ObjectiveTo investigate characteristics of motor semiology of epileptic seizure originated from dorsolateral frontal lobe. MethodsRetrospectively analysis the clinical profiles of patients who were diagnosed dorsolateral frontal lobe epilepsy (FLE) based on stereoelectroencephalography (SEEG) and underwent respective surgeries subsequently. Component of motor semiology in a seizure can be divided into elementary motor (EM, include tonic, versive, clonic, and myoclonic seizures) and complex motor (CM, include automotor, hypermotor, and so on). A Talairach coordinate system was constructed in the sagittal series of MRI images in each case. From the cross point of VAC and the Sylvian Fissure, a line was drawn antero-superiorly, which made an angle of 60° with the AC-PC line, then the frontal lobe could be divided into anterior and posterior portion. The epileptogenic zone, which was defined as ictal onset and early spreading zone in SEEG, was classified into three types, according to the positional relationship of the responding electrodes contacts and the "60° line": the anterior, posterior, and intermediate FLE. The correlation of the components of motor semiology in seizures and the location of the epileptogenic zone was analyzed. ResultsFive cases (26.3%) were verified as anterior FLE, among which there were 2 of EM, one of CM, and 2 of EM+CM. In 7 cases (36.8%) of intermediate FLE, there were one of EM, none of CM, and 6 of EM+CM. In the rest 7 cases of posterior FLE, there were 6 of EM, none of CM, and one of EM+CM. Compared with the cases that the epileptogenic zone involved anterior portion, the posterior FLE is more likely to present EM seizures (85.7%), and less likely to show CM components (P < 0.05). And Compared with the anterior FLE and posterior FLE, the intermediate FLE is more likely to present EM+CM seizures (85.7%)(P < 0.05). ConclusionThe motor seizure semiology of dorsolateral FLE has significant correlation with the localization of the epileptogenic zone. Posterior FLE mainly present a pure elementary motor seizure, and once the epileptogenic zone involved anteriorly beyond the "60° line", the component of complex motor seizure would be seen. Intermediate FLE, as its specialty of transboundary, is more likely to show "comprised semiology" of EM and CM. Construction of the "60° line" with AC-PC coordinate system in the MRI images may play an useful role in semiology analysis in presurgical evaluation of FLE.
ObjectiveTo study the clinical characteristics of patients onset epilepsy Dentatorubral-pallidoluysian atropy (DRPLA) in Epilepsy Center of Guangdong 999 Brain Hospital and improve understanding of the disease. MethodsCollected five patients from August 2014 to August 2016 in Guangdong 999 Brain Hospital, whom diagnosed through genetic testing of DRPLA, analysed their disease course, family history, video-EEG, brain MRI and treatment data. ResultsDRPLA performed as neurodegenerative diseases, and epilepsy population mainly performed as progressive myoclonic epilepsy (Progressive myoclonus epilepsy, PME). ConclusionDRPLA is autosomal dominant neurodegenerative disease. In patients with cerebellar atrophy, neurological regression, ataxia, drug refractory epilepsy, it is recommended routinely to detect ATN1 gene, so that timely diagnosis and genetic counseling.
ObjectivesPost-encephalitic epilepsy could be of great chance of pharmaco-resistant, even surgery may not achieve seizure free. The aim of this study is to mapping epileptogenic area of pharmaco-resistant post-encephalitic temporal lobe epilepsy, to find whether "temporal plus" epilepsy is the main type and its surgery outcome, based on stereo-EEG(SEEG) study.MethodWe retrospectively studied 15 patients with pharmaco-resistant temporal lobe epilepsy. Scalp EEG, seizure semiology, MRI, FDG-PET, and SEEG were reviewed for all patients. According to epileptogenic area which was analysed by SEEG, 15 patients were divided into 2 groups, temporal lobe epilepsy(TLE) group and temporal plus epilepsy(TPE) group. Clinical characteristics were compared with each group, by t-test or Fisher exact test when data needed.ResultsThere were 8 patients in TLE group, with 6 mesial TLE, 1 lateral TLE, 1 mesial-lateral TLE. And 7 patients in TPE group. Age of seizure onset (P=0.548), duration of epilepsy (P=0.099), age of remote encephalitis (P=0.385), type of semiology (P=0.315) and lateralization of MR lesions (P=1.000), interictal FDG-PET hypometabalism (P=1.000) or intracranial implantation (P=0.619) were of no statistically difference between TLE group and TPE group. Surgery was performed in all patients. Better outcome was obtained in TLE group(5/8 class Ⅰ), and poor was in TPE group(3/7class Ⅰ).ConclusionMesial-TLE and temporal plus epilepsy were common types of pharmaco-resistant post-encephalitic TLE. There was no way to differentiate clinically, except by SEEG. Mesial-TLE had a better outcome after surgery, but temporal plus epilepsy did not.