scholarly journals Alternate Seizure Spread with Agenesis of the Corpus Callosum

2021 ◽  
Vol 11 (1) ◽  
pp. 100-105
Author(s):  
Lohit Velagapudi ◽  
Caio M. Matias ◽  
Timothy M. Ambrose ◽  
Michael R. Sperling ◽  
Maromi Nei ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
Temporal Lobe Epilepsy ◽  
Cognitive Deficits ◽  
Surgical Approach ◽  
Refractory Epilepsy ◽  
Anterior Commissure ◽  
Brain Malformation ◽  
Developmental Defects ◽  
Medically Refractory Epilepsy

Agenesis of the corpus callosum is a brain malformation that can occur in isolation or in conjunction with other congenital or developmental defects. The clinical sequelae of this condition include epilepsy, cognitive deficits, developmental delay, and various neurological and psychiatric disorders. Here we present the case of a patient with congenital complete agenesis of the corpus callosum and medically refractory epilepsy who underwent stereoelectroencephalography. This identified a left frontal ictal focus and revealed that contralateral spread occurred though the anterior commissure, a rare and interesting occurrence. Left frontal resection resulted in significant improvement. This demonstrates the role of the anterior commissure in ictal spread and the potential for novel methods of seizure spread in patients with temporal lobe epilepsy that must be considered in a surgical approach.

scholarly journals Role of Electroencephalography in Presurgical Evaluation of Temporal Lobe Epilepsy

2012 ◽  
Vol 2012 ◽  
pp. 1-18 ◽  
Author(s):  
Seetharam Raghavendra ◽  
Javeria Nooraine ◽  
Seyed M. Mirsattari
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Refractory Epilepsy ◽  
Therapeutic Option ◽  
Presurgical Evaluation ◽  
Video Eeg ◽  
Medically Refractory Epilepsy

Surgery remains a therapeutic option for patients with medically refractory epilepsy. Comprehensive presurgical evaluation includes electroencephalography (EEG) and video EEG in identifying patients who are likely to benefit from surgery. Here, we discuss in detail the utility of EEG in presurgical evaluation of patients with temporal lobe epilepsy along with illustrative cases.

Systematic Analysis of Brain MRI Findings in Adaptor Protein Complex 4–Associated Hereditary Spastic Paraplegia

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012836
Author(s):  
Darius Ebrahimi-Fakhari ◽  
Julian E Alecu ◽  
Marvin Ziegler ◽  
Gregory Geisel ◽  
Catherine Jordan ◽  
...  
Keyword(s):  
White Matter ◽  
Corpus Callosum ◽  
Brain Development ◽  
Hereditary Spastic Paraplegia ◽  
Anterior Commissure ◽  
Brain Mri ◽  
Adaptor Protein ◽  
Spastic Paraplegia ◽  
Mri Findings

Background and Objectives:AP-4-associated hereditary spastic paraplegia (AP-4-HSP: SPG47, SPG50, SPG51, SPG52) is an emerging cause of childhood-onset hereditary spastic paraplegia and mimic of cerebral palsy. This study aims to define the spectrum of brain MRI findings in AP-4-HSP and to investigate radio-clinical correlations.Methods:A systematic qualitative and quantitative analysis of 107 brain MRI studies from 76 individuals with genetically-confirmed AP-4-HSP and correlation with clinical findings including surrogates of disease severity.Results:We define AP-4-HSP as a disorder of gray and white matter and demonstrate that abnormal myelination is common and that metrics of reduced white matter volume correlate with severity of motor symptoms. We identify a common diagnostic imaging signature consisting of (1) a thin splenium of the corpus callosum, (2) an absent or thin anterior commissure, (3) characteristic signal abnormalities of the forceps minor (“ears of the grizzly sign”), and (4) periventricular white matter abnormalities. The presence of two or more of these findings has a sensitivity of ∼99% for detecting AP-4-HSP, while the combination of all four is found in ∼45% of cases. Compared to other HSP with a thin corpus callosum, the absent anterior commissure appears to be specific to AP-4-HSP. Our analysis further identified a subset of AP-4-HSP patients with polymicrogyria, underscoring the role of AP-4 in early brain development. Of clinical importance, these patients displayed a higher prevalence of seizures and status epilepticus, many at a young age.Discussion:Our findings define the MRI spectrum of AP-4-HSP providing opportunities for early diagnosis, identification of individuals at risk for complications, and a window into the role of the AP-4 complex in brain development and neurodegeneration.

Anatomic hemispherectomy for intractable epilepsy in a patient with unilateral schizencephaly

2008 ◽  
Vol 2 (2) ◽  
pp. 146-149 ◽  
Author(s):  
Gregory G. Heuer ◽  
Douglas A. Hardesty ◽  
Kareem A. Zaghloul ◽  
Erin M. Simon Schwartz ◽  
A. Reghan Foley ◽  
...  
Keyword(s):  
Ventriculoperitoneal Shunt ◽  
Seizure Control ◽  
Refractory Epilepsy ◽  
Intractable Epilepsy ◽  
Cerebral Hemispheres ◽  
Communicating Hydrocephalus ◽  
Brain Shift ◽  
Brain Malformation ◽  
Viable Option ◽  
Medically Refractory Epilepsy

Schizencephaly is a rare congenital cortical brain malformation defined by unilateral or bilateral clefts of the cerebral hemispheres. These malformations are often associated with medically intractable epilepsy. Surgical solutions include lesionectomy, lobectomy, or hemispherectomy. The authors describe the case of an anatomic hemispherectomy for medically intractable epilepsy in an 8-year-old boy with a large schizencephalic cleft. Seven years prior to his epilepsy surgery, the patient underwent placement of a ventriculoperitoneal shunt for communicating hydrocephalus that resulted in severe left-to-right shift. Subsequently, medically refractory epilepsy developed and the patient underwent an anatomic hemispherectomy for seizure control. The preoperative brain shift remained after the surgery, although the patient tolerated the procedure well and was seizure free postoperatively. Anatomic hemispherectomy is a viable option for treating medically intractable epilepsy in a schizencephalic pediatric patient—even one with considerable brain shift.

