text:
Hanlin Academy

The Hanlin Academy was an academic and administrative institution of higher learning founded in the 8th century Tang China by Emperor Xuanzong in Chang'an. It has also been translated as "College of Literature" and "Academy of the Forest of Pencils."

Membership in the academy was confined to an elite group of scholars, who performed secretarial and literary tasks for the court. One of its primary duties was to decide on an interpretation of the Chinese classics. This formed the basis of the Imperial examinations, which aspiring government bureaucrats had to pass to attain higher-level government posts. Painters working for the court were also attached to the academy.

Academy members
Some of the more famous academicians of Hanlin were:
 * Li Bai (701–762) – Poet
 * Bai Juyi (772–846) – Poet
 * Su Shi (1037 – 1101) – Poet
 * Yan Shu (991–1055) – Poet, calligrapher, (prime minister, 1042)
 * Ouyang Xiu (1007–1072) – Historian
 * Shen Kuo (1031–1095) – Chancellor
 * Zhang Zeduan (1085–1145) – Painter
 * Zhao Mengfu (1254–1322) – Painter, calligrapher, poet (rector, 1314–1320)
 * Huang Zicheng (1350–1402) – Imperial scholar
 * Li Dongyang (1447–1516) – Imperial officer, poet, served as 'Grand Historian'
 * Ni Yuanlu (1593–1644) – Calligrapher, painter, high-ranking official
 * Wu Renchen (1628–1689) – Historian and mathematician
 * Chen Menglei (1650–1741) – Scholar, writer (Editor in Chief of the Complete Classics Collection of Ancient China)
 * Zhang Tingyu (1672–1755) – Politician and historian
 * Ji Xiaolan (1724–1805) – Scholar, poet (Editor in Chief of the Complete Library of the Four Treasuries)
 * Yao Nai (1731–1815) – Scholar
 * Gao E (1738–1815) – Scholar and editor
 * He Changling (1785–1848) – Scholar and official
 * Zeng Guofan (1811–1872) – Scholar and later key military official
 * Chen Lanbin (1816–1895) – Diplomat (ambassador to the U.S., Spain and Peru)
 * Weng Tonghe (1830–1904) – Imperial Tutor
 * Cai Yuanpei (1868–1940) – Educator
 * Qu Hongji (1850–1918) – Politician

Bureau of Translators
Subordinated to the Hanlin Academy was the Bureau of Translators. Founded by the Ming dynasty in 1407, after the first expedition of Zheng He to the Indian Ocean, the Bureau dealt with the memorials delivered by foreign ambassadors and trained foreign language specialists. It included departments for many languages such as the Jurchen, "Tartar" (Mongol),   Korean, Ryukyuan, Japanese, Tibetan, "Huihui" (the "Muslim" language, Persian)  Vietnamese and Burmese languages, as well as for the languages of the "various barbarian tribes" (Bai yi 百夷, i.e., Shan ethnic groups on China's southwestern borders), "Gaochang" (people of Turfan, i.e. Old Uyghur language),      and Xitian (西天; (Sanskrit, spoken in India). In 1511 and 1579 departments for the languages of Ba bai (八百; Lao) and Thai were added, respectively. A Malay language vocabulary (Manlajia Guan Yiyu) 滿剌加館譯語 (Words-list of Melaka Kingdom) for the Malay spoken in the Malacca Sultanate was compiled.   A Cham language vocabulary 占城館 was created for the language spoken in the Champa Kingdom.

When the Qing dynasty revived the Ming Siyiguan 四夷館, the Manchus, who "were sensitive to references to barbarians", changed the name from yi 夷 "barbarian" to yi 彝 "Yi people", and changed the Shan exonym from Baiyi 百夷 "hundred barbarians" to Baiyi 百譯 "hundred translations".

The later Tongwen Guan set up by the Qing dynasty for translating western languages was subordinated to the Zongli Yamen and not the Hanlin.

1900 fire
The Beijing Hanlin Academy and its library were severely damaged in a fire during the Siege of the International Legations in Peking (now known as Beijing) in 1900 by the Kansu Braves while fighting against the Eight-Nation Alliance. On June 24, the fire spread to the academy: "The old buildings burned like tinder with a roar which drowned the steady rattle of musketry as Tung Fu-shiang's Moslems fired wildly through the smoke from upper windows. Some of the incendiaries were shot down, but the buildings were an inferno and the old trees standing round them blazed like torches. An attempt was made to save the famous Yung Lo Ta Tien, but heaps of volumes had been destroyed, so the attempt was given up."

- eyewitness Lancelot Giles, son of Herbert Giles

The flames destroyed many ancient texts.

The academy operated continuously until its closure during the 1911 Xinhai Revolution.

Foreign language vocabularies

 * 華夷譯語 – 達達館（蒙古語）Mongol language
 * 華夷譯語(一) – 暹羅館（泰語） 天文門. Thai language
 * 華夷譯語(二) – 緬甸館譯語 緬甸館來文（緬甸語）通用門. Burmese language
 * 華夷譯語(三) – 百夷館（雲南傣語）天文門. Baiyi (Dai, Shan)
 * 華夷譯語(四) – 百夷館（雲南傣語）天文門和地理門. Baiyi (Dai, Shan)
 * 華夷譯語(五) – 回回館《回回館雜字》（波斯語）天文門. Persian language
 * 華夷譯語(六) – 回回館《回回館雜字》（波斯語）天文門和地理門. Persian language
 * 高昌館來文 – 高昌館來文（高昌）回鶻語 (畏兀兒館（回鶻語）. Old Uyghur language
 * 譯文備覽 – 譯文備覽 西番館（藏語）. Tibetan language
 * 西番譯語 – 《西番譯語》西番館（藏語）Tibetan language
 * 華夷譯語/朝鮮館譯語 Korean language
 * 使琉球錄 (陳侃) Ryukyuan language
 * 使琉球錄 (蕭崇業)/附 – 使琉球錄 夷語 夷字 Ryukyuan language
 * 使琉球錄 (夏子陽)/卷下 Ryukyuan language
 * 中山傳信錄/卷六 Ryukyuan language
 * 重修使琉球錄 Ryukyuan language
 * 使琉球录三种-夷语（附）_国学导航 Ryukyuan language
 * 0-使琉球录-明-陈侃 Ryukyuan language
 * 國朝典故卷之一百二　　使琉球錄（明）陳侃 撰 Ryukyuan language
 * 《女直館》（女真語） 《女真譯語》《女真館雜字》在 Die Sprache und Schrift der Jučen by Wilhem Grube by Wilhem Grube 《女真文和女真語》作者：葛祿博  Jurchen language

title: Hanlin Academy


text:
Attundaland

Attundaland (or the land of the eight hundreds) was the name given to the southeastern part of the present day province of Uppland, north of Stockholm. Its name refers to its role of providing 800 men and 32 ships for the leidang of the Swedish kings at Uppsala.

Snorri Sturluson relates that Tiundaland was the richest and most fertile region of Sweden. It was the seat of the Swedish kings at Uppsala and later the Swedish Archbishopric. All the Swedish lawspeakers were subordinate to the lawspeaker of Tiundaland.

The name of Attunda was revived as Attunda district court (Attunda tingsrätt) in April 2007, through the fusion of Sollentuna and Södra Roslags district courts. The seat of Attunda district court is situated in Sollentuna Municipality.

license: Creative Commons - Attribution Share-Alike - https://creativecommons.org/licenses/by-sa/4.0/

title: Attundaland


text:
Still Cruisin'

Still Cruisin' is the twenty-sixth studio album by the Beach Boys, their thirty-fifth official album (counting compilations and live packages), and their last release of the 1980s. It is also the last album of new material released during a brief return to Capitol Records.

Released on August 28, 1989, the album was panned by critics, with AllMusic referring to the band's sound by producer Terry Melcher as sounding "like a professional '60s cover band". Despite the poor critical reception, the album was Certified Gold by RIAA in 1989 and has since gone on to be Certified Platinum for sales of 1,000,000 copies.

Still Cruisin' was left out of Capitol's Beach Boys re-issue campaign in 2000 and 2001, along with their following album Summer in Paradise.

Background
After "Kokomo" (when released as a single from the Cocktail film soundtrack) gave the Beach Boys their first number one hit in the US since 1966's "Good Vibrations", the band decided to put together an album of recent and classic songs. The classic songs included had been heard in recent films. The songs "Still Cruisin'", "Somewhere Near Japan", and "Island Girl" were recorded for the album by the touring Beach Boys band as well as studio musicians and producer Terry Melcher. Due to his ongoing relationship with Dr. Eugene Landy, Brian Wilson's lone contribution to this album was "In My Car", a song credited as being co-written by Landy and girlfriend Alexandra Morgan. However, as subsequent court actions have seen Landy's name removed from other period songs, such as those on Wilson's 1988 album, these credits may be negated.

"Kokomo" was a recent single, as was "Wipe Out", a duet with American rap group the Fat Boys. (The song was originally to be recorded with Run-DMC, but Mike Love apparently struck a deal with the other group.) "Make It Big" was recorded for the film Troop Beverly Hills, and the remaining three songs - "I Get Around", "Wouldn't It Be Nice", and "California Girls" – were the "classic" recordings, ones from the group's earlier period, that had been used in recent films. The inclusion of these hits gave the album a more acceptable running time.

At least one song was recorded during or around the time of the sessions for this album but was not included. "Happy Endings" is a ballad which was a collaboration with Little Richard; it was produced by Terry Melcher and was (recorded in June and October 1987 and) released as a non-album single on November 3, 1987. (Its B-side was a live version of "California Girls".) Also not included were "Rock 'n' Roll to the Rescue", "California Dreamin", and "Lady Liberty" (all of which were released as singles in 1986), along with "The Spirit of Rock and Roll" (recorded between August 1986 and January 1987), a Brian Wilson composition included as the final song in the band's 25th anniversary concert/TV special. "The Spirit of Rock and Roll" was not released. In addition, the 1988 remake of "Don't Worry Baby" from the film Tequila Sunrise with the Everly Brothers was not included.

Mike Love said of the album: "The theme of that album was to have been songs that have been in movies. It was basically a repackage. But then it got watered down with politics, meaning Brian's Dr. Landy forcing a song called "In My Car," which was never in a movie, and a song by [Al] Jardine, which ultimately ended up on the album, called "Island Girl," which was never in a movie either. So to me the concept was a little bit diluted there politically."

