Microcephaly: Difference between revisions
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==Microcephaly and culture== |
==Microcephaly and culture== |
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Numerous [[superstition]]s revolve around microcephalic people in [[Pakistan]], ranging from being condemned as outcasts and institutionally shunned to having divine powers attributed to them.<ref name="ratppl">{{cite journal |author=Miles M, Beer D |title=Pakistan's microcephalic chuas of Shah Daulah: cursed, clamped or cherished |journal=Hist Psychiatry |volume=7 |issue=28 pt 4 |pages=571–89 |year=1996 |month=December |pmid=11618756 }}</ref> 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, [[curse|cursing]] them into being a "chooha", which means "mouse" or "rat" in Hindi and 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.<ref>{{cite web |author=Leroi, Armand |title=What Makes Us Human? |date=08 January 2006 |publisher=Telegraph |url=http://www.telegraph.co.uk/connected/main.jhtml;jsessionid=U3FKJXNUVXGKJQFIQMFSFFOAVCBQ0IV0?xml=/connected/2006/08/01/echuman01.xml}}</ref> |
Numerous [[superstition]]s revolve around microcephalic people in [[Pakistan]], ranging from being condemned as outcasts and institutionally shunned to having divine powers attributed to them.<ref name="ratppl">{{cite journal |author=Miles M, Beer D |title=Pakistan's microcephalic chuas of Shah Daulah: cursed, clamped or cherished |journal=Hist Psychiatry |volume=7 |issue=28 pt 4 |pages=571–89 |year=1996 |month=December |pmid=11618756 }}</ref> 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, [[curse|cursing]] them into being a "chooha", which means "mouse" or "rat" in Hindi and 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.<ref>{{cite web |author=Leroi, Armand |title=What Makes Us Human? |date=08 January 2006 |publisher=Telegraph |url=http://www.telegraph.co.uk/connected/main.jhtml;jsessionid=U3FKJXNUVXGKJQFIQMFSFFOAVCBQ0IV0?xml=/connected/2006/08/01/echuman01.xml}}</ref> |
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Microcephalics were sometimes sold to [[freak shows]] in North America and Europe in the 19th and early 20th century, where they would go under the name pinheads. Many of them were presented as different species (e.g., "monkey man") and described as being the [[Missing Link|missing link]], but it was also common for them to be presented as the last surviving [[Aztec]].<ref>{{cite journal | author=Mateen FJ, Boes CJ |title="Pinheads": the exhibition of neurologic disorders at "The Greatest Show on Earth" | journal= Neurology | volume=30 | pages=2028–32 | pmid=21115959}}</ref> Famous examples are [[Zip the Pinhead]] (although he may not have had microcephaly)<ref>"[http://thehumanmarvels.com/blog/?p=36]?" 16 October 2010.</ref> and [[Schlitzie]] the Pinhead,<ref>"[http://thehumanmarvels.com/blog/?p=130]?" 16 October 2010.</ref> who also starred in the 1932 movie [[Freaks]]. |
Microcephalics were sometimes sold to [[freak shows]] in North America and Europe in the 19th and early 20th century, where they would go under the name pinheads. Many of them were presented as different species (e.g., "monkey man") and described as being the [[Missing Link|missing link]], but it was also common for them to be presented as the last surviving [[Aztec]].<ref>{{cite journal | author=Mateen FJ, Boes CJ |title="Pinheads": the exhibition of neurologic disorders at "The Greatest Show on Earth" | journal= Neurology | volume=30 | pages=2028–32 | pmid=21115959}}</ref> Famous examples are [[Zip the Pinhead]] (although he may not have had microcephaly)<ref>"[http://thehumanmarvels.com/blog/?p=36]?" 16 October 2010.</ref> and [[Schlitzie]] the Pinhead,<ref>"[http://thehumanmarvels.com/blog/?p=130]?" 16 October 2010.</ref> who also starred in the 1932 movie [[Freaks]]. |
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Revision as of 20:20, 11 January 2012
 | This article needs additional citations for verification. (April 2009) |
| Microcephaly | |
|---|---|
| Specialty | Medical genetics  |
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.
