolution rate are poorly quantified in these specific environment. In this context, La Reunion Island, insular department of France located in Indian Ocean, constitute a favoured laboratory. This high volcanic island possesses 25 km of carbonate beaches which experience hydrodynamic forcing specific from tropical environment: cyclonic swell during summer and long period swell during winter. Because of degraded coral reef health and high anthropogenic pressure, 50% of the beaches are in erosion since 1970s. Beach survey has been conducted since 1990s by scientist and are now encompassed as pilot site within a French observatory network which guarantee long-term survey with high resolution observational techniques. Thus, La Reunion Island is one of the rare carbonate beach to be surveyed since 20 years. This study aims to examined and quantify beach response at decadal scale on carbonate sandy beaches of Reunion Island. The study focus on 12 km of beaches from Cap Champagne to the Passe de Trois-Bassins. The analyze of 15 beach profile data originated from historical and DGPS beach topographic data confirm long term trend to erosion. Sediment lost varies between 0.5 and 2 m3.yr-1 since 1998. However longshore current have led to accretion of some part of beach compartment with rate of 0.7 to 1.6 m3.yr-1. Wave climate was examined from in-situ measurement over 15 years and show that extreme waves associated with tropical cyclones and long period swell play a major role in beach dynamics

  1. The sandy beach meiofauna and free-living nematodes from De Panne (Belgium)


    Gheskiere, T.; Hoste, E.; Kotwicki, L.; Degraer, S.; Vanaverbeke, J.; Vincx, M.


    Despite their rather barren and arid appearance, European sandy beaches harbour a highly diverse fauna and flora and some of them are even highly productive. In contrast to tropical sandy beaches little is known about the structural and functional diversity of the different benthic components. This study aims to investigate the structural diversity of the meiobenthos, emphasizing on free-living marine nematodes on a Belgian sandy beach.The samples were collected on the sandy beach of De Panne...

  2. Temporal shift of sea turtle nest sites in an eroding barrier island beach (United States)

    Fujisaki, Ikuko; Lamont, Margaret M.; Carthy, Raymond R.


    Shoreline changes affect functionality of a sandy beach as a wildlife habitat and coastal erosion is among the primary causes of the changes. We examined temporal shifts in locations where loggerheads placed nests in relation to coastal erosion along a barrier island beach in the northern Gulf of Mexico. We first confirmed consistency in long-term (1855–2001), short-term (1976–2001), and more recent (2002–2012) shoreline change rates in two adjacent beach sections, one historically eroding (west beach) and the other accreting (east beach). The mean annual shoreline change rate in the two sections was significantly different in all time periods. The recent (1998–2012) mean change rate was −10.9 ± 9.9 m/year in the west beach and −2.8 ± 4.9 m/year in the east beach, which resulted in the loss of about 70% and 30% of area in the west and east beaches, respectively. Loggerheads nested significantly closer to the vegetation line in 2012 than in 2002 in the west beach but the difference between the two time periods was not significant in the east beach. However, the distance from nests to the vegetation line from 2002 to 2014 was significantly reduced annually in both beaches; on average, loggerheads nested closer to the vegetation line by 9 m/year in the west beach and 5.8 m/year in the east beach. The observed shoreline change rate and corresponding shift of nest placement sites, combined with the forecasted future beach loss, highlighted the importance of addressing the issue of beach erosion to conserve sandy beach habitats.

  3. Plastic Pollution at a Sea Turtle Conservation Area in NE Brazil: Contrasting Developed and Undeveloped Beaches.




    Sea turtles are highly susceptible to plastic ingestion and entanglement. Beach debris were surveyed along the most important sea turtle nesting beaches in Brazil (Costa dos Coqueiros, Bahia State). No significant differences among developed and undeveloped beaches were observed in terms of total number of items. Local sources (tourism activities) represented 70% of debris on developed beaches, where cigarette butts, straws, paper fragments, soft plastic fragments, and food packaging...

  4. Observation on foreshore morphodynamics of microtidal sandy beaches

    Digital Repository Service at National Institute of Oceanography (India)

    Dora, G.U.; SanilKumar, V.; Philip, C.S.; Johnson, G.

    river mouth runs for ~6.2 km along the shore- line. Both sites are well-known fishing zones along west coast of India and also popular tourist places. However, there was no field data available on the beach morphody- namics covering different annual... Tables, Indian and selected Foreign Ports. Survey of India, Government of India, New Delhi, 2008–2011, p. 238. 10. Beach Morphology Analysis Package, Coastal Engineering Tech- nical Note, CETN-11/34, US Army Engineer Waterways Experi- ment Station...

  5. A Parametric Model for Barred Equilibrium Beach Profiles (United States)


    to shallow water. Bodge (1992) and Komar and McDougal (1994) suggested an exponential form as a preferred solution that exhibited finite slope at the...performance measures were computed from the shoreline to hsea, the active volume of sand bar variability. 3.1. Duck, NC, example Tests were carried out...applications. J. Coast. Res. 7, 53–84. Komar, P.D., McDougal ,W.G., 1994. The analysis of beach profiles and nearshore processes using the exponential beach

  6. Psychiatric and cognitive symptoms in Huntington's disease are modified by polymorphisms in catecholamine regulating enzyme genes

    DEFF Research Database (Denmark)

    Vinther-Jensen, T; Nielsen, Troels Tolstrup; Budtz-Jørgensen, E


    Huntington's disease (HD) is an autosomal dominantly inherited neurodegenerative disorder characterized by motor, psychiatric, and cognitive manifestations. HD is caused by a CAG repeat expansion in the Huntingtin (HTT) gene but the exact pathogenesis remains unknown. Dopamine imbalance has......-described cohort of Danish HD gene-expansion carriers. We show that cognitive impairment and psychiatric symptoms in HD are modified by polymorphisms in the monoamine oxidase A (MAOA) and catechol-O-methyltransferase (COMT) genes and by the 4p16.3 B haplotype. These results support the theory of dopamine imbalance...

  7. Derivation of Huntington Disease affected Genea046 human embryonic stem cell line

    Directory of Open Access Journals (Sweden)

    Biljana Dumevska


    Full Text Available The Genea046 human embryonic stem cell line was derived from a donated, fully commercially consented ART blastocyst, carrying HTT gene CAG expansion of 45 repeats, indicative of Huntington Disease. Following ICM outgrowth on inactivated human feeders, karyotype was confirmed as 46, XX by CGH and STR analysis demonstrated a female Allele pattern. The hESC line had pluripotent cell morphology, 85% of cells expressed Nanog, 92% Oct4, 75% Tra1–60 and 99% SSEA4 and demonstrated Alkaline Phosphatase activity. The cell line was negative for Mycoplasma and visible contamination.

  8. El papel de la terapia ocupacional en la enfermedad de Huntington


    Hernández Lozano, David; Fernández Hawrylak, María; Grau Rubio, Claudia


    se analiza el papel del terapeuta ocupacional en el trabajo con las personas afectadas por la enfermedad de Huntington. se realizó una investigación por objetivos a través del diseño y puesta en práctica de un programa de intervención encaminado a mitigar el deterioro producido por la enfermedad mediante el entrenamiento en actividades de la vida diaria y de estimulación cognitiva, y a desarrollar competencias en la familia. la investigación se desarrolló en la Asociación de corea de Huntingt...

  9. Assessing and Modulating Kynurenine Pathway Dynamics in Huntington's Disease: Focus on Kynurenine 3-Monooxygenase. (United States)

    Sathyasaikumar, Korrapati V; Breda, Carlo; Schwarcz, Robert; Giorgini, Flaviano


    The link between disturbances in kynurenine pathway (KP) metabolism and Huntington's disease (HD) pathogenesis has been explored for a number of years. Several novel genetic and pharmacological tools have recently been developed to modulate key regulatory steps in the KP such as the reaction catalyzed by the enzyme kynurenine 3-monooxygenase (KMO). This insight has offered new options for exploring the mechanistic link between this metabolic pathway and HD, and provided novel opportunities for the development of candidate drug-like compounds. Here, we present an overview of the field, focusing on some novel approaches for interrogating the pathway experimentally.

  10. A Case of Juvenile Huntington Disease in a 6-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Jun-Sang Sunwoo


    Full Text Available Huntington disease is a neurodegenerative disorder distinguished by the triad of dominant inheritance, choreoathetosis and dementia, usually with onset in the fourth and fifth decades. It is caused by an unstable cytosine-adenine-guanine (CAG trinucleotide repeat expansion in the gene IT15 in locus 4p16.3. Juvenile HD that constitutes about 3% to 10% of all patients is clinically different from adult-onset form and characterized by a larger number of CAG repeats typically exceeding 60. We report a case of a 6-year-old boy with myoclonic seizure and 140 CAG repeats confirmed by molecular genetic analysis.

  11. 77 FR 21662 - Amendment of Class D Airspace; Cocoa Beach, FL (United States)


    ...-0099; Airspace Docket No. 12-ASO-11] Amendment of Class D Airspace; Cocoa Beach, FL AGENCY: Federal... Class D airspace at Cape Canaveral Skid Strip, Cocoa Beach, FL, by correcting the geographic coordinates... of Cape Canaveral Skid Strip, Cocoa Beach, FL. Also, the geographic coordinates for the airport need...

  12. 77 FR 28243 - Amendment of Class D Airspace; Cocoa Beach, FL (United States)


    ...-0099; Airspace Docket No. 12-ASO-11] Amendment of Class D Airspace; Cocoa Beach, FL AGENCY: Federal... Federal Register on April 11, 2012 that amends Class D airspace at Cocoa Beach, FL. DATES: Effective 0901...), amends Class D airspace at Cape Canaveral Skid Strip, Cocoa Beach, FL. A typographical error was made in...

  13. 36 CFR 3.17 - What regulations apply to swimming areas and beaches? (United States)


    ... swimming areas and beaches? 3.17 Section 3.17 Parks, Forests, and Public Property NATIONAL PARK SERVICE, DEPARTMENT OF THE INTERIOR BOATING AND WATER USE ACTIVITIES § 3.17 What regulations apply to swimming areas and beaches? (a) The superintendent may designate areas as swimming areas or swimming beaches in...

  14. 76 FR 77119 - Special Local Regulations; Pompano Beach Holiday Boat Parade, Intracoastal Waterway, Pompano... (United States)


    ...-AA08 Special Local Regulations; Pompano Beach Holiday Boat Parade, Intracoastal Waterway, Pompano Beach... Pompano Beach Holiday Boat Parade on Sunday, December 11, 2011. The marine parade will consist of... Friday, except Federal holidays. FOR FURTHER INFORMATION CONTACT: If you have questions on this temporary...

  15. Gone to the Beach — Using GIS to infer how people value ... (United States)

    Estimating the non-market value of beaches for saltwater recreation is complex. An individual’s preference for a beach depends on their perception of beach characteristics. When choosing one beach over another, an individual balances these personal preferences with any additional costs including travel time and/or fees to access the beach. This trade-off can be used to infer how people value different beach characteristics; especially when beaches are free to the public, beach value estimates rely heavily on accurate travel times. A current case study focused on public access on Cape Cod, MA will be used to demonstrate how travel costs can be used to determine the service area of different beaches, and model expected use of those beaches based on demographics. We will describe several of the transportation networks and route services available and compare a few based on their ability to meet our specific requirements of scale and seasonal travel time accuracy. We are currently developing a recreational demand model, based on visitation data and beach characteristics, that will allow decision makers to predict the benefits of different levels of water quality improvement. An important part of that model is the time required for potential recreation participants to get to different beaches. This presentation will describe different ways to estimate travel times and the advantages/disadvantages for our particular application. It will go on to outline how freely a

  16. 76 FR 54375 - Safety Zone; Thunder on the Gulf, Gulf of Mexico, Orange Beach, AL (United States)


    ...-AA00 Safety Zone; Thunder on the Gulf, Gulf of Mexico, Orange Beach, AL AGENCY: Coast Guard, DHS... portion of the Gulf of Mexico for the waters off Orange Beach, Alabama. This action is necessary for the... conduct a high speed boat race on the Gulf of Mexico, south of Orange Beach, Alabama to occur from October...

  17. RIP current zones along beaches in Goa, west coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; SanilKumar, V.; Jena, B.K.

    Goa has a 125-km-long coastline of which two-thirds consists of beautiful sandy beaches. There are mainly 17 beaches having significant importance of tourism. Sporadically, surf drownings have been reported at a few stretches of the beach. Longshore...

  18. Geographic relatedness and predictability of Escherichia coli along a peninsular beach complex of Lake Michigan (United States)

    Nevers, M.B.; Shively, D.A.; Kleinheinz, G.T.; McDermott, C.M.; Schuster, W.; Chomeau, V.; Whitman, R.L.


    To determine more accurately the real-time concentration of fecal indicator bacteria (FIB) in beach water, predictive modeling has been applied in several locations around the Great Lakes to individual or small groups of similar beaches. Using 24 beaches in Door County, Wisconsin, we attempted to expand predictive models to multiple beaches of complex geography. We examined the importance of geographic location and independent variables and the consequential limitations for potential beach or beach group models. An analysis of Escherichia coli populations over 4 yr revealed a geographic gradient to the beaches, with mean E. coli concentrations decreasing with increasing distance from the city of Sturgeon Bay. Beaches grouped strongly by water type (lake, bay, Sturgeon Bay) and proximity to one another, followed by presence of a storm or creek outfall or amount of shoreline enclosure. Predictive models developed for beach groups commonly included wave height and cumulative 48-h rainfall but generally explained little E. coli variation (adj. R2 = 0.19-0.36). Generally low concentrations of E. coli at the beaches influenced the effectiveness of model results presumably because of low signal-to-noise ratios and the rarity of elevated concentrations. Our results highlight the importance of the sensitivity of regressors and the need for careful methods evaluation. Despite the attractiveness of predictive models as an alternative beach monitoring approach, it is likely that FIB fluctuations at some beaches defy simple prediction approaches. Regional, multi-beach, and individual beach predictive models should be explored alongside other techniques for improving monitoring reliability at Great Lakes beaches. Copyright ?? 2009 by the American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America. All rights reserved.

