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 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

Microcephaly is a type of cephalic disorder. This is a disorder characterized by a small brain and may be caused by a disturbance in the proliferation 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). 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.

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

Microcephaly and culture

"Rat People" of Pakistan

Microcephalics are known as "chuas" or "rat people" in Pakistan for appearance of sloping forehead and narrow face ^[1]. Numerous superstitions revolve around microcephalics in the country, ranging from being condemned as outcastes and institutionally shunned to having divine powers attributed to them^[1]. 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 "chua". 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 chuas as well. Other Pakistanis believe that priests, chua-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. http://www.telegraph.co.uk/connected/main.jhtml;jsessionid=U3FKJXNUVXGKJQFIQMFSFFOAVCBQ0IV0?xml=/connected/2006/08/01/echuman01.xml The disorder occurs primarily among Muslims rather than Hindus, Sikhs or Christians in the country.^[2] Microcephaly has been observed among British Pakistanis in recent years^[2].

Notable Microcephalics

Beetlejuice

Beetlejuice, a member of Howard Stern's "Wack Pack" is a microcephalic dwarf.

References

1. ↑ ^{a b} Miles M, Beer D.,"Pakistan's microcephalic chuas of Shah Daulah: cursed, clamped or cherished".Hist Psychiatry. 1996 Dec;7(28 pt 4):571-89.
2. ↑ ^{a b} J . Ainsworth, "Micro syndrome in Muslim Pakistan children", Journal of Ophthalmology , Volume 108 , Issue 3 , Pages 491 - 497

See also

• Hydrocephaly
• Macrocephaly
• Seckel syndrome
• Anencephaly

External links

• NINDS Microcephaly Information Page
• Microcephaly Support Group
• The Rat People of Pakistan
• NINDS Overview
• Schlitzie The Pinhead
• Beetlejuice Official Website

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