Riding on the coattails of "Kokomo", Still Cruisin' went gold in the US and Austria and gave the Beach Boys their best chart showing since 1976. During Capitol's Beach Boys re-issue campaign in 2000 and 2001 however, Still Cruisin' was left behind and allowed to go out of print, and it has remained out of print ever since.

Singles

 * "Kokomo" b/w "Tutti Frutti" (Little Richard) (Elektra), July 18, 1988 – US No. 1; UK No. 25
 * "Still Cruisin'" b/w "Kokomo" (Capitol), August 7, 1989 – US No. 93
 * "Somewhere Near Japan" b/w "Wipe Out" (Capitol), January 1990

Music videos were produced for "Still Cruisin'", "Somewhere Near Japan", "Kokomo" and "Wipe Out".

Commercial performance
Still Cruisin' reached number 46 on the US Billboard 200 during a chart stay of 22 weeks. It reached number 10 in Australia, number 12 in Austria. number 25 in Switzerland and number 43 in Sweden.

Personnel
Partial credits; credits exclude those for the 1960s songs.

The Beach Boys
 * Mike Love – lead vocals, harmony and backing vocals
 * Al Jardine – lead vocals, harmony and backing vocals, guitar
 * Carl Wilson – lead vocals, harmony and backing vocals, guitar, keyboards
 * Bruce Johnston – lead vocals, harmony and backing vocals, keyboards, bass guitar
 * Brian Wilson – lead vocals, harmony and backing vocals; keyboards and synthesizers on "In My Car"

Additional musicians
 * The Fat Boys – rapping on "Wipe Out"
 * Adam Jardine – backing vocals on “Island Girl”
 * Matt Jardine – backing vocals on “Island Girl”
 * Jeffrey Foskett – backing vocals on “Island Girl” and acoustic guitar on "Kokomo"
 * Craig Trippand Fall – lead guitar on "Still Crusin'" and "Somewhere Near Japan," bass guitar, mandolin
 * Joseph Brasler – lead guitar on "In My Car"
 * Rod Clark – bass on "Kokomo"
 * Keith Wechsler – drums and keyboards on "Still Cruisin'"
 * Mike Kowalski – drums on “Island Girl”
 * Vinnie Colaiuta – drums on "In My Car"
 * Jim Keltner – drums on "Somewhere Near Japan" and "Kokomo"
 * Van Dyke Parks – accordion on "Kokomo"
 * Joel Peskin – saxophone on "Kokomo"
 * Chili con Charles – percussion on "Kokomo"
 * "Vince", "Milton", and "Mike" (last names unknown) – steel drums on "Kokomo"
 * James Grunke – synth programming on "Island Girl"
 * Michael Bernard – synth programming on "In My Car"

Production staff
 * Terry Melcher – producer
 * Brian Wilson – producer ("In My Car")
 * Al Jardine – producer ("Island Girl")
 * Albert Calbrera – producer ("Wipe Out")
 * Tony Moran – producer ("Wipe Out")
 * Gary Usher – vocal producer ("Wipe Out"; The Beach Boys' vocal overdubs only)

title: Still Cruisin'


text:
Herstmonceux Castle

Herstmonceux Castle is a brick-built castle, dating from the 15th century, near Herstmonceux, East Sussex, England. It is one of the oldest significant brick buildings still standing in England. The castle was renowned for being one of the first buildings to use that material in England, and was built using bricks taken from the local clay, by builders from Flanders. It dates from 1441. Construction began under the then-owner, Sir Roger Fiennes, and was continued after his death in 1449 by his son, Lord Dacre. The castle has been owned by Queen's University at Kingston, a Canadian university, since 1993.

The parks and gardens of Herstmonceux Castle and Place are Grade II* listed on the Register of Historic Parks and Gardens. Other listed structures on the Herstmonceux estate include the Grade II listed walled garden to the north of the castle, and the Grade II* listed telescopes and workshops of the Herstmonceux Science Centre.

Early history
The first written evidence of the existence of the Herst settlement appears in William the Conqueror's Domesday Book of 1086, which reports that one of William's closest supporters granted tenancy of the manor at Herst to a man named 'Wilbert'. By the end of the twelfth century, the family at the manor house at Herst had considerable status. Written accounts mention a lady called Idonea de Herst, who married a Norman nobleman named Ingelram de Monceux. Around this time, the manor began to be called the "Herst of the Monceux", a name that eventually became Herstmonceux.

A descendant of the Monceux family, Roger Fiennes, was ultimately responsible for the construction of Herstmonceux Castle in the County of Sussex. Sir Roger was appointed Treasurer of the Household of Henry VI of England and needed a house fitting a man of his position, so construction of the castle on the site of the old manor house began in 1441. It was this position as treasurer which enabled him to afford the £3,800 construction of the original castle.

In 1541, Sir Thomas Fiennes, Lord Dacre, was tried for murder and robbery of the King's deer after his poaching exploits on a neighboring estate resulted in the death of a gamekeeper. He was convicted and hanged as a commoner, and the Herstmonceux estate was temporarily confiscated by Henry VIII of England, but was restored to the Fiennes family during the reign of one of Henry's children.

The profligacy of the 15th Baron Dacre, heir to the Fiennes family, forced him to sell in 1708 to George Naylor, a lawyer of Lincoln's Inn in London. Bethaia Naylor, who became the heiress of Herstmonceux on the death of her brother's only daughter, married Francis Hare and produced a son, Francis, who inherited in turn, his mother's property. The castle eventually came into the possession of Robert Hare-Naylor, who, upon the insistence of his second wife, Henrietta Henckell, followed the architect Samuel Wyatt's advice to reduce the Castle to a picturesque ruin by demolishing the interior. Thomas Lennard, 17th Baron Dacre, was sufficiently exercised as to commission James Lambert of Lewes to record the building in 1776. The castle was dismantled in 1777 leaving the exterior walls standing and remained a ruin until the early 20th century.

20th-century restoration
Radical restoration work was undertaken by Colonel Claude Lowther in 1913 to transform the ruined building into a residence and, based on a design by the architect, Walter Godfrey, this work was completed by Sir Paul Latham in 1933. The existing interiors largely date from that period, incorporating architectural antiques from England and France. The one major change in planning was the combination of the four internal courtyards into one large one. The restoration work, regarded as the apex of Godfrey's architectural achievement, was described by the critic Sir Nikolaus Pevsner as executed 'exemplarily'.

Royal Greenwich Observatory
The Royal Observatory was founded by King Charles II at Greenwich in 1675. Observing conditions at Greenwich deteriorated following the urban growth of London, and plans were made in the early 20th century to relocate the observatory to a rural location with clearer, darker skies. Herstmonceux Castle and estate were put up for sale by their private owners and were sold in 1946 to the Admiralty, which then operated the Royal Observatory on behalf of the British government. The relocation of the observatory took place over a decade, and was complete by 1957. A number of new buildings were erected in the castle grounds. The institution at Herstmonceux Castle was known as the Royal Greenwich Observatory, where it remained until 1988, when the observatory relocated to Cambridge.

Several of the telescopes remain but the largest telescope, the 100 inch (254 cm) aperture Isaac Newton Telescope was moved to La Palma, in the Canary Islands, in the 1970s. The estate provides housing for the Equatorial Telescope Buildings, which have been converted for use as an interactive science centre for schoolchildren. The empty dome for the Newton Telescope remains on this site and is a landmark, visible from afar.

University Study Centre
In 1992 Alfred Bader, an alumnus of Queen's University at Kingston, learned of the castle's vacancy and offered to purchase the castle for his wife; she declined, joking that there would be "too many rooms to clean". But in 1994, after intensive renovations, the Queen's International Study Centre was opened. It hosts primarily undergraduate students studying arts, science, or commerce through the Canadian University Study Abroad Program (CUSAP), as well as graduate students studying Public International Law or International Business Law. Specialty summer programmes (May–June) including engineering (Global Project Management), archaeology, international health sciences, and law have become popular in recent years with students from both Queen's and other universities. In late January 2009, the ISC was renamed the Bader International Study Centre. As part of the 25th anniversary celebrations, new science and innovation labs were opened on the campus to increase the ability for first year science-tracked students to attend. In 2022, the Bader International Study Centre was renamed Bader College. On 13 November 2023, in response to engineering investigations, the university suspended operations at Bader College and future admissions to Bader College programs until structural remediation activities can be completed. It is expected that these activities will take at least 18 months, with the scope of works required still under investigation.

Historical retinue and events
Herstmonceux Castle is associated with a retinue of historical re-enactment troops including archers, knights, and falconers, who fly their birds over the grounds. The castle is host to a large medieval weekend in August of each year, and is also hired out for weddings and weekend events.

In popular culture
The castle was used for filming part of The Silver Chair, a 1990 BBC adaptation of the book (one of The Chronicles of Narnia) by C. S. Lewis. The castle and gardens were used by comedians Reeves and Mortimer for one of their Mulligan and O'Hare sketches. In August 2002, The Coca-Cola Company rented the castle for use as part of a prize in a Harry Potter-themed sweepstakes—the castle served as "Hogwarts" in a day of Harry Potter-related activities for the sweepstakes winners. A "painting" of the castle was used as a magical cursed object in the U.S. television show Charmed – episode 2.3 "The Painted World". Due to its suitable appearance, Herstmonceux Castle was a filming location for the series My Lady Jane.