  19. Wireless Time Tracking Improves Productivity at CSU Long Beach. (United States)

    Charmack, Scott; Walsh, Randy


    Describes California State University Long Beach's implementation of new maintenance management software, which integrated maintenance, inventory control, and key control and allows technicians to enter and receive information through handheld wireless devices for more accurate time accounting. The school estimates a 10 percent increase in…

  20. Aggregations of the sandy-beach isopod, Tylos granulatus ...

    African Journals Online (AJOL)

    ... lives as a scavenger in the intertidal zone of sandy beaches on the west coast of South Africa. Individuals emerge with the receding tide leaving exit holes, then forage for about two hours before returning to the vicinity of the high-water mark where they aggregate to bury themselves, leaving behind cone-shaped mounds.

  1. Effectiveness of the Call in Beach Volleyball Attacking Play

    Directory of Open Access Journals (Sweden)

    Künzell Stefan


    Full Text Available In beach volleyball the setter has the opportunity to give her or his hitter a “call”. The call intends that the setter suggests to her or his partner where to place the attack in the opponent’s court. The effectiveness of a call is still unknown. We investigated the women’s and men’s Swiss National Beach Volleyball Championships in 2011 and analyzed 2185 attacks. We found large differences between female and male players. While men called in only 38.4% of attacks, women used calls in 85.5% of attacks. If the male players followed a given call, 63% of the attacks were successful. The success rate of attacks without any call was 55.8% and 47.6% when the call was ignored. These differences were not significant (χ2(2 = 4.55, p = 0.103. In women’s beach volleyball, the rate of successful attacks was 61.5% when a call was followed, 35% for attacks without a call, and 42.6% when a call was ignored. The differences were highly significant (χ2(2 = 23.42, p < 0.0005. Taking into account the findings of the present study, we suggested that the call was effective in women’s beach volleyball, while its effect in men’s game was unclear. Considering the quality of calls we indicate that there is a significant potential to increase the effectiveness of a call.

  2. Spatial variability of macrobenthic zonation on exposed sandy beaches (United States)

    Veiga, Puri; Rubal, Marcos; Cacabelos, Eva; Maldonado, Cristina; Sousa-Pinto, Isabel


    We analysed the consistence of vertical patterns of distribution (i.e. zonation) for macrofauna at different spatial scales on four intermediate exposed beaches in the North of Portugal. We tested the hypothesis that biological zonation on exposed sandy beaches would vary at the studied spatial scales. For this aim, abundance, diversity and structure of macrobenthic assemblages were examined at the scales of transect and beach. Moreover, the main environmental factors that could potentially drive zonation patterns were investigated. Univariate and multivariate analyses revealed that the number of biological zones ranged from two to three depending on the beach and from indistinct zonation to three zones at the scale of transect. Therefore, results support our working hypothesis because zonation patterns were not consistent at the studied spatial scales. The median particle size, sorting coefficient and water content were significantly correlated with zonation patterns of macrobenthic assemblages. However, a high degree of correlation was not reached when the total structure of the assemblage was considered.

  3. Meiofauna of some sandy beaches of Andaman Islands

    Digital Repository Service at National Institute of Oceanography (India)

    Ansari, Z.A.; Ingole, B.S.

    Comparatively high meiofauna numbers were recorded from beaches having fine sand particles than those having coarse to medium sand particles The fauna was dominated mainly by nematodes (53.7%), harpacticoids (13.7%) and polychaetes (12.8%) On all...

  4. The effects of large beach debris on nesting sea turtles (United States)

    Fujisaki, Ikuko; Lamont, Margaret M.


    A field experiment was conducted to understand the effects of large beach debris on sea turtle nesting behavior as well as the effectiveness of large debris removal for habitat restoration. Large natural and anthropogenic debris were removed from one of three sections of a sea turtle nesting beach and distributions of nests and false crawls (non-nesting crawls) in pre- (2011–2012) and post- (2013–2014) removal years in the three sections were compared. The number of nests increased 200% and the number of false crawls increased 55% in the experimental section, whereas a corresponding increase in number of nests and false crawls was not observed in the other two sections where debris removal was not conducted. The proportion of nest and false crawl abundance in all three beach sections was significantly different between pre- and post-removal years. The nesting success, the percent of successful nests in total nesting attempts (number of nests + false crawls), also increased from 24% to 38%; however the magnitude of the increase was comparably small because both the number of nests and false crawls increased, and thus the proportion of the nesting success in the experimental beach in pre- and post-removal years was not significantly different. The substantial increase in sea turtle nesting activities after the removal of large debris indicates that large debris may have an adverse impact on sea turtle nesting behavior. Removal of large debris could be an effective restoration strategy to improve sea turtle nesting.

  5. The investigation of radionuclides distributions in beach sand by ...

    African Journals Online (AJOL)

    The investigation of radionuclides distributions in beach sand by means of GIS techniques. ... The PDF file you selected should load here if your Web browser has a PDF reader plug-in installed (for example, a recent version of Adobe Acrobat Reader). If you would like more information about how to print, save, and work ...

  6. Sediment budget of a portion of Trivandrum beach (Kerala)

    Digital Repository Service at National Institute of Oceanography (India)

    Varma, P.U.; RamaRaju, V.S.; Pylee, A.; Swamy, G.N.

    to be deposited back when the erosion phase is over. Eventhough a net loss has been noticed during the period of study, it is considered not very significant from the point of view of long-term changes which the beach may undergo...

  7. Beaches and Bluffs of Puget Sound and the Northern Straits (United States)


    sand up to pebbles, cobbles, and occasionally boulders, often also containing shelly material. Puget Sound beaches commonly have two distinct...very limited historic wind records (wave hind- casting ). Drift directions indicated in the Atlas have repeatedly been proven inaccurate (Johannessen

  8. Surfin’ California with Whitman and The Beach Boys

    DEFF Research Database (Denmark)

    Balle, Søren Hattesen

    of American literary Romanticism, whereas The Beach Boys became the sixties’ most well-known pop icon of surf music and surf culture. Nevertheless, their common interest in California as a particular topographic image of the American West invites comparison and further study. This paper aims to make...

  9. Pollution of some recreation beaches of Mumbai, Maharashtra

    Digital Repository Service at National Institute of Oceanography (India)

    Ingole, S.A.; Kadam, A.N.

    Pollution in terms of water and sediment qualities of Madh. Juhu, Dadar and Girgaon recreation beaches was studied. Water quality in respect of low pH (7.8-8.3), low salinity (15.1-34.5 ppl), high microbial population (coliform count up to 18000...

  10. White shark and other chondrichthyan interactions with the beach ...

    African Journals Online (AJOL)

    Public perception has been that an apparent increase in the nearshore occurrence of white sharks Carcharodon carcharias in False Bay, on the south coast of South Africa, can at least be partly attributed to beach-seine (treknet) operations attracting sharks into this coastal area. To assess the merit of these concerns, ...

  11. Economic valuation of illegal fishing: An empirical study of beach ...

    African Journals Online (AJOL)

    Beach seining was banned in Kenya in 2001 largely due to growth overfishing. To-date compliance to this regulation remains a challenge to managers and policy makers. This paper analyses enforcement records in Lake Victoria between 2001 and 2012 and applies the model of rational criminality to estimate the economic ...

  12. Disturbance of beach sediment by off-road vehicles (United States)

    Anders, Fred J.; Leatherman, Stephen P.


    A three-year investigation was undertaken to examine the effects of off-road vehicles (ORVs) on the beach at Fire Island, New York. Within the National Seashore over 45,000 vehicle trips per year are concentrated in the zone seaward of the dune toe. The experimental approach was adopted in order to assess the environmental effects of ORVs. Specially developed instrumentation was used to measure the direct displacement of sand by vehicles traversing the beach. Direct displacement data were reduced graphically and analyzed by stepwise linear regression. The results of 89 field experiments (788 cases) showed that slope, sand compaction, and number of vehicle passes in the same track were the principal factors controlling the measured net seaward displacement of sand. The data suggest that ORV use levels within the National Seashore could be contributing to the overall erosion rate by delivering large quantities of sand to the swash zone (max. of 119,300 m3/yr). However, with proper management downslope movement of sand could be reduced by an order of magnitude. While vehicular passage over the open beach displaces sand seaward, it is not known if such activity actually increases the amount of erosion, measured as net loss to the beach face.

  13. Organisms associated with the sandy-beach bivalve Donax serra ...

    African Journals Online (AJOL)

    57: 134-136. BROWN, AC. & WEBB, S.c. 1994. Organisms associated \\.,.,ith burrowing whelks of the genus Bullia. S Afr. 1. Zool. 29: 144-151. BROWN, A.C., STENTON-DOZEY, J.~.E. & TRUEMAN, E.R.. 1989. Sandy-beach bivalves and gastropods; a comparison between Donax serra and Ruilia digitalis. Adv. mar. Bioi. 25:.

  14. Modeling the Movement of Beach Alluvia in the Alongshore Direction

    Directory of Open Access Journals (Sweden)

    Elena V. Bondareva


    Full Text Available The authors have worked out a design model for the dynamics of a mixed-composition beach in the vicinity of transverse structures. The model uses a modified formula for calculating alluvia, which is based on modified energy dependencies. The authors provide an algorithm for performing these calculations.

  15. The investigation of radionuclides distributions in beach sand by ...

    African Journals Online (AJOL)

    A transportable and robust gamma ray detection system (GISPI) was employed to determine the concentrations of naturally occurring radionuclides on various beaches in and around Saldanha Bay, located on the West Coast of South Africa. Several mathematical techniques were applied to obtain GIS overlay that could be ...

  16. Aggregations of the sandy-beach isopod, Tylos granulatu ...

    African Journals Online (AJOL)


    Dec 14, 1995 ... The advantages of this are analysed in terms of energy conservation, and as a means of reducing ...... permanently unless washed out by the waves (Brown. Sten- .... The bc:- havior and life history or a sand-beach isopod.

  17. Effect of sillimanite beach sand composition on mullitization and ...

    Indian Academy of Sciences (India)


    Effect of sillimanite beach sand composition on mullitization and properties of Al2O3–SiO2 system ... Presence of zircon in Z-variety increases the hardness and fracture toughness. Alumina addition ... The ratio of charge to grinding media was ...

  18. Cluster analysis of radionuclide concentrations in beach sand

    NARCIS (Netherlands)

    de Meijer, R.J.; James, I.; Jennings, P.J.; Keoyers, J.E.

    This paper presents a method in which natural radionuclide concentrations of beach sand minerals are traced along a stretch of coast by cluster analysis. This analysis yields two groups of mineral deposit with different origins. The method deviates from standard methods of following dispersal of

  19. March 1977 Sandy beaches are unstable, often very exposed

    African Journals Online (AJOL)

    COMPOSITION, DISTRIBUTION, ABUNDANCE AND BIOMASS OF THE ... beach giving macrofauna values of 17,5-16553,3 '/transect and meiofauna values .... and evenness, the two components of diversity, were calculated as done by ..... feeding on particulate matter and plankton, and Bullia occupies the niche feeding on.

  20. Experiences of returning to elite beach volleyball after shoulder injury. (United States)

    Bele, Sofie; Östenberg, Anna Hafsteinsson; Sjöström, Rita; Alricsson, Marie


    The purpose of this study was to examine beach volleyball players' experience regarding shoulder injury and how it affects their return to play. To achieve the research aims a qualitative design with semi-structured interviews had been conducted, five elite beach volleyball players, four men and one woman aged 27-42 participated in the study. All participants had suffered a severe shoulder injury, with absence from training and competing for at least 28 days. The findings of this study indicate that it is the individual's inner motivation, together with a clear goal and support from the community, family, teammate and coach that are the most important factors when going through rehabilitation and getting back to playing beach volleyball after a shoulder injury. All participants had been affected by their injury in some way; some of the participants had been affected in a positive way since they had become mentally stronger and had developed better volleyball technique after rehabilitation. The conclusions of this study indicate that there are three distinct factors that increase the chances of getting back to playing beach volleyball after shoulder injury; it is the players' self motivation, together with a clear goal and support from the community.

  1. Sun behaviour on the beach monitored by webcam photos. (United States)

    Heerfordt, I M; Philipsen, P A; Wulf, H C


    To prevent skin cancer, the general population is recommended to limit time in the sun, to wear clothes and to seek shade around noon. This study aimed to investigate the number of beachgoers, the duration of sun exposure, and clothing worn during the day on a beach in Copenhagen. Observational, descriptive study. On 11 beach days in 2014 and 2015, beachgoers were counted every hour from 8:00 to 20:00. It was noted if they wore clothes or swimwear. To estimate the duration of sun exposure, it was noted how long cars were parked by the beach. Of the counted beachgoers 46% were present from noon to 15:00. The number of beachgoers peaked at 15:00 on weekend days (Saturdays and Sunday) and at 16:00 on working days (Monday to Friday). Both on weekend days and working days, the percentage of beachgoers wearing clothes was lowest at 13:00 when about 90% wore only swimwear. Cars were parked for 117 min on average. Around noon, the mean time expanded to 142 min. We assume this to reflect the duration of a beach visit. The results indicate a weak tendency to limit time in the sun and to seek shade when the ultraviolet radiation is strongest in the midday sun. Hopefully information about actual sun behaviour can be used to adjust campaigns. Copyright © 2017 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.

