Thursday, August 25, 2016

Microcephaly (+ Notable Cases)

Kokuvi, a seventeen-year-old student at the Volta School for the Mentally Challenged who has microcephaly Allison Stillwell
Microphallus is a neurodevelopmental disorder in which the circumference of the head is more than two standard deviations smaller than average for the person's age and sex. Microcephaly may be congenital or it may develop in the first few years of life. The disorder may stem from a wide variety of conditions that cause abnormal growth of the brain, or from syndromes associated with chromosomal abnormalities. Two copies of a loss-of-function mutation in one of the microcephalin genes causes primary microcephaly.

In general, life expectancy for individuals with microcephaly is reduced and the prognosis for normal brain function is poor. The prognosis varies depending on the presence of associated abnormalities.
Microcephaly is a type of cephalic disorder. This is a disorder characterized by a small head and may be caused by a disturbance in the rapid growing of nerve cells. Microcephaly may also be associated with maternal problems such as alcoholism (which can result in the fetal alcohol syndrome disability), diabetes, or rubella (German measles). After the dropping of atomic bombs on Hiroshima and Nagasaki, a large percentage of women who had been pregnant at the time gave birth to children with microcephaly.A genetic factor may play a role in causing some cases of microcephaly. Affected newborns generally have striking neurological defects and seizures. Severely impaired intellectual development is common, but disturbances in motor functions may not appear until later in life.

Infants with microcephaly are born with either a normal or reduced head size. Subsequently the head fails to grow while the face continues to develop at a normal rate, producing a child with a small head and a receding forehead, and a loose, often wrinkled scalp. As the child grows older, the smallness of the skull becomes more obvious, although the entire body also is often underweight and dwarfed. Development of motor functions and speech may be delayed. Hyperactivity and mental retardation are common occurrences, although the degree of each varies. Convulsions may also occur. Motor ability varies, ranging from clumsiness in some to spastic quadriplegia in others.

Side-view illustration of a baby with microcephaly (left) compared to a baby with a typical head size

Generally there is no specific treatment for microcephaly. Treatment is symptomatic and supportive.

Numerous superstitions revolve around microcephalics in Pakistan, ranging from being condemned as outcasts and institutionally shunned to having divine powers attributed to them.Local myths concerning their origins involve infertile women who practice religious rituals in order to facilitate childbirth, and local religious clergies take the health of the firstborn as a price, cursing them into being a "chooha", mouse/rat in Hindi & Urdu. That child has to be given back to the shrine where it would be raised, and live, as an acolyte. Should she fail to do so, all future children will be born choohas as well. Other Pakistanis believe that priests, chooha-masters, or perhaps even parents, purposefully deform healthy infants by placing pots or metal clamps on the heads of healthy infants and so retard the growth of the brain.



Notable microcephalics

Schlitzie, a circus performer best known for his role in the 1932 film Freaks.
Beetlejuice, a member of Howard Stern's "Wack Pack".
Zip the Pinhead (Circus "freak show" performer William Henry Johnson) is commonly associated with microcephaly, although he may not have actually had it.



Schlitzie

Schlitzie (September 10, 1901 – September 24, 1971), possibly born Simon Metz, and legally Schlitze Surtees, was a notable American sideshow performer and occasional actor, best known for his role in the 1932 movie Freaks and his life-long career on the outdoor entertainment circuit as a major sideshow attraction.

His name is alternately spelled "Schlitze" or "Shlitze".

Schlitzie's true birth date, name and location is unknown; it is commonly accepted, at least by what was inscribed on his death certificate and gravesite, that he was born on September 10, 1901 in The Bronx, New York City, New York, though 1891 has also been strongly considered as a possibility. Additionally, some sources have claimed that he was born in Santa Fe, New Mexico. Claims that he was born in Yucat√°n, Mexico, are probably mistaken reflections of Schlitzie's occasional fanciful billing as "The Last of the Aztecs". Schlitzie's born identity may never be known, the information having been lost as he was handed off to various carnivals in a long line of mostly informal guardianship throughout his career.