Owners of Herstmonceux Manor/Castle
Owners have been as follows:
 * 1066 – Edmer, a priest
 * 1086 – Wilbert, tenant-in-chief
 * c.1200 – Idonea de Herst (married Ingelram de Monceux)
 * 1211 – Her son Waleran de Monceux
 * 1216 – His son William de Monceux
 * ?  – His son Waleran de Monceux
 * 1279 – His son John de Monceux
 * 1302 – His son John de Monceux
 * 1316 – His son John de Monceux
 * 1330 – His sister Maud de Monceux (married Sir John Fiennes)
 * 1351 – Her eldest son William Fiennes
 * 1359 – His son Sir William Fiennes
 * 1402 – His son Sir Roger Fiennes (built Herstmonceux Castle)
 * 1449 – His son Sir Richard Fiennes (married Joan Dacre, 7th Baroness Dacre)
 * 1483 – His grandson Sir Thomas Fiennes
 * 1533 – Sir Thomas Fiennes
 * 1541 – His eldest son Thomas Fiennes
 * 1553 – His brother Gregory Fiennes
 * 1594 – His sister Margaret Fiennes (married Sampson Lennard).
 * 1612 – Her son Henry Lennard, 12th Baron Dacre
 * 1616 – His son Richard Leonard
 * 1630 – His son Francis Leonard
 * 1662 – His son Thomas Leonard
 * 1708 – Estate purchased by George Naylor for £38,215
 * 1730 – His nephew Francis Naylor
 * 1775 – His half-brother Robert Hare who demolished the castle in 1776
 * ?  – His son Francis Hare Naylor
 * 1807 – Purchased by Thomas Read Kemp
 * 1819 – Purchased for John Gillon MP
 * 1846 – Purchased by Herbet Barrett Curteis MP
 * ?  – His son Herbert Mascall Curteis
 * ?  – His son Herbert Curteis
 * 1911 – Purchased by Lieutenant-Colonel Claude Lowther (restoration began)
 * 1929 – Purchased by Reginald Lawson
 * 1932 – Purchased by Sir Paul Latham (completed restoration under Walter Godfrey)
 * 1946 – Purchased by H.M. Admiralty for The Royal Observatory
 * 1965 – Transferred to the Science Research Council
 * 1989 – Purchased by James Developments, transfers to a receiver, the Guinness Mahon Bank
 * 1993 – Purchased for Queen's University, Ontario (Canada) as a gift from Drs. Alfred and Isabel Bader

title: Herstmonceux Castle


text:
Michael Turner (comics)

Michael Layne Turner (April 21, 1971 – June 27, 2008) was an American comics artist known for his work on Witchblade, Fathom, Superman/Batman, Soulfire, and various covers for DC Comics and Marvel Comics. He was also the president of the entertainment company Aspen MLT.

Early life
Michael Turner was born in Crossville, Tennessee on April 21, 1971. He was a student at the University of Tennessee, and graduated from the International Performing Arts Academy after which he moved to San Diego, California, where he developed an interest in comics.

Career
Turner was discovered by Marc Silvestri at a convention and hired by Silvestri's Top Cow Productions as an artist. He initially did background illustrations for Top Cow titles before co-creating Witchblade. In the summer of 1998 he debuted the creator-owned Fathom, having also worked on his new series Soulfire.

As well as an artist, Turner was an award-winning water skier, held an instructor-level red sash in martial arts, and was an avid video game player. In March 2000, Turner was diagnosed with chondrosarcoma, a form of cancer, in the right pelvis. He was treated at the Ronald Reagan UCLA Medical Center with surgery in which he lost a hip, 40% of his pelvis, and three pounds of bone. The surgery was followed by nine months of radiation therapy.

Turner departed Top Cow in late 2002 to found his own comic book publishing company, Aspen MLT Inc. (where MLT stands for Michael Layne Turner), located in Santa Monica, with a studio in Marina del Rey, California. The release of comics from Aspen was delayed by a year-long lawsuit with Top Cow Productions over the rights to Fathom and over the rights to the unreleased titles Soulfire (initially called Dragonfly) and Ekos, both of which Turner had begun developing before leaving Top Cow and before his diagnosis of cancer. Aspen and Top Cow settled the lawsuit out of court in 2003.

In 2004 Turner contributed covers to various DC Comics titles, including The Flash and Identity Crisis. He provided cover art and co-wrote the "Godfall" story arc that ran in the three main Superman titles in early 2004 and illustrated the six-issue Supergirl story arc in Superman/Batman. His creator-owned title Soulfire began publication in 2004 and Fathom resumed publication in that year as well, though this time with Aspen MLT rather than Top Cow.

On August 6, 2005, Marvel Comics announced the signing of Michael Turner to a work-for-hire deal for a six-issue project and covers. This would turn out to be at least the variant covers for the miniseries Civil War and the Wolverine ongoing series Wolverine: Origins. In addition Turner had been announced as the artist on Ultimate Wolverine.

Turner created online comic adaptations for the NBC television series Heroes.

Death and tribute
Turner died June 27, 2008, at the Santa Monica Hospital in Santa Monica, California, of complications from bone cancer. He is survived by his mother Grace Crick, his brother Jake Turner, and his fiancée Kelly Carmichael. Fathom vol. 3 #1, which was published on Wednesday, August 6, 2008, featured a tribute to Turner in the form of a stylized blue ribbon in the upper right-hand corner of its cover, and its first page was a memorial to him. Aspen MLT also published a trade paperback collection of writings from people who knew Turner, titled A Tribute to Michael Turner, which features a cover painted by Alex Ross. Volume #21 of The Boys included a page dedicated to Michael Turner as well.

Aspen MLT

 * Soulfire #0–7 (2004–2005)

DC Comics

 * Superman/Batman #8–13 (2004)

Image Comics/Top Cow Productions

 * Ballistic #1–3 (1995)
 * Codename: Strykeforce #14 (among other artists) (1995)
 * Fathom #0–14 (1998–2002)
 * Tomb Raider #25 (2002)
 * Tomb Raider/Witchblade, one-shot, (with Brian Ching) #1 (1997)
 * Witchblade #1–8, 10–23, 25 (1995–1998)
 * Witchblade/Tomb Raider Special #1 (with Keu Cha)(1998)
 * Wolverine/Witchblade #1 (1997)
 * Witchblade/Elektra  #1 (1997)

Aspen MLT

 * Fathom vol. 2, Beginnings, Prelude #0, 1–4, 7–8, 10–11 (2005–2006)
 * Shrugged #0, 1–6 (2006–2007)
 * Soulfire Preview, #0, 1–10 (2004–2009)

DC Comics

 * Action Comics #812–813 (2004)
 * The Adventures of Superman #625–626 (2004)
 * The Flash vol. 2 #207–211 (2004)
 * Identity Crisis #1–7 (2004)
 * Justice League of America vol. 2 #0–12 (2006–2007)
 * Supergirl vol. 5 #1–3, 5, 50 (2005)
 * Superman vol. 2, #202–203, 205 (2004)
 * Superman/Batman #8–13, 19, 26 (2006)
 * Teen Titans vol. 3 #1 (2003)

Image Comics / Top Cow Productions

 * Darkness #7, 11 (1997–1998)
 * Tomb Raider #1, 9, 25, 46 (1999–2004)
 * Witchblade #1–25, 27, 50, 86, 100, 103 (1995–2007)

Image Comics / Hurricane Ent.

 * ''Violent Messiahs: Lamenting Pain #1 (2002)

Marvel Comics

 * Black Panther vol. 4 #18, 23–25 (2006–2007)
 * Civil War #1–7 (2006–2007)
 * Fantastic Four #544–553 (2007)
 * Hulk vol. 2 #1, 6 (2008)
 * The Incredible Hulk vol. 3 #100 (2006)
 * Ms. Marvel vol. 2 #1 (2006)
 * Onslaught: Reborn #1 (2006)
 * Spider-Man/Red Sonja #1–5 (2007)
 * Sub-Mariner vol. 2 #1, 6 (2007–2008)
 * Thor vol. 3 #1 (2007)
 * Ultimate X-Men #75 (2006)
 * Uncanny X-Men #500 (2008)
 * World War Hulk #1 (2007)
 * Wolverine: Origins #1 (2006)
 * Wolverine vol. 3 #66 (2008)

title: Michael Turner (comics)


text:
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title: User talk:Dbastos~enwiki


text:
User:Rye 212

Mike, not living in Dublin

Wikipedia life
First visited at 15:45PST Fri Oct 8, 2004 via Google result #6 for Australian PM John Howard when Alexa ranked the site as approx the 250th most popular. Voting had just started in the Australian 2004 elections, and Howard was seeking a record 4th term. I wanted more info on his terms of office - and found it on WP. Twenty-four hours later Howard had won the election, and was featured on the WP home-page.

I was surprised to find the Oct 1st re-election of Irish President Mary McAleese already recorded, which demonstrated the accuracy of the site. I made my first contributions to pages on Irish politicians John O'Donoghue and Mary Harney to reflect the recent cabinet reshuffle.

Next day, to test WP, I researched my favourite topic - What is the formal date of Irish independence? The answers were there on Irish Republic. I added to that info, made a few more contributions and joined WikiPedia on Sun 10th October, 2004. Enjoy Looking fwd to being a constructive member. - Rye1967 20:57, Oct 11, 2004 (UTC)

Made my 1500th logged-in contribution with a new page on Eddie Wade on 1st Dec 2005 and 2000th on March 20, 2006 with a change to Esera Tuaolo, 2500 on 16 May, 2006, change to Gerald Boland, 4600 on 26 March 2007 change to Ian Paisley (Impending First Min Nom.), 6621 edits by Mar 2011, 7588 as of 25 Sep 2023. Slowed down! Most recent was on Merab Sharikadze Georgian Rugby International.

Made some anon contributions as follows: I agree to multi-license all my contributions, with the exception of my user pages, as described below:
 * 64.32.180.82 in 2004 and 2005
 * 24.136.82.123 in October 2004
 * 75.80.109.78 on Dec 11, 2006
 * 89.127.164.8 in 2007
 * 89.100.154.154 in 2007 and 2008
 * 83.141.89.154 in April 2008, June 2009 and in July 2010
 * 109.255.22.196 in 2010
 * 109.255.141.254 in 2011
 * 109.255.235.7 in 2012
 * 178.250.114.154 in August 2013, February 2014, November 2014 and May 2015

23 Apr 2013: My first view of anon-IP contributions logged with an IPv6 address. . Vandalism, sadly.

When were topics 'born' Wikipedia

 * Alan Turing started Nov 12, 2001
 * Google started Oct 30, 2001, Googol started Aug 8, 2001
 * France started Jan 29, 2001 (small entry, amusing)
 * User:JimboWales made his first contribution to ThomasEdison Jan 23rd 2001, became User:Jimbo Wales in March 2001
 * See also History of Wikipedia
 * the first entry on the Sept 11 attacks at 10:02 EST. Much written since, including early updates from Jimbo wales.
 * Wikipedia.com launched Jan 15th 2001 - see History of Wikipedia
 * From WP I learned that Mikheil Saakashvili of Georgia was born just one day before me
 * Articles that have not been edited in a long, long time

Some famous (to me) old people from WP
Created Feb 2006, Last update Sep 2023, Last extension Sep 2023

