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Brain Malformations in Children
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BRAIN MALFORMATIONS
What are other brain malformations?
Just as malformations can occur in the brain, other malformations can occur in the spine and spinal cord such as meningocele, meningomyelocele and spina bifida.
See "Spina bifida" for more details.
What is encephalocele?
Encephalocele (pronounced as N-ceph-a-low-seal) is a condition where brain, or its outer lining - the meninges protrude out due to a defect in the skull bone. Patients have a fluctuant swelling over the skull. Depending on the part of the brain that is involved, patients may have visual problems, seizures or mental retardation. If only meninges are protruding, prognosis is good.
What is anencephaly?
Anencephaly (pronounced as N-N-ceph-a-lee) is a fatal brain malformation in which the brain lobes are absent. Infant is born with a defect in the skull bones with soft rudimentary brain seen from the defect. Most infants are still born or die shortly after birth. Commonest cause of anencephaly is Folic Acid deficiency and if mother takes folic acid supplement during pregnancy, the risk significantly decreases.
What is holoprosencephaly?
Holoprosencephaly (pronounced as holo-pro-zen-ceph-a-lee) is a condition in which brain fails to divide into two lobes and only a single lobe is present. This condition is common in patients with genetic syndromes. Most patients have severe delayed development, spasticity and seizures.
Figure 4: Holoprosencephaly
What is megelencephaly?
Large brain is called megelencephaly. Large head is called macrocephaly. Large head does not necessarily mean large brain. Large head can occur due to variety of causes such as large brain, increase in fluid in brain (hydrocephalus) or due to bleed in the head. Megelencephaly occurs with various genetic disorders in which abnormal substance is stored in the brain such as in Canavan and Alexander leukodystrophy. Sometimes large brains can run in families without any pathological abnormality. In patients with genetic disorders and large head, they can have seizures and mental retardation.
What is the treatment of brain malformations?
Most of the brain malformations cannot be corrected as they are due to abnormal development of brain. Seizures will be controlled by anti-fit medications and spasticity will require physiotherapy. Conditions such as spina bifida and encephalocele can be treated surgically whereby the skull defect or spine defect is closed and brain or spinal cord is pushed back into the space it occupies. However prognosis would depend on the amount of brain damage that is already present.
Last Updated On 26/2/2011
Just as malformations can occur in the brain, other malformations can occur in the spine and spinal cord such as meningocele, meningomyelocele and spina bifida.
See "Spina bifida" for more details.
What is encephalocele?
Encephalocele (pronounced as N-ceph-a-low-seal) is a condition where brain, or its outer lining - the meninges protrude out due to a defect in the skull bone. Patients have a fluctuant swelling over the skull. Depending on the part of the brain that is involved, patients may have visual problems, seizures or mental retardation. If only meninges are protruding, prognosis is good.
What is anencephaly?
Anencephaly (pronounced as N-N-ceph-a-lee) is a fatal brain malformation in which the brain lobes are absent. Infant is born with a defect in the skull bones with soft rudimentary brain seen from the defect. Most infants are still born or die shortly after birth. Commonest cause of anencephaly is Folic Acid deficiency and if mother takes folic acid supplement during pregnancy, the risk significantly decreases.
What is holoprosencephaly?
Holoprosencephaly (pronounced as holo-pro-zen-ceph-a-lee) is a condition in which brain fails to divide into two lobes and only a single lobe is present. This condition is common in patients with genetic syndromes. Most patients have severe delayed development, spasticity and seizures.
Figure 4: Holoprosencephaly
What is megelencephaly?
Large brain is called megelencephaly. Large head is called macrocephaly. Large head does not necessarily mean large brain. Large head can occur due to variety of causes such as large brain, increase in fluid in brain (hydrocephalus) or due to bleed in the head. Megelencephaly occurs with various genetic disorders in which abnormal substance is stored in the brain such as in Canavan and Alexander leukodystrophy. Sometimes large brains can run in families without any pathological abnormality. In patients with genetic disorders and large head, they can have seizures and mental retardation.
What is the treatment of brain malformations?
Most of the brain malformations cannot be corrected as they are due to abnormal development of brain. Seizures will be controlled by anti-fit medications and spasticity will require physiotherapy. Conditions such as spina bifida and encephalocele can be treated surgically whereby the skull defect or spine defect is closed and brain or spinal cord is pushed back into the space it occupies. However prognosis would depend on the amount of brain damage that is already present.
Last Updated On 26/2/2011
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