(redirected from Lissencephaly syndrome)


agyria. adj., adj lissencephal´ic.


Congenital lack or underdevelopment of the convolutional pattern of the cerebral cortex.
[G. a- priv. + gyros, circle]


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


James Q., U.S. physician.
Miller-Dieker syndrome - arrested brain development. Synonym(s): lissencephaly; Norman-Roberts syndrome
References in periodicals archive ?
A wide range of partial aneuploidy, including dup (3q), dup (11p) [10], inv (11), dup (1q), del (1q), dup (4q), dup (5p), dup (6q), del (9p), dup (15q) [11], dup (17q), dup (7q)/del (21q) [12], dup (3q)/del (9q) [13], Pallister-Killian syndrome with mosaic tetrasomy 12p and Miller-Dieker lissencephaly syndrome with deletion of 17p13.3, and uniparental disomy (UPD) such as UPD11 and UPD14, is also reported to be associated with omphalocele [4, 10, 11, 14, 15].
Salih, "Lethal congenital muscular dystrophy in two sibs with arthrogryposis multiplex: new entity or variant of cobblestone lissencephaly syndrome?" Neuropediatrics, vol.
A persistently and significantly small fetal head with normal-appearing sonographic anatomy may, for example, reveal a lissencephaly syndrome on MRI exam.