1889 to 1914

 * 1889 September 14 María Capovilla (d. August 27 2006, aged 116)
 * 1902 January 2   Dan Keating (d. October 2 2007, aged 105)
 * 1910 July 4      Gloria Stuart Titanic actress (d. September 26 2010, aged 100)
 * 1912 December 22 Lady Bird Johnson (d. July 11 2007, aged 94)
 * 1913 July 14     Gerald Ford (d. December 26 2006, aged 93)
 * 1913 July 23     Michael Foot (d. March 3 2010, aged 96)
 * 1913 August 17   W. Mark Felt (d. December 18 2008, aged 95)
 * 1914 October 16  Mohammed Zahir Shah (d. July 23 2007, aged 92)

1915

 * January 13  Kit Ahern (d. December 27 2007, aged 92)
 * February 4  Norman Wisdom (d. October 4 2010, aged 95)
 * September 15 Eddie Filgate (d. January 19, 2017, aged 101)
 * October 15  Yitzhak Shamir (d. June 30, 2012, aged 96)
 * November 9  Sargent Shriver (d. January 18 2011, aged 95)
 * November 25 Augusto Pinochet (d. December 10 2006, aged 91)

1916

 * January 12  Pieter Willem Botha (d. October 31 2006, aged 90)
 * May 8       João Havelange (d. August 16, 2016, aged 100)
 * June 9      Robert Mcnamara (d. July 6 2009, aged 93)
 * July 11     Gough Whitlam (d. October 21 2014, aged 98)
 * November 4  Walter Cronkite (d. July 17 2009, aged 92)
 * November 10 Louis le Brocquy (d. April 25 2012, aged 95)
 * December 8  T. K. Whitaker (d. January 9, 2017, aged 100)
 * December 9  Kirk Douglas (d.February 5, 2020, aged 103)

1917

 * January 5   Jane Wyman (d. September 10 2007, aged 90)
 * February 2  Đỗ Mười (d. October 1, 2018, aged 101)
 * February 6  Zsa Zsa Gabor (d. December 18, 2016, aged 99)
 * March 20    Vera Lynn (d. 18 June 2020, aged 103)
 * August 18   Caspar Weinberger (d. March 28 2006, aged 88)
 * August 30   Denis Healey (d. October 30 2015, aged 98
 * October 1   Cahal Cardinal Daly (d. December 31 2009, aged 92)
 * November 3  Conor Cruise O'Brien (d. December 18 2008, aged 91)
 * November 20 Robert Byrd (d. June 28 2010, aged 92)
 * December 16 Arthur C. Clarke (d. March 19 2008, aged 90)

1918

 * March 12    Pádraig Faulkner (d. June 1 2012, aged 94)
 * April 8     Betty Ford (d. July 8 2011, aged 93)
 * May 27      Yasuhiro Nakasone (d. 29 November 2019, aged 101)
 * July 18     Nelson Mandela (d. Dec 5 2013, aged 95)
 * November 7  Billy Graham (d. February 21, 2018, aged 99)
 * December 21 Kurt Waldheim (d. June 14 2007, aged 88)
 * December 23 Helmut Schmidt (d. November 10, 2015 aged 96)

1919

 * January 1   J. D. Salinger (d. January 27 2010, aged 91)
 * January 14  Giulio Andreotti (d. May 6 2013, aged 94)
 * May 3       Pete Seeger (d. January 27 2014, aged 94)
 * July 20     Edmund Hillary (d. January 11 2008, aged 88)

1920

 * January 19  Javier Pérez de Cuéllar (d. March 4, 2020, aged 100)
 * April 13    Liam Cosgrave (d. October 4, 2017, aged 97)
 * April 20    John Paul Stevens (d. July 16, 2019, aged 99)
 * August 4    Helen Thomas (d. July 20, 2013, aged 92)
 * August 17   Maureen O'Hara (d. October 24 2015, aged 95)
 * September 23 Mickey Rooney (d. April 6, 2014 aged 93)
 * December 9  Carlo Azeglio Ciampi (d. September 16, 2016, aged 95)
 * December 13 George P. Shultz (d. February 6, 2021, aged 100)

1921

 * April 21    Tiede Herrema (d. 24 April 2020, aged 99)
 * June 10     Prince Philip, Duke of Edinburgh (d. April 9, 2021, aged 99)
 * July 10     Nancy Davis Reagan (d. March 6, 2016, aged 95)
 * July 18     John Glenn (d. December 8, 2016, aged 95)
 * November 29 Jackie Stallone (d. September 21, 2020, aged 98)

1922

 * January 17  Betty White (d. December 31, 2021, aged 99)
 * February 6  Denis Norden (d. September 18, 2018, aged 96)
 * April 3     Doris Day (d. May 13, 2019, aged 97)
 * April 4     Máire Mhac an tSaoi (d. October 16, 2021, aged 99)
 * July 2      Pierre Cardin (d. December 29, 2020, aged 98)
 * November 14 Boutros Boutros-Ghali (d. February 16, 2016, aged 93)

1923

 * May 27      Henry Kissinger (d. November 29, 2023, aged 100)
 * May 27      Sumner Redstone (d. August 11, 2020, aged 97)
 * July 22     Bob Dole (d. December 5, 2021, aged 98)
 * August 2    Shimon Peres (d. September 28, 2016, aged 93)
 * September 16 Lee Kuan Yew (d. March 23, 2015, aged 91)
 * October 10  Murray Walker (d. March 13, 2021, aged 97)
 * October 10  Nicholas Parsons (d. 28 January 2020, aged 96)
 * December 12 Bob Barker (d. 26 August 2023, aged 99)

1924

 * February 20 Gloria Vanderbilt (d. June 17, 2019, aged 95)
 * February 21 Robert Mugabe (d. 6 September 2019, aged 95)
 * April 28    Kenneth Kaunda (d. 17 June 2021, aged 97)
 * June 12     George H. W. Bush (d. November 30, 2018, aged 94)
 * September 2 Daniel arap Moi (d. 4 February 2020, aged 95)
 * October 1   Jimmy Carter
 * October 15  Lee Iacocca (d. July 2, 2019, aged 94)
 * December 7  Mário Soares (d. January 7, 2017, aged 92)
 * December 25 Atal Bihari Vajpayee (d. August 16, 2018, aged 93)

1925

 * March 19    Brent Scowcroft (d. August 6, 2020, aged 95)
 * June 29     Giorgio Napolitano (d. September 22, 2023, aged 98)
 * July 10     Mahathir Mohamad
 * July 20     Jacques Delors (d. December 27, 2023, aged 98)
 * October 19  Angela Lansbury (d. October 11 2022, aged 96)
 * Dec 13      Dick Van Dyke

1926

 * February 2  Valéry Giscard d'Estaing (d. December 2, 2020, aged 94)
 * March 6     Alan Greenspan
 * April 21    Elizabeth II (d. September 8, 2022, aged 96)
 * May 8       David Attenborough
 * June 28     Mel Brooks
 * July 8      John Dingell (d. February 7, 2019, aged 92)
 * August 3    Tony Bennett (d. July 21, 2023, aged 96)

1927

 * January 17 John A. Murphy (d. February 28, 2022, aged 95)
 * February 20 Sidney Poitier (d. January 6, 2022, aged 94)
 * March 1    Harry Belafonte (d. April 25, 2023, aged 96)
 * April 16   Pope Benedict XVI (d. December 31, 2022, aged 95)
 * August 18  Rosalynn Carter (d. November 19, 2023, aged 96)
 * October 28 Cleo Laine
 * Unknown Imogen Stuart

1928

 * January 24 Desmond Morris
 * March 18 Fidel V. Ramos (d. July 31, 2022, aged 94)
 * April 6 James Watson
 * April 11 Ethel Kennedy
 * May 4 Thomas Kinsella (d. December 22, 2021, aged 93)
 * May 12 Burt Bacharach (d. February 8 2023, aged 94)
 * June 4 Ruth Westheimer
 * June 7 James Ivory
 * June 20 Jean-Marie Le Pen
 * June 28 Hans Blix
 * December 7 Noam Chomsky

1929

 * February 17 Patricia Routledge
 * May 2 Édouard Balladur
 * July 2 Imelda Marcos
 * September 5 Bob Newhart
 * September 25 Barbara Walters (d. December 30, 2022, aged 93)
 * December 20 Milan Panić

1930

 * January 4 Tras Honan (d. November 25, 2023, aged 93)
 * January 12 Jennifer Johnston (novelist)
 * January 20 Buzz Aldrin
 * January 30 Gene Hackman
 * February 10 Robert Wagner
 * February 27 Joanne Woodward
 * March 3 Ion Iliescu
 * March 8 Douglas Hurd
 * March 22 Pat Robertson (d. March 22, 2023, aged 93)
 * March 26 Sandra Day O'Connor (d. December 1, 2023, aged 93)
 * March 30 Rolf Harris (d. May 10, 2023, aged 93)
 * April 15 Vigdís Finnbogadóttir
 * April 28 James Baker
 * May 20 Betty DeGeneres
 * May 31 Clint Eastwood
 * July 7 Theodore McCarrick
 * August 12 George Soros
 * August 20 Mícheál Ó Muircheartaigh
 * August 30 Warren Buffet
 * October 28 Bernie Ecclestone
 * December 15 Edna O'Brien

Nonsense
I am responsible for including the following in the Robert Marjolin article in 2006:
 * ...he was head of State Department responsible for producing the Pineau aperitif...

which was later corrected to
 * ...he was a member of the staff of the socialist minister of foreign affairs, Christian Pineau...

That nonsense was a consequence of applying Google translate to the German wikipedia page! Four years later it translates the same sentence correctly:
 * ...he...was...Cabinet Secretary in the Ministry of Foreign Affairs under Pineau ...