  2. Plastics Distribution and Degradation on Lake Huron Beaches (United States)

    Zbyszewski, M.; Corcoran, P.


    The resistivity of plastic debris to chemical and mechanical weathering processes poses a serious threat to the environment. Numerous marine beaches are littered with plastic fragments that entangle and become ingested by organisms including birds, turtles and plankton. Although many studies have been conducted to determine the amount and effects of plastics pollution on marine organisms, relatively little is known about the distribution and quantity of polymer types along lacustrine beaches. Plastic particles sampled from selected beaches on Lake Huron were analyzed using Fourier Transform Infrared Spectroscopy (FTIR) to determine polymer composition. The majority of the plastic fragments are industrial pellets composed of polypropylene and polyethylene. Varying degrees of oxidation are indicated by multiple irregular peaks in the lower wavenumber region on the FTIR spectra. The oxidized pellets also represent the plastic particles with the most pronounced surface textures, as identified using Scanning Electron Microscopy (SEM). Crazes and flakey, fibrous, or granular textures are consistent with chemical weathering processes, whereas gauges and pits occur through abrasion during mechanical weathering. Further textural and compositional analysis will indicate which polymer types are more resistant to weathering processes. Additional investigation of the distribution of plastic debris along the beaches of Lake Huron will indicate the amount and primary transport directions of resistant plastic debris polluting one of Ontario's Great Lakes.

  3. Continuous deep sedation, physician-assisted suicide, and euthanasia in Huntington's disorder. (United States)

    Lindblad, Anna; Juth, Niklas; Fürst, Carl Johan; Lynöe, Niels


    To investigate the attitudes among Swedish physicians and the general public towards continuous deep sedation (CDS) as an alternative treatment for a competent, not imminently dying patient with Huntington's disorder requesting physician-assisted suicide (PAS) and euthanasia. A questionnaire was distributed to 1200 physicians in Sweden and 1201 individuals in Stockholm. It consisted of three parts: 1) A vignette about a competent patient with Huntington's disease requesting PAS. When no longer competent, relatives request euthanasia on behalf of the patient. Responders were asked about their attitudes towards these requests and whether CDS would be an acceptable alternative. 2) General questions about PAS and euthanasia. 3) Background variables. The response rate was 56% (physicians) and 52% (general public). The majority of the general public and a fairly large proportion of physicians reported more liberal views on CDS than are expressed in current Swedish and international recommendations. In light of the results, we suggest that there is a need for a broader discussion about the recommendations for CDS, with a special focus on the needs of patients with progressive neurodegenerative disorders.

  4. Insights into gait disorders: walking variability using phase plot analysis, Huntington's disease. (United States)

    Collett, Johnny; Esser, Patrick; Khalil, Hanan; Busse, Monica; Quinn, Lori; DeBono, Katy; Rosser, Anne; Nemeth, Andrea H; Dawes, Helen


    Huntington's disease (HD) is a progressive inherited neurodegenerative disorder. Identifying sensitive methodologies to quantitatively measure early motor changes have been difficult to develop. This exploratory observational study investigated gait variability and symmetry in HD using phase plot analysis. We measured the walking of 22 controls and 35 HD gene carriers (7 premanifest (PreHD)), 16 early/mid (HD1) and 12 late stage (HD2) in Oxford and Cardiff, UK. The unified Huntington's disease rating scale-total motor scores (UHDRS-TMS) and disease burden scores (DBS) were used to quantify disease severity. Data was collected during a clinical walk test (8.8 or 10 m) using an inertial measurement unit attached to the trunk. The 6 middle strides were used to calculate gait variability determined by spatiotemporal parameters (co-efficient of variation (CoV)) and phase plot analysis. Phase plots considered the variability in consecutive wave forms from vertical movement and were quantified by SDA (spatiotemporal variability), SDB (temporal variability), ratio ∀ (ratio SDA:SDB) and Δangleβ (symmetry). Step time CoV was greater in manifest HD (p0.05). Phase plot analysis identified differences between manifest HD and controls for SDB, Ratio ∀ and Δangle (all pplot analysis may be a sensitive method of detecting gait changes in HD and can be performed quickly during clinical walking tests. Copyright © 2014 Elsevier B.V. All rights reserved.

  5. Electronic Transport in Single-Stranded DNA Molecule Related to Huntington's Disease (United States)

    Sarmento, R. G.; Silva, R. N. O.; Madeira, M. P.; Frazão, N. F.; Sousa, J. O.; Macedo-Filho, A.


    We report a numerical analysis of the electronic transport in single chain DNA molecule consisting of 182 nucleotides. The DNA chains studied were extracted from a segment of the human chromosome 4p16.3, which were modified by expansion of CAG (cytosine-adenine-guanine) triplet repeats to mimics Huntington's disease. The mutated DNA chains were connected between two platinum electrodes to analyze the relationship between charge propagation in the molecule and Huntington's disease. The computations were performed within a tight-binding model, together with a transfer matrix technique, to investigate the current-voltage (I-V) of 23 types of DNA sequence and compare them with the distributions of the related CAG repeat numbers with the disease. All DNA sequences studied have a characteristic behavior of a semiconductor. In addition, the results showed a direct correlation between the current-voltage curves and the distributions of the CAG repeat numbers, suggesting possible applications in the development of DNA-based biosensors for molecular diagnostics.

  6. Adenyl cyclase activator forskolin protects against Huntington's disease-like neurodegenerative disorders

    Directory of Open Access Journals (Sweden)

    Sidharth Mehan


    Full Text Available Long term suppression of succinate dehydrogenase by selective inhibitor 3-nitropropionic acid has been used in rodents to model Huntington's disease where mitochondrial dysfunction and oxidative damages are primary pathological hallmarks for neuronal damage. Improvements in learning and memory abilities, recovery of energy levels, and reduction of excitotoxicity damage can be achieved through activation of Adenyl cyclase enzyme by a specific phytochemical forskolin. In this study, intraperitoneal administration of 10 mg/kg 3-nitropropionic acid for 15 days in rats notably reduced body weight, worsened motor cocordination (grip strength, beam crossing task, locomotor activity, resulted in learning and memory deficits, greatly increased acetylcholinesterase, lactate dehydrogenase, nitrite, and malondialdehyde levels, obviously decreased adenosine triphosphate, succinate dehydrogenase, superoxide dismutase, catalase, and reduced glutathione levels in the striatum, cortex and hippocampus. Intragastric administration of forskolin at 10, 20, 30 mg/kg dose-dependently reversed these behavioral, biochemical and pathological changes caused by 3-nitropropionic acid. These results suggest that forskolin exhibits neuroprotective effects on 3-nitropropionic acid-induced Huntington's disease-like neurodegeneration.

  7. Nonparametric modeling and analysis of association between Huntington's disease onset and CAG repeats. (United States)

    Ma, Yanyuan; Wang, Yuanjia


    Huntington's disease (HD) is a neurodegenerative disorder with a dominant genetic mode of inheritance caused by an expansion of CAG repeats on chromosome 4. Typically, a longer sequence of CAG repeat length is associated with increased risk of experiencing earlier onset of HD. Previous studies of the association between HD onset age and CAG length have favored a logistic model, where the CAG repeat length enters the mean and variance components of the logistic model in a complex exponential-linear form. To relax the parametric assumption of the exponential-linear association to the true HD onset distribution, we propose to leave both mean and variance functions of the CAG repeat length unspecified and perform semiparametric estimation in this context through a local kernel and backfitting procedure. Motivated by including family history of HD information available in the family members of participants in the Cooperative Huntington's Observational Research Trial (COHORT), we develop the methodology in the context of mixture data, where some subjects have a positive probability of being risk free. We also allow censoring on the age at onset of disease and accommodate covariates other than the CAG length. We study the theoretical properties of the proposed estimator and derive its asymptotic distribution. Finally, we apply the proposed methods to the COHORT data to estimate the HD onset distribution using a group of study participants and the disease family history information available on their family members. Copyright © 2013 John Wiley & Sons, Ltd.

  8. [Predictors of mental and physical quality of life in Huntington's disease]. (United States)

    Brugger, F; Hepperger, C; Hametner, E-M; Holl, A K; Painold, A; Schusterschitz, C; Bonelli, R; Holas, C; Wenning, G K; Poewe, W; Seppi, K


    The assessment of health-related quality of life (hrQoL) is an important tool in therapy studies and in the treatment of patients with Huntington's disease (HD). In the absence of causal interventions, HD therapy targets the alleviation of symptoms aiming to improve impaired hrQoL. The aim of this study was to determine the impact of disease characteristics on hrQoL in HD. A total of 80 genetically confirmed HD patients underwent an assessment using the Unified Huntington's Disease Rating Scale, the Beck Depression Inventory, the Hamilton Rating Scale and the SF-36, a scale for the assessment of physical and mental QoL. Multiple regression analysis revealed that health-related physical and mental QoL was considerably influenced by the functional capacity. The mental QoL also correlated with the degree of depressive symptoms, age and the number of CAG repeats. However, there was no statistical relation between QoL and motor and cognitive abilities. This study underlines the relationship between function capacity and depressive symptoms with mental and physical QoL. This is the first time that hrQoL has been investigated in a German speaking cohort. The results are in accordance with previous studies of hrQoL in HD.

  9. Neurotensin receptor binding levels in basal ganglia are not altered in Huntington's chorea or schizophrenia

    International Nuclear Information System (INIS)

    Palacios, J.M.; Chinaglia, G.; Rigo, M.; Ulrich, J.; Probst, A.


    Autoradiographic techniques were used to examine the distribution and levels of neurotensin receptor binding sites in the basal ganglia and related regions of the human brain. Monoiodo ( 125 I-Tyr3)neurotensin was used as a ligand. High amounts of neurotensin receptor binding sites were found in the substantia nigra pars compacta. Lower but significant quantities of neurotensin receptor binding sites characterized the caudate, putamen, and nucleus accumbens, while very low quantities were seen in both medial and lateral segments of the globus pallidus. In Huntington's chorea, the levels of neurotensin receptor binding sites were found to be comparable to those of control cases. Only slight but not statistically significant decreases in amounts of receptor binding sites were detected in the dorsal part of the head and in the body of caudate nucleus. No alterations in the levels of neurotensin receptor binding sites were observed in the substantia nigra pars compacta and reticulata. These results suggest that a large proportion of neurotensin receptor binding sites in the basal ganglia are located on intrinsic neurons and on extrinsic afferent fibers that do not degenerate in Huntington's disease

  10. Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model

    DEFF Research Database (Denmark)

    Aidt, Frederik Heurlin; Nielsen, Signe Marie Borch; Kanters, Jørgen


    Metabolic dysfunction and mitochondrial involvement are recognised as part of the pathology in Huntington's Disease (HD). Post-mortem examinations of the striatum from end-stage HD patients have shown a decrease in the in vitro activity of complexes II, III and IV of the electron transport system...

  11. Transgenic Rat Model of Huntington's Disease: A Histopathological Study and Correlations with Neurodegenerative Process in the Brain of HD Patients.

    Czech Academy of Sciences Publication Activity Database

    Mazurová, Y.; Anděrová, Miroslava; Němečková, I.; Bezrouk, A.


    Roč. 2014, Aug 03 (2014), s. 291531 ISSN 2314-6133 R&D Projects: GA ČR(CZ) GBP304/12/G069 Grant - others:GA MŠk(CZ) Prvouk P37 Institutional support: RVO:68378041 Keywords : Huntington's Disease * neurodegenerative process in the brain Subject RIV: FH - Neurology Impact factor: 1.579, year: 2014

  12. Assessment of motor function, sensory motor gating and recognition memory in a novel BACHD transgenic rat model for Huntington disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Nguyen, Huu Phuc; Schreiber, Rudy; Ellenbroek, Bart


    Rationale: Huntington disease (HD) is frequently first diagnosed by the appearance of motor symptoms; the diagnosis is subsequently confirmed by the presence of expanded CAG repeats (> 35) in the HUNTINGTIN (HTT) gene. A BACHD rat model for HD carrying the human full length mutated HTT with 97

  13. Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington's Disease Gene-expansion Carriers

    DEFF Research Database (Denmark)

    Unmack Larsen, Ida; Vinther-Jensen, Tua; Gade, Anders


    Executive functions (EF) and psychomotor speed (PMS) has been widely studied in Huntington's disease (HD). Most studies have focused on finding markers of disease progression by comparing group means at different disease stages. Our aim was to investigate performances on nine measures of EF and PMS...

  14. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    John P Miller

    Full Text Available A genome-scale RNAi screen was performed in a mammalian cell-based assay to identify modifiers of mutant huntingtin toxicity. Ontology analysis of suppressor data identified processes previously implicated in Huntington's disease, including proteolysis, glutamate excitotoxicity, and mitochondrial dysfunction. In addition to established mechanisms, the screen identified multiple components of the RRAS signaling pathway as loss-of-function suppressors of mutant huntingtin toxicity in human and mouse cell models. Loss-of-function in orthologous RRAS pathway members also suppressed motor dysfunction in a Drosophila model of Huntington's disease. Abnormal activation of RRAS and a down-stream effector, RAF1, was observed in cellular models and a mouse model of Huntington's disease. We also observe co-localization of RRAS and mutant huntingtin in cells and in mouse striatum, suggesting that activation of R-Ras may occur through protein interaction. These data indicate that mutant huntingtin exerts a pathogenic effect on this pathway that can be corrected at multiple intervention points including RRAS, FNTA/B, PIN1, and PLK1. Consistent with these results, chemical inhibition of farnesyltransferase can also suppress mutant huntingtin toxicity. These data suggest that pharmacological inhibition of RRAS signaling may confer therapeutic benefit in Huntington's disease.

