Agyria-pachygyria is a disease of neuronal migration characterized by total or partial absence of sulci and thickened cortical mantle.
Pachygyria was considered as a less severe form of agyria-pachygyria complex
and had a broader spectrum of clinical features than agyria
. In many cases, agyria and pachygyria coexist in the same patient.
Although some of these disorders have a genetic basis, the cause of the migrational arrest is still unknown.
and development delay are the most common presentations.
These patients are always retarded in motor or intellectual function.
The advent of CT and MRI have made the diagnosis of agyria-pachygyria during life possible.
In addition to neuroimaging
studies, EEG abnormalities are also helpful in the diagnosis of agyria-pachygyria
These abnormalities in agyria-pachygyria described previously including generalized high amplitude fast activity, high amplitude sharp- and slow-wave complexes and an alternating pattern consisting of bursts of sharp waves alternating with periods of electrocerebral depression
Agyria is often associated with dysgenetic syndromes such as Miller-Dieker (A developmental defect of the brain caused by incomplete neuronal migration and characterized by smoothness of the surface of the brain (lissencephaly
) occurring in association with absence of the sulci and gyri (agyria) and thickening of the cerebral cortex
with four rather than six layers (pachygyria), microcephaly
, characteristic facial appearance, retarded growth and mental development, neurological complications, and multiple abnormalities of the brain, kidneys, heart, gastrointestinal tract, and other organs.) and Walker-Warburg (cobblestone lissencephaly II).
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