lissencephaly


Also found in: Dictionary, Acronyms, Wikipedia.

lissencephaly

 [lis″en-sef´ah-le]
agyria. adj., adj lissencephal´ic.

a·gy·ri·a

(ā-jī'rē-ă),
Congenital lack or underdevelopment of the convolutional pattern of the cerebral cortex.
[G. a- priv. + gyros, circle]

lissencephaly

/lis·sen·ceph·a·ly/ (lis″en-sef´ah-le) agyria.lissencephal´ic

lissencephaly

See agyria.

lissencephaly

Neurology A brain malformation that is isolated or associated with other syndromes–eg, Miller-Dieker syndrome, Norman-Roberts syndrome, characterized by absence–agyria or incomplete development–pachygyria of cerebral gyri Clinical Microcephaly, seizures, profound mental retardation, feeding difficulties, growth retardation, impaired motor activity

Miller,

James Q., U.S. physician.
Miller-Dieker syndrome - arrested brain development. Synonym(s): lissencephaly; Norman-Roberts syndrome

lissencephaly

a malformation in which the gyri of the cerebral cortex are not normally developed. In dogs it has been associated with behavioral abnormalities, visual deficits, ataxia and seizures. Called also agyria.
References in periodicals archive ?
One support group that is very specific for the family of a child with polymicrogyria is the Lissencephaly Network which has a Web site (http://www.
Reduced levels of the protein called LIS1 have been shown to cause lissencephaly, which is characterized by brain malformations, seizures, severe mental retardation and very early death in human infants.
As Lissencephaly is such a rare condition - affecting just four children born every year in the UK - there is no dedicated charity support available like there is for other illnesses such as cancer.
Tragically, many children with Lissencephaly do not live beyond a couple years.
Helen Sands and five-year-old Holly Sands from the charity, which was set up in memory of Rebecca Sands and to support children living with lissencephaly and other neurological conditions, declared the store officially open on Thursday after store manager Paul Welham presented them with a PS500 donation on behalf of the supermarket.
Millie, 10, suffers from the incurable brain disease lissencephaly, which has resulted in her not being able to walk or talk.
Felix Forbes, who lives in Penarth Marina, was born with Lissencephaly with Miller-Dieker.
For instance, mutations in the ARX gene is both causative of a phenotype: West syndrome and a structural abnormality: lissencephaly (11,12,13,14,15,16,17).
The process could shed light on several brain disorders, including lissencephaly, in which the folds in the brain s cortex fail to develop, as well as maladies diagnosed later in development, such as autism and schizophrenia, Kriegstein said.
Noreen Noone's 22-year-old daughter Orla has lissencephaly - a rare condition which leaves her in a wheelchair.
Failure of this process results in microcephaly, megalencephaly, heterotopia, focal cortical dysplasia, polymicrogyria, lissencephaly, hemimegalencephaly, schizencephaly, anomalies of operculization, and phakomatoses.
Abigail, who was diagnosed with the rare brain disorder Lissencephaly aged just six months, also attended Acorns for respite care.