  15. First molecular modeling report on novel arylpyrimidine kynurenine monooxygenase inhibitors through multi-QSAR analysis against Huntington's disease: A proposal to chemists! (United States)

    Amin, Sk Abdul; Adhikari, Nilanjan; Jha, Tarun; Gayen, Shovanlal


    Huntington's disease (HD) is caused by mutation of huntingtin protein (mHtt) leading to neuronal cell death. The mHtt induced toxicity can be rescued by inhibiting the kynurenine monooxygenase (KMO) enzyme. Therefore, KMO is a promising drug target to address the neurodegenerative disorders such as Huntington's diseases. Fiftysix arylpyrimidine KMO inhibitors are structurally explored through regression and classification based multi-QSAR modeling, pharmacophore mapping and molecular docking approaches. Moreover, ten new compounds are proposed and validated through the modeling that may be effective in accelerating Huntington's disease drug discovery efforts. Copyright © 2016 Elsevier Ltd. All rights reserved.

  16. Disentangling diversity patterns in sandy beaches along environmental gradients. (United States)

    Barboza, Francisco R; Gómez, Julio; Lercari, Diego; Defeo, Omar


    Species richness in sandy beaches is strongly affected by concurrent variations in morphodynamics and salinity. However, as in other ecosystems, different groups of species may exhibit contrasting patterns in response to these environmental variables, which would be obscured if only aggregate richness is considered. Deconstructing biodiversity, i.e. considering richness patterns separately for different groups of species according to their taxonomic affiliation, dispersal mode or mobility, could provide a more complete understanding about factors that drive species richness patterns. This study analyzed macroscale variations in species richness at 16 Uruguayan sandy beaches with different morphodynamics, distributed along the estuarine gradient generated by the Rio de la Plata over a 2 year period. Species richness estimates were deconstructed to discriminate among taxonomic groups, supralittoral and intertidal forms, and groups with different feeding habits and development modes. Species richness was lowest at intermediate salinities, increasing towards oceanic and inner estuarine conditions, mainly following the patterns shown for intertidal forms. Moreover, there was a differential tolerance to salinity changes according to the habitat occupied and development mode, which determines the degree of sensitivity of faunal groups to osmotic stress. Generalized (additive and linear) mixed models showed a clear increase of species richness towards dissipative beaches. All taxonomic categories exhibited the same trend, even though responses to grain size and beach slope were less marked for crustaceans and insects than for molluscs or polychaetes. However, supralittoral crustaceans exhibited the opposite trend. Feeding groups decreased from dissipative to reflective systems, deposit feeders being virtually absent in the latter. This deconstructive approach highlights the relevance of life history strategies in structuring communities, highlighting the relative

  17. Ground-Penetrating Radar Study of Fort Morgan Peninsula Holocene Beach Ridges as Sea-level Indicators (United States)

    Philbin, A.; Frederick, B.; Blum, M. D.; Tsoflias, G. P.


    Holocene sea-level change along the northern Gulf of Mexico (GoM) coast is controversial. One view interprets basal peats from the Mississippi Delta to indicate continual sea-level (SL) rise for the GoM as a whole. An alternate view proposes that data from the subsiding delta is primarily a subsidence signal, and that sandy non-deltaic shorelines indicate that regional SL reached present elevations by the middle Holocene, with minor oscillations since then. In fact, new regional long-term subsidence records from biostratigraphic indicators display significant subsidence in deltaic areas where basal-peat data were collected, and negligible rates along the GoM shoreline to the east. However, the use of sandy progradational shorelines, commonly known as "beach ridge systems", has been criticized for a lack of precise sea-level indicators, and therefore discounted. This research focuses on developing Holocene progradational sandy shorelines along the Alabama coast in the eastern GoM as SL indicators. Sandy shorelines in this area are ideal to examine SL change because they are well preserved, sufficiently distant from the subsiding delta, well mapped, and ages are known from previous work. Two-dimensional ground-penetrating radar imaging of well-dated beach-ridge successions is used here to examine and identify changes through time in the elevation of the shoreface clinoform topset-foreset break, which represents the transition between flat-lying foreshore and seaward-dipping shoreface facies, and forms in the intertidal zone. Beach-ridge successions with optical luminescence ages of ca. 5500-4800 yrs BP display topset-foreset breaks at current mean sea-level elevation, whereas beach-ridge successions from ca. 3500-2400 yrs BP display topset-foreset breaks that are 1 m above present mean SL and the elevation of modern topset-foreset breaks. These data support the view that current sea-level was reached by the middle Holocene, and was higher than present for at least

  18. Data on nearshore wave process and surficial beach deposits, central Tamil Nadu coast, India. (United States)

    Joevivek, V; Chandrasekar, N


    The chronicles of nearshore morphology and surficial beach deposits provide valuable information about the nature of the beach condition and the depositional environment. It imparts an understanding about the spatial and temporal relationship of nearshore waves and its influence over the distribution of beach sediments. This article contains data about wave and sediment dynamics of the ten sandy beaches along the central Tamil Nadu coast, India. This present dataset comprises nearshore wave parameters, breaker wave type, beach morphodynamic state, grain size distribution and weight percentage of heavy and light mineral distribution. The dataset will figure out the beach morphology and hydrodynamic condition with respect to the different monsoonal season. This will act as a field reference to realize the coastal dynamics in an open sea condition. The nearshore entities were obtained from the intensive field survey between January 2011 and December 2011, while characteristics of beach sediments are examined by the chemical process in the laboratory environment.

  19. Impact of erosion and accretion on the distribution of enterococci in beach sands. (United States)

    Gast, Rebecca J; Gorrell, Levi; Raubenheimer, Britt; Elgar, Steve


    Bacterial pathogens in coastal sediments may pose a health risk to users of beaches. Although recent work shows that beach sands harbor both indicator bacteria and potential pathogens, it is not known how deep within beach sands the organisms may persist nor if they may be exposed during natural physical processes. In this study, sand cores of approximately 1 m depth were collected at three sites across the beach face in Kitty Hawk, North Carolina before, during and after large waves from an offshore hurricane. The presence of DNA from the fecal indicator bacterium Enterococci was detected in subsamples at different depths within the cores by PCR amplification. Erosion and accretion of beach sand at the three sites also was determined for each sampling day. The results indicate that ocean beach sands with persisting enterococci signals could be exposed and redistributed when wind, waves, and currents cause beach erosion or accretion.

  20. 75 FR 20802 - Safety Zone; New York Air Show at Jones Beach State Park, Atlantic Ocean off of Jones Beach... (United States)


    ... meeting would be beneficial. If we determine that one would aid this rulemaking, we will hold one at a... to the shows, as well as providing additional time should they run over the scheduled period. The... running east along the shoreline of Jones Beach State Park to approximate position 40[deg]35'49'' N, 073...

  1. 78 FR 33969 - Special Local Regulations; Daytona Beach Grand Prix of the Sea, Atlantic Ocean; Daytona Beach, FL (United States)


    ... through Sunday, June 14-16, 2013, Powerboat P1-USA will host the Daytona Beach Grand Prix of the Sea, a... private sector of $100,000,000 (adjusted for inflation) or more in any one year. Though this rule will not...

  2. Post-monsoon equilibrium beach profiles and longshore sediment transport rates at Candolim, Miramar and Keri beaches of Goa, India.

    Digital Repository Service at National Institute of Oceanography (India)

    Jayakumar, S.; Yadhunath, E.M.; Jishad, M.; Gowthaman, R.; Rajasekaran, C; Pednekar, P.S.

    ) and the profile shape factor m, which are inversely proportional to each other, have been analysed for their application to the study site. Also, the variation of these two parameters with beach slope and grain size is studied. Longshore sediment...

  3. Water Quality, Weather and Environmental Factors Associated with Fecal Indicator Organism Density in Beach Sand at Two Recreational Marine Beaches (United States)

    Recent studies showing an association between fecal indicator organisms (FIOs and gastrointestinal (GI) illness among beachgoers wit sand contact have important public health implicatons because of the large numbers of people who recreate at beaches and engage in sand contact act...

  4. Impact of seawater [Ca

    NARCIS (Netherlands)

    Mewes, A.; Langer, G.; Thoms, S.; Nehrke, G.; Reichart, G.J.; de Nooijer, L.J.; Bijma, J.


    Mg / Ca ratios in foraminiferal tests are routinely used as paleotemperature proxies, but on long timescales, they also hold the potential to reconstruct past seawater Mg / Ca. The impact of both temperature and seawater Mg / Ca on Mg incorporation in Foraminifera has been quantified by a number of

  5. Beach ridge sedimentology: field observation and palaeoenvironmental interpretation for Anegada Island, British Virgin Islands. (United States)

    Cescon, Anna Lisa; Cooper, J. Andrew G.; Jackson, Derek W. T.


    Beach ridge landforms have been observed in different environments and in settings that range from polar to tropical. Their stratigraphy and sedimentology has received a limited amount of discussion in the literature (Tamura, 2012). In coastal geomorphology a beach ridge can be seen as a transitional deposit between onshore and offshore environments. They are regarded as representing high level wave action along a coastline. In the Caribbean the origin of beach ridges has been variously attributed to one of three extreme wave events: extreme swell, extreme storm or tsunami waves. Beach ridges are arranged in beach ridge plains where there is succession of the landforms and can be several kilometres long. Beach ridge accumulation is not continuous and the coast shows alternating accretion and erosion periods. The use of beach ridges as palaeostorm archives is therefore not straightforward. The temporal continuity of beach ridge formation is being assessed on the beach ridge plains of Anegada, British Virgin Islands (Lesser Antilles). This carbonate platform surrounded by a fringing reef contains two beach ridge plains. There are more than 30 ridges in the Atlantic facing- coast and around 10 in the south, Caribbean- facing coast. The sediments of the modern beaches are dominated by the sand fraction and are 100% biogenic origin due to the isolation of Anegada from terrestrial sediment sources. The beach ridge sections have been studied in different area of Anegada beach ridge plains and present low angle seaward-dipping bedding. The sand fraction is dominant in the stratigraphy with a few intact shells. At only one site were coral pebbles deposited in association with the sand fraction. Aeolian deposits represent the upper part of the beach ridges and reflect the stabilization of the beach ridges with ongoing accretion. The sedimentology of the contemporary beach and dunes will be discussed in terms of their implications for understanding beach ridge genesis and its

  6. New marketing strategy has CA hospital saying 'in with the old and out with the new'. (United States)


    Redesigning the marketing department can offer a sweet surprise of savings. Long Beach (CA) Memorial Hospital revamped its marketing operations as an afterthought to a hospitalwide reengineering effort. Little did they know they could slash close to $1 million out of their advertising and marketing budget and redesign job functions to meet the changes taking shape within the entire facility. Learn their cost-saving secrets.

  7. New insights on water level variability for Lake Turkana for the past 15 ka and at 150 ka from relict beaches (United States)

    Forman, S. L.; Wright, D.


    Relict beaches adjacent to Lake Turkana provide a record of water level variability for the Late Quaternary. This study focused on deciphering the geomorphology, sedimentology, stratigraphy and 14C chronology of strand plain sequences in the Kalokol and Lothagam areas. Nine >30 m oscillations in water level were documented between ca. 15 and 4 ka. The earliest oscillation between ca. 14.5 and 13 ka is not well constrained with water level to at least 70 m above the present surface and subsequently fell to at least 50 m. Lake level increased to ~ 90 m between ca. 11.2 and 10.4 ka, post Younger Dryas cooling. Water level fell by >30 m by 10.2 ka, with another potential rise at ca. 8.5 ka to >70 m above current level. Lake level regressed by > 40 m at 8.2 ka coincident with cooling in the equatorial Eastern Atlantic Ocean. Two major >70 m lake level oscillations centered at 6.6 and 5.2 ka may reflect enhanced convection with warmer sea surface temperatures in the Western Indian Ocean. The end of the African Humid Period occurred from ca. 8.0 to 4.5 ka and was characterized by variable lake level (± > 40 m), rather than one monotonic fall in water level. This lake level variability reflects a complex response to variations in the extent and intensity of the East and West African Monsoons near geographic and topographic limits within the catchment of Lake Turkana. Also, for this closed lake basin excess and deficits in water input are amplified with a cascading lake effect in the East Rift Valley and through the Chew Bahir Basin. The final regression from a high stand of > 90 m began at. 5.2 ka and water level was below 20 m by 4.5 ka; and for the remainder of the Holocene. This sustained low stand is associated with weakening of the West African Monsoon, a shift of the mean position of Congo Air Boundary west of the Lake Turkana catchment and with meter-scale variability in lake level linked to Walker circulation across the Indian Ocean. A surprising observation is

  8. Periodontitis determining the onset and progression of Huntington's disease: review of the literature

    Directory of Open Access Journals (Sweden)

    María Lourdes Rodríguez Coyago


    Full Text Available La enfermedad de Hungtinton es un trastorno neurodegenerativo, causado por la expansión de un triplete de citosina, adenina, guanina en el gen de la huntingtina. Se manifiesta con un deterioro físico, cognitivo y psiquiátrico a distintas edades en el adulto, con un pronóstico fatal. Además del número de repeticiones del triplete, existirían otros factores que explicarían el inicio de esta enfermedad a más temprana edad. Se sabe que la neuroinflamación es un protagonista en los trastornos neurodegenerativos, no siendo la enfermedad de Huntington una excepción. La neuroinflamación exacerba el daño neuronal producido por la mutación, al existir activación aberrante de la célula microglía, disfunción de astrocitos y células dendríticas; compromiso de la barrera hematoencefálica y activación de complemento, todas ellas como efecto directo e indirecto de la mutante y otros estímulos como infecciones crónicas. Es el interés del presente trabajo analizar la periodontitis, como modelo de infección bucodental crónica y fuente de inflamación sistémica. Hipotetizamos que el potencial rol de la periodontitis en la enfermedad de Huntington y los mecanismos por los cuales contribuiría a la manifestación temprana y progreso de dicha enfermedad, para lo cual se consideraron revisiones sistemáticas, metanálisis y estudios experimentales publicados tanto en español como en inglés obtenidos del PubMed y SciELO. Son diversos los mecanismos que generan inflamación en el cerebro de estos pacientes, adquiriendo especial protagonismo los mecanismos de la inmunidad innata. Las infecciones buco dentarias crónicas, como la enfermedad periodontal, pueden constituir un factor exacerbante de la neuroinflamación que per se asocia la enfermedad de Huntington.

