Microcephaly 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
microcephalinMicrocephalin is one of six genes causing primary microcephaly when non-functional mutations exist in the homozygous state...
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
Microcephaly is a type of
cephalic disorderCephalic disorders are congenital conditions that stem from damage to, or abnormal development of, the budding nervous system...
. This is a disorder characterized 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
alcoholismAlcoholism is a term with multiple and sometimes conflicting definitions. In common and historic usage, alcoholism is any condition that results in the continued consumption of alcoholic beverages, despite health problems and negative social consequences...
(which can result in the
fetal alcohol syndromeFetal alcohol syndrome is a disorder that can occur to the embryo when a pregnant woman ingests alcohol during pregnancy. An ingestion of alcohol does not always result in FAS...
disability), diabetes, or
rubellaRubella, commonly known as German measles, is a disease caused by the rubella virus. The name "rubella" is derived from the Latin, meaning little red. Rubella is also known as German measles because the disease was first described by German physicians in the mid-eighteenth century. This disease is...
(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.
InfantAn infant or baby is the term used to refer to the very young offspring of humans and other primates.-Infant:The term infant derives from the Latin word infans, meaning "unable to speak."...
s 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
skullThe skull is a bony structure found in the head of many animals. The skull supports the structures of the face and protects the head against injury....
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
quadriplegiaQuadriplegia, also known as tetraplegia, is paralysis caused by illness or injury to a human that results in the partial or total loss of use of all of their limbs and torso; paraplegia is similar but does not affect the arms...
in others.
Generally there is no specific treatment for microcephaly. Treatment is symptomatic and supportive.
Microcephaly and culture
Numerous
superstitionSuperstition is a credulous belief or notion, not based on reason, knowledge, or experience. The word is often used pejoratively to refer to folk beliefs deemed irrational. This leads to some superstitions being called "old wives' tales"...
s revolve around microcephalics in
PakistanPakistan , officially the Islamic Republic of Pakistan, is a country located at the crossroads of South Asia, the Middle East, and Central Asia...
, ranging from being condemned as
outcastAn outcast is a person with social stigmaOutcast also may refer to:In literature:*Guardians of Ga'Hoole Book 8: The Outcast*Outcast , a book by Iraqi-Israeli author Shimon Ballas...
s 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,
cursingA curse is any expressed wish that some form of adversity or unhappiness will befall another person or persons. In particular, a wish that harm or hurt will be inflicted by any supernatural power, such as a spell, a prayer, an imprecation, an execration, magic, witchcraft, a god, a natural force,...
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
acolyteThis article is about religious acolytes. For other uses, see Acolyte .In many Christian denominations, an acolyte is anyone who performs ceremonial duties such as lighting altar candles...
. 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
Schlitzie , 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...
, a circus performer best known for his role in the 1932 film FreaksFreaks is a horror film about sideshow performers, directed and produced by Tod Browning and released by Metro-Goldwyn-Mayer, with a cast mostly composed of actual carnival performers. The film was based on Tod Robbins' short story "Spurs"...
.
- Beetlejuice
Lester Napoleon Green , known as legally Beetlejuice , is a frequent guest on The Howard Stern Show and a member of Stern's Wack Pack. He has appeared in feature films as well as performed voice-over work...
, a member of Howard Stern's "Wack Pack".
- Zip the Pinhead
Zip the Pinhead, born William Henry Johnson , was an American freak show performer famous for his oddly tapered head.- Early life :...
(Circus "freak showA freak show is an exhibition of rarities, "freaks of nature" — such as unusually tall or short humans, and people with both male and female secondary sexual characteristics or other extraordinary diseases and conditions — and performances that are expected to be shocking to the viewers...
" performer William Henry Johnson) is commonly associated with microcephaly, although he may not have actually had it.
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