ObjectiveTo investigate the lateralization of ictal scalp EEG in different times in focal epilepsy.Methods356 surface ictal EEG of 41 patients were reviewed retrospectively in focal epilepsy arising from the mesial frontal, lateralfrontal, mesialtemporal, neocorticaltemporal, insular lobes and posterior cortex from July, 2010 to at, 2016. Each ictal scalp EEG was subdivided into ten epoches (E1-E10), then the lateralization of every epoch was analyzed. Ten epochs EEG were merged into three timesas E1-E3, E4-E6 and E7-E10. The ratio of lateralization, mislateralization and non-lateralization of each timeEEG were studied. Ictal onset zone (IOZ) were precise localized by intracranial EEG. The results of epileptogenic zone corresponded with surgical outcomes as seizure free or decreased.Results62% seizures were lateralized by surface ictal EEG in all epilepsies. Lateralized ictal scalp EEG were seen in nearly 80% of seizures in all times in temporal lobe epilepsy (TLE). The highest lateralization of 89% occurred inE4-E6 andfalse lateralization up to 30% in E1-E3 in mesial temporal lobe epilepsy (MTLE), whereas 95% lateralized seizures emerged in E1-E3 in neocortical temporal lobe epilepsy (NTLE). Apparent non-lateralization in all times were higher than lateralization in frontal lobe epilepsy (FLE), especially in mesial frontal lobe epilepsy (MFLE). Lateralization in E1-E3 was only 24% higher than other times. In addition, False lateralization never occurred in all times in lateral frontal lobe epilepsy (LFLE). There were maximum of 83%lateralized seizures in E1-E3 in LFLE and 93% in E1-E3 in posterior cortex epilepsy (PCE). Seizures arising from insular lobe epilepsy (ILE) tendedto predict less lateralization in all times.ConclusionsIctal scalp EEG of E1-E3 are valuable in the lateralization in all epilepsies particularly in LFLE, NTLE and PCE. Lateralized E4-E6 and E7-10 are very useful in MTLE.
ObjectiveTo analyse the seizure semeiology of MRI negative insular epilepsy.MethodsA case of patient with insular epilepsy who presented in Epileptic Center, Guangdong 999 Brain Hospital was collected. Related literatures were thoroughly reviewed.ResultsThe patient was diagnosised as insular epilepsy by SEEG and postoperative pathology reported focal cortical dysplasia Ib.ConclusionsThe insula lobe is a part of the limbic system, and the seizure semeiology in insular epilepsy is varied. Seizure semeiology analysis is particularly important in the evaluation of presurgical especially in a MRI negative case. In this paper, we summarized the semeiology characteristics of various parts of insula lobe, and provided some references for the diagnosis and treatment of insular epilepsy.
ObjectiveTo clarify the characteristic of secondary cerebral amyloidoma which is relapsing in one year after seven years gamma knife radiosurgery and review relevant literature.MethodsTo analyze the clinical manifestation, preoperative and postoperative MRI imaging, inter-ictal and ictal electroencephalogram (EEG) and histopathological evaluation.ResultsThe patient suffered from epilepsy (mainly autonomic seizure and global tonic-clonic seizure) at the age of 22 and took a gamma knife radiosurgery for right medial temporal epilepsy as the refractory seizures occurred at the age of 36. In her 43 and 44 years’ old, she suddenly found left hemiplegia and mental retardation, the MRI showed right frontal and parietal space-occupying lesion and relapsed after the partial excision respectively, the inter-ictal and ictal EEG displayed persistent slow wave in the right hemisphere and spike wave located in the right posterior temporal and central-parietal, after the surgery, we found amyloid in the histopathological evaluation.ConclusionOne of the delayed complications of gamma knife radiosurgery is secondary cerebral amyloidoma, and partial excision may induced relapsing easily.