scholarly journals The Putative Role of mTOR Inhibitors in Non-tuberous Sclerosis Complex-Related Epilepsy

2021 ◽  
Vol 12 ◽  
Author(s):  
Hannah E. Goldstein ◽  
Jason S. Hauptman
Keyword(s):  
Tuberous Sclerosis ◽  
Seizure Frequency ◽  
Tuberous Sclerosis Complex ◽  
Refractory Epilepsy ◽  
Mtor Inhibitors ◽  
Serine Threonine Kinase ◽  
Cortical Dysplasias ◽  
Highly Correlated ◽  
Medically Refractory Epilepsy

Epilepsy affects ~5 out of every 10,000 children per year. Up to one-third of these children have medically refractory epilepsy, with limited to no options for improved seizure control. mTOR, a ubiquitous 289 kDa serine/threonine kinase in the phosphatidylinositol 3-kinase (PI3K)-related kinases (PIKK) family, is dysregulated in a number of human diseases, including tuberous sclerosis complex (TSC) and epilepsy. In cell models of epilepsy and TSC, rapamycin, an mTOR inhibitor, has been shown to decrease seizure frequency and duration, and positively affect cell growth and morphology. Rapamycin has also been shown to prevent or improve epilepsy and prolong survival in animal models of TSC. To date, clinical studies looking at the effects of mTOR inhibitors on the reduction of seizures have mainly focused on patients with TSC. Everolimus (Novartis Pharmaceuticals), a chemically modified rapamycin derivative, has been shown to reduce seizure frequency with reasonable safety and tolerability. Mutations in mTOR or the mTOR pathway have been found in hemimegalencephaly (HME) and focal cortical dysplasias (FCDs), both of which are highly correlated with medically refractory epilepsy. Given the evidence to date, a logical next step is to investigate the role of mTOR inhibitors in the treatment of children with medically refractory non-TSC epilepsy, particularly those children who have also failed resective surgery.

Consequences of mesial temporal sparing temporal lobe surgery in medically refractory epilepsy

2021 ◽  
Vol 115 ◽  
pp. 107642
Author(s):  
Lilach Goldstein ◽  
Mitra Dehghan Harati ◽  
Kathryn Devlin ◽  
Joseph Tracy ◽  
Maromi Nei ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Refractory Epilepsy ◽  
Medically Refractory Epilepsy

A historical cohort of temporal lobe surgery for medically refractory epilepsy: a systematic review and meta-analysis to guide future nonrandomized controlled trial studies

2020 ◽  
Vol 133 (1) ◽  
pp. 71-78 ◽  
Author(s):  
Anthony T. Lee ◽  
John F. Burke ◽  
Pranathi Chunduru ◽  
Annette M. Molinaro ◽  
Robert Knowlton ◽  
...  
Keyword(s):  
Systematic Review ◽  
Temporal Lobe ◽  
Epilepsy Surgery ◽  
Refractory Epilepsy ◽  
Meta Analysis ◽  
Small Sample ◽  
Seizure Freedom ◽  
Level I ◽  
Study Designs ◽  
Medically Refractory Epilepsy

OBJECTIVERecent trials for temporal lobe epilepsy (TLE) highlight the challenges of investigating surgical outcomes using randomized controlled trials (RCTs). Although several reviews have examined seizure-freedom outcomes from existing data, there is a need for an overall seizure-freedom rate estimated from level I data as investigators consider other methods besides RCTs to study outcomes related to new surgical interventions.METHODSThe authors performed a systematic review and meta-analysis of the 3 RCTs of TLE in adults and report an overall surgical seizure-freedom rate (Engel class I) composed of level I data. An overall seizure-freedom rate was also collected from level II data (prospective cohort studies) for validation. Eligible studies were identified by filtering a published Cochrane meta-analysis of epilepsy surgery for RCTs and prospective studies, and supplemented by searching indexed terms in MEDLINE (January 1, 2012–April 1, 2018). Retrospective studies were excluded to minimize heterogeneity in patient selection and reporting bias. Data extraction was independently reverified and pooled using a fixed-effects model. The primary outcome was overall seizure freedom following surgery. The historical benchmark was applied in a noninferiority study design to compare its power to a single-study cohort.RESULTSThe overall rate of seizure freedom from level I data was 72.4% (55/76 patients, 3 RCTs), which was nearly identical to the overall seizure-freedom rate of 71.7% (1325/1849 patients, 18 studies) from prospective cohorts (z = 0.134, p = 0.89; z-test). Seizure-freedom rates from level I and II studies were consistent over the years of publication (R2< 0.01, p = 0.73). Surgery resulted in markedly improved seizure-free outcomes compared to medical management (RR 10.82, 95% CI 3.93–29.84, p < 0.01; 2 RCTs). Noninferiority study designs in which the historical benchmark was used had significantly higher power at all difference margins compared to using a single cohort alone (p < 0.001, Bonferroni’s multiple comparison test).CONCLUSIONSThe overall rate of seizure freedom for temporal lobe surgery is approximately 70% for medically refractory epilepsy. The small sample size of the RCT cohort underscores the need to move beyond standard RCTs for epilepsy surgery. This historical seizure-freedom rate may serve as a useful benchmark to guide future study designs for new surgical treatments for refractory TLE.

Sign in / Sign up

Export Citation Format

Share Document