【PHILIPS case per day】No gyrus malformation with corpus callosum dysplasia CT case picture image diagnosis

[Clinical history]: child, F/0.
5Y
.
Epilepsy, which has been
frequent in the past month.
The facial features are acceptable, the size of the head is slightly smaller than that of children of the same age, and the intelligence is low (usually the eyes and movements of the family are slow when teasing children).

【Image image】: CT image

[Image performance]: It shows that the cortex of both cerebral hemispheres is significantly thickened, the surface is smooth, there is no gyrus and sulci, and the subcortical white matter is rare
.
Bilateral lateral fission is shallow and concave, the entire bilateral cerebral hemispheres are in a "figure-8" shape, and the ventricular system is enlarged
.
The anterior longitudinal fissure widens and deepens, communicating with the third ventricle
.
Bilateral ventricles enlarge, separate.

[Imaging diagnosis]: Lissencephaly associated with Corpus Callosum dysplasia

[Key points of diagnosis]: no gyrus malformation with corpus callosum dysplasia

【Discussion】: Anencephalic gyrus and/or megaencephaly: Aencephaly refers to the smooth surface of the brain and the absence of gyrus structure; Giant encephalyphalis refers to the brain staying in the primitive stage, with a wide and flat
gyrus.
The two are only different in the degree of teragenesis and can exist in different parts of
the brain at the same time.
The former is mostly in the parietal occipital part, and the latter is mostly located in the
forehead.
The former microscopic tube has abnormal cerebral cortex structure, incomplete or incomplete cortical stratification, and immature nerve cells
are common.
The latter cortex retains the 4 nerve cell structures of the original cortex, and the nerve cells are immature
.
Ectopic nerve cells are often found in white matter, sometimes nodular.

【Clinical manifestations】 Children without gyrus often have microcephaly and slight facial abnormalities in the neonatal period, and later show intellectual retardation
.
Most people with complete gyrus die
before the age of 2.
Survivors of the giant gyrus often have mental retardation and epilepsy
.

【CT performance】 No gyrus malformation and/or giant gyrus malformation, the former is mainly manifested as smooth brain surface, gyrus and sulci disappear, cortical thickening, and white matter reduction
.
The latter is mainly manifested as shallow and widened lateral fission, widening of the gyrus, increased cortex, smooth inner surface, reduced white matter, and enlargement
of the ventricular system on the affected side.
In addition, the sides of the anencephalic gyrus are shallow and appear in a "figure 8" shape
.