Causes
Microcephaly is a type of cephalic disorder.
A genetic factor may play a role in causing some cases of microcephaly. Relations have been found between autism, duplications of chromosomes and macrocephaly on one side. On the other side a relation has been found between schizophrenia, deletions of chromosomes and microcephaly.[1][2][3]
Microencephaly
This is a disorder characterized by a small head and may be caused by a disturbance in the rapid growing of nerve cells. The term "microencephaly" is used to specifically denote a small brain. ("Cephalus" denotes the head, and "encephalon" denotes the brain.) In ICD-10, "Microcephaly" is classified under "congenital malformations of the nervous system", as opposed to macrocephaly which is classified under "congenital malformations and deformations of the musculoskeletal system".
Other
Microcephaly can also be associated with other conditions that are only indirectly associated with the nervous system:
- alcoholism (which can result in the fetal alcohol syndrome disability)
- diabetes
- varicella zoster virus (Chickenpox)
- rubella (German measles)
- radiation. After the dropping of atomic bombs on Hiroshima and Nagasaki, several women close to ground zero who had been pregnant at the time gave birth to children with microcephaly.[4] A total of seven of the in utero children at Hiroshima were affected.[5] Microcephaly prevalence was 7 out of a group of 11 pregnant women who held the distinction of surviving the blast at an distance of ≈1 km from ground zero. Due to their proximity to the bomb, the pregnant women's in utero children received a biologically significant radiation dosage that was relatively high due to the massive neutron output of the lower explosive-yielding Little Boy.[6] Microcephaly is the only proven malformation, or congenital abnormality, found in the children of Hiroshima and Nagasaki.[7]
Presentation
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.
Prognosis
Generally there is no specific treatment for microcephaly. Treatment is symptomatic and supportive.
Microcephaly and culture
Numerous superstitions revolve around microcephalic people in Pakistan, ranging from being condemned as outcasts and institutionally shunned to having divine powers attributed to them.[8] 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", which means "mouse" or "rat" in Hindi and 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.[9]
Microcephalics were sometimes sold to freak shows in North America and Europe in the 19th and early 20th century, where they would go under the name pinheads. Many of them were presented as different species (e.g., "monkey man") and described as being the missing link, but it was also common for them to be presented as the last surviving Aztec.[10] Famous examples are Zip the Pinhead (although he may not have had microcephaly)[11] and Schlitzie the Pinhead,[12] who also starred in the 1932 movie Freaks.
Notable microcephalic persons
- 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.
See also
References
- ^ Crespi B, Stead P, Elliot M (2010). "Evolution in health and medicine Sackler colloquium: Comparative genomics of autism and schizophrenia". Proc. Natl. Acad. Sci. U.S.A. 107 (Suppl 1): 1736–41. doi:10.1073/pnas.0906080106. PMC 2868282. PMID 19955444.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ "Rare chromosomal deletions and duplications increase risk of schizophrenia". Nature. 455 (7210): 237–41. 2008. doi:10.1038/nature07239. PMID 18668038.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Dumas L, Sikela JM (2009). "DUF1220 domains, cognitive disease, and human brain evolution". Cold Spring Harb. Symp. Quant. Biol. 74: 375–82. doi:10.1101/sqb.2009.74.025. PMC 2902282. PMID 19850849.
- ^ Hiroshima Peace Site
- ^ Teratology in the Twentieth Century Plus Ten
- ^ Teratology in the Twentieth Century Plus Ten
- ^ Teratology in the Twentieth Century Plus Ten
- ^ Miles M, Beer D (1996). "Pakistan's microcephalic chuas of Shah Daulah: cursed, clamped or cherished". Hist Psychiatry. 7 (28 pt 4): 571–89. PMID 11618756.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Leroi, Armand (08 January 2006). "What Makes Us Human?". Telegraph.
{{cite web}}: Check date values in:|date=(help) - ^ Mateen FJ, Boes CJ. ""Pinheads": the exhibition of neurologic disorders at "The Greatest Show on Earth"". Neurology. 30: 2028–32. PMID 21115959.
- ^ "[1]?" 16 October 2010.
- ^ "[2]?" 16 October 2010.