Schlitzie Microcephaly
Schlitzie was born with microcephaly, a neurodevelopmental disorder that left him with an unusually small brain and skull, a small stature (he stood about four feet tall (122cm)), myopia and moderate to severe mental retardation. It was said Schlitzie had the cognizance of a three year-old: he was unable to fully care for himself and he could only speak in monosyllabic words and form a few simple phrases. However, he was still able to perform simple tasks and it is believed that he could understand most of what was said to him, as he had a very quick reaction time and the ability to mimic. Those who knew Schlitzie described him as an affectionate, exuberant, sociable person who loved dancing, singing and being the center of attention, performing for anyone he could stop and talk to.

In accordance with the common practices of the carnival business of the time, it can be assumed that Schlitzie was either given to or purchased by a sideshow from his (thus far unknown or unidentified) birth parents. His guardians were usually his employers, sometimes by law and sometimes simply de facto. Responsibility for him tended to change hands as shows traded attractions. Most accounts tell that he was widely known, well cared for and treated affectionately throughout his carnival years.


CAREER

Schlitzie Microcephaly
On the sideshow circuit, microcephalics were usually promoted as "pinheads" or "missing links", and Schlitzie was billed under such titles as "The Last of the Aztecs", "The Monkey Girl" or simply "What Is It?", or was paired up with other microcephalics. One notable example of the latter was Schlitzie's possible pairing with a microcephalic woman named Athelia, exhibited together as "Aurora and Natalia, the Aztec sisters". This has led to claims that Aurora actually was Schlitzie's sister, but these claims are unsubstantiated.

Schlitzie was often dressed in a muumuu and either presented as a female or left androgynous to add to the mystique of his odd appearance. Those who knew him alternately referred to him as "he" and "she". Most sources say the choice to dress him like this was also due to his incontinence, as Schlitzie had to wear diapers and such clothing made it easier to care for him. However, even this is debatable, as most pictures show that Schlitzie was in fact wearing pants under his dress and some who knew him have said that he didn't develop incontinence until later in life.

The sideshow circuit was a tremendous success for Schlitzie, and throughout the 1920s and 1930s, he was employed by many upscale circuses, including Ringling Bros. and Barnum & Bailey Circus, Clyde Beatty Circus, Tom Mix Circus, Crafts 20 Big Shows and Foley & Burke Carnival. In 1928, Schlitzie made his film debut in the The Sideshow, a drama that took place at a circus, featuring a variety of actual sideshow performers including himself.

FREAKS AND LATER WORKS

In 1932, Schlitzie landed his most known role as an actor in Tod Browning's iconic horror film Freaks. Not unlike The Sideshow, Freaks also took place at a carnival and featured a number of genuine sideshow performers, among them conjoined twins Daisy and Violet Hilton and Prince Randian, "The Living Torso". A group of "pinheads" appear throughout the film, and Schlitzie (who is referred to in the feminine sense) has a scene of (unintelligible) dialogue opposite actor Wallace Ford.

Freaks' use of a deformed cast proved extremely controversial upon its release, and the film was ultimately a financial failure. Additionally, it was banned for thirty years in the United Kingdom and Browning struggled to find work following its release, his last with a major studio. Schlitzie appeared in bit roles in various movies, and is credited with a role in the 1934 exploitation film Tomorrow's Children, in a brief role as a mentally defective criminal who undergoes forced sterilization. He is often widely cited as appearing in 1933's Island of Lost Souls, in a seconds-long role as "Furry Mammal", However, there have been some doubts about whether or not the performer in these two films is actually Schlitzie or just a look-alike.

Schlitzie Microcephaly
In 1935, while Schlitzie was performing with the Tom Mix Circus, George Surtees, a chimpanzee trainer also appearing at the show with a trained chimp act, adopted him, becoming Schlitzie's legal guardian. In 1941, Schlitzie appeared in his final film role as "Princess Betsy", a sideshow attraction, in Meet Boston Blackie.

