Ulegyria Research Articles

Outcome analysis of surgical resections in patients with refractory epilepsy—A review of 15-year experience at a single center

Objective To summarize the epileptogenic localizations, pathological findings and long-term surgical outcomes of patients with refractory epilepsy at a single center during past 15 years. Methods A total of 2 424 patients who underwent epilepsy surgeries at Department of Functional Neurosurgery between April 2001 and December 2016 were included in this retrospective study. The traits of epileptogenic localization were analyzed. Outcomes of all patients who were followed up longer than 1 year post surgeries were studied in two stages, and the rate of seizure free was calculated. Results Among all 2 424 patients, 1 299 (53.6%) had obvious lesions on MRI. There were 1 007 (41.5%) patients who underwent intracranial electrode placement and 65.5% of those had no definite lesions on MRI. There were 1 030 (42.5%) cases who were diagnosed as temporal lobe epilepsy (TLE) and 1 394 (57.5%) diagnosed as extratemporal lobe epilepsy (ETLE). The common pathological types included focal cortical dysplasia (37.5%, 909), mesial temporal sclerosis (17.7%, 429), ulegyria (15.7%, 381) and low grade tumor (9.2%, 223). There was a double pathological change in 303 (12.5%) patients. A total of 1 424 (59.0%) patients were followed up for more than 1 year post surgery. At the first stage of follow-up (April 2001 to July 2012), 846 patients completed follow-up lasting at least 1 year. The seizure-free rate was 64.7% (547/846) at 1 year, 52.5% (241/459) at 3 years and 49.6% (119/240) at 5 years post surgery. For the patients with TLE, the seizure-free rate was 57.9% (210/363) at 1 year, 57.8% (108/187) at 3 years and 56.3% (58/103) at 5 years post surgery. For the patients with ETLE, the seizure-free rate was 59.4% (287/483) at 1 year, 47.8% (130/272) at 3 years and 46.7% (64/137) at 5 years post operation. At the second stage of follow-up (August 2012 to September 2015), 578 patients completed follow-up lasting at least 1 year. The seizure-free rate was 64.7% (374/578) at 1 year and 57.3% (133/232) at 3 years post surgery. For the patients with TLE, the seizure-free rate was 71.4% (187/262) at 1 year and 66.4% (64/95) at 3 years post operation. For patients with ETLE, the seizure-free rate was 59.5% (188/316) and 51.1% (70/137) at 1 year and 3 years post operation respectively. However, the difference in the seizure free rate was significant only at the first year post surgery in the patients with TLE between the 2 stages (57.9% vs. 71.4%, P=0.001), and there was no significant difference afterwards. Conclusions Long-term intracranial recording seems to be indispensable for identification of epileptogenic focus for some patients. Although surgical efficacy might decrease to some extent with extension of postoperative time, surgical treatment is beneficial and postoperative outcome is relatively stable for patients with either temporal or extratemporal epilepsy. Key words: Epilepsy; Neurosurgical procedures; Treatment outcome; Temporal lobe epilepsy; Extratemporal lobe epilepsy

Clinicopathologic analysis of cerebral hemisphere ulegyria in 26 patients with refractory epilepsy

Objective To investigate the clinicopathologic features of cerebral hemisphere ulegyria in children with refractory epilepsy. Methods The clinical and pathologic findings were reviewed in 26 children who underwent resection of lesion and epileptogenic lobectomy operation in the Neurosurgery Department of Haidian Hospital, Haidian District of Peking University Third Hospital, from January 2011 to August 2015, and pathological diagnosis was cerebral hemisphere ulegyria. Results All children including 19 male and 7 female had medically intractable seizures.The mean ages of seizure onset and disease duration were 3.93 years old (from 10 days to 12 years old) and 5.42 years (from 1 month to 13 years), respectively.Eight cases had seizures because of perinatal period injury, and 12 cases developed seizures owing to infancy injury and others had no cause of disease.The mean operation age was 9.35 years old (5-14 years old). Fourteen cases underwent multilobar resection and the whole corpus callosum incision, and 12 cases were given modified anatomical cerebral hemisphere resection.The pathological diagnoses of brain tissues were cerebral hemisphere ulegyria with focal cortical dysplasia(FCD)Ⅲd and dual pathology.Seizure outcome after the operation revealed that 19 cases (73.08%) had an Engel gradeⅠ, 3 cases(11.54%)had an Engel gradeⅡ, 2 cases(7.69%)had an Engel grade Ⅲ, and 2 cases(7.69%) missed the follow-up. Conclusions Acquired brain injury during the period of infant can cause ulegyria and cortical dysplasia, resulting in intractable epilepsy.The treatment of improved anatomical cerebral hemisphere resection and multilobar resection is significantly effective. Key words: Epilepsy; Neurosurgery; Malformations of cortical development

