"Agyria" redirects here. Not to be confused with Argyria.
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Medical condition
Lissencephaly
Lissencephalic brain of a human, lacking surface convolutions (gyrification)
Specialty
Medical genetics, neurology
Causes
Absence of gyrification
Treatment
See below
Prognosis
Usually die young; see below for details
Lissencephaly (/ˌlɪs.ɛnˈsɛf.əl.i/, meaning 'smooth brain')[1] is a set of rare brain disorders whereby the whole or parts of the surface of the brain appear smooth.[2] 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).[3] 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.
^"Lissencephaly Information Page". National Institute of Neurological Disorders and Stroke. Retrieved 2019-10-31.
^Toy, Eugene; Simpson, Ericka; Tinter, Ron (2013). Case Files. Neurology (2nd ed.). McGraw Hill. p. 421. ISBN 978-0-07-176170-3.
^Dobyns WB (1987). "Developmental aspects of lissencephaly and the lissencephaly syndromes". Birth Defects Original Article Series. 23 (1): 225–41. PMID 3472611.
differ across the two main types of lissencephaly, Classical (Type I) and Cobble Stone (Type 2). In Classical lissencephaly the cortex becomes thickened and...
disorders; most commonly disorders relating to varied degrees of lissencephaly. Lissencephaly is present in 1 of 85,470 births and the life span of those affected...
S2CID 37380445. Dobyns WB (1987). "Developmental aspects of lissencephaly and the lissencephaly syndromes". Birth Defects Original Article Series. 23 (1):...
several neuronal migration disorders; most commonly relating to lissencephaly. Lissencephaly (smooth brain) is a rare congenital brain malformation caused...
expression are controversial. Total lack of reelin causes a form of lissencephaly. Reelin may also play a role in Alzheimer's disease, temporal lobe epilepsy...
extent of gyrification in various species. Lissencephaly is a human disease state. For humans with lissencephaly, a large number of neurons fail to reach...
in X-linked lissencephaly and the double cortex syndrome, and the clinical manifestations are sex-linked. In males, X-linked lissencephaly produces a smooth...
syndromes, including agenesis of the corpus callosum, Chiari malformation, lissencephaly, and microcephaly. Although the exact cause of colpocephaly is not known...
neurons in that area, and it is suggested to regulate fiber releasing. Lissencephaly, or 'smooth brain', is a disorder in which the brain does not properly...
lead to cortical disorders, which includes cobblestone lissencephaly. Cobblestone lissencephaly is typically seen in three different human congenital muscular...
mutations and an intragenic deletion in LIS1, the lissencephaly causative gene in isolated lissencephaly sequence and Miller-Dieker syndrome". Human Molecular...
Dandy–Walker malformation, hydrocephalus, microencephaly, megalencephaly, lissencephaly, polymicrogyria, holoprosencephaly, and agenesis of the corpus callosum...
may lead to a variety of symptoms and diseases, which include type I lissencephaly, cerebellar hypoplasia, and atherosclerosis. VLDLR is a member of the...
brain disorder that combines severe microcephaly (small head) with lissencephaly (smooth brain surface due to absent sulci and gyri). Microlissencephaly...
Your Pain, was published, about his daughter Georgia’s struggle with lissencephaly. In 2017, Veach’s third book, Faith Forward Future: Moving Past Your...
development of the cortex. A viral infection is one of the causes of lissencephaly, which results in a smooth cortex without gyrification. A type of electrocorticography...
posterior predominant lissencephaly in a 2020 study, added under #618873 in the OMIM catalogue, where it received the name of lissencephaly 10. Centrosome -...
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