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BackgroundCLN2 disease (Neuronal Ceroid Lipofuscinosis Type 2) is an ultra-rare, neurodegenerative lysosomal storage disease, caused by an enzyme deficiency of tripeptidyl peptidase 1 (TPP1). Lack of disease awareness and the non-specificity of presenting symptoms often leads to delayed diagnosis. These guidelines provide robust evidence-based, expert-agreed recommendations on the risks/benefits of disease-modifying treatments and the medical interventions used to manage this condition.MethodsAn expert mapping tool process was developed ranking multidisciplinary professionals, with knowledge of CLN2 disease, diagnostic or management experience of CLN2 disease, or family support professionals. Individuals were sequentially approached to identify two chairs, ensuring that the process was transparent and unbiased. A systematic literature review of published evidence using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidance was independently and simultaneously conducted to develop key statements based upon the strength of the publications. Clinical care statements formed the basis of an international modified Delphi consensus determination process using the virtual meeting (Within3) online platform which requested experts to agree or disagree with any changes. Statements reaching the consensus mark became the guiding statements within this manuscript, which were subsequently assessed against the Appraisal of Guidelines for Research and Evaluation (AGREEII) criteria.ResultsTwenty-one international experts from 7 different specialities, including a patient advocate, were identified. Fifty-three guideline statements were developed covering 13 domains: General Description and Statements, Diagnostics, Clinical Recommendations and Management, Assessments, Interventions and Treatment, Additional Care Considerations, Social Care Considerations, Pain Management, Epilepsy / Seizures, Nutritional Care Interventions, Respiratory Health, Sleep and Rest, and End of Life Care. Consensus was reached after a single round of voting, with one exception which was revised, and agreed by 100% of the SC and achieved 80% consensus in the second voting round. The overall AGREE II assessment score obtained for the development of the guidelines was 5.7 (where 1 represents the lowest quality, and 7 represents the highest quality).ConclusionThis program provides robust evidence- and consensus-driven guidelines that can be used by all healthcare professionals involved in the management of patients with CLN2 disease and other neurodegenerative disorders. This addresses the clinical need to complement other information available.

This article reports on a 1995 survey of students with visual impairments in Japan (4,537 in special schools and 233 in visual impairments units in mainstream schools). Although there appears to be a decrease in the number of students with visual impairments, the proportion of those with additional disabilities is increasing. (Contains references.) (Author/CR)

Competition from weeds can reduce grain yields in both conventional and organic systems. Plant height, tillering, and elevated photosynthetically active radiation interception are some of the traits thought to help confer competitive ability in cereal grains. Crop cultivars developed before the advent of modern, high-input agriculture may be better suited to lower soil nutrient levels and elevated weed competition. Twenty-seven spring bread wheat (Triticum aestivum L.) cultivars, representing 114 yr of Canadian wheat breeding, were grown at conventionally and organically managed sites in north central Alberta over a 3-yr period. Average conventional yields were 63% greater than organic yields, and average overall weed biomass was significantly greater under organic management. Earlier flowering and maturity were more important for achieving high grain yield in organic fields than in conventional fields. Greater numbers of spikes [m.sup.-2] were associated with increased grain yield in organic fields but were not in conventional fields. In organic fields, increased plant height and early maturity were associated with reduced weed biomass, while strong early season vigor was related to increased yield, increased spikes [m.sup.-2], and reduced weed biomass. A competitive crop ideotype for organically grown spring wheat in northern growing regions of the Canadian Prairies should include taller plants, with fast early season growth, early maturity, and elevated fertile tiller number.

Radial glia functions as neural progenitors and as scaffolding supporting neuronal migration during CNS development. A study identifies brain lipid-binding protein as the first predominantly CNS-specific Notch target gene and mediates some aspects of Notch signaling in radial glia.

Gallup Youth Survey is conducted to find the views of American teens on sex and violence in the movies. Teens seem to be relatively unconcerned about the issue of too much sex and violence in the movies as 73% said there is no much violence while 59% disagreed with the issue of sex in movies.

A personal narrative is presented which explores the author's experience of arriving at a point of advocating for herself in her profession and following her own path in it.

A poll survey viewed the opinion of Americans on whether their government and society could do something to prevent shootings like the one held in Red Lake school, Minneapolis in March 2005.The result suggested that only 36% believed the government and society could effectively prevent such shootings, while 60% feel that school shootings would happen regardless of what measures are taken.

A survey is conducted to know if teens are worried that they or someone else in their families will become victims of terrorism. The results depict that 30% are 'very' or 'somewhat' worried, while 71% are 'not too worried' or 'not at all worried' of becoming a victim of terrorism.

