September 30, 2006
Q: Can a person live without a brain?- Layperson
A: Yes, for a brief period, longer with medical intervention. The medical term is anencephaly. Anencephaly is a cephalic disorder that results from a neural tube defect that occurs when the cephalic (head) end of the neural tube fails to close, usually between the 23rd and 26th day 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.
There are case studies involveing victims of an ailment known as hydrocephalus, more commonly known as water on the brain, with no brain matter. The condition results from an abnormal build up of cerebrospinal fluid and can cause severe retardation and death if not treated.
Two young children with hydrocephalus referred to Lorber presented with normal mental development for their age. In both children, there was no evidence of a cerebral cortex. One of the children died at age 3 months, the second at 12 months. He was still following a normal development profile with the exception of the apparent lack of cerebral tissue shown by repeated medical testing. An account of the children was published in Developmental Medicine and Child Neurology.
Later, a colleague at Sheffield University became aware of a young man with a larger than normal head. He was referred to Lorber even though it had not caused him any difficulty. Although the boy had an IQ of 126 and had a first class honours degree in mathematics, he had "virtually no brain". A noninvasive measurement of radio density known as CAT scan showed the boy's skull was lined with a thin layer of brain cells to a millimeter in thickness. The rest of his skull was filled with cerebrospinal fluid. The young man continues a normal life with the exception of his knowledge that he has no brain.
Although anecdotal accounts may be found in medical literature, Lorber is the first to provide a systematic study of such cases. He has documented over 600 scans of people with hydrocephalus and has broken them into four groups:
Of the last group, which comprised less than 10% of the study, half were profoundly retarded. The remaining half had IQs greater than 100. Skeptics have claimed that it was an error of interpretation of the scans themselves. Lorber himself admits that reading a CAT scan can be tricky. He also has said that he would not make such a claim without evidence. In answer to attacks that he has not precisely quantified the amount of brain tissue missing, he added, "I can't say whether the mathematics student has a brain weighing 50 grams or 150 grams, but it is clear that it is nowhere near the normal 1.5 kilograms."
Many neurologists feel that this is a tribute to the brain's redundancy and its ability to reassign functions. Others, however, are not so sure. Patrick Wall, professor of anatomy at University College, London states "To talk of redundancy is a cop-out to get around something you don't understand."
Norman Geschwind, a neurologist at Boston's Beth Israel Hospital agrees: "Certainly the brain has a remarkable capacity for reassigning functions following trauma, but you can usually pick up some kind of deficit with the right tests, even after apparently full recovery."
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, which controls autonomic and regulatory function, 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.
Most of the foetuses who are affected by anencephaly die in the womb of the mother, during childbirth or in the next few hours after they have exited the mother's body. In some cases, however, it has been known that certain infants have lived up to a week and a half, and the current record lifespan is twelve years.
There is no cure or standard treatment for anencephaly and the prognosis for affected individuals is poor. Most anencephalic babies do not survive birth. 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. The maternal serum alpha-fetoprotein (AFP screening) and detailed fetal ultrasound can be useful for screening for neural tube defects such as spina bifida or anencephaly.
In almost all cases anencephalic infants are not aggressively resuscitated since there is no chance of the infant ever achieving a conscious existence. Instead, the usual clinical practice is to offer hydration, nutrition and comfort measures and to "let nature take its course". Artificial ventilation, surgery (to fix any co-existing congenital defects), and drug therapy (such as antibiotics) are usually regarded as being pointless. Some clinicians see no point in even providing nutrition and hydration, arguing that euthanasia is morally and clinically appropriate in such cases.
Rate of occurrence
In the United States, approximately 1,000 to 2,000 babies are born with anencephaly each year. Female babies are more likely to be affected by the disorder. About 95% of women who learn that they will have an anencephalic baby choose to have an abortion. Of the remaining 5%, about 55% are stillborn. The rest usually live only a few hours or days.
The cause of anencephaly is unknown. Neural tube defects do not follow direct patterns of heredity. Studies show that a woman who has had one child with a neural tube defect such as anencephaly, has about a 3% risk to have another child with a neural tube defect. This risk can be reduced to about 1% if the woman takes high dose (4mg/day) folic acid before and during pregnancy.
It is known that women taking certain medication for epilepsy and women with insulin dependent diabetes have a higher chance of having a child with a neural tube defect. Genetic counseling is usually offered to women at a higher risk of having a child with a neural tube defect to discuss available testing.
Recent studies have shown that the addition of folic acid to the diet of women of child-bearing age may significantly reduce, although not eliminate, 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, especially those attempting to conceive or who may possibly conceive. It is not advisable to wait until pregnancy has begun, since by the time a woman knows she is pregnant, the critical time for the formation of a neural tube defect has usually already passed. A physician may prescribe even higher dosages of folic acid (4 mg/day) for women who have had a previous pregnancy with a neural tube defect.
One must pause and ask just where does consciousness, intelligence, personality, and so on, exist? Perhaps in the heart of the soul.
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