  9. Current status of PET imaging in Huntington's disease

    Energy Technology Data Exchange (ETDEWEB)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios [King' s College London, Neurodegeneration Imaging Group, Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), Camberwell, London (United Kingdom)


    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading ''Huntington Disease'' combined with text and key words ''Huntington Disease'', ''Neuroimaging'' and ''PET''. Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([{sup 18}F]FDG and [{sup 15}O]H{sub 2}O), presynaptic ([{sup 18}F]fluorodopa, [{sup 11}C]β-CIT and [{sup 11}C]DTBZ) and postsynaptic ([{sup 11}C]SCH22390, [{sup 11}C]FLB457 and [{sup 11}C]raclopride) dopaminergic function, phosphodiesterases ([{sup 18}F]JNJ42259152, [{sup 18}F]MNI-659 and [{sup 11}C]IMA107), and adenosine ([{sup 18}F]CPFPX), cannabinoid ([{sup 18}F]MK-9470), opioid ([{sup 11}C]diprenorphine) and GABA ([{sup 11}C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed

  10. Exchange Standards for Electronic Product Data (United States)


    Implementation Stds Product Definition Stds Graphics Stds j- Image Archival Stds 3 VDMNAPLPS GKS Manipulation I Core PHIGS VDI Textual Stds [ - Simple...Douglas Astronautics 5301 Bolsa Avenue Huntington Beach, CA 92647- 2048 Mr. Siegfried Goldstein Siegfried Enterprises, Inc. P. 0. Box 2308 North

  11. Short- and medium-term response to storms on three Mediterranean coarse-grained beaches (United States)

    Grottoli, Edoardo; Bertoni, Duccio; Ciavola, Paolo


    The storm response of three Italian coarse-grained beaches was investigated to better understand the morphodynamics of coarse-clastic beaches in a microtidal context. Two of the studied sites are located on the eastern side of the country (Portonovo and Sirolo) and the third one (Marina di Pisa) is on the western side. Portonovo and Sirolo are mixed sand and gravel beaches where the storms approach from two main directions, SE and NE. Marina di Pisa is a coarse-grained, gravel-dominated beach, exposed to storms driven by SW winds. Gravel nourishments were undertaken in recent years on the three sites. Beach topography was monitored measuring the same network of cross sections at a monthly (i.e. short-term) to seasonal frequency (i.e. medium-term). Geomorphic changes were examined before and after storm occurrences by means of profile analyses and shoreline position evaluations. The beach orientation and the influence of hard structures are the main factors controlling the transport and accumulation of significant amount of sediments and the consequent high variability of beach morphology over the medium-term. For Marina di Pisa, storms tend to accumulate material towards the upper part of the beach with no shoreline rotation and no chance to recover the initial configuration. Sirolo and Portonovo showed a similar behaviour that is more typical of pocket beaches. Both beaches show shoreline rotation after storms in a clockwise or counter-clockwise direction according to the incoming wave direction. The wider and longer beach at Sirolo allows the accumulation of a thin layer of sediment during storms, rather than at Portonovo where, given its longshore and landward boundaries, the beach material tends to accumulate in greater thickness. After storms, Sirolo and especially Portonovo can quickly recover the initial beach configuration, as soon as another storm of comparable energy approaches from the opposite direction of the previous one. Large morphological

  12. On the profile evolution of three artificial pebble beaches at Marina di Pisa, Italy (United States)

    Bertoni, Duccio; Sarti, Giovanni


    In this paper, the profiles of three artificial coarse-grained beaches located at Marina di Pisa (Tuscany, Italy) were monitored from April 2008 to May 2009 in order to define the response of the beaches to major storms that occurred during the study. Two beaches are similar, the third differs in length and in the level of protection, being less than half the length of the others and devoid of an offshore submerged breakwater. The work was achieved by means of accurate topographic surveys intended to reconstruct the beach profile from the backshore up to the foreshore-upper shoreface transition (step). The surveys were performed with an RTK-GPS instrument, which provided extremely precise recording of the beach. The most significant features of the beaches were tracked during each survey; in particular, the landward foot of the storm berm, the crest of the storm berm, the coastline, and the step crest were monitored. Five cross-shore transects were traced on each beach. Along these transects, any meaningful slope change was recorded to obtain accurate sections of the beach. The field datasets were processed with AutoCAD software to compare the beach profile evolution during the year-long research. The results showed a comparable evolution of the twin beaches: the resulting storm berm retreat of about 15 to 19 m is a remarkable feature considering the coarse grain size and the offshore protection. Due to the absence of the breakwater, the third beach was characterized by even higher values of recession (over 20 m), and showed hints of wave reflection-related processes after the huge, steep storm berm had been formed and grown after the high energy events. These processes were not as evident on the twin beaches. These results underline the different response of three similar protection schemes, and the importance that frequent monitoring of the beach morphology holds when it comes to coastal management issues.

  13. Documenting the global impacts of beach sand mining (United States)

    Young, R.; Griffith, A.


    For centuries, beach sand has been mined for use as aggregate in concrete, for heavy minerals, and for construction fill. The global extent and impact of this phenomenon has gone relatively unnoticed by academics, NGOs, and major news sources. Most reports of sand mining activities are found at the very local scale (if the mining is ever documented at all). Yet, sand mining in many localities has resulted in the complete destruction of beach (and related) ecosystems along with severe impacts to coastal protection and tourism. The Program for the Study of Developed Shorelines at Western Carolina University and have initiated the construction of a global database of beach sand mining activities. The database is being built through a combination of site visits and through the data mining of media resources, peer reviewed papers, and reports from private and governmental entities. Currently, we have documented sand mining in 35 countries on 6 continents representing the removal of millions of cubic meters of sand. Problems extend from Asia where critical infrastructure has been disrupted by sand mining to the Caribbean where policy reform has swiftly followed a highly publicized theft of sand. The Program for the Study of Developed Shorelines recently observed extensive sand mining in Morocco at the regional scale. Tens of kilometers of beach have been stripped of sand and the mining continues southward reducing hope of a thriving tourism-based economy. Problems caused by beach sand mining include: destruction of natural beaches and the ecosystems they protect (e.g. dunes, wetlands), habitat loss for globally important species (e.g. turtles, shorebirds), destruction of nearshore marine ecosystems, increased shoreline erosion rates, reduced protection from storms, tsunamis, and wave events, and economic losses through tourist abandonment and loss of coastal aesthetics. The threats posed by sand mining are made even more critical given the prospect of a

  14. Coastal morphology and dynamics of two beaches of Favignana

    International Nuclear Information System (INIS)

    Pasanisi, Francesco; Tebano, Carlo; Grauso, Sergio


    Results of an investigation on the coastal morphology and dynamics of two pocket beaches of Favignana, Cala Azzurra and Lido Burrone, are presented. Four detailed hydrographic surveys were performed using multibeam echo sounder with sidescan sonar and differential marine GPS. Surveys were repeated in different periods following the same navigation project. Moreover, incident wave climate and coastal hydrodynamics were investigated using state-of-the-art numerical models. Results of in-situ activities indicate little bathymetric variations among different surveys and suggest a substantial stability of submerged beach profiles limited to surveyed area. Slightly greater bathymetric changes and a generally more intense coastal dynamics were observed at Cala Azzurra compared to Lido Burrone. Simulations of wave propagation and nearshore circulation currents provided results consistent with field observations [it

  15. Development of a numerical 2-dimensional beach evolution model

    DEFF Research Database (Denmark)

    Baykal, Cüneyt


    This paper presents the description of a 2-dimensional numerical model constructed for the simulation of beach evolution under the action of wind waves only over the arbitrary land and sea topographies around existing coastal structures and formations. The developed beach evolution numerical model...... is composed of 4 submodels: a nearshore spectral wave transformation model based on an energy balance equation including random wave breaking and diffraction terms to compute the nearshore wave characteristics, a nearshore wave-induced circulation model based on the nonlinear shallow water equations...... to compute the nearshore depth-averaged wave-induced current velocities and mean water level changes, a sediment transport model to compute the local total sediment transport rates occurring under the action of wind waves, and a bottom evolution model to compute the bed level changes in time based...

  16. Bryophytes of beach forests in Chon Buri Province, Thailand

    Directory of Open Access Journals (Sweden)

    Phiangphak Sukkharak


    Full Text Available An investigation of bryophyte diversity of three beach forests including Had Tung Prong, Had Tein Talay, and the beach forest in Thai Island and Sea Natural History Museum in Chon Buri Province, Thailand, was carried out. From 137 enumerated specimens, 16 species (6 mosses, 10 liverworts in 12 genera (5 mosses, 7 liverworts and eight families (5 mosses, 3 liverworts were found. Among those the most common families of mosses are Fissidentaceae (2 species and the most common families of liverwort are Lejeuneaceae (8 species. A comparison of species richness among the three areas revealed that the highest species richness of bryophytes was found in Had Tung Prong. Moreover, of all bryophyte species found, Weissia edentula Mitt. was the most common one.

  17. Achieving Energy Savings in Municipal Construction in Long Beach California

    Energy Technology Data Exchange (ETDEWEB)



    Long Beach Gas and Oil (LBGO), the public gas utility in Long Beach, California, partnered with the U.S. Department of Energy (DOE) to develop and implement solutions to build a new, low-energy modular office building that is at least 50% below requirements set by Energy Standard 90.1-2007 of the American Society of Heating, Refrigerating, and Air-conditioning Engineers (ASHRAE), the American National Standards Institute (ANSI), and the Illuminating Engineering Society of America (IESNA) as part of DOE’s Commercial Building Partnerships (CBP) program. The LBGO building, which demonstrates that modular construction can be very energy efficient, is expected to exceed the ASHRAE baseline by about 45%.

  18. Modulation at Age of Onset in Tunisian Huntington Disease Patients: Implication of New Modifier Genes

    Directory of Open Access Journals (Sweden)

    Dorra Hmida-Ben Brahim


    Full Text Available Huntington’s disease (HD is an autosomal dominant neurodegenerative disorder. The causative mutation is an expansion of more than 36 CAG repeats in the first exon of IT15 gene. Many studies have shown that the IT15 interacts with several modifier genes to regulate the age at onset (AO of HD. Our study aims to investigate the implication of CAG expansion and 9 modifiers in the age at onset variance of 15 HD Tunisian patients and to establish the correlation between these modifiers genes and the AO of this disease. Despite the small number of studied patients, this report consists of the first North African study in Huntington disease patients. Our results approve a specific effect of modifiers genes in each population.

  19. Neuropsychological correlates of brain atrophy in Huntington's disease: a magnetic resonance imaging study

    International Nuclear Information System (INIS)

    Starkstein, S.E.; Brandt, J.; Bylsma, F.; Peyser, C.; Folstein, M.; Folstein, S.E.


    Magnetic resonance imaging and a comprehensive cognitive evaluation were carried out in a series of 29 patients with mild to moderate Huntington's disease (HD). A factor analysis of the neuropsychological test scores provided three factors: A memory/speed-of-processing factor, a 'frontal' factor, and a response inhibition factor. The memory/speed factor correlated significantly with measures of caudate atrophy, frontal atrophy, and atrophy of the left (but not the right) sylvian cistern. There were no significant correlations between the 'frontal' or response inhibition factors and measures of cortical or subcortical brain atrophy. Our findings confirm that subcortical atrophy is significantly correlated with specific cognitive deficits in HD, and demonstrate that cortical atrophy also has important association with the cognitive deficits of patients with HD. (orig.)

  20. Liver function in Huntington's disease assessed by blood biochemical analyses in a clinical setting

    DEFF Research Database (Denmark)

    Nielsen, Signe Marie Borch; Vinther-Jensen, Tua; Nielsen, Jørgen E.


    dysfunction are seen. Blood levels of a wide range of hormones, metabolites and proteins have been analyzed in HD patients, identifying several changes associated with the disease. However, a comprehensive panel of liver function tests (LFT) has not been performed. We investigated a cohort of manifest......Huntington's disease (HD) is a dominantly inherited, progressive neurological disorder caused by a CAG repeat elongation in the huntingtin gene. In addition to motor-, psychiatric- A nd cognitive dysfunction, peripheral disease manifestations in the form of metabolic changes and cellular...... and premanifest HD gene-expansion carriers and controls, using a clinically applied panel of LFTs. Here, we demonstrate that the level of alkaline phosphatase is increased in manifest HD gene-expansion carriers compared to premanifest HD gene-expansion carriers and correlate with increased disease severity...

  1. Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients. (United States)

    van Roon-Mom, Willeke M C; Roos, Raymund A C; de Bot, Susanne T


    On December 11 of 2017, Ionis Pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their HTTRx Phase 1/2a study in Huntington disease (HD) patients. The results from this Ionis trial have gained much attention from the patient community and the oligonucleotide therapeutics field, since it is the first trial targeting the cause of HD, namely the mutant huntingtin protein, using antisense oligonucleotides (ASOs). The press release also states that the primary endpoints of the study (safety and tolerability) were met, but does not contain data. This news follows the approval of another therapeutic ASO nusinersen (trade name Spinraza) for a neurological disease, spinal muscular atrophy, by the U.S. Food and Drug Administration and European Medicines Agency, in 2016 and 2017, respectively. Combined, this offers hope for the development of the HTTRx therapy for HD patients.