Mobile Phones

 * Nokia 1610 Dec 1996-2000 (end: needed to get an Irish account, got phone that could send txt messages)
 * ? 2000-2001 (end: Wanted more modern phone)
 * Siemens SL42 2001-2003 (end: Wanted more modern phone)
 * Nokia 6310i 2003-Dec 2007 (end: got given new phone for birthday)
 * Motorola v400 2004-Dec 2006 (first camera phone, end:Moved back to Ireland from USA)
 * Nokia E65 Apr-Aug 2007 (end: Lost in pub)
 * Nokia N70 Dec 2007-Jan 2010 (end: wanted more modern phone)
 * Nokia 5800 XpressMusic Jan 2010-July 2011 (end: Screen was broken, couldn't read)
 * HTC Desire S July 2011-Jan 2012 (end: Lost in taxi)
 * HTC Wildfire S Jan 2012-Feb 2013 (end: Qualified for an upgrade to faster phone)
 * Sony Xperia T Feb 2013-July 2014 (end: Camera focus damaged by car)
 * Sony Xperia Z1 Compact July 2014 - May 2016 (end: Touchscreen failure due to broken glass)
 * Sony Xperia Z5 Compact May 2016 - Dec 2016 (end: iPhone gift)
 * iPhone 6 Dec 2016 - Dec 2017 (end: iPhone 7 gift)
 * iPhone 7 Dec 2017 - Aug 2018 (end: iPhone X gift)
 * iPhone X Aug 2018 - Dec 2019 (end: iPhone 11 gift)
 * iPhone 11 Dec 2019 - Dec 2021 (end: iPhone 13 gift)
 * iPhone 13 Dec 2021 -

To do

 * User:Rye1967/KerryProj
 * search for (exact match) of 'Death of Former Member: Expressions of Sympathy' in http://debates.oireachtas.ie/DDebates.aspx?Dail=29&Type=DAL
 * create a list of irish political party leaders, with terms in service
 * Clann_na_Talmhan says it is 1943 onwards, but it was in the 1927 general election - see link from there, however wiki.politics.ie say that was the Farmers Union
 * according to Government of the 23rd Dail Albert Reynolds was temporarily min for Trade, Commeerce & Tourism in 1982, according to Irish_Minister_for_Industry_%26_Commerce it was Paddy Power (politician)
 * improve sentence structure in Fianna Fáil
 * remove links to Fianna Fail
 * define Rapprochement in Wikictionary.com and link from Bertie Ahern, search for other occurences in WikiPedia.com
 * review Irish categories at approx [|here]
 * review all links to Irish and see if they should instead go to one of the others, or not be a link at all

title: User:Rye 212


text:
User talk:Mazadillon

Leave notes for me here and I'll read them and then move them into the archive.

Notes Archive
You can use your user page for information about you. This "ytalk page" is for other Wikipeidans to leave you messages. Please do not edit the {welcome} template; it's used by all Wikipedians to welcome others. Any questions, feel free to leave a note on my talk page (use the link next to my name). Cheers, Whosyourjudas (talk) 21:06, 11 Oct 2004 (UTC)

it's no problem - i made all the newbie mistakes too (i still mess up sometimes!). don't worry about them; someone will catch any mistakes you might make. the best way to revert a change is to go to the page's history, pick the version you want, and save it to the article. good luck! Whosyourjudas (talk) 21:13, 11 Oct 2004 (UTC)

Fair use disputed for Image:Dancing lasha tumbai cover.jpg
Thanks for uploading Image:Dancing lasha tumbai cover.jpg. However, there is a concern that the rationale you have provided for using this image under "fair use" may be invalid. Please read the instructions at Non-free content carefully, then go to the image description page and clarify why you think the image qualifies for fair use. Using one of the templates at Fair use rationale guideline is an easy way to ensure that your image is in compliance with Wikipedia policy, but remember that you must complete the template. Do not simply insert a blank template on an image page.

If it is determined that the image does not qualify under fair use, it will be deleted within a couple of days according to our Criteria for speedy deletion. If you have any questions please ask them at the Media copyright questions page. Thank you.BetacommandBot 23:22, 13 September 2007 (UTC)

Disputed fair use rationale for Image:AVH NYC Beat.jpg
Thanks for uploading Image:AVH NYC Beat.jpg. However, there is a concern that the rationale you have provided for using this image under "fair use" may be invalid. Please read the instructions at Non-free content carefully, then go to the image description page and clarify why you think the image qualifies for fair use. Using one of the templates at Fair use rationale guideline is an easy way to ensure that your image is in compliance with Wikipedia policy, but remember that you must complete the template. Do not simply insert a blank template on an image page.

If it is determined that the image does not qualify under fair use, it will be deleted within a couple of days according to our criteria for speedy deletion. If you have any questions please ask them at the media copyright questions page. Thank you.BetacommandBot 03:54, 26 October 2007 (UTC)

Disputed fair use rationale for Image:David Whitfield Cara Mia.jpg
Thanks for uploading Image:David Whitfield Cara Mia.jpg. However, there is a concern that the rationale you have provided for using this image under "fair use" may be invalid. Please read the instructions at Non-free content carefully, then go to the image description page and clarify why you think the image qualifies for fair use. Using one of the templates at Fair use rationale guideline is an easy way to ensure that your image is in compliance with Wikipedia policy, but remember that you must complete the template. Do not simply insert a blank template on an image page.

If it is determined that the image does not qualify under fair use, it will be deleted within a couple of days according to our criteria for speedy deletion. If you have any questions please ask them at the media copyright questions page. Thank you.BetacommandBot 10:46, 27 October 2007 (UTC)

PPS3
The PPS3 article has the following:
 * Creation of sustainable, inclusive and mised communities

mised? Is that a typo? I can't figure it out! Rjm at sleepers (talk) 17:48, 7 May 2008 (UTC)

Nomination of Family and Parenting Institute for deletion
A discussion is taking place as to whether the article Family and Parenting Institute is suitable for inclusion in Wikipedia according to Wikipedia's policies and guidelines or whether it should be deleted.

The article will be discussed at Articles for deletion/Family and Parenting Institute until a consensus is reached, and anyone is welcome to contribute to the discussion. The nomination will explain the policies and guidelines which are of concern. The discussion focuses on good quality evidence, and our policies and guidelines.

Users may edit the article during the discussion, including to improve the article to address concerns raised in the discussion. However, do not remove the article-for-deletion template from the top of the article.  Dennis The Tiger  (Rawr and stuff) 17:52, 21 December 2011 (UTC)

title: User talk:Mazadillon


text:
Ataxia–telangiectasia

Ataxia–telangiectasia (AT or A–T), also referred to as ataxia–telangiectasia syndrome or Louis–Bar syndrome, is a rare, neurodegenerative disease causing severe disability. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels, both of which are hallmarks of the disease. A–T affects many parts of the body:
 * It impairs certain areas of the brain including the cerebellum, causing difficulty with movement and coordination.
 * It weakens the immune system, causing a predisposition to infection.
 * It prevents repair of broken DNA, increasing the risk of cancer.

Symptoms most often first appear in early childhood (the toddler stage) when children begin to sit or walk. Though they usually start walking at a normal age, they wobble or sway when walking, standing still or sitting. In late pre-school and early school age, they develop difficulty moving their eyes in a natural manner from one place to the next (oculomotor apraxia). They develop slurred or distorted speech, and swallowing problems. Some have an increased number of respiratory tract infections (ear infections, sinusitis, bronchitis, and pneumonia). Because not all children develop in the same manner or at the same rate, it may be some years before A–T is properly diagnosed. Most children with A–T have stable neurologic symptoms for the first 4–5 years of life, but begin to show increasing problems in early school years.

Causes
A–T has an autosomal recessive pattern of inheritance.

A–T is caused by a defect in the ATM gene, named after this disease, which is involved in the recognition and repair of damaged DNA. Heterozygotes will not experience the characteristic symptoms but it has been reported they have higher risks of cancer and heart disease. The prevalence of A–T is estimated to be as high as 1 in 40,000 to as low as 1 in 300,000 people.

Symptoms and signs
There is substantial variability in the severity of features of A–T among affected individuals, and at different ages. The following symptoms or problems are either common or important features of A–T:
 * Ataxia (difficulty with control of movement) that is apparent early but worsens in school to pre-teen years
 * Oculomotor apraxia (difficulty with coordination of head and eye movement when shifting gaze from one place to the next)
 * Involuntary movements
 * Telangiectasia (dilated blood vessels) over the white (sclera) of the eyes, making them appear bloodshot. These are not apparent in infancy and may first appear at age 5–8 years. Telangiectasia may also appear on sun-exposed areas of skin.
 * Problems with infections, especially of the ears, sinuses and lungs
 * Increased incidence of cancer (primarily, but not exclusively, lymphomas and leukemias)
 * Delayed onset or incomplete pubertal development, and very early menopause
 * Slowed rate of growth (weight and/or height)
 * Drooling particularly in young children when they are tired or concentrating on activities
 * Dysarthria (slurred, slow, or distorted speech)
 * Diabetes in adolescence or later
 * Premature changes in hair and skin

Many children are initially misdiagnosed as having cerebral palsy. The diagnosis of A–T may not be made until the preschool years when the neurologic symptoms of impaired gait, hand coordination, speech and eye movement appear or worsen, and the telangiectasia first appear. Because A–T is so rare, doctors may not be familiar with the symptoms, or methods of making a diagnosis. The late appearance of telangiectasia may be a barrier to the diagnosis. It may also take some time before doctors consider A–T as a possibility because of the early stability of symptoms and signs. There are patients who have been diagnosed with A-T only in adulthood due to an attenuated form of the disease, and this has been correlated with the type of their gene mutation.

Ataxia and other neurologic problems
The first indications of A–T usually occur during the toddler years. Children start walking at a normal age, but may not improve much from their initial wobbly gait. Sometimes they have problems standing or sitting still and tend to sway backward or from side to side. In primary school years, walking becomes more difficult, and children will use doorways and walls for support. Children with A–T often appear better when running or walking quickly in comparison to when they are walking slowly or standing in one place. Around the beginning of their second decade, children with the more severe ("classic") form of A–T start using a wheelchair for long distances. During school years, children may have increasing difficulty with reading because of impaired coordination of eye movement. At the same time, other problems with fine-motor functions (writing, coloring, and using utensils to eat), and with speech (dysarthria) may arise. Most of these neurologic problems stop progressing after the age of about 12 – 15 years, though involuntary movements may start at any age and may worsen over time. These extra movements can take many forms, including small jerks of the hands and feet that look like fidgeting (chorea), slower twisting movements of the upper body (athetosis), adoption of stiff and twisted postures (dystonia), occasional uncontrolled jerks (myoclonic jerks), and various rhythmic and non-rhythmic movements with attempts at coordinated action (tremors).

Telangiectasia
Prominent blood vessels (telangiectasia) over the white (sclera) of the eyes usually occur by the age of 5–8 years, but sometimes appear later or not at all. The absence of telangiectasia does not exclude the diagnosis of A–T. Potentially a cosmetic problem, the ocular telangiectasia do not bleed or itch, though they are sometimes misdiagnosed as chronic conjunctivitis. It is their constant nature, not changing with time, weather or emotion, that marks them as different from other visible blood vessels. Telangiectasia can also appear on sun-exposed areas of skin, especially the face and ears. They occur in the bladder as a late complication of chemotherapy with cyclophosphamide, have been seen deep inside the brain of older people with A–T, and occasionally arise in the liver and lungs.

