Table 1.
Clinical and radiological summaries of 21 children with schizencephaly
| Case | Age at MR | Neurodevelopmental information | Schizencephaly: Number Laterality Symmetry | Schizencephaly: Location/type | Other brain abnormalities | Fornix location | Septum pellucidum on MR | SOD suspected clinically |
|---|---|---|---|---|---|---|---|---|
| P1* | 7 years | Microcephaly, quadriparetic cerebral palsy, global developmental delay | 2 Bilateral Symmetric |
Right: paracentral lobule/type 2 (closed lip) Left: paracentral lobule/type 2 (closed lip) |
Extensive bilateral PMG | Normal | Present | No |
| P2* | 7 months | Microcephaly, in utero growth restriction, quadriparetic cerebral palsy | 2 Bilateral Symmetric |
Right: paracentral lobule/type 2 (closed lip) Left: paracentral lobule/type 2 (closed lip) |
Extensive bilateral PMG | Normal | Present | No |
| P3 | 2 years | Global developmental delay, visual impairment | 2 Bilateral Symmetric |
Right: paracentral lobule/type 3 (open lip) Left: paracentral lobule/type 3 (open lip) |
Extensive bilateral PMG | Superior aspect of the 3rd ventricle | Absent | Yes |
| P4 | 14 months | Hemiparetic cerebral palsy (right) | 1 Unilateral | Left: middle frontal gyrus/type 2 (closed lip) | Contralateral posterior peri-sylvian PMG | Superior aspect of the 3rd ventricle | Absent | Yes |
| P5 | 2 years | Global developmental delay, epilepsy | 3 Bilateral Non-symmetric |
Right: a. superior frontal gyrus/type 2 (closed lip) Right: b. superior temporal gyrus/type 1 (no cleft) Left: superior frontal gyrus/type 1 (no cleft) |
ACC, type 2 cysts, extensive nodular heterotopia | Aberrant | Absent | No |
| P6 | 3 years | Epilepsy | 1 Unilateral | Right: superior temporal gyrus/type 2 (closed lip) | No | Normal | Present | No |
| P7 | 13 years | Microcephaly, global developmental delay, epilepsy | 2 Bilateral Symmetric |
Right: extensive frontal/type 3 (open lip) Left: extensive frontal/type 3 (open lip) | No | Normal | Disrupted | No |
| P8 | 8 days | Hypoglycaemia, hyponatraemia | 1 Unilateral | Left: paracentral lobule/type 2 (closed lip) | No | Superior aspect of the 3rd ventricle | Absent | Yes |
| P9 | 2 months | Unilateral ptosis (right) | 1 Unilateral | Right: paracentral lobule/type 2 (closed lip) | Contralateral heterotopia | Normal | Disrupted | Yes |
| P10 | 10 months | Epilepsy | 1 Unilateral | Right: paracentral lobule/type 2 (closed lip) | Mirror contralateral PMG | Superior aspect of the 3rd ventricle | Absent | Yes |
| P11 | 7 months | Microcephaly, global developmental delay, epilepsy | 3 Bilateral Non-symmetric |
Right: paracentral lobule/type 3 (open lip) Left: a. paracentral lobule/type 2 (closed lip) Left: b. inferior frontal gyrus (pars opercularis)/type 3 (open lip) |
Extensive bilateral PMG Hypogenesis CC, absent splenium |
Superior aspect of the 3rd ventricle | Absent | Yes |
| P12 | 3 years | Diplegic cerebral palsy | 2 Bilateral Symmetric |
Right: paracentral lobule/type 2 (closed lip) Left: paracentral lobule/type 2 (closed lip) |
No | Normal | Present | No |
| P13 | 11 months | Hemiparetic cerebral palsy (left) | 1 Unilateral | Right: paracentral lobule/type 2 (closed lip) | Mirror contralateral PMG | Superior aspect of the 3rd ventricle | Absent | No |
| P14 | 2 years | Global developmental delay, epilepsy | 2 Bilateral Symmetric |
Right: middle frontal gyrus/type 2 (closed lip) Left: middle frontal gyrus/type 2 (closed lip) |
Extensive bilateral PMG | Superior aspect of the 3rd ventricle | Absent | No |
| P15 | 4 years | Epilepsy | 1 Unilateral | Right: middle frontal gyrus/type 1 (no cleft) | No | Normal | Present | No |
| P16 | 13 months | Hemiparetic cerebral palsy (right) | 2 Bilateral Non-symmetric |
Right: middle frontal gyrus/type 2 (closed lip) Left: extensive frontal/parietal lobes/type 3 (open lip) | Extensive bilateral PMG Body of CC absent? disrupted |
Superior aspect of the 3rd ventricle | Absent | No |
| P17 | 4 years | Global developmental delay, epilepsy | 1 Unilateral | Right: paracentral lobule/type 2 (closed lip) | No | Superior aspect of the 3rd ventricle | Absent | No |
| P18 | 9 years | Global developmental delay, epilepsy | 1 Unilateral | Right: superior frontal gyrus/type 2 (closed lip) | No | Superior aspect of the 3rd ventricle | Absent | Yes |
| P19 | 2 years | Hemiparetic cerebral palsy (left) | 2 Unilateral | Right: a. paracentral lobule/type 2 (closed lip) Right: b. inferior frontal gyrus (pars orbitalis)/type 2 (closed lip) |
Hypogenesis CC (genu/anterior body absent) Chiari 1 malformation | Aberrant | Absent | Yes |
| P20 | 12 months | Global developmental delay, epilepsy, macrocephaly | 1 Unilateral | Left: supramarginal gyrus/type 1 (no cleft) | Hypogenesis CC (posterior body/splenium absent) | Aberrant | Disrupted | No |
| P21 | 5 years | Global developmental delay, epilepsy | 2 Bilateral Symmetric |
Right: inferior frontal gyrus (pars opercularis)/type 3 (open lip) Left: inferior frontal gyrus (pars opercularis)/type 3 (open lip) |
Agenesis CC, bilateral PMG, fused nucleus accumbens septi | Superior aspect of the 3rd ventricle | Absent | Yes |