HOSPITALIZATION AND FINAL YEARS

Under the care of Surtees, Schlitzie continued performing the sideshow circuit until Surtees' death in the early 1960s, after which Surtees' daughter, who was not in show business, committed Schlitzie to a Los Angeles county hospital.

Schlitzie remained hospitalized for some time until he was recognized by sword swallower Bill Unks, who happened to be working at the hospital during the off season. According to Unks, Schlitzie seemed to miss the carnival dearly, and being away from the public eye had made him very sad and depressed. Hospital authorities determined that the best care for Schlitzie would be to make him a ward of Unks' employer, showman Sam Kortes, and return him to the sideshow.

In his later years, Schlitzie lived in Los Angeles, California, occasionally performing on various sideshow circuits both locally and internationally (he frequently performed in Hawaii and London, and his last major appearance was at the 1968 Dobritch International Circus held at the Los Angeles Sports Arena). Schlitzie also became a notable attraction performing on the streets of Hollywood, his caretakers selling his stock carnival souvenir pictures. Schlitzie spent time in his final days visiting MacArthur Park, feeding the pigeons and ducks with his guardian and performing for people as they passed by.

On September 24, 1971, at the age of 70, Schlitzie died from bronchial pneumonia at Fountain View Convalescent Home. His death certificate listed his official name as "Shlitze Surtees" and his birth date as 1901. He was interred in an unmarked grave at Queen of Heaven Cemetery in Rowland Heights. Schlitzie's grave remained unmarked until August 2008, when members of the website www.findadeath.com raised almost $400 to purchase him a proper headstone, which displays his name as "Schlitze Surtees" and his birth date as September 10, 1901.


Zip the Pinhead

Zip the Pinhead
Zip the Pinhead, born William Henry Johnson (1842/1843 in Liberty Corner, New Jersey – April 9, 1926 in New York City, New York), was an American freak show performer famous for his oddly tapered head.

EARLY LIFE

William Henry Johnson was born to a very poor African-American family. His parents were William and Mahalia Johnson, former slaves. As he grew his body developed normally but his head remained small. His tapering cranium and heavy jaw made him attractive to agents from van Emburgh's Circus in Somerville, New Jersey. His unusual appearance caused many to believe that he was a "pinhead", or microcephalic. Microcephalics are characterized by a small, tapering cranium and impaired mental faculty. It is arguable, however, that William Henry was not mentally deficient.

William Henry's parents agreed to allow the circus to display him in return for money. He was billed as a missing link supposedly caught in Africa and displayed in a cage. He was a popular draw and his success led young William Henry's agent to show his charge to P.T. Barnum.

Barnum purchased the right to display William Henry Johnson from the circus and gave him a new look. A furry suit was made to fit him, and his hair was shaped to a tiny point that further accented his sloping brow. Finally, he was given the name, "Zip the Pinhead," the "What-Is-It?


AS 'ZIP the PINHEAD'

Zip the Pinhead
Zip's early performances were set against a background story. It was told to the audience that a tribe of "missing links" had been discovered in Africa, and that Zip was one of these. It was further explained that the "wild man", the "What-Is-It", subsisted on raw meat, nuts, and fruit, but was learning to eat more civilized fare such as bread and cake.

Zip would then be revealed in a cage where he could rattle the bars and screech. This act was tremendously successful for Barnum, and Zip was as big a draw to his American Museum as the famous Siamese twins, Chang and Eng Bunker.

In later years Zip became more "civilized" in his act. He shared the stage with other prodigies, including his friends Jim Tarver, the Texas Giant; Jack Earle, the Tallest Man in the World; Koo-Koo the Bird Girl and many others. Zip also traveled extensively with the Ringling Brothers circus.

Zip drew the attention of important figures of the time. In 1860 he was visited at the Museum by the Prince of Wales; his photo was taken by famed Civil War photographer Mathew Brady.

