Neurology Commons^™

Ultradian Rhythms In Accelerometric And Autonomic Data Vary Based On Seizure Occurrence In Paediatric Epilepsy Patients, Solveig Vieluf, Sarah Cantley, Vaishnav Krishnan, Tobias Loddenkemper

Faculty, Staff and Students Publications

Ultradian rhythms are physiological oscillations that resonate with period lengths shorter than 24 hours. This study examined the expression of ultradian rhythms in patients with epilepsy, a disease defined by an enduring seizure risk that may vary cyclically. Using a wearable device, we recorded heart rate, body temperature, electrodermal activity and limb accelerometry in patients admitted to the paediatric epilepsy monitoring unit. In our case–control design, we included recordings from 29 patients with tonic–clonic seizures and 29 non-seizing controls. We spectrally decomposed each signal to identify cycle lengths of interest and compared average spectral power- and period-related markers between groups. …

Screening For Adhd In Adult Patients With Epilepsy: Prevalence Of Symptoms And Challenges To Diagnosis, Trung Nguyen, Emily Xiao, Allison Clark, Afroz Shamim, Atul Maheshwari

Faculty, Staff and Students Publications

OBJECTIVE: Given the complex nature of seizure disorders and their treatments, ADHD may be underdiagnosed in this population. We hypothesized that a higher percentage of patients presenting to a seizure clinic would endorse ADHD symptoms compared to rates reported in the general population and that formal screening for ADHD symptoms would identify patients with previously undiagnosed comorbid ADHD.

METHODS: In this study, we surveyed 312 adults in a seizure clinic using the Adult ADHD Self-Report Scale (ASRS-v1.1).

RESULTS: We found that 90 patients (28.8%) screened positive with the ASRS-v1.1, but only nine of these patients were able to complete neuropsychological …

Proceedings Of The 11th Annual Deep Brain Stimulation Think Tank: Pushing The Forefront Of Neuromodulation With Functional Network Mapping, Biomarkers For Adaptive Dbs, Bioethical Dilemmas, Ai-Guided Neuromodulation, And Translational Advancements, Kara A Johnson, Nico U F Dosenbach, Evan M Gordon, Cristin G Welle, Kevin B Wilkins, Helen M Bronte-Stewart, Valerie Voon, Takashi Morishita, Yuki Sakai, Amanda R Merner, Gabriel Lázaro-Muñoz, Theresa Williamson, Andreas Horn, Ro'ee Gilron, Jonathan O'Keeffe, Aryn H Gittis, Wolf-Julian Neumann, Simon Little, Nicole R Provenza, Sameer A Sheth, Alfonso Fasano, Abbey B Holt-Becker, Robert S Raike, Lisa Moore, Yagna J Pathak, David Greene, Sara Marceglia, Lothar Krinke, Huiling Tan, Hagai Bergman, Monika Pötter-Nerger, Bomin Sun, Laura Y Cabrera, Cameron C Mcintyre, Noam Harel, Helen S Mayberg, Andrew D Krystal, Nader Pouratian, Philip A Starr, Kelly D Foote, Michael S Okun, Joshua K Wong

Faculty, Staff and Students Publications

The Deep Brain Stimulation (DBS) Think Tank XI was held on August 9-11, 2023 in Gainesville, Florida with the theme of "Pushing the Forefront of Neuromodulation". The keynote speaker was Dr. Nico Dosenbach from Washington University in St. Louis, Missouri. He presented his research recently published in Nature inn a collaboration with Dr. Evan Gordon to identify and characterize the somato-cognitive action network (SCAN), which has redefined the motor homunculus and has led to new hypotheses about the integrative networks underpinning therapeutic DBS. The DBS Think Tank was founded in 2012 and provides an open platform where clinicians, engineers, and …

Prognostic Value Of The 5-Sense Score To Predict Focality Of The Seizure-Onset Zone As Assessed By Stereoelectroencephalography: A Prospective International Multicentre Validation Study, Alexandra Astner-Rohracher, Alyssa Ho, John Archer, Fabrice Bartolomei, Milan Brazdil, Melita Cacic Hribljan, James Castellano, Irena Dolezalova, Martin Ejler Fabricius, Mercedes Garcés-Sanchez, Kahina Hammam, Akio Ikeda, Kristin Ikeda, Philippe Kahane, Giridhar Kalamangalam, Gudrun Kalss, Mays Khweileh, Katsuya Kobayashi, Patrick Kwan, Joshua Andrew Laing, Markus Leitinger, Samden Lhatoo, Julia Makhalova, Aileen Mcgonigal, Iona Mindruta, Mary Margaret Mizera, Andrew Neal, Irina Oane, Prachi Parikh, Piero Perucca, Francesca Pizzo, Rodrigo Rocamora, Philippe Ryvlin, Victoria San Antonio Arce, Stephan Schuele, Andreas Schulze-Bonhage, Ana Suller Marti, Alexandra Urban, Vincente Villanueva, Laura Vilella Bertran, Benjamin Whatley, Sandor Beniczky, Eugen Trinka, Georg Zimmermann, Birgit Frauscher