Immune problems
About two-thirds of people with A–T have abnormalities of the immune system. The most common abnormalities are low levels of one or more classes of immunoglobulins (IgA, IgM, and IgG subclasses), not making antibodies in response to vaccines or infections, and having low numbers of lymphocytes (especially T-lymphocytes) in the blood. Some people have frequent infections of the upper (colds, sinus and ear infections) and lower (bronchitis and pneumonia) respiratory tract. All children with A–T should have their immune systems evaluated to detect those with severe problems that require treatment to minimize the number or severity of infections. Some people with A–T need additional immunizations (especially with pneumonia and influenza vaccines), antibiotics to provide protection (prophylaxis) from infections, and/or infusions of immunoglobulins (gamma globulin). The need for these treatments should be determined by an expert in the field of immunodeficiency or infectious diseases.

Cancer
People with A–T have a highly increased incidence (approximately 25% lifetime risk) of cancers, particularly lymphomas and leukemia, but other cancers can occur.

Women who are A–T carriers (who have one mutated copy of the ATM gene), have approximately a two-fold increased risk for the development of breast cancer compared to the general population. This includes all mothers of A–T children and some female relatives. Current consensus is that special screening tests are not helpful, but all women should have routine cancer surveillance.

Skin
A–T can cause features of early aging such as premature graying of the hair. It can also cause vitiligo (an auto-immune disease causing loss of skin pigment resulting in a blotchy "bleach-splashed" look), and warts which can be extensive and recalcitrant to treatment. A small number of people develop a chronic inflammatory skin disease (granulomas).

Lung disease
Chronic lung disease develops in more than 25% of people with A–T.

Lung function tests (spirometry) should be performed at least annually in children old enough to perform them, influenza and pneumococcal vaccines given to eligible individuals, and sinopulmonary infections treated aggressively to limit the development of chronic lung disease.

Feeding, swallowing, and nutrition
Feeding and swallowing can become difficult for people with A–T as they get older.

Involuntary movements may make feeding difficult or messy and may excessively prolong mealtimes. It may be easier to finger feed than use utensils (e.g., spoon or fork). For liquids, it is often easier to drink from a closed container with a straw than from an open cup. Caregivers may need to provide foods or liquids so that self-feeding is possible, or they may need to feed the person with A–T. In general, meals should be completed within approximately 30 minutes. Longer meals may be stressful, interfere with other daily activities, and limit the intake of necessary liquids and nutrients.

If swallowing problems (dysphagia) occur, they typically present during the second decade of life. Dysphagia is common because of the neurological changes that interfere with coordination of mouth and pharynx (throat) movements that are needed for safe and efficient swallowing. Coordination problems involving the mouth may make chewing difficult and increase the duration of meals. Problems involving the pharynx may cause liquid, food, and saliva to be inhaled into the airway (aspiration). People with dysphagia may not cough when they aspirate (silent aspiration). Swallowing problems and especially swallowing problems with silent aspiration may cause lung problems due to inability to cough and clear food and liquids from the airway.

 * Warning signs of a swallowing problem
 * Choking or coughing when eating or drinking
 * Poor weight gain (during ages of expected growth) or weight loss at any age
 * Excessive drooling
 * Mealtimes longer than 40 – 45 minutes, on a regular basis
 * Foods or drinks previously enjoyed are now refused or difficult
 * Chewing problems
 * Increase in the frequency or duration of breathing or respiratory problems
 * Increase in lung infections

Eye and vision

 * Most people develop telangiectasia (prominent blood vessels) in the membrane that covers the white part (sclera) of the eye.
 * Vision (ability to see objects in focus) is normal.
 * Control of eye movement is often impaired, affecting visual functions that require fast, accurate eye movements from point to point (e.g. reading).
 * Eye misalignments (strabismus) are common, but may be treatable.
 * There may be difficulty in coordinating eye position and shaping the lens to see objects up close.

Orthopedics
Many individuals with A–T develop deformities of the feet that compound the difficulty they have with walking due to impaired coordination. Early treatment may slow progression of this deformity. Bracing or surgical correction sometimes improves stability at the ankle sufficient to enable an individual to walk with support, or bear weight during assisted standing transfers from one seat to another. Severe scoliosis is relatively uncommon, but probably does occur more often than in those without A–T. Spinal fusion is only rarely indicated.

Genetics
A–T is caused by mutations in the ATM (ATM serine/threonine kinase or ataxia–telangiectasia mutated) gene, which was cloned in 1995. ATM is located on human chromosome 11 (11q22.3) and is made up of 69 exons spread across 150kb of genomic DNA.

The mode of inheritance for A–T is autosomal recessive. Each parent is a carrier, meaning that they have one normal copy of the A–T gene (ATM) and one copy that is mutated. A–T occurs if a child inherits the mutated A–T gene from each parent, so in a family with two carrier parents, there is 1 chance in 4 that a child born to the parents will have the disorder. Prenatal diagnosis (and carrier detection) can be carried out in families if the errors (mutation) in an affected child's two ATM genes have been identified. The process of getting this done can be complicated and, as it requires time, should be arranged before conception.

Looking for mutations in the ATM gene of an unrelated person (for example, the spouse of a known A–T carrier) presents significant challenges. Genes often have variant spellings (polymorphisms) that do not affect function. In a gene as large as ATM, such variant spellings are likely to occur and doctors cannot always predict whether a specific variant will or will not cause disease. Genetic counseling can help family members of an A–T patient understand what can or cannot be tested, and how the test results should be interpreted.

Carriers of A–T, such as the parents of a person with A–T, have one mutated copy of the ATM gene and one normal copy. They are generally healthy, but there is an increased risk of breast cancer in women. This finding has been confirmed in a variety of different ways, and is the subject of current research. Standard surveillance (including monthly breast self-exams and mammography at the usual schedule for age) is recommended, unless additional tests are indicated because the individual has other risk factors (e.g., family history of breast cancer).

Pathophysiology
How loss of the ATM protein creates a multisystem disorder A–T has been described as a genome instability syndrome, a DNA repair disorder and a DNA damage response (DDR) syndrome. ATM, the gene responsible for this multi-system disorder, encodes a protein of the same name which coordinates the cellular response to DNA double strand breaks (DSBs). Radiation therapy, chemotherapy that acts like radiation (radiomimetic drugs) and certain biochemical processes and metabolites can cause DSBs. When these breaks occur, ATM stops the cell from making new DNA (cell cycle arrest) and recruits and activates other proteins to repair the damage. Thus, ATM allows the cell to repair its DNA before the completion of cell division. If DNA damage is too severe, ATM will mediate the process of programmed cell death (apoptosis) to eliminate the cell and prevent genomic instability.

Cancer and radiosensitivity
In the absence of the ATM protein, cell-cycle check-point regulation and programmed cell death in response to DSBs are defective. The result is genomic instability which can lead to the development of cancers.

Irradiation and radiomimetic compounds induce DSBs which are unable to be repaired appropriately when ATM is absent. Consequently, such agents can prove especially cytotoxic to A–T cells and people with A–T.

Delayed pubertal development (gonadal dysgenesis)
Infertility is often described as a characteristic of A–T. Whereas this is certainly the case for the mouse model of A–T, in humans it may be more accurate to characterize the reproductive abnormality as gonadal atrophy or dysgenesis characterized by delayed pubertal development. Because programmed DSBs are generated to initiate genetic recombinations involved in the production of sperm and eggs in reproductive organs (a process known as meiosis), meiotic defects and arrest can occur when ATM is not present.

Immune system defects and immune-related cancers
As lymphocytes develop from stem cells in the bone marrow into mature lymphocytes in the periphery, they rearrange special segments of their DNA [V(D)J recombination process]. This process requires them to make DSBs, which are difficult to repair in the absence of ATM. As a result, most people with A–T have reduced numbers of lymphocytes and some impairment of lymphocyte function (such as an impaired ability to make antibodies in response to vaccines or infections). In addition, broken pieces of DNA in chromosomes involved in the above-mentioned rearrangements have a tendency to recombine with other genes (translocation), making the cells prone to the development of cancer (lymphoma and leukemia).

Progeric changes
Cells from people with A–T demonstrate genomic instability, slow growth and premature senescence in culture, shortened telomeres and an ongoing, low-level stress response. These factors may contribute to the progeric (signs of early aging) changes of skin and hair sometimes observed in people with A–T. For example, DNA damage and genomic instability cause melanocyte stem cell (MSC) differentiation which produces graying. Thus, ATM may be a "stemness checkpoint" protecting against MSC differentiation and premature graying of the hair.

Telangiectasia
The cause of telangiectasia or dilated blood vessels in the absence of the ATM protein is not yet known.

Increased alpha-fetoprotein (AFP) levels
Approximately 95% of people with A–T have elevated serum AFP levels after the age of two, and measured levels of AFP appear to increase slowly over time. AFP levels are very high in the newborn, and normally descend to adult levels over the first year to 18 months. The reason why individuals with A–T have elevated levels of AFP is not yet known.

Neurodegeneration
A–T is one of several DNA repair disorders that result in neurological abnormalities or degeneration. Arguably some of the most devastating symptoms of A–T are a result of progressive cerebellar degeneration, characterized by the loss of Purkinje cells and, to a lesser extent, granule cells (located exclusively in the cerebellum). The cause of this cell loss is not known, though many hypotheses have been proposed based on experiments performed both in cell culture and in the mouse model of A–T. Current hypotheses explaining the neurodegeneration associated with A–T include the following: These hypotheses may not be mutually exclusive and more than one of these mechanisms may underlie neuronal cell death when there is an absence or deficiency of ATM. Further, cerebellar damage and loss of Purkinje and granule cells do not explain all of the neurologic abnormalities seen in people with A–T. The effects of ATM deficiency on the other areas of the brain outside of the cerebellum are being actively investigated.
 * Defective DNA damage response in neurons which can lead to
 * Failed clearance of genomically damaged neurons during development
 * Transcription stress and abortive transcription including topoisomerase 1 cleavage complex (TOP1cc) dependent lesions
 * Aneuploidy
 * Defective response to oxidative stress characterized by elevated ROS and altered cellular metabolism
 * Mitochondrial dysfunction
 * Defects in neuronal function:
 * Inappropriate cell cycle re-entry of post-mitotic (mature) neurons
 * Synaptic/vesicular dysregulation
 * HDAC4 dysregulation
 * Histone hypermethylation and altered epigenetics
 * Altered protein turnover

Radiation exposure
People with A–T have an increased sensitivity to ionizing radiation (X-rays and gamma rays). Therefore, X-ray exposure should be limited to times when it is medically necessary, as exposing an A–T patient to ionizing radiation can damage cells in such a way that the body cannot repair them. The cells can cope normally with other forms of radiation, such as ultraviolet light, so there is no need for special precautions from sunlight exposure.