Throughout this period Zip's best friend and manager was Captain O.K. White. White conscientiously looked after Zip's interests. He also gave Zip one of his prized possessions, a tuxedo. He would wear the tuxedo on special occasions such as birthdays.

One of his other possessions was a fiddle. It was said that he purchased the fiddle in Kentucky and that it had once belonged to Daniel Boone. Zip was most unskillful with the instrument, but it is reported that audiences loved seeing Zip play his fiddle and dance about with it.

In his later years Zip eschewed traveling in favor of displaying himself at Coney Island. One Sunday afternoon in 1925, Zip heard a little girl cry for help. He noticed the girl waving her arms in the ocean and swam out to rescue her. All who witnessed cheered his valor, but he left the scene to avoid their accolades.

Zip caught bronchitis in early 1926, and despite the wishes of his doctor and Captain White he continued to perform his part in a stage play. Upon the closing of the play he returned to his home in Bound Brook, New Jersey, where he was cared for by his doctor, Captain White, and his sister. When his condition worsened he was moved to Bellevue Hospital in New York City where he died.

Zip the Pinhead
It is estimated that during his 67 years in show business, Zip entertained more than one hundred million people. He was termed "The Dean of Freaks". His funeral was attended by the greatest side show acts of the days, including Lady Olga Roderick, the Bearded Lady; Frank Graf, the tattooed man; and many more. During the ceremonies the distraught Captain White collapsed. He died three days later.

Although there seem to be discrepancies on the certificate concerning age and date of death; Zip the Pinhead, William Henry Johnson, was buried in Plot 399 of the Bound Brook Cemetery on April 26 1926. A small gravestone bearing the inscription "William H. Johnson, 1857–1926" marks his resting place. However, it is written on Zip's burial certificate that Zip was 83 years old, which would have meant he was born in 1842 or 1843, which seems more likely considering his 1860 photograph; 1857 is more likely the year he began his career.

Johnson is partly the inspiration for Bill Griffith's comics character, Zippy the Pinhead. He was featured in the "Freak Show Tech" episode of the History Channel series Wild West Tech.


MENTAL CAPACITY

William Henry Johnson may not have been a true microcephalic; he may have merely had an oddly-shaped head. Additionally, he possibly did not suffer the mental retardation that microcephalics often suffer. There has been some interest in ascertaining Zip's actual mental capacity.

William Henry's sister, Sarah van Duyne, claimed in a 1926 interview that her brother would "converse like the average person, and with fair reasoning power".

Zip's last words were to his sister, Mrs. van Duyne. He is quoted as saying, "Well, we fooled 'em for a long time, didn't we?"


Beetlejuice (entertainer)

Beetlejuice" on The Howard Stern Show
Height 4 ft 3 in (1.30 m) Photo by Paul Rudman
Lester Napoleon Green (born June 2, 1968), better known by his stage name Beetlejuice (often abbreviated to Beetle or Beet), is a frequent guest on The Howard Stern Show and a member of Stern's Wack Pack. In 2015, the Howard Stern Show named Beetlejuice the greatest Wack Packer of all time. He has appeared in feature films as well as performed voice-over work. Beetlejuice has performed with various Howard Stern Show related comedians, including those that perform under The Killers of Comedy Tour banner.

Beetlejuice is a dwarf, standing about 4 feet 3 inches tall. He also has microcephaly, which gives him his characteristic small head relative to his already small body. He had to be taught how to bathe himself by his manager while working on The Howard Stern Show. Lester's nickname was given to him by children in Marion Garden projects in Jersey City, New Jersey, where he lived with his sister Lisa. The children used to pick on him because of a scene in the film Beetlejuice featuring a character's shrunken head.

On the October 2, 2007 broadcast of The Howard Stern Show, Jerry O'Connell revealed that he knew Lester while he was growing up. O'Connell's mother was Beetle's special-ed teacher, and Beetle was such a good student that he did special work as her "assistant".



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Source(s): wikipedia | wikipedia | wikipedia

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