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Neurology

Lissencephaly

Lissencephaly means smooth brain. It is a rare disorder marked outwardly by abnormal smallness of the head (microcephaly) and inwardly by the lack of normal folds and grooves in the surface of the brain.

Cause
Lissencephaly results when nerve cells in the fetal neural tube fail to move to the proper location (called defective neuronal migration) as the fetus's central nervous system develops. This may be caused by intrauterine or fetal viral infections during the 1st trimester, insufficient blood supply to the brain early in pregnancy, or a genetic disorder.

Signs and Symptoms
Babies born with lissencephaly may present with an unusual facial appearance, difficulty swallowing, severe psychomotor retardation, muscle spasms, seizures, and/or deformities of the hands, fingers, or toes.

Diagnosis
The condition is diagnosed at or soon after birth. Doctors usually confirm the diagnosis with ultrasound, computed tomography (CT scan) or magnetic resonance imaging (MRI scan).

Treatment
Treatment for lissencephaly is symptomatic, depending on the nature and severity of the brain malformations. Supportive care and nursing may be needed. Medication may be prescribed to control seizures, and a shunt may be required to control hydrocephalus. When swallowing is a problem, a gastrostomy tube may be necessary.

Prognosis
Prognosis depends on the degree of brain malformation. While some have nearly normal development and intelligence, most show no significant development beyond that of a normal child who is 3 to 5 months old. Many lissencephalic children die of respiratory problems before age 2.

Additional Information

The Lissencephaly Launch Pad

 The Cerebral Palsy Network©1997/2014. All graphics are the exclusive property of CPN, unless otherwise indicated. Contact Cerebral Palsy Network   for further information. Last updated 05/04/14