Objective To investigate the task group’s effectiveness in language evaluation based on the task group's functional Magnetic resonance (fMRI) results’ agreement with the fixation side of the Wada language area. MethodsWe collected 90 patients with intractable epilepsy of 90 patients from December 13, 2018 to January 3, 2020 from the Epilepsy Center of Guangdong 999 Brain Hospital. We used two simple fMRI tasks. Among them, 25 patients completed the Wada experimental examination, and 8 patients completed the electrode implantation and subsequent preoperative language area mapping. Adopt block experimental design, ABBA style presentation, and use AFNI software to process fMRI data, lateralization index calculation, and multiple regression analysis. ResultsfMRI results from 90 patients showed that the results from both the sentence-completion task and the image-naming task were more stable than those from either task. The results were then compared with the results of the “gold standard” Wada test in 25 patients with fMRI-located language dominance in the hemisphere. The results showed that the accuracy of the single task was between 70% and 80%, but the accuracy of the combined results of the two tasks was 93.3%. Conclusions Compared with the results of a single task, the results of multiple fMRI tasks are more stable in the judgment of activation range and language dominance hemisphere. fMRI and Wada language area siding accuracy 93.3%, fMRI task siding valid and replicable.
ObjectiveTo investigate the application of stereoelectroencephalography (SEEG) in the refractory epilepsy related to periventricular nodular heterotopia (PNH). MethodsTen patients with drug-resistant epilepsy related to PNHs from Guangdong Sanjiu Brain Hospital and the First Affiliated Hospital of Jinan University from April 2017 to February 2021 were studied. Electrodes were implanted based on non-invasive preoperative evaluation. Then long-term monitoring of SEEG was carried out. The patterns of epileptogenic zone (EZ) were divided into four categories based on the ictal SEEG: A. only the nodules started; B. nodules and cortex synchronous initiation; C. the cortex initiation with early spreading to nodules; D. only cortex initiation. All patients underwent SEEG-guided radiofrequency thermocoagulation (RFTC), with a follow-up of at least 12 months. ResultsAll cases were multiple nodules. Four cases were unilateral and six bilateral. Eight cases were distributed in posterior pattern, and one in anterior pattern and one in diffused pattern, respectively. Seven patients had only PNH (pure PNH) and three patients were associated with other overlying cortex malformations (PNH plus). The EZ patterns of all cases were confirmed by the ictal SEEG: six patients were in pure type A, two patients were in pure type B, one patient in type A+B and one in type A+B+C, respectively. In eight patients SEEG-guided RF-TC was targeted only to PNHs; and in two patients RFTC was directed to both heterotopias and related cortical regions. The mean follow up was (33.4±14.0) months (12 ~ 58 months). Eight patients (in pure type A or type A included) were seizure free. Two patients were effective. None of the patients had significant postoperative complications or sequelae. ConclusionThe epileptic network of Epilepsy associated with nodular heterotopia may be individualized. Not all nodules are always epileptogenic, the role of each nodule in the epileptic network may be different. And multiple epileptic patterns may occur simultaneously in the same patient. SEEG can provide individualized diagnosis and treatment, be helpful to prognosis.
ObjectiveThe study aimed to investigate the clinical characteristics of epilepsy patients with DEPDC5 mutation, and to improve the understanding of familial hereditary focal epilepsy.MethodsThree families with familial hereditary focal epilepsy were enrolled in this study from September 2014 to September 2017 at the Sanjiu Brain Hospital of Guangdong Province. Epilepsy-related gene in peripheral blood was detected by the second generation sequencing. The medical history, family history, magnetic resonance imaging, electroencephalo-groph, treatment programs and other data were collected and aralyzed.ResultsThere were 8 patients in the three families, seizures of whom originate mostly from the frontal or temporal lobe. Cognitive function and other system function was basically normal fron patients treated with antiepilepsy drugs.ConclusionsThe mutations of DEPDC5 gene are common in familial hereditary focal epilepsy, which leads to the main clinical symptom of complex partial seizure. Antiepilepsy drug therapy is effective to most patients. However, to those drug resistant patients, prognosis can improved by surgical treatment.
