Lissencephaly, which literally means "smooth brain," is a rare, gene-linked brain malformation characterized by the absence of normal convolutions (folds) in the cerebral cortex and an abnormally small head (microcephaly). In the usual condition of lissencephaly, children usually have a normal sized head at birth. In children with reduced head size at birth, the condition microlissencephaly is typically diagnosed.
Lissencephaly cannot be reversed. Treatments aim to support and comfort affected children. For example, children who have difficulty feeding and swallowing may require a gastrostomy tube. This is a tube that is inserted directly into a child’s stomach. If a child experiences hydrocephalus, or an excessive accumulation of cerebrospinal fluid, a surgical procedure that shunts fluid away from the brain may be necessary.
If a child experiences seizures as a result of lissencephaly, he or she may require medications to control seizures.
Prognosis for a Patient with Lissencephaly
The outlook for a child with lissencephaly depends on the condition’s severity. For example, severe cases may result in the child failing to develop mentally beyond a three- to-five month old function. Children with severe lissencephaly have a life expectancy of about 10 years and will show no significant development beyond a 3-5 month-old level. according to The National Institute of Neurological Disorders. Common causes of death include aspiration (choking on) food or fluids, respiratory disease, or seizures.
Children with mild lissencephaly can experience near-normal development and brain function.
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