Faculty, Staff and Student Publications

Introduction: Epilepsy surgery is the only curative treatment for patients with drug-resistant focal epilepsy. Stereoelectroencephalography (SEEG) is the gold standard to delineate the seizure-onset zone (SOZ). However, up to 40% of patients are subsequently not operated as no focal non-eloquent SOZ can be identified. The 5-SENSE Score is a 5-point score to predict whether a focal SOZ is likely to be identified by SEEG. This study aims to validate the 5-SENSE Score, improve score performance by incorporating auxiliary diagnostic methods and evaluate its concordance with expert decisions.

Methods and analysis: Non-interventional, observational, multicentre, prospective study including 200 patients with drug-resistant …

Saint Paul, The Apostle, And The Gastaut-Geschwind Syndrome, Leonardo Cruz De Souza, Guilherme Nogueira Mendes De Oliveira, Leandro Boson Gambogi, Ana Paula Gonçalves, Antônio Lúcio Teixeira

Faculty, Staff and Student Publications

The interface between epilepsy and religiosity has been a long-standing matter of debate. Epilepsy has affected several religious leaders throughout history. Hyperreligiosity may be observed in patients with temporal lobe epilepsy as a component of the so-called Gastaut-Geschwind syndrome which involves other behavioral and personality traits such as hyposexuality, viscosity, philosophical concerns, sense of personal destiny, hypergraphy, emotionality, and irritability. Saint Paul, the Apostle, probably had temporal lobe epilepsy. He was a genius man of intellectual excellence and refined culture, whose life and writings exerted a decisive influence on Western history. The current paper investigates the elements of Gastaut-Geschwind syndrome …

Responsive Neurostimulation For People With Drug-Resistant Epilepsy And Autism Spectrum Disorder, Madeline C Fields, Christina Marsh, Onome Eka, Emily A Johnson, Lara V Marcuse, Churl-Su Kwon, James J Young, Maite Lavega-Talbott, Mohankumar Kurukumbi, Gretchen Von Allmen, John Zempel, Daniel Friedman, Nathalie Jette, Anuradha Singh, Ji Yeoun Yoo, Leah Blank, Fedor Panov, Saadi Ghatan

Faculty, Staff and Student Publications

PURPOSE: Individuals with autism spectrum disorder (ASD) have comorbid epilepsy at much higher rates than the general population, and about 30% will be refractory to medication. Patients with drug-resistant epilepsy (DRE) should be referred for surgical evaluation, yet many with ASD and DRE are not resective surgical candidates. The aim of this study was to examine the response of this population to the responsive neurostimulator (RNS) System.

METHODS: This multicenter study evaluated patients with ASD and DRE who underwent RNS System placement. Patients were included if they had the RNS System placed for 1 year or more. Seizure reduction and …

Healthcare Utilization And Clinical Characteristics Of Genetic Epilepsy In Electronic Health Records, Christian M Boßelmann, Alina Ivaniuk, Mark St John, Sara C Taylor, Gokul Krishnaswamy, Alex Milinovich, Costin Leu, Ajay Gupta, Elia M Pestana-Knight, Imad Najm, Dennis Lal

Faculty, Staff and Student Publications

Understanding the clinical characteristics and medical treatment of individuals affected by genetic epilepsies is instrumental in guiding selection for genetic testing, defining the phenotype range of these rare disorders, optimizing patient care pathways and pinpointing unaddressed medical need by quantifying healthcare resource utilization. To date, a matched longitudinal cohort study encompassing the entire spectrum of clinical characteristics and medical treatment from childhood through adolescence has not been performed. We identified individuals with genetic and non-genetic epilepsies and onset at ages 0–5 years by linkage across the Cleveland Clinic Health System. We used natural language processing to extract medical terms and …

