lissencephaly
Sign in to saveAlso known as Nerve cell migration disorder, Neuronal migration defect
Lissencephaly (, meaning 'smooth brain') is a set of rare brain disorders whereby the whole or parts of the surface of the brain are smooth. It is caused by defective neuronal migration during the 12th to 24th weeks of gestation, resulting in a lack of development of brain folds (gyri) and grooves (sulci). It is a form of cephalic disorder. Terms such as agyria (no gyri) and pachygyria (broad gyri) are used to describe the appearance of the surface of the brain.
Key facts
- Medical condition (new).image
- Lissencephaly.jpg
- Medical condition (new).caption
- Lissencephalic brain of a human, lacking surface convolutions (gyrification)
- Medical condition (new).causes
- Absence of gyrification
- Medical condition (new).treatment
- See below
- Medical condition (new).prognosis
- Usually die young; see below for details
via Wikipedia infobox
Research
2,037 papersvia PubMed
~12 min read
Encyclopedic overview
15 sectionsContents
- Signs and symptoms
- Causes
- Neuronal migration
- ''LIS1''
- ''DCX''
- ''ARX''
- ''RELN''
- Viral infection
- Diagnosis
- Classification
- Treatment
- Prognosis
- See also
- References
- External links
Lissencephaly (, meaning 'smooth brain') is a set of rare brain disorders whereby the whole or parts of the surface of the brain are smooth. It is caused by defective neuronal migration during the 12th to 24th weeks of gestation, resulting in a lack of development of brain folds (gyri) and grooves (sulci). It is a form of cephalic disorder. Terms such as agyria (no gyri) and pachygyria (broad gyri) are used to describe the appearance of the surface of the brain.
Children with lissencephaly generally have significant developmental delays, but these vary greatly from child to child depending on the degree of brain malformation and seizure control. Life expectancy can be shortened, generally due to respiratory problems.
Excerpted from Wikipedia’s “lissencephaly” article, available under the CC BY-SA 4.0 licence.