Lissencephaly, literally meaning smooth brain, is a rare gene-linked brain malformation in which the normal convolutions in the cerebral cortex is absent and an abnormally small head occurs. Children who are born with a reduced head size at the time of birth, the condition microlissencephaly is typically diagnosed. When a defective neuronal migration during embryonic development takes place, lissencephaly occurs. In this process, the nerve cells move from their place of origin to their permanent location within the cerebral cortex gray matter.
The symptoms of lissencephaly include:
Lissencephaly may have a relation with other diseases also. These disease include isolated lissencephaly sequence, Miller-Dieker syndrome and Walker-Warbug syndrome. At times it can get difficult to distinguish between these conditions.
Although there is no cure for lissencephaly but, children can show some progress in development over time. Care may be needed to comfort, feed and nurse the needs of the patient. Seizures can be troublesome but, anticonvulsant medications can be useful.
The prognosis for children suffering from lissencephaly depends on the degree of malformation. Many children die before the age of 10 years. Usually, the death is caused by aspiration of food or fluids, respiratory disease or severe seizures.
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Read more on Lissencephaly Signs and Symptoms.
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