Clinicopathologic features and surgery treatment of intractable epilepsy in childhood

Objective To study the pathological features and the timing and effects of epilepsy surgery in children with intractable epilepsy.Methods The outcomes of 71 pediatric cases that underwent epilepsy surgery in our department were collected.All the patients had at least 1 year of follow-up.The effects of operation were evaluated with Engel class.Results Seizure freedom (Engel Class Ⅰ outcome) was achieved in 45 cases (63％),26 patients (37％) continued to have seizures (Engel Class Ⅱ ～ Ⅳ outcome) (P ＜ 0.05).The major pathological findings in these patients included:focal cortical dysplasia in 31 cases,low grade neoplasm in 18,ulegyria in 13,polymicrogyia malformation in 6,tuberous sclerosis in 3,and dual pathology in 9.There was no death or permanent disability.Conclusions Early surgical intervention was safe and effective for the treatment of intractable epilepsy in children.The relation between the pathological features and surgical effects should be paid attention for further study. Key words: Child ; Intractable epilepsy; Pathology; Surgical procedures

Bilateral perisylvian ulegyria: An under‐recognized, surgically remediable epileptic syndrome

Interest in the association of epilepsy and pseudobulbar palsy was rekindled since the identification through magnetic resonance imaging (MRI) of bilateral perisylvian polymicrogyria (PMG). Seizures are often intractable, but resective epilepsy surgery has not been recommended. However, a similar clinical picture can be encountered in patients with bilateral perisylvian destructive lesions, which fit the description of ulegyria (ULG). We report a series of patients with epilepsy and pseudobulbar palsy due to bilateral perisylvian ULG (BP-ULG), show that hippocampal sclerosis (HS) is often associated and highlight the fact that in this entity, unlike in malformative bilateral perisylvian PMG, seizures may be surgically treated. The motor, cognitive, epileptologic, and imaging features of 12 patients with perisylvian ULG followed at three institutions are described. For patients with refractory seizures, we detail extracranial and intracranial electrographic recordings, surgical strategies, histopathologic analyses of the resected tissue, and outcome of surgical treatment. Descriptive statistics were used for quantitative and categorical variables. Student's t-test was used to compare means, and a p < 0.05 was considered significant. Pseudobulbar palsy and mental retardation were present in all patients with symmetrical BP-ULG. Five had refractory seizures. There was no relationship between the severity of the pseudobulbar palsy or of the mental retardation and the degree of seizure control with medication. The five patients in whom seizures were refractory to medication had significantly earlier age of onset and longer duration of epilepsy (p < 0.05). Dual pathology with associated unilateral HS was present in four. One patient with dual pathology had a temporolimbic electroclinical picture and had an anterior temporal lobectomy (ATL) based upon noninvasive evaluation. The other four had ictal semiology suggesting involvement of both temporolimbic and perisylvian cortex. Intracranial electroencephalography (EEG) showed concomitant seizure onset in the anterior temporal region and in the ipsilateral ULG in three of the four with dual pathology and in the ulegyric cortex in the one without HS. Resection guided by a combination of semiology, MRI, and extra and intracranial EEG led to complete seizure control in two and almost complete seizure control (Engel class II) in two other patients. The only surgical failure was an isolated ATL in a patient with dual pathology, and concomitant seizure onset in both lesions according to semiology and intracranial EEG. Our findings suggest that BP-ULG mimics the clinical features of bilateral perisylvian PMG. In patients with refractory seizures, recognition of this entity should lead to consideration of resective surgery despite the bilateral ULG.