Diagnosis
The diagnosis of A–T is usually suspected by the combination of neurologic clinical features (ataxia, abnormal control of eye movement, and postural instability) with telangiectasia and sometimes increased infections, and confirmed by specific laboratory abnormalities (elevated alpha-fetoprotein levels, increased chromosomal breakage or cell death of white blood cells after exposure to X-rays, absence of ATM protein in white blood cells, or mutations in each of the person's ATM genes).

A variety of laboratory abnormalities occur in most people with A–T, allowing for a tentative diagnosis to be made in the presence of typical clinical features. Not all abnormalities are seen in all patients. These abnormalities include:
 * Elevated and slowly increasing alpha-fetoprotein levels in serum after 2 years of age
 * Immunodeficiency with low levels of immunoglobulins (especially IgA, IgM, IgG, and IgG subclasses) and low number of lymphocytes in the blood
 * Chromosomal instability (broken pieces of chromosomes)
 * Increased sensitivity of cells to x-ray exposure (cells die or develop even more breaks and other damage to chromosomes)
 * Cerebellar atrophy on MRI scan

The diagnosis can be confirmed in the laboratory by finding an absence or deficiency of the ATM protein in cultured blood cells, an absence or deficiency of ATM function (kinase assay), or mutations in both copies of the cell's ATM gene. These more specialized tests are not always needed, but are particularly helpful if a child's symptoms are atypical.

Differential diagnosis
There are several other disorders with similar symptoms or laboratory features that physicians may consider when diagnosing A–T. The three most common disorders that are sometimes confused with A–T are: Each of these can be distinguished from A–T by the neurologic exam and clinical history.
 * Cerebral palsy
 * Friedreich's ataxia
 * Cogan oculomotor apraxia

Cerebral palsy (CP) describes a non-progressive disorder of motor function stemming from malformation or early damage to the brain. CP can manifest in many ways, given the different manner in which the brain can be damaged; in common to all forms is the emergence of signs and symptoms of impairment as the child develops. However, milestones that have been accomplished and neurologic functions that have developed do not deteriorate in CP as they often do in children with A–T in the late pre-school years. Most children with ataxia caused by CP do not begin to walk at a normal age, whereas most children with A–T start to walk at a normal age even though they often "wobble" from the start. Pure ataxia is a rare manifestation of early brain damage or malformation, however, and the possibility of an occult genetic disorder of brain should be considered and sought for those in whom ataxia is the chief manifestation of CP. Children with ataxic CP will not manifest the laboratory abnormalities associated with A–T.

Cogan occulomotor apraxia is a rare disorder of development. Affected children have difficulty moving their eyes only to a new visual target, so they will turn their head past the target to "drag" the eyes to the new object of interest, then turn the head back. This tendency becomes evident in late infancy and toddler years, and mostly improves with time. This contrasts to the oculomotor difficulties evident in children with A–T, which are not evident in early childhood but emerge over time. Cogan's oculomotor apraxia is generally an isolated problem, or may be associated with broader developmental delay.

Friedreich ataxia (FA) is the most common genetic cause of ataxia in children. Like A–T, FA is a recessive disease, appearing in families without a history of the disorder. FA is caused by mutation in the frataxin gene, most often an expansion of a naturally occurring repetition of the three nucleotide bases GAA from the usual 5–33 repetitions of this trinucleotide sequence to greater than 65 repeats on each chromosome. Most often the ataxia appears between 10 and 15 years of age, and differs from A–T by the absence of telangiectasia and oculomotor apraxia, a normal alpha fetoprotein, and the frequent presence of scoliosis, absent tendon reflexes, and abnormal features on the EKG. Individuals with FA manifest difficulty standing in one place that is much enhanced by closure of the eyes (Romberg sign) that is not so apparent in those with A–T – even though those with A–T may have greater difficulty standing in one place with their eyes open.

There are other rare disorders that can be confused with A–T, either because of similar clinical features, a similarity of some laboratory features, or both. These include: Ataxia–oculomotor apraxia type 1 (AOA1) is an autosomal recessive disorder similar to A–T in manifesting increasing problems with coordination and oculomotor apraxia, often at a similar age to those having A–T. It is caused by mutation in the gene coding for the protein aprataxin. Affected individuals differ from those with A–T by the early appearance of peripheral neuropathy, early in their course manifest difficulty with initiation of gaze shifts, and the absence of ocular telangiectasia, but laboratory features are of key importance in the differentiation of the two. Individuals with AOA1 have a normal AFP, normal measures of immune function, and after 10–15 years have low serum levels of albumin. Genetic testing of the aprataxin gene can confirm the diagnosis. There is no enhanced risk for cancer.
 * Ataxia–oculomotor apraxia type 1 (AOA1)
 * Ataxia–oculomotor apraxia type 2 (AOA2 also known as SCAR1)
 * Ataxia–telangiectasia like disorder (ATLD)
 * Nijmegen breakage syndrome (NBS)

Ataxia–oculomotor apraxia type 2 (AOA2) is an autosomal recessive disorder also similar to A–T in manifesting increasing problems with coordination and peripheral neuropathy, but oculomotor apraxia is present in only half of affected individuals. Ocular telangiectasia do not develop. Laboratory abnormalities of AOA2 are like A–T, and unlike AOA1, in having an elevated serum AFP level, but like AOA1 and unlike A–T in having normal markers of immune function. Genetic testing of the senataxin gene (SETX) can confirm the diagnosis. There is no enhanced risk for cancer.

Ataxia–telangiectasia like disorder (ATLD) is an extremely rare condition, caused by mutation in the hMre11 gene, that could be considered in the differential diagnosis of A–T. Patients with ATLD are very similar to those with A–T in showing a progressive cerebellar ataxia, hypersensitivity to ionizing radiation and genomic instability. Those rare individuals with ATLD who are well described differ from those with A–T by the absence of telangiectasia, normal immunoglobulin levels, a later onset, and a slower progression of the symptoms. Because of its rarity, it is not yet known whether or not ATLD carries an increased risk to develop cancer. Because those mutations of Mre11 that severely impair the MRE11 protein are incompatible with life, individuals with ATLD all have some partial function of the Mre11 protein, and hence likely all have their own levels of disease severity.

Nijmegen breakage syndrome (NBS) is a rare genetic disorder that has similar chromosomal instability to that seen in people with A–T, but the problems experienced are quite different. Children with NBS have significant microcephaly, a distinct facial appearance, short stature, and moderate cognitive impairment, but do not experience any neurologic deterioration over time. Like those with A–T, children with NBS have enhanced sensitivity to radiation, disposition to lymphoma and leukemia, and some laboratory measures of impaired immune function, but do not have ocular telangiectasia or an elevated level of AFP.

The proteins expressed by the hMre11 (defective in ATLD) and Nbs1 (defective in NBS) genes exist in the cell as a complex, along with a third protein expressed by the hRad50 gene. This complex, known as the MRN complex, plays an important role in DNA damage repair and signaling and is required to recruit ATM to the sites of DNA double strand breaks. Mre11 and Nbs1 are also targets for phosphorylation by the ATM kinase. Thus, the similarity of the three diseases can be explained in part by the fact that the protein products of the three genes mutated in these disorders interact in common pathways in the cell.

Differentiation of these disorders is often possible with clinical features and selected laboratory tests. In cases where the distinction is unclear, clinical laboratories can identify genetic abnormalities of ATM, aprataxin and senataxin, and specialty centers can identify abnormality of the proteins of potentially responsible genes, such as ATM, MRE11, nibrin, TDP1, aprataxin and senataxin as well as other proteins important to ATM function such as ATR, DNA-PK, and RAD50.

Ataxia and other neurologic problems
There is no treatment known to slow or stop the progression of the neurologic problems.

Immune problems
All individuals with A–T should have at least one comprehensive immunologic evaluation that measures the number and type of lymphocytes in the blood (T-lymphocytes and B-lymphocytes), the levels of serum immunoglobulins (IgG, IgA, and IgM) and antibody responses to T-dependent (e.g., tetanus, Hemophilus influenzae b) and T-independent (23-valent pneumococcal polysaccharide) vaccines. For the most part, the pattern of immunodeficiency seen in an A–T patient early in life (by age five) will be the same pattern seen throughout the lifetime of that individual. Therefore, the tests need not be repeated unless that individual develops more problems with infection. Problems with immunity sometimes can be overcome by immunization. Vaccines against common bacterial respiratory pathogens such as Hemophilus influenzae, pneumococci and influenza virus (the "flu") are commercially available and often help to boost antibody responses, even in individuals with low immunoglobulin levels. If the vaccines do not work and the patient continues to have problems with infections, gamma globulin therapy (IV or subcutaneous infusions of antibodies collected from normal individuals) may be of benefit. A small number of people with A–T develop an abnormality in which one or more types of immunoglobulin are increased far beyond the normal range. In a few cases, the immunoglobulin levels can be increased so much that the blood becomes thick and does not flow properly. Therapy for this problem must be tailored to the specific abnormality found and its severity.

If an individual patient's susceptibility to infection increases, it is important to reassess immune function in case deterioration has occurred and a new therapy is indicated. If infections are occurring in the lung, it is also important to investigate the possibility of dysfunctional swallow with aspiration into the lungs (see above sections under Symptoms: Lung Disease and Symptoms: Feeding, Swallowing and Nutrition.)

