[P2-39] Clinical and Neuroimaging Findings in Children with Schizencephaly: a Single Institution Experience
[Introduction] To describe the clinical, neuroimaging features, outcome, and other associated clinical spectrum of childhood schizencephaly in a single tertiary hospital in Taiwan.
[Methodology] We retrospectively reviewed the medical records and magnetic resonance images (MRI) of 21 patients with schizencephaly between January 2000 and December 2014. The MRI findings were recorded along with the presence of associated cerebral malformations. The clinical, electrophysiological and associated systemic malformation data were also recorded.
[Results] A total of 21 patients, 13 males and 8 females, were enrolled in the study. Their diagnostic ages ranged from 1 month to 18 years with a mean age of 3 years 2 months. According to the location of schizencephaly, patients were classified into two groups: unilateral (16) and bilateral (5). The phenotypic spectrum in our population differed from that described previously. In unilateral schizencephaly, the most common mode of evaluation was hemiparesis; however, seizures were the most common in bilateral group. The most common associated cerebral malformation was ventriculomegaly in both groups. The majority of patients had developmental delay, intellectual deficit, and speech impairment, especially in bilateral group. Seventeen patients suffered from epileptic seizures with seven developing refractory epilepsy.
[Conclusions] The clinical features of schizencephaly vary broadly and their severity is closely related to the importance of the cleft. Determination of schizencephaly type and its extent is useful for management planning and predicting prognosis.
[Methodology] We retrospectively reviewed the medical records and magnetic resonance images (MRI) of 21 patients with schizencephaly between January 2000 and December 2014. The MRI findings were recorded along with the presence of associated cerebral malformations. The clinical, electrophysiological and associated systemic malformation data were also recorded.
[Results] A total of 21 patients, 13 males and 8 females, were enrolled in the study. Their diagnostic ages ranged from 1 month to 18 years with a mean age of 3 years 2 months. According to the location of schizencephaly, patients were classified into two groups: unilateral (16) and bilateral (5). The phenotypic spectrum in our population differed from that described previously. In unilateral schizencephaly, the most common mode of evaluation was hemiparesis; however, seizures were the most common in bilateral group. The most common associated cerebral malformation was ventriculomegaly in both groups. The majority of patients had developmental delay, intellectual deficit, and speech impairment, especially in bilateral group. Seventeen patients suffered from epileptic seizures with seven developing refractory epilepsy.
[Conclusions] The clinical features of schizencephaly vary broadly and their severity is closely related to the importance of the cleft. Determination of schizencephaly type and its extent is useful for management planning and predicting prognosis.