ObjectiveWe report a special case to explain seizure semiology and epileptogenic network of seizure arising from ventral motor cortex, and to explore Focal cortical dycplasia (FCD) features on MR of epileptic patients with DEPDC5 mutation.MethodsA drug-resistant focal epilepsy patient with DEPDC5 mutation was underwent a detailed presurgical evaluation. The epileptogenic area(EA) was localized with SEEG and removed later by surgery. Related literatures were thoroughly reviewed.ResultsSubtle FCD of ventral branch of inferior precentral sulcus(IPv) on MR(1.5T) was noticed. With SEEG recording, seizure onset zone was detected on IPv with the probable lesion, early spreading to anterior insula, central operculum and ventral precentral gyrus. According to the architectures of ventral motor trend, seizure semiology with evolution from contralateral dystonia to ipsilateral chorea movement could be better comprehended. Seizure was controlled after totally resection on the sites of IPv, anterior insula, and central operculum. Pathological change was FCD type I. Other literatures reported that DEPDC5 mutation related FCD may be located in motor system, and seizure onset could also be in anterior insula cortex besides motor cortex in other SEEG cases.ConclusionsEarly contralateral dystonia and chorea movement could be definite figures of seizure arising from inferior precentral sulcus; DEPDC5 mutation maybe a clue to find subtle FCD in motor cortex.
ObjectiveTo improve the knowledge of a rare disease named pyridoxine-dependent epilepsy.MethodsHigh-throughput sequencing and Sanger sequencing were used to validate the genes of epilepsy. Mutation gene validation was performed on two probands and their parents. Analyze clinical manifestations, electroencephalogram (EEG), imaging and prognostic features of the two probands.ResultsProbands 1, seizure onset at 4 months, progress as drug-refractory epilepsy, manifested as seizures types origin of multi-focal lesions. Head MRI and fluorodeoxyglucose-positron-based tomography (FDG-PET) were both normal. Gene detection showed that Aldehydedehydrogenase (ALDH7A1) gene has a complex heterozygous mutation contain c.1442G> and c.1046C> T.Proband 2, seizure onset at 5 months, manifested as a tonic-clonic seizure. Intermittent EEG and head MRI were both normal. Genotyping revealed ALDH7A1 gene contain a complex heterozygous mutation c.1547A> G and c.965C> T. Two cases were both seizure free by vitamin B6 therapy and gradually reduce the antiepileptic drugs.ConclusionsPyridoxine-dependent epilepsy may be late onset, some patient can be atypical and early experimental treatment can help to identify and the diagnosis should be confirmed by gene test.
ObjectiveTo improve the understanding of clinicians by reports and literature review of patients with rare diseases of Menkes disease.MethodsHigh-throughput sequencing and Sanger sequencing were used to verify the genes of epilepsy, and the mutations were verified in three probands and two parents. The patient's clinical manifestations, EEG, imaging, gene and prognostic characteristics were analyzed.ResultsAll the three patients developed onset in infancy, with hair thinning and curling, and various forms of seizures. Three patients had epileptiform discharges during the EEG interval, and all clinical seizures were recorded. Skull MR showed white matter long T1, long T2 abnormal signal, cerebral artery tortuosity, proband 3 appeared subdural effusion. Three patients had poor efficacy after taking anti-epileptic drugs. The proband one and the proband two did not show significant progress after using histidine copper, but could not alleviate the existing neurological damage.ConclusionMenkes disease occurs frequently in infants, clinical manifestations may be different, some clinical manifestations may be atypical, and currently it is an incurable disease, but the use of histidine copper in the neonatal period can improve survival and reduce nervous system injury. It should be diagnosed early. and the treatment of indications should not be guided by the patient's genotype.