Most people with A–T have low lymphocyte counts in the blood. This problem seems to be relatively stable with age, but a rare number of people do have progressively decreasing lymphocyte counts as they get older. In the general population, very low lymphocyte counts are associated with an increased risk for infection. Such individuals develop complications from live viral vaccines (measles, mumps, rubella and chickenpox), chronic or severe viral infections, yeast infections of the skin and vagina, and opportunistic infections (such as pneumocystis pneumonia). Although lymphocyte counts are often as low in people with A–T, they seldom have problems with opportunistic infections. (The one exception to that rule is that problems with chronic or recurrent warts are common.) The number and function of T-lymphocytes should be re-evaluated if a person with A–T is treated with corticosteroid drugs such as prednisone for longer than a few weeks or is treated with chemotherapy for cancer. If lymphocyte counts are low in people taking those types of drugs, the use of prophylactic antibiotics is recommended to prevent opportunistic infections.

If the tests show significant abnormalities of the immune system, a specialist in immunodeficiency or infectious diseases will be able to discuss various treatment options. Absence of immunoglobulin or antibody responses to vaccine can be treated with replacement gamma globulin infusions, or can be managed with prophylactic antibiotics and minimized exposure to infection. If antibody function is normal, all routine childhood immunizations including live viral vaccines (measles, mumps, rubella and varicella) should be given. In addition, several "special" vaccines (that is, licensed but not routine for otherwise healthy children and young adults) should be given to decrease the risk that an A–T patient will develop lung infections. The patient and all household members should receive the influenza (flu) vaccine every fall. People with A–T who are less than two years old should receive three doses of a pneumococcal conjugate vaccine (Prevnar) given at two month intervals. People older than two years who have not previously been immunized with Prevnar should receive two doses of Prevnar. At least 6 months after the last Prevnar has been given and after the child is at least two years old, the 23-valent pneumococcal vaccine should be administered. Immunization with the 23-valent pneumococcal vaccine should be repeated approximately every five years after the first dose.

In people with A–T who have low levels of IgA, further testing should be performed to determine whether the IgA level is low or completely absent. If absent, there is a slightly increased risk of a transfusion reaction. "Medical Alert" bracelets are not necessary, but the family and primary physician should be aware that if there is elective surgery requiring red cell transfusion, the cells should be washed to decrease the risk of an allergic reaction.

People with A–T also have an increased risk of developing autoimmune or chronic inflammatory diseases. This risk is probably a secondary effect of their immunodeficiency and not a direct effect of the lack of ATM protein. The most common examples of such disorders in A–T include immune thrombocytopenia (ITP), several forms of arthritis, and vitiligo.

Lung disease
Recurrent sinus and lung infections can lead to the development of chronic lung disease. Such infections should be treated with appropriate antibiotics to prevent and limit lung injury. Administration of antibiotics should be considered when children and adults have prolonged respiratory symptoms (greater than 7 days), even following what was presumed to have been a viral infection. To help prevent respiratory illnesses from common respiratory pathogens, annual influenza vaccinations should be given and pneumococcal vaccines should be administered when appropriate. Antibiotic treatment should also be considered in children with chronic coughs that are productive of mucous, those who do not respond to aggressive pulmonary clearance techniques and in children with muco-purulent secretions from the sinuses or chest. A wet cough can also be associated with chronic aspiration which should be ruled out through proper diagnostic studies, however, aspiration and respiratory infections are not necessarily exclusive of each other. In children and adults with bronchiectasis, chronic antibiotic therapy should be considered to slow chronic lung disease progression.

Culturing of the sinuses may be needed to direct antibiotic therapy. This can be done by an Ear Nose and Throat (ENT) specialist. In addition, diagnostic bronchoscopy may be necessary in people who have recurrent pneumonias, especially those who do not respond or respond incompletely to a course of antibiotics.

Clearance of bronchial secretions is essential for good pulmonary health and can help limit injury from acute and chronic lung infections. Children and adults with increased bronchial secretions can benefit from routine chest therapy using the manual method, an a cappella device or a chest physiotherapy vest. Chest physiotherapy can help bring up mucous from the lower bronchial tree, however, an adequate cough is needed to remove secretions. In people who have decreased lung reserve and a weak cough, use of an insufflator-exsufflator (cough-assist) device may be useful as a maintenance therapy or during acute respiratory illnesses to help remove bronchial secretions from the upper airways. Evaluation by a Pulmonology specialist, however, should first be done to properly assess patient suitability.

Children and adults with chronic dry cough, increased work of breathing (fast respiratory rate, shortness of breath at rest or with activities) and absence of an infectious process to explain respiratory symptoms should be evaluated for interstitial lung disease or another intrapulmonary process. Evaluation by a Pulmonologist and a CT scan of the chest should be considered in individuals with symptoms of interstitial lung disease or to rule other non-infectious pulmonary processes. People diagnosed with interstitial lung disease may benefit from systemic steroids.

Feeding, swallowing and nutrition
Oral intake may be aided by teaching persons with A–T how to drink, chew and swallow more safely. The propriety of treatments for swallowing problems should be determined following evaluation by an expert in the field of speech-language pathology. Dieticians may help treat nutrition problems by recommending dietary modifications, including high calorie foods or food supplements.

A feeding (gastrostomy) tube is recommended when any of the following occur:
 * A child cannot eat enough to grow or a person of any age cannot eat enough to maintain weight;
 * Aspiration is problematic;
 * Mealtimes are stressful or too long, interfering with other activities.

Education and socialization
Most children with A–T have difficulty in school because of a delay in response time to visual, verbal or other cues, slurred and quiet speech (dysarthria), abnormalities of eye control (oculomotor apraxia), and impaired fine motor control. Despite these problems, children with A–T often enjoy school if proper accommodations to their disability can be made. The decision about the need for special education classes or extra help in regular classes is highly influenced by the local resources available. Decisions about proper educational placement should be revisited as often as circumstances warrant. Despite their many neurologic impairments, most individuals with A–T are very socially aware and socially skilled, and thus benefit from sustained peer relationships developed at school. Some individuals are able to function quite well despite their disabilities and a few have graduated from community colleges.

Many of the problems encountered will benefit from special attention, as problems are often related more to "input and output" issues than to intellectual impairment. Problems with eye movement control make it difficult for people with A–T to read, yet most fully understand the meaning and nuances of text that is read to them. Delays in speech initiation and lack of facial expression make it seem that they do not know the answers to questions. Reduction of the skilled effort needed to answer questions, and an increase of the time available to respond, is often rewarded by real accomplishment. It is important to recognize that intellectual disability is not regularly a part of the clinical picture of A–T although school performance may be suboptimal because of the many difficulties in reading, writing, and speech. Children with A–T are often very conscious of their appearance, and strive to appear normal to their peers and teachers. Life within the ataxic body can be tiring. The enhanced effort needed to maintain appearances and increased energy expended in abnormal tone and extra movements all contribute to physical and mental fatigue. As a consequence, for some a shortened school day yields real benefits.

General recommendations

 * All children with A–T need special attention to the barriers they experience in school. In the United States, this takes the form of a formal IEP (Individualized Education Program).
 * Children with A–T tend to be excellent problem solvers. Their involvement in how to best perform tasks should be encouraged.
 * Speech-language pathologists may facilitate communication skills that enable persons with A–T to get their messages across (using key words vs. complete sentences) and teach strategies to decrease frustration associated with the increase time needed to respond to questions (e.g., holding up a hand and informing others about the need to allow more time for responses). Rarely helpful are traditional speech therapies that focus on the production of specific sounds and strengthening of the lip and tongue muscles.
 * Classroom aides may be appropriate, especially to help with scribing, transportation through the school, mealtimes and toileting. The impact of an aide on peer relationships should be monitored carefully.
 * Physical therapy is useful to maintain strength and general cardiovascular health. Horseback therapy and exercises in a swimming pool are often well tolerated and fun for people with A–T. However, no amount of practice will slow the cerebellar degeneration or improve neurologic function. Exercise to the point of exhaustion should be avoided.
 * Hearing is normal throughout life. Books on tape may be a useful adjunct to traditional school materials.
 * Early use of computers (preschool) with word completion software should be encouraged.
 * Practicing coordination (e.g. balance beam or cursive writing exercises) is not helpful.
 * Occupational therapy is helpful for managing daily living skills.
 * Allow rest time, shortened days, reduced class schedule, reduced homework, modified tests as necessary.
 * Like all children, those with A–T need to have goals to experience the satisfaction of making progress.
 * Social interactions with peers are important, and should be taken into consideration for class placement. For everyone long-term peer relationships can be the most rewarding part of life; for those with A–T establishing these connections in school years can be helpful.

Treatment
No curative medication has been approved for the treatment of inherited cerebellar ataxias, including Ataxia-Telangiectasia.

N-Acetyl-Leucine
N-Acetyl-Leucine is an orally administered, modified amino acid that is being developed as a novel treatment for multiple rare and common neurological disorders by IntraBio Inc (Oxford, United Kingdom).

N-Acetyl-Leucine has been granted multiple orphan drug designations from the U.S. Food & Drug Administration (FDA) and the European Medicines Agency (EMA) for the treatment of various genetic diseases, including Ataxia-Telangiectasia. N-Acetyl-Leucine has also been granted Orphan Drug Designations in the US and EU for related inherited cerebellar ataxias, such as Spinocerebellar Ataxias. U.S. Food & Drug Administration (FDA) and the European Medicines Agency (EMA).

Published case series studies have demonstrated the positive clinical benefit of treatment with N-Acetyl-Leucine various inherited cerebellar ataxias.

A multinational clinical trial investigating N-Acetyl-L-Leucine for the treatment Ataxia-Telangiectasia began in 2019.

IntraBio is also conducting two parallel clinical trials with N-Acetyl-L-Leucine for the treatment Niemann-Pick disease type C and GM2 Gangliosidosis (Tay-Sachs and Sandhoff Disease) Future opportunities to develop N-Acetyl-Leucine include Lewy Body Dementia, Amyotrophic lateral sclerosis, Restless Leg Syndrome, Multiple Sclerosis, and Migraine.

Prognosis
Median survival in two large cohorts studies was 25 and 19 years of age, with a wide range.

Life expectancy does not correlate well with severity of neurological impairment.

Epidemiology
Individuals of all races and ethnicities are affected equally. The incidence worldwide is estimated to be between 1 in 40,000 and 1 in 100,000 people.

Research directions
An open-label Phase II clinical trial studying the use of red blood cells (erythrocytes) loaded with dexamethasone sodium phosphate found that this treatment improved symptoms and appeared to be well tolerated. This treatment uses a unique delivery system for medication by using the patient's own red blood cells as the delivery vehicle for the drug.

title: Ataxia–telangiectasia