A survey is conducted to determine what U.S. teenagers think about man's origin and development. The results depict that a majority of teens believed that God guided human evolution over millions of years, while only 18% felt that humans evolved over millions of years with no help from God.

A survey was conducted among Americans, Canadians and Britons to rate the seriousness of the crime problem in their respective country. The result showed that 50% of Britons, 34% of Canadians, and 42% of Americans crime problems extremely serious in their countries.

The percentage of teen youth who were prescribed medications for various disorders and their satisfaction levels in terms of these medications were studied. The result showed that 9% of the teens claim that they were prescribed medication for depression at some point, 4% revealed they were prescribed medication for anxiety, while 25% of the teens believed medications were of no help.

A survey was conducted to know the view of Americans on whether they feel the amount of sleep they need and get is satisfactory. The result showed that Americans reported an average of 6.8 hours of sleep per night and around 55% feel they get as much sleep as they need while 44% would feel better if they got more sleep.

A survey was conducted among Americans, Canadians and Britons to rate the quality of healthcare in their country. The result showed that 20% of Americans find the quality of healthcare in their country to be excellent as compared with 9% of Canadians and 8% of Briton.

A survey was conducted among Americans, Canadians, and Britons about the moral acceptability of sex between unmarried men and woman. The result showed that 60% of the Americans, 78% of the Canadians, and 85% of the Britons considered premarital sex morally acceptable while 36%, 20%, and 13% of the Americans, Canadians, and Britons respectively said it is morally wrong.

A survey was conducted to show the level of religiosity among Canadians and Britons. The result show that 71% of the Canadians believed in God where as 12% said they did not believe and the 16% were unsure. In contrast, barely half that is 52% of the Britons believed in God and more than quarter did not believe and 19% was unsure.

A survey was conducted in 7-10 Nov 2004 to rate the current most important problem facing the country.40% of Americans rated economy, unemployment and the deficit to be issues of concern and 23% mentioned concerns about the Iraq war. Other issues included in the survey were social security/Medicare, national security, dissatisfaction with the government and others.

Measurement of Palestinians leader Yasser Arafat's, favorability among Americans taken in May 2002 showed 80% unfavorable opinion. After intifada, a Palestinian uprising, which manifested in numerous suicide bombings by militants and subsequent attacks in the West Bank by the Israeli military, began in September 2000, the favorability rate among Americans was greatly affected.

Pledge of Allegiance, which was recited by the American children in school more than 100 years ago, has become one of the most common expressions of American patriotism in American's schools. The data from the August 2004 Gallop Youth survey reflects the ongoing popularity of the pledge in schools where majority of U.S. teenagers say that they recite it in school daily (50%) or weekly (9%), 18% of teens rarely recite the pledge at school and 23% say they never recite it.

Teenagers believe that it takes more than love to make a marriage. Teenagers have expresses their views about marriage and love.

Britain, U.S., and Canada are following different paths toward comprehensive smoking bans. Americans tend to believe the government should do less, rather than more, while Britons and Canadians generally take the opposite view.

The Gallup Youth Survey invited 13- to 17-year-olds to name their three favorite male and female athletes. A substantial number of players did not name any athletes as their favorites, basketball players were among the most popular male athletic stars and tennis players seem to have an advantage among female stars.

The series of commercials sponsored by the Swift Boat Veterans for Truth that question Democratic candidate John Kerry's military record sparked an extraordinary amount of debate about the 'fairness' of the political dialogue. Conventions fueled that debate, as the Rev. Al Sharpton and Sen. Zell Miller unleashed vitriolic diatribes against Bush and Kerry, respectively.

A Gallup poll was conducted to find out the confidence of people on federal government for food supply safety. It was found that 54% say that they have a fair amount of confidence, while 31% say that they have high confidence.

A Gallup poll was conducted to find which party wants to improve education. It was found that 42% say Democratic Party, while 35% say Republican Party is more interested in improving education in the country.

A Gallup poll was conducted on 1000 unmarried Americans to find whether they would consider marrying someone who holds political views significantly different from their own. It was found that majority of singles- 57%- said yes.

The Gallup's study in the United States in 2004 supports the idea that teens that drink or use marijuana are more likely than other teens to exhibit signs of alienation. Aggregated data from two recent Gallup Youth Surveys show that 10% of U.S. teenagers aged 13 to 17 think about their own deaths 'almost always' or 'often', while 62% think about them 'hardly at all' or 'not at all'.