  2. Map showing selected surface-water data for the Huntington 30 x 60-minute quadrangle, Utah (United States)

    Price, Don


    This is one of a series of maps that describe the geology and related natural resources of the Huntington 30 x 60-minute quadrangle, Utah. Streamflow records used to compile this map were collected by the U.S. Geological Survey in cooperation with the Utah Department of Natural Resources, Division of Water Rights, and the Utah Department of Transportation. The principal runoff-producing area shown on the map was delineated from a work map (scale 1:250,000) compiled to estimate water yields in Utah (Bagley and others, 1964). Sources of information about recorded floods resulting from cloudbursts included Woolley (1946) and Butler and Marsell (1972); sources of information about the chemical quality of streamflow included Mundorff (1972) and Mundorff and Thompson (1982).

  3. Therapeutic strategies for circadian rhythm and sleep disturbances in Huntington disease. (United States)

    van Wamelen, Daniel J; Roos, Raymund Ac; Aziz, Nasir A


    Aside from the well-known motor, cognitive and psychiatric signs and symptoms, Huntington disease (HD) is also frequently complicated by circadian rhythm and sleep disturbances. Despite the observation that these disturbances often precede motor onset and have a high prevalence, no studies are available in HD patients which assess potential treatments. In this review, we will briefly outline the nature of circadian rhythm and sleep disturbances in HD and subsequently focus on potential treatments based on findings in other neurodegenerative diseases with similarities to HD, such as Parkinson and Alzheimer disease. The most promising treatment options to date for circadian rhythm and sleep disruption in HD include melatonin (agonists) and bright light therapy, although further corroboration in clinical trials is warranted.

  4. Age at onset in Huntington's disease: replication study on the association of HAP1. (United States)

    Karadima, Georgia; Dimovasili, Christina; Koutsis, Georgios; Vassilopoulos, Demetris; Panas, Marios


    In recent years two association studies investigating the HAP1 T441M (rs4523977) polymorphism as a potential modifying factor of the age at onset (AAO) of Huntington's disease (HD), have been reported. Initially evidence for association was found between the M441 risk allele and the AAO. Subsequently, a second study, although failing to replicate these findings, found evidence for association between the same risk allele and AAO of motor symptoms (mAAO). In the present study, the role of the HAP1 T441M polymorphism as a modifier of the AAO in HD was investigated in a cohort of 298 Greek HD patients. In this cohort the CAG repeat number accounted for 55% of the variance in AAO. No association was found between the HAP1 T441M polymorphism and the AAO of HD. © 2012 Elsevier Ltd. All rights reserved.

  5. Brain energy metabolism and dopaminergic function in Huntington's disease measured in vivo using positron emission tomography

    International Nuclear Information System (INIS)

    Leenders, K.L.; Frackowiak, R.S.; Quinn, N.; Marsden, C.D.


    A 48-year-old man with typical Huntington's disease was investigated with computed tomography (CT) and positron emission tomography. Regional cerebral blood flow, oxygen extraction, oxygen and glucose utilization, L-Dopa uptake, and dopamine (D2) receptor binding were measured using several positron-labelled tracers. CT showed slight atrophy of the head of caudate but no cortical atrophy, although distinct frontal lobe dysfunction was present on psychometric testing. Oxygen and glucose metabolism and cerebral blood flow were decreased in the striata and to a lesser extent in frontal cortex. Cerebral blood flow was in the low normal range throughout the remainder of the brain. A normal metabolic ratio was found in all regions, since the changes in glucose utilization paralleled those in oxygen consumption. The capacity of the striatum to store dopamine as assessed by L-[ 18 F]-fluorodopa uptake was normal, but dopamine (D2) receptor binding was decreased when compared to normal subjects

  6. Tics as an initial manifestation of juvenile Huntington's disease: case report and literature review. (United States)

    Cui, Shi-Shuang; Ren, Ru-Jing; Wang, Ying; Wang, Gang; Chen, Sheng-Di


    Huntington's disease (HD) is an autosomal dominant disorder, typically characterized by chorea due to a trinucleotide repeat expansion in the HTT gene, although the clinical manifestations of patients with juvenile HD (JHD) are atypical. A 17-year-old boy with initial presentation of tics attended our clinic and his DNA analysis demonstrated mutation in the HTT gene (49 CAG repeats). After treatment, his symptoms improved. Furthermore, we performed literature review through searching the databases and summarized clinical features in 33 JHD patients. The most prevalent symptoms are ataxia, and two cases reported that tics as initial and prominent manifestation in JHD. Among them, 88% patients carried CAG repeats beyond 60 and most of them have family history. This case here illustrates the variable range of clinical symptoms of JHD and the necessity of testing for the HD mutation in young patients with tics with symptoms unable to be explained by Tourette's syndrome (TS).

  7. Social Cognition, Executive Functions and Self-Report of Psychological Distress in Huntington's Disease

    DEFF Research Database (Denmark)

    Larsen, Ida Unmack; Vinther-Jensen, Tua; Nielsen, Jørgen Erik


    OBJECTIVE: Huntington's disease (HD) is characterized by motor symptoms, psychiatric symptoms and cognitive impairment in, inter alia, executive functions and social cognition. The aim of this study was to investigate the relationship between subjective feeling of psychological distress using...... a self-report questionnaire and performances on tests of executive functions and social cognition in a large consecutive cohort of HD patients. METHOD: 50 manifest HD patients were tested in social cognition and executive functions and each answered a self-report questionnaire about current status...... psychological distress was significantly associated with worse performances on social cognitive tests (mean absolute correlation .34) and that there were no significant correlations between perceived psychological distress and performance on tests of executive functions. The correlations between perceived...

  8. Oxidative Stress and Huntington's Disease: The Good, The Bad, and The Ugly. (United States)

    Kumar, Amit; Ratan, Rajiv R


    Redox homeostasis is crucial for proper cellular functions, including receptor tyrosine kinase signaling, protein folding, and xenobiotic detoxification. Under basal conditions, there is a balance between oxidants and antioxidants. This balance facilitates the ability of oxidants, such as reactive oxygen species, to play critical regulatory functions through a direct modification of a small number of amino acids (e.g. cysteine) on signaling proteins. These signaling functions leverage tight spatial, amplitude, and temporal control of oxidant concentrations. However, when oxidants overwhelm the antioxidant capacity, they lead to a harmful condition of oxidative stress. Oxidative stress has long been held to be one of the key players in disease progression for Huntington's disease (HD). In this review, we will critically review this evidence, drawing some intermediate conclusions, and ultimately provide a framework for thinking about the role of oxidative stress in the pathophysiology of HD.

  9. Aerial gamma ray and magnetic survey, Huntington quadrangle: Ohio, West Virginia and Kentucky. Final report

    International Nuclear Information System (INIS)


    The Huntington quadrangle of Kentucky, Ohio, and West Virginia covers 7250 square miles of the easternmost Midwestern Physiographic Province. Paleozoic exposures dominate the surface. These Paleozoics deepen toward the east from approximately 500 feet to a maximum depth of 8000 feet. Precambrian basement is thought to underlie the entire area. No known uranium deposits exist in the area. One hundred anomalies were found using the standard statistical analysis. Some high uranium concentration anomalies that may overlie the stratigraphic equivalent of the Devonian-Mississippian New Albany or Chattanooga Shales may represent significant levels of naturally occurring uranium. Future studies should concentrate on this unit. Magnetic data are largely in concurrence with existing structural interpretations but suggest some complexities in the underlying Precambrian

  10. Is gardening a stimulating activity for people with advanced Huntington's disease? (United States)

    Spring, Josephine A; Viera, Marc; Bowen, Ceri; Marsh, Nicola


    This study evaluated adapted gardening as an activity for people with advanced Huntington's disease (HD) and explored its therapeutic aspects. Visitors and staff completed a questionnaire and participated in structured interviews to capture further information, whereas a pictorial questionnaire was designed for residents with communication difficulties. Staff reported that gardening was a constructive, outdoor activity that promoted social interaction, physical activity including functional movement and posed cognitive challenges. Half the staff thought the activity was problem free and a third used the garden for therapy. Visitors used the garden to meet with residents socially. Despite their disabilities, HD clients enjoyed growing flourishing flowers and vegetables, labelling plants, being outside in the sun and the quiet of the garden. The garden is valued by all three groups. The study demonstrates the adapted method of gardening is a stimulating and enjoyable activity for people with advanced HD. © The Author(s) 2013 Reprints and permissions:

  11. Gardening with Huntington's disease clients--creating a programme of winter activities. (United States)

    Spring, Josephine Anne; Baker, Mark; Dauya, Loreane; Ewemade, Ivie; Marsh, Nicola; Patel, Prina; Scott, Adrienne; Stoy, Nicholas; Turner, Hannah; Viera, Marc; Will, Diana


    A programme of garden-related indoor activities was developed to sustain a gardening group for people with mid to late stage Huntington's disease during the winter. The activities were devised by the horticulturist, working empirically, involving the services occupational therapist, physiotherapist, occupational therapy art technician, computer room, recreation and leisure staff. The programme was strongly supported by the nursing and care staff. Feedback on the effectiveness of the activities was sought from the clients, team members and unit staff. The clients' interest in gardening was sustained by a multidisciplinary programme of indoor growing and using plant products in creative activities, computing and group projects. The clients enjoyed all activities except one that they said lacked contact with plants. The inexpensive programme of activities enabled creativity and self-expression, stimulated social contact and helped with therapeutic goals of the clients. In addition, it engaged the multi-disciplinary team and the unit staff, was practical and enhanced the environment.

  12. Beach-ridge architecture constrained by beach topography and Ground-Penetrating Radar, Itilleq (Lakse Bugt), Disko, Greenland – Implications for sea-level reconstructions

    DEFF Research Database (Denmark)

    Emerich Souza, Priscila; Kroon, Aart; Nielsen, Lars


    Detailed topographical data and high-resolution ground-penetrating radar (GPR) reflection data are presented from the present-day beach and across successive raised beach-ridges at Itilleq (Disko, West Greenland). In the western part of our study area, the present low-tide level is well-marked by......Detailed topographical data and high-resolution ground-penetrating radar (GPR) reflection data are presented from the present-day beach and across successive raised beach-ridges at Itilleq (Disko, West Greenland). In the western part of our study area, the present low-tide level is well...... beach-ridge GPR profiles. Most of them are located at the boundary between a unit with reflection characteristics representing palaeo foreshore deposits, and a deeper and more complex radar unit characterized by diffractions, which, however, is not penetrated to large depths by the GPR signals. Based...

  13. Vertical distribution of meiofauna on reflective sandy beaches

    Directory of Open Access Journals (Sweden)

    Mariana de Oliveira Martins


    Full Text Available Abstract Extreme physical conditions usually limit the meiofauna occurrence and distribution in highly hydrodynamic environments such as reflective beaches. Despite sediment grains of the upper layers being constantly resuspended and deposited, the high energy of the swash zone besides depositing coarse sediments allows an ample vertical distribution of meiofaunal organisms. The effect of physical, chemical and sediment variables on the vertical distribution of meiofaunal organims and nematodes was analysed on two reflective exposed beaches. Sampling was conducted at three sampling points on each beach in the swash zone. The sediment collected was divided into four 10-cm strata (0-10 cm, 10-20 cm, 20-30 cm, 30-40 cm. The statistical differences between strata due to factors previously established (i.e. meiofaunal composition, density of most abundant taxa were tested using a hierarchical PERMANOVA applied under similarity and euclidian distances. An inverse relation among average grain size, content of organic matter and sediment sorting was evident. Coarser sediment characterized the upper layers, while at deeper layers the sediment was very poorly sorted and presented a higher content of organic matter. A similar pattern in the vertical distribution of meiofaunal and nematofaunal composition and density was detected. The lowest densities were associated with the first stratum (0-10 cm, highly affected by hydrodynamics. The vertical distribution of organisms was statistically different only when the interaction among factors was considered. This result suggests that zonation and vertical distribution of meiofaunal organisms are determined by the within-beach variability.

  14. Identifying Preserved Storm Events on Beaches from Trenches and Cores (United States)

    Wadman, H. M.; Gallagher, E. L.; McNinch, J.; Reniers, A.; Koktas, M.


    Recent research suggests that even small scale variations in grain size in the shallow stratigraphy of sandy beaches can significantly influence large-scale morphology change. However, few quantitative studies of variations in shallow stratigraphic layers, as differentiated by variations in mean grain size, have been conducted, in no small part due to the difficulty of collecting undisturbed sediment cores in the energetic lower beach and swash zone. Due to this lack of quantitative stratigraphic grain size data, most coastal morphology models assume that uniform grain sizes dominate sandy beaches, allowing for little to no temporal or spatial variations in grain size heterogeneity. In a first-order attempt to quantify small-scale, temporal and spatial variations in beach stratigraphy, thirty-five vibracores were collected at the USACE Field Research Facility (FRF), Duck, NC, in March-April of 2014 using the FRF's Coastal Research and Amphibious Buggy (CRAB). Vibracores were collected at set locations along a cross-shore profile from the toe of the dune to a water depth of ~1m in the surf zone. Vibracores were repeatedly collected from the same locations throughout a tidal cycle, as well as pre- and post a nor'easter event. In addition, two ~1.5m deep trenches were dug in the cross-shore and along-shore directions (each ~14m in length) after coring was completed to allow better interpretation of the stratigraphic sequences observed in the vibracores. The elevations of coherent stratigraphic layers, as revealed in vibracore-based fence diagrams and trench data, are used to relate specific observed stratigraphic sequences to individual storm events observed at the FRF. These data provide a first-order, quantitative examination of the small-scale temporal and spatial variability of shallow grain size along an open, sandy coastline. The data will be used to refine morphological model predictions to include variations in grain size and associated shallow stratigraphy.

