Nhân hai trường hợp Schizencephaly

Schizencephaly – 2 cases report

SUMMARY

Introduction: Schizencephaly is a congentital brain malformation, which is characterized by clefts extending from pial surface of cerebral mantle to ventricle. This morphologically malformation divides into 2 types “closed-lip” and “open-lip”. Clinical symptoms ranged from simple to complicated depend on the location of clefts. This is a rare malformation, according to statistics, the incidence of the disease is approximately 1-2: 100.000 populations. Therefore, we are describing 2 cases encountered in Hue UniversityHospital within 2 years.

Approach:Case 1: 2-year-old male patient, presenting the clinical symptom of half body weakness since 8 months old. Pregnant history of his mother were been unexplained fever in 8th month pregnancy, untreated. He underwent brain CT scanner and MRI. Case 2: 33-year-old male, hospitalization by presenting the epilepsy. He has been the half body weakness since neonatal phase. He underwent brain MRI.

Result: Case 1: Presenting a large cleft lined by grey matter between right ventricle and pial surface of right hemisphere. Right parietal, temporal, and frontal lobeswere atrophic; the septum pellucidum is absent. Posterior part of corpus callosum was atrophic Case 2: Large size cleft lined by grey matter between right ventricle and pial surface of right hemisphere. Right frontal lobe is small. Right frontal gyri and lined-cleft gyri are small, hyperdensity. Medial line deviates toward left side. Corpus callosum is normal.

Conclusion: Schizencephaly malformation occurs in any ages with different clinical symptoms. Typical character is the cleft, which is lined by grey matter from ventricle to arachinoid space. This accompanies other abnormalities such as the absence of septum pellucidum, polygyri, atrophy of corpus callosum. Diagnostic imaging, especially MRI, is crucially important to define this malformation and accompied abnormalities.

Key words: Schizencephaly, MRI.