Schizencephaly là một khuyết tật phát triển hệ thần kinh, được định nghĩa là khe nứt ở mô não phát triển từ bề mặt vỏ não đến não thất. Tùy hình thái dị dạng, được chia làm hai thể môi đóng và môi hở. Triệu chứng lâm sàng đơn giản hay phức tạp phụ thuộc vị trí xuất hiện đường nứt. | NHÂN HAI TRƯỜNG HỢP CHIZENCEPHALY DIỄN ĐÀN Schizencephaly 2 cases report MEDICAL FORUM Nguyễn Lê Thảo Trâm Nguyễn Thị Ngọc Tỷ Nguyễn Thanh Thảo Hoàng Minh Lợi 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 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