  15. Markkinointisuunnitelma digitaaliseen markkinointiin Case Baan Lee Beach Resort & Sauna


    Lähteenmäki, Essi


    Tämän opinnäytetyön tavoitteena on kehittää Baan Lee Beach Resort & Saunan digitaalista markkinointia markkinointisuunnitelman avulla. Yritys on aloittamassa liiketoimintaansa erittäin kilpailulla alalla, jossa digitaalinen markkinointi on tärkeä osa kilpailussa menestymistä ja se on otettava huomioon yrityksen markkinointistrategian tärkeänä osana. Markkinointisuunnitelmalla pyritään takaamaan markkinointistrategian mukainen toiminta. Markkinointisuunnitelmassa kuvataan yrityksen nykyti...

  16. The functional implications of motor, cognitive, psychiatric, and social problem-solving states in Huntington's disease. (United States)

    Van Liew, Charles; Gluhm, Shea; Goldstein, Jody; Cronan, Terry A; Corey-Bloom, Jody


    Huntington's disease (HD) is a genetic, neurodegenerative disorder characterized by motor, cognitive, and psychiatric dysfunction. In HD, the inability to solve problems successfully affects not only disease coping, but also interpersonal relationships, judgment, and independent living. The aim of the present study was to examine social problem-solving (SPS) in well-characterized HD and at-risk (AR) individuals and to examine its unique and conjoint effects with motor, cognitive, and psychiatric states on functional ratings. Sixty-three participants, 31 HD and 32 gene-positive AR, were included in the study. Participants completed the Social Problem-Solving Inventory-Revised: Long (SPSI-R:L), a 52-item, reliable, standardized measure of SPS. Items are aggregated under five scales (Positive, Negative, and Rational Problem-Solving; Impulsivity/Carelessness and Avoidance Styles). Participants also completed the Unified Huntington's Disease Rating Scale functional, behavioral, and cognitive assessments, as well as additional neuropsychological examinations and the Symptom Checklist-90-Revised (SCL-90R). A structural equation model was used to examine the effects of motor, cognitive, psychiatric, and SPS states on functionality. The multifactor structural model fit well descriptively. Cognitive and motor states uniquely and significantly predicted function in HD; however, neither psychiatric nor SPS states did. SPS was, however, significantly related to motor, cognitive, and psychiatric states, suggesting that it may bridge the correlative gap between psychiatric and cognitive states in HD. SPS may be worth assessing in conjunction with the standard gamut of clinical assessments in HD. Suggestions for future research and implications for patients, families, caregivers, and clinicians are discussed.

  17. Somatostatin receptor 1 and 5 double knockout mice mimic neurochemical changes of Huntington's disease transgenic mice.

    Directory of Open Access Journals (Sweden)

    Padmesh S Rajput

    Full Text Available Selective degeneration of medium spiny neurons and preservation of medium sized aspiny interneurons in striatum has been implicated in excitotoxicity and pathophysiology of Huntington's disease (HD. However, the molecular mechanism for the selective sparing of medium sized aspiny neurons and vulnerability of projection neurons is still elusive. The pathological characteristic of HD is an extensive reduction of the striatal mass, affecting caudate putamen. Somatostatin (SST positive neurons are selectively spared in HD and Quinolinic acid/N-methyl-D-aspartic acid induced excitotoxicity, mimic the model of HD. SST plays neuroprotective role in excitotoxicity and the biological effects of SST are mediated by five somatostatin receptor subtypes (SSTR1-5.To delineate subtype selective biological responses we have here investigated changes in SSTR1 and 5 double knockout mice brain and compared with HD transgenic mouse model (R6/2. Our study revealed significant loss of dopamine and cAMP regulated phosphoprotein of 32 kDa (DARPP-32 and comparable changes in SST, N-methyl-D-aspartic acid receptors subtypes, calbindin and brain nitric oxide synthase expression as well as in key signaling proteins including calpain, phospho-extracellular-signal-regulated kinases1/2, synapsin-IIa, protein kinase C-α and calcineurin in SSTR1/5(-/- and R6/2 mice. Conversely, the expression of somatostatin receptor subtypes, enkephalin and phosphatidylinositol 3-kinases were strain specific. SSTR1/5 appears to be important in regulating NMDARs, DARPP-32 and signaling molecules in similar fashion as seen in HD transgenic mice.This is the first comprehensive description of disease related changes upon ablation of G- protein coupled receptor gene. Our results indicate that SST and SSTRs might play an important role in regulation of neurodegeneration and targeting this pathway can provide a novel insight in understanding the pathophysiology of Huntington's disease.

  18. R6/2 Huntington's disease mice develop early and progressive abnormal brain metabolism and seizures. (United States)

    Cepeda-Prado, Efrain; Popp, Susanna; Khan, Usman; Stefanov, Dimitre; Rodríguez, Jorge; Menalled, Liliana B; Dow-Edwards, Diana; Small, Scott A; Moreno, Herman


    A hallmark feature of Huntington's disease pathology is the atrophy of brain regions including, but not limited to, the striatum. Though MRI studies have identified structural CNS changes in several Huntington's disease (HD) mouse models, the functional consequences of HD pathology during the progression of the disease have yet to be investigated using in vivo functional MRI (fMRI). To address this issue, we first established the structural and functional MRI phenotype of juvenile HD mouse model R6/2 at early and advanced stages of disease. Significantly higher fMRI signals [relative cerebral blood volumes (rCBVs)] and atrophy were observed in both age groups in specific brain regions. Next, fMRI results were correlated with electrophysiological analysis, which showed abnormal increases in neuronal activity in affected brain regions, thus identifying a mechanism accounting for the abnormal fMRI findings. [(14)C] 2-deoxyglucose maps to investigate patterns of glucose utilization were also generated. An interesting mismatch between increases in rCBV and decreases in glucose uptake was observed. Finally, we evaluated the sensitivity of this mouse line to audiogenic seizures early in the disease course. We found that R6/2 mice had an increased susceptibility to develop seizures. Together, these findings identified seizure activity in R6/2 mice and show that neuroimaging measures sensitive to oxygen metabolism can be used as in vivo biomarkers, preceding the onset of an overt behavioral phenotype. Since fMRI-rCBV can also be obtained in patients, we propose that it may serve as a translational tool to evaluate therapeutic responses in humans and HD mouse models.

  19. Spectral responses of gravel beaches to tidal signals (United States)

    Geng, Xiaolong; Boufadel, Michel C.


    Tides have been recognized as a major driving forcing affecting coastal aquifer system, and deterministic modeling has been very effective in elucidating mechanisms caused by tides. However, such modeling does not lend itself to capture embedded information in the signal, and rather focuses on the primary processes. Here, using yearlong data sets measured at beaches in Alaska Prince William Sound, we performed spectral and correlation analyses to identify temporal behavior of pore-water pressure, temperature and salinity. We found that the response of the beach system was characterized by fluctuations of embedded diurnal, semidiurnal, terdiurnal and quarterdiurnal tidal components. Hydrodynamic dispersion of salinity and temperature, and the thermal conductivity greatly affected pore water signals. Spectral analyses revealed a faster dissipation of the semi-diurnal component with respect to the diurnal components. Correlation functions showed that salinity had a relatively short memory of the tidal signal when inland freshwater recharge was large. In contrast, the signature of the tidal signal on pore-water temperature persisted for longer times, up to a week. We also found that heterogeneity greatly affected beach response. The response varied from a simple linear mapping in the frequency domain to complete modulation and masking of the input frequencies.

  20. Microplastic resin pellets on an urban tropical beach in Colombia. (United States)

    Acosta-Coley, Isabel; Olivero-Verbel, Jesus


    Microplastics are a problem in oceans worldwide. The current situation in Latin America is not well known. This paper reports, for the first time, the presence of microplastics on an urban Caribbean beach in Cartagena, Colombia. Pellet samples were collected from a tourist beach over a 5-month period covering both dry and rainy seasons. Pellets were classified by color and their surface analyzed by stereomicroscopy, and some were characterized by infrared spectroscopy. The most abundant pellets were white, presenting virgin surfaces, with few signs of oxidation. This is congruent with a short residence time in the marine environment and primary sources possibly located nearby. The frequency of white pellets did not change with sampling period. Surface features identified in the pellets included cracks, material loss, erosion, adhesion, granulation, color change, and glazed surfaces. Reticulated granular pellets exhibited the greatest degradation, easily generating submicroplastics. Sample composition was mostly polyethylene, followed by polypropylene. This pollution problem must be addressed by responsible authorities to avoid pellet deposition in oceans and on beaches around the world.

  1. Biorock Electric Reefs Grow Back Severely Eroded Beaches in Months

    Directory of Open Access Journals (Sweden)

    Thomas J. F. Goreau


    Full Text Available Severely eroded beaches on low lying islands in Indonesia were grown back in a few months—believed to be a record—using an innovative method of shore protection, Biorock electric reef technology. Biorock shore protection reefs are growing limestone structures that get stronger with age and repair themselves, are cheaper than concrete or rock sea walls and breakwaters, and are much more effective at shore protection and beach growth. Biorock reefs are permeable, porous, growing, self-repairing structures of any size or shape, which dissipate wave energy by internal refraction, diffraction, and frictional dissipation. They do not cause reflection of waves like hard sea walls and breakwaters, which erodes the sand in front of, and then underneath, such structures, until they collapse. Biorock reefs stimulate settlement, growth, survival, and resistance to the environmental stress of all forms of marine life, restoring coral reefs, sea grasses, biological sand production, and fisheries habitat. Biorock reefs can grow back eroded beaches and islands faster than the rate of sea level rise, and are the most cost-effective method of shore protection and adaptation to global sea level rise for low lying islands and coasts.

  2. Coastal geomorphological study of pocket beaches in Crete, with the use of planview indices. (United States)

    Alexandrakis, George; Karditsa, Aikaterini; Poulos, Serafim; Kampanis, Nikos


    The formation of pocket beaches is a result of a large number of processes and mechanisms that vary on space and time scales. This study aims in defining the planform characteristics of pocket beaches in Crete Isl. and to determine their sheltering effect, embaymentization and their status of equilibrium. Thus, data from 30 pocket beaches along the coastline of Crete, with different geomorphological and hydrodynamical setting, were collected. Planform parameters were applied and coastal planview indices from the bibliography were applied. The parameters included: length and orientation of the headlands between the pocket beach; length between the bay entrance and the center of the beach; lengths of the i) embayed shoreline, ii) embayed beach, iii) beach segment located at the shadow of a headland; linear distance and orientation between the edges of the embayed beach; direction of the incident wave energy flux; wave crest obliquity to the control line; beach area, maximum beach width and headland orientation and river/ torrent catchment areas in beach zones that an active river system existed (Bowman et al.2009). For the morphological mapping of the study areas, 1:5000 orthophoto maps were used. Wave regime has been calculated with the use of prognostic equations and utilising local wind data (mean annual frequency of wind speed and direction), provided by the Wind and Wave Atlas of the Eastern Mediterranean Sea. The diffraction and refraction of the waves has been simulated with the use of numerical models. The study shows that Cretan pocket beaches display a wide range of indentation, suggesting that is the result of several parameters that include tectonics, coastal hydrodynamics and river catchment areas. The more indented bays are, the shorter their beaches become, while low-indented pocket beaches are the widest and the longest ones. Beaches with headland with large length appear to be more protected and receive smaller amount of wave energy. Most of the


    International Development Research Centre (IDRC) Digital Library (Canada)

    et des enfants d'Afrique. INITIATIVE CONCERTÉE. Innovation pour la santé des mères et des enfants d'Afrique. Centre de recherches pour le développement international. CP Box 8500 Ottawa ON Canada K1G 3H9. Téléphone : +1 613 236 6163 • Télécopieur : +1 613 657 7749 |

  4. Beach dynamics and nest distribution of the leatherback turtle (Dermochelys coriacea at Grande Riviere Beach,Trinidad &Tobago

    Directory of Open Access Journals (Sweden)

    Lori Lee Lum


    Full Text Available Grande Riviere Beach in Trinidad and Tobago is an important nesting site in the Caribbean for the Critically Endangered leatherback sea turtle,Dermochelys coriacea .Community members were concerned that beach erosion and seasonal river flooding were destroying many of the nests deposited annually and thought that a hatchery was a possible solution.Over the 2001 turtle nesting season,the Institute of Marine Affairs (IMA assessed the spatial and temporal distribution of nests using the Global Positioning System recorded to reference points,and beach dynamics using permanent bench mark profile stations,to determine areas of high risk and more stable areas for nesting.A total of 1449 leatherback nests were positioned.It was evident that at the start of the season in March,the majority of leatherback nests were deposited at the eastern section of the beach. After May,there was a continuing westward shift in nest distribution as the season progressed until August and beach erosion in the eastern section became predominant.The backshore remained relatively stable along the entire beach throughout the nesting season,and erosion was predominant in the foreshore at the eastern section of the beach,from the middle to the end of the season.Similar trends in accretion and erosion were observed in 2000.River flooding did not occur during the study period or in the previous year.With both high risk and more stable regions for turtle nesting available at Grande Riviere Beach,there was no compelling evidence to justify the need for a hatchery.La playa de Grande Riviere en Trinidad y Tobago es un sitio importante de anidación en el Caribe de la tortuga baula,Dermochelys coriacea ;una tortuga marina en peligro de extinción.Los residentes estaban preocupados de que la erosión de la playa y las inundaciones estacionales estaban destruyendo muchos de los nidos y pensaron que un criadero era una solución.Durante la temporada de anidación del 2001,el Instituto de


    Directory of Open Access Journals (Sweden)

    Shahdevi Nandar Kurniawan


    Full Text Available Ca2+ signaling functions to regulate many cellular processes. Dynamics of Ca2+ signaling or homeostasis is regulated by the interaction between ON and OFF reactions that control Ca2+ flux in both the plasma membrane and internal organelles such as the endoplasmic reticulum (ER and mitochondria. External stimuli activate the ON reactions, which include Ca2+ into the cytoplasm either through channels in the plasma membrane or from internal storage like in ER. Most of the cells utilize both channels/sources, butthere area few cells using an external or internal source to control certain processes. Most of the Ca2+ entering the cytoplasm adsorbed to the buffer, while a smaller part activate effect or to stimulate cellular processes. Reaction OFF is pumping of cytoplasmic Ca2+ using a combination mechanism of mitochondrial and others. Changes in Ca2+ signal has been detected in various tissues isolated from animals induced into diabetes as well as patients with diabetes. Ca2+ signal interference is also found in sensory neurons of experimental animals with diabetes. Ca2+ signaling is one of the main signaling systems in the cell.

