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Cephalic Disorders

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Dr Kaplens Column

 

ANENCEPHALY

is a neural tube defect  that occurs when the cephalic (head) end of the neural tube fails to close, usually between the 23rd and 26th days of pregnancy, resulting in the absence of a major portion of the brain, skull, and scalp. Infants with this disorder are born without a forebrain the largest part of the brain consisting mainly of the cerebrum, which is responsible for thinking and coordination. The remaining brain tissue is often exposed not covered by bone or skin.

Infants born with anencephaly are usually blind, deaf, unconscious, and unable to feel pain. Although some individuals with anencephaly may be born with a rudimentary brainstem, the lack of a functioning cerebrum permanently rules out the possibility of ever gaining consciousness. Reflex actions such as respiration (breathing) and responses to sound or touch may occur.

The disorder is one of the most common disorders of the fetal central nervous system. Approximately 1,000 to 2,000 American babies are born with anencephaly each year. The disorder affects females more often than males.

The cause of anencephaly is unknown. Although it is believed that the mother’s diet and vitamin intake may play a role, scientists agree that many other factors are also involved.

There is no cure or standard treatment for anencephaly and the prognosis for affected individuals is poor. Most infants do not survive infancy. If the infant is not stillborn, then he or she will usually die within a few hours or days after birth. Anencephaly can often be diagnosed before birth through an ultrasound examination.

Recent studies have shown that the addition of folic acid to the diet of women of child-bearing age may significantly reduce the incidence of neural tube defects. Therefore it is recommended that all women of child-bearing age consume 0.4 mg of folic acid daily.

The following are some of the latest updates and support networks for those families whom are dealing with Anencephaly now or in the past. 

Anencephaly NEWS

This site is for those whom choose to carry to term

Carrying to Term

Anencephaly Support Foundation

Websites offering a vast range of issues concerning anencephaly including organ donation.

http://www.anencephaly.net/ 

Below is some updated information on Anecephaly. (credit for this information goes to Neurology Channel)

Anencephaly is one of the most serious cephalic disorders and afflicts roughly 1000 to 2000 babies born in the United States annually. Female infants are affected more often than males. Infants born with this disorder have no forebrain—the main portion of the cerebrum, responsible for thinking and coordination. Brain tissue that does develop often is not covered by bone or skin.

Causes
The cause of anencephaly remains unknown. A developmental failure occurs between the 23rd and 26th days of pregnancy. The cephalic
(or head) end of the neural tube fails to close, and major portions of the brain, skull, and scalp do not develop. Recent studies suggest the addition of folic acid to the diet of women in their child-bearing years may reduce the incidence of neural tube defects, suggesting that factors associated with diet and vitamins may play a role.

Signs and Symptoms
Anencephalic infants typically are born blind, deaf, unconscious, and insensitive to pain. Some may have a rudimentary brainstem that permits reflex actions such as breathing and, in some cases, responsiveness to sound or touch.

Diagnosis
Anencephaly often can be diagnosed before birth through an ultrasound examination.

Treatment
The condition is untreatable and incurable.

Prognosis
Without a functioning cerebrum, anencephalic infants cannot gain consciousness. Most anencephalic infants are stillborn or die within a few hours or days after birth.

 

 The Cerebral Palsy Network©1997/2014. All graphics are the exclusive property of CPN, unless otherwise indicated. Contact Cerebral Palsy Network   for further information. Last updated 05/04/14