lissencephaly
Pronunciation: lis-en-SEF-ah-lee
A rare neurological condition characterized by abnormal brain development resulting in a smooth cerebral surface with few or no gyri.
Full Definition
Lissencephaly, literally meaning "smooth brain," is a severe brain malformation where the normal folding pattern of the cerebral cortex fails to develop properly. This condition results in developmental delays, seizures, and intellectual disability. The severity varies depending on the grade of lissencephaly, with Type I being more severe than Type II. It is often caused by genetic mutations affecting neuronal migration during fetal development.
Usage
Usage note: Often confused with related cortical malformation terms; specify grade when known.
In Context
- "The MRI findings revealed grade II lissencephaly with simplified gyral pattern." — Radiological report
- "Genetic testing confirmed a LIS1 mutation associated with classical lissencephaly." — Clinical documentation