  6. Huntington's Disease (United States)

    ... monitor a disease) for HD. A large and related NINDS-supported study aims to identify additional genetic factors in people that influence the course of the disease. Other research hopes to identify variations in the genomes of individuals with HD that may point to new targets ...

  7. Comparing different methods for fast screening of microbiological quality of beach sand aimed at rapid-response remediation. (United States)

    Testolin, Renan C; Almeida, Tito C M; Polette, Marcus; Branco, Joaquim O; Fischer, Larissa L; Niero, Guilherme; Poyer-Radetski, Gabriel; Silva, Valéria C; Somensi, Cleder A; Corrêa, Albertina X R; Corrêa, Rogério; Rörig, Leonardo R; Itokazu, Ana Gabriela; Férard, Jean-François; Cotelle, Sylvie; Radetski, Claudemir M


    There is scientific evidence that beach sands are a significant contributor to the pathogen load to which visitors are exposed. To develop beach quality guidelines all beach zones must be included in microbiological evaluations, but monitoring methods for beach sand quality are relatively longstanding, expensive, laborious and require moderate laboratory infrastructure. This paper aimed to evaluate the microorganism activity in different beach zones applying and comparing a classical method of membrane filtration (MF) with two colorimetric screening methods based on fluorescein (FDA) and tetrazolium (TTC) salt biotransformation to evaluate a new rapid and low-cost method for beach sand microbiological contamination assessments. The colorimetric results can help beach managers to evaluate rapidly and at low cost the microbiological quality of different beach zones in order to decide whether remedial actions need to be adopted to prevent exposure of the public to microbes due to beach sand and/or water contamination. Copyright © 2017. Published by Elsevier Ltd.

  8. Sandy berm and beach-ridge formation in relation to extreme sea-levels

    DEFF Research Database (Denmark)

    Bendixen, Mette; Clemmensen, Lars B; Kroon, Aart


    The formation of berms and their transformation into beach ridges in a micro-tidal environment is coupled to wave run-up and overtopping during extreme sea levels. A straight-forward comparison between extreme sea levels due to storm-surges and active berm levels is impossible in the semi...... prograding spit on the south-eastern Baltic shores of Zealand, Denmark. The modern, sandy beach at this location consists of a beachface with a shallow incipient berm, a mature berm, and a dune-covered beach ridge. It borders a beach-ridge plain to the west, where more than 20 N–S oriented beach ridges...... and swales are present. Measured water-level data from 1991 to 2012 and topographical observations, carried out during fair weather period and during a storm event, provided the basis for a conceptual model exhibiting berm formation and transformation into the local beach-ridge system. The character...

  9. Research on pathogens at Great Lakes beaches: sampling, influential factors, and potential sources (United States)



    The overall mission of this work is to provide science-based information and methods that will allow beach managers to more accurately make beach closure and advisory decisions, understand the sources and physical processes affecting beach contaminants, and understand how science-based information can be used to mitigate and restore beaches and protect the public. The U.S. Geological Survey (USGS), in collaboration with many Federal, State, and local agencies and universities, has conducted research on beach health issues in the Great Lakes Region for more than a decade. The work consists of four science elements that align with the USGS Beach Health Initiative Mission: real-time assessments of water quality; coastal processes; pathogens and source tracking; and data analysis, interpretation, and communication. The ongoing or completed research for the pathogens and source tracking topic is described in this fact sheet.

  10. Bacteriological Monitoring and Sustainable Management of Beach Water Quality in Malaysia: Problems and Prospects (United States)

    Dada, Ayokunle Christopher; Asmat, Ahmad; Gires, Usup; Heng, Lee Yook; Deborah, Bandele Oluwaseun


    Despite the growing demand of tourism in Malaysia, there are no resolute efforts to develop beaches as tourist destinations. With no incentives to monitor public beaches or to use them in a sustainable manner, they might eventually degenerate in quality as a result of influx of pollutants. This calls for concerted action plans with a view to promoting their sustainable use. The success of such plans is inevitably anchored on the availability of robust quality monitoring schemes. Although significant efforts have been channelled to collation and public disclosure of bacteriological quality data of rivers, beach water monitoring appears left out. This partly explains the dearth of published information related to beach water quality data. As part of an on-going nation-wide surveillance study on the bacteriological quality of recreational beaches, this paper draws on a situation analysis with a view to proffering recommendations that could be adapted for ensuring better beach water quality in Malaysia. PMID:22980239

  11. Bacteriological monitoring and sustainable management of beach water quality in Malaysia: problems and prospects. (United States)

    Dada, Ayokunle Christopher; Asmat, Ahmad; Gires, Usup; Heng, Lee Yook; Deborah, Bandele Oluwaseun


    Despite the growing demand of tourism in Malaysia, there are no resolute efforts to develop beaches as tourist destinations. With no incentives to monitor public beaches or to use them in a sustainable manner, they might eventually degenerate in quality as a result of influx of pollutants. This calls for concerted action plans with a view to promoting their sustainable use. The success of such plans is inevitably anchored on the availability of robust quality monitoring schemes. Although significant efforts have been channelled to collation and public disclosure of bacteriological quality data of rivers, beach water monitoring appears left out. This partly explains the dearth of published information related to beach water quality data. As part of an on-going nation-wide surveillance study on the bacteriological quality of recreational beaches, this paper draws on a situation analysis with a view to proffering recommendations that could be adapted for ensuring better beach water quality in Malaysia.

  12. Cardiovascular consequence of reclining vs. sitting beach-chair body position for induction of anesthesia

    DEFF Research Database (Denmark)

    Larsen, Soren L.; Lyngeraa, Tobias S.; Maschmann, Christian P.


    The sitting beach-chair position is regularly used for shoulder surgery and anesthesia may be induced in that position. We tested the hypothesis that the cardiovascular challenge induced by induction of anesthesia is attenuated if the patient is placed in a reclining beach-chair position....... Anesthesia was induced with propofol in the sitting beach-chair (n = 15) or with the beach-chair tilted backwards to a reclining beach-chair position (n = 15). The last group was stepwise tilted to the sitting beach-chair position prior to surgery. Hypotension was treated with ephedrine. Continuous...... ± 12 vs. 45 ± 15 % reduction from baseline, p = 0.04) and ScO2 (7 ± 6 vs. 1 ± 8% increase from baseline, p = 0.02) and received less ephedrine (mean: 4 vs. 13 mg, p = 0.048). The higher blood pressure and lower need of vasopressor following induction of anesthesia in the reclining compared...

  13. Morphdynamics of Beaches in the Tróia-Sines Littoral Ribbon (SW Portugal)


    Gama, Cristina; Andrade, César; Taborda, Rui; Freitas, Conceição


    In the Tróia-Sines littoral ribbon five beaches were monitored in order to evaluate morphological and textural changes. The textural analysis reveals a southward coarsening trend that reflects an increase in the wave energy. The morphodynamic data indicate that the modal stages are intermediate to reflective, and that the available beach volume increases southwards. During storm periods the volumetric changes reach 15% to 82% of the beach envelope corresponding to magnitudes of 6x10 3 to 2x10...

  14. Limits of Wave Runup and Corresponding Beach-Profile Change from Large-Scale Laboratory Data (United States)


    A nearly vertical scarp developed after 40 min of wave action, with the upper limit of beach change identified at the toe of the dune scarp. and...change UL was found to approximately equal the vertical excursion of total wave runup, Rtw. An exception was runs where beach or dune scarps were...approximately equal the vertical excursion of total wave runup, Rtw. An exception was runs where beach or dune scarps were produced, which substantially limit the

  15. Environmental factors controlling macrofaunal assemblages on six microtidal beaches of the Ligurian Sea (NW Mediterranean) (United States)

    Covazzi Harriague, Anabella; Albertelli, Giancarlo


    Six microtidal beaches along the Ligurian coast (NW Mediterranean, Italy) were sampled in order to study their macrofaunal assemblages. All six beaches are subject to heavy tourism in the swimming season and three were subject to nourishment activities during the study period (May 2000). The beaches of Lavagna, Varazze and Pietra Ligure were sampled three times: before the nourishment and the onset of the swimming season (March 2000), after the nourishment (June 2000) and at the end of the swimming season (October 2000). The beaches of Varigotti, Albisola and Loano were sampled twice: before and after the swimming season (March and October 2000, respectively). Sampling was performed along two transects (T1 and T2), about 500 m apart, each transect having three sampling stations: one placed in the swash zone, one in the surf zone and one in the subtidal zone (depth of 3-5 m), in order to verify how far the nourishment material reached. The beaches were characterised by coarse sediments that became finer towards the sub-littoral station. The Beach Deposit Index and Beach Index classified the beaches as reflective (Lavagna, Varazze, Albisola and Varigotti) or intermediate (Pietra Ligure and Loano). Species richness showed a clearly increasing pattern from the swash zone (average 7) to the subtidal zone (average 103). The beach communities were dominated by polychaetes, in particular Saccocirrus papillocercus, which was mainly responsible for the dissimilarity between the beach and subtidal stations. The highest abundance was observed at the surf station (average 118.6 ind. m -2) and the lowest at the subtidal station (average 82.1 ind. m -2). The sediment composition and macrofaunal assemblages were not affected by the beach nourishment. The beach communities responded to different environmental descriptors: species richness seemed to be governed by environmental harshness, while abundance seemed to be linked to the degree of homogeneity of the sediments and the

  16. The STS-95 crew participates in a parade in Cocoa Beach (United States)


    STS-95 Commander Curtis L. Brown Jr. (in front), along with the other crew members behind him, waves to the crowd as he leads a parade of 1999 C-5 Corvette convertibles down State Road A1A in nearby Cocoa Beach. Organizers of the parade include the Cocoa Beach Area Chamber of Commerce, the Brevard County Tourist Development Council, and the cities of Cape Canaveral and Cocoa Beach. The parade is reminiscent of those held after missions during the Mercury Program.

  17. STS-95 Payload Specialist Glenn greets baseball legend Williams following a parade in Cocoa Beach (United States)


    STS-95 Payload Specialist John H. Glenn Jr. (left) greets baseball legend Ted Williams at a reception at the Double Tree Oceanfront Hotel following a parade down State Road A1A in nearby Cocoa Beach. Organizers of the parade included the Cocoa Beach Area Chamber of Commerce, the Brevard County Tourist Development Council, and the cities of Cape Canaveral and Cocoa Beach. The parade is reminiscent of those held after missions during the Mercury Program.

  18. STS-95 Payload Specialist Mukai participates in a parade in Cocoa Beach (United States)


    STS-95 Payload Specialist Chiaki Mukai is perched on the back of a red 1999 C-5 Corvette convertible during a parade down State Road A1A in nearby Cocoa Beach. Organizers of the parade include the Cocoa Beach Area Chamber of Commerce, the Brevard County Tourist Development Council, and the cities of Cape Canaveral and Cocoa Beach. The parade is reminiscent of those held after missions during the Mercury Program.

  19. STS-95 Payload Specialist Glenn participates in a parade in Cocoa Beach (United States)


    STS-95 Payload Specialist John H. Glenn Jr. waves to a dense crowd of well-wishers from the back of a silver 1999 C-5 Corvette convertible during a parade down State Road A1A in nearby Cocoa Beach. Organizers of the parade include the Cocoa Beach Area Chamber of Commerce, the Brevard County Tourist Development Council, and the cities of Cape Canaveral and Cocoa Beach. The parade is reminiscent of those held after missions during the Mercury Program.

  20. Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure? (United States)

    Morea, Veronica; Bidollari, Eris; Colotti, Gianni; Fiorillo, Annarita; Rosati, Jessica; De Filippis, Lidia; Squitieri, Ferdinando; Ilari, Andrea


    Huntington's disease (HD) or Huntington's chorea is the most common inherited, dominantly transmitted, neurodegenerative disorder. It is caused by increased CAG repeats number in the gene coding for huntingtin (Htt) and characterized by motor, behaviour and psychiatric symptoms, ultimately leading to death. HD patients also exhibit alterations in glucose and energetic metabolism, which result in pronounced weight loss despite sustained calorie intake. Glucose metabolism decreases in the striatum of all the subjects with mutated Htt, but affects symptom presentation only when it drops below a specific threshold. Recent evidence points at defects in glucose uptake by the brain, and especially by neurons, as a relevant component of central glucose hypometabolism in HD patients. Here we review the main features of glucose metabolism and transport in the brain in physiological conditions and how these processes are impaired in HD, and discuss the potential ability of strategies aimed at increasing intracellular energy levels to counteract neurological and motor degeneration in HD patients.