Personalization Of Sudep Risk: A Survey Of Transient Subclinical Comorbid Changes, Kristina A Simeone, Dawn M Martenz, Shruthi H Iyer, Cameron P Booth, Shelby E Herr, Stephanie A Matthews, Samantha B Draves, Laura L Heinemann, Pierce L Greenberg, Samden D Lhatoo, Elizabeth Donner, Timothy A Simeone

Faculty, Staff and Student Publications

Objective: Preclinical data report within subject modifiable ailments emerge weeks prior to SUDEP, including sleep disorders and cardiorespiratory changes; findings which support anecdotal clinical data. Here, we bridge preclinical findings with future clinical/preclinical studies, and survey whether caretakers or family members of victims noticed transient changes prior to SUDEP. The aim of this pilot study is to identify potential modifiable changes that may synergistically increase SUDEP risk for future research.

Methods: A mobile electronic survey was posted on SUDEP community websites. The survey queried whether changes in seizures, sleep, physical well-being, emotional well-being, cognition, breathing, or heart rate were noticed …

Epilepsy Networks And Their Surgical Relevance, Kevin Hines, Chengyuan Wu

Department of Neurosurgery Faculty Papers

Surgical epilepsy is a rapidly evolved field. As the understanding and concepts of epilepsy shift towards a network disorder, surgical outcomes may shed light on numerous components of these systems. This review documents the evolution of the understanding of epilepsy networks and examines the data generated by resective, ablative, neuromodulation, and invasive monitoring surgeries in epilepsy patients. As these network tools are better integrated into epilepsy practice, they may eventually inform surgical decisions and improve clinical outcomes.

Sleep Duration, Sleep Quality, And Weapon Carrying In A Sample Of Adolescents From Texas, Arthur Stefanski, Eduardo Pérez-Palma, Tobias Brünger, Ludovica Montanucci, Cornelius Gati, Chiara Klöckner, Katrine M Johannesen, Kimberly Goodspeed, Marie Macnee, Alexander T Deng, Ángel Aledo-Serrano, Artem Borovikov, Maina Kava, Arjan M Bouman, M J Hajianpour, Deb K Pal, Marc Engelen, Eveline E O Hagebeuk, Marwan Shinawi, Alexis R Heidlebaugh, Kathryn Oetjens, Trevor L Hoffman, Pasquale Striano, Amanda S Freed, Line Futtrup, Thomas Balslev, Anna Abulí, Leslie Danvoye, Damien Lederer, Tugce Balci, Maryam Nabavi Nouri, Elizabeth Butler, Sarah Drewes, Kalene Van Engelen, Katherine B Howell, Jean Khoury, Patrick May, Marena Trinidad, Steven Froelich, Johannes R Lemke, Jacob Tiller, Amber N Freed, Jing-Qiong Kang, Arthur Wuster, Rikke S Møller, Dennis Lal

Faculty, Staff and Student Publications

Genetic variants in the SLC6A1 gene can cause a broad phenotypic disease spectrum by altering the protein function. Thus, systematically curated clinically relevant genotype-phenotype associations are needed to understand the disease mechanism and improve therapeutic decision-making.

We aggregated genetic and clinical data from 172 individuals with likely pathogenic/pathogenic (lp/p) SLC6A1 variants and functional data for 184 variants (14.1% lp/p). Clinical and functional data were available for a subset of 126 individuals. We explored the potential associations of variant positions on the GAT1 3D structure with variant pathogenicity, altered molecular function and phenotype severity using bioinformatic approaches.

The GAT1 transmembrane domains …

Altered Amygdala Volumes And Microstructure In Focal Epilepsy Patients With Tonic-Clonic Seizures, Ictal, And Post-Convulsive Central Apnea, Claudia Zeicu, Antoine Legouhy, Catherine A Scott, Joana F A Oliveira, Gavin P Winston, John S Duncan, Sjoerd B Vos, Maria Thom, Samden Lhatoo, Hui Zhang, Ronald M Harper, Beate Diehl

Faculty, Staff and Student Publications

Objectives

Sudden unexpected death in epilepsy (SUDEP) is a leading cause of death for patients with epilepsy; however, the pathophysiology remains unclear. Focal‐to‐bilateral tonic–clonic seizures (FBTCS) are a major risk factor, and centrally‐mediated respiratory depression may increase the risk further. Here, we determined the volume and microstructure of the amygdala, a key structure that can trigger apnea in people with focal epilepsy, stratified by the presence or absence of FBTCS, ictal central apnea (ICA), and post‐convