What are the Different Kinds of Cephalic Disorders?
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 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.
COLPOCEPHALY is a disorder in which there is an abnormal enlargement of the occipital horns - the posterior or rear portion of the lateral ventricles (cavities or chambers) of the brain. This enlargement occurs when there is an underdevelopment or lack of thickening of the white matter in the posterior cerebrum. Colpocephaly is characterized by microcephaly (abnormally small head) and mental retardation. Other features may include motor abnormalities, muscle spasms, and seizures.
Although the cause is unknown, researchers believe that the disorder results from an intrauterine disturbance that occurs between the second and sixth months of pregnancy.
Colpocephaly may be diagnosed late in pregnancy, although it is often misdiagnosed as hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain). It may be more accurately diagnosed after birth when signs of mental retardation, microcephaly, and seizures are present.
There is no definitive treatment for colpocephaly. Anticonvulsant medications can be given to prevent seizures, and doctors try to prevent contractures (shrinkage or shortening of muscles). The prognosis for individuals with colpocephaly depends on the severity of the associated conditions and the degree of abnormal brain development. Some children benefit from special education.
HOLOPROSENCEPHALY is a disorder characterized by the failure of the prosencephalon (the forebrain of the embryo) to develop. During normal development the forebrain is formed and the face begins to develop in the fifth and sixth weeks of pregnancy.
Holoprosencephaly is caused by a failure of the embryo's forebrain to divide to form bilateral cerebral hemispheres (the left and right halves of the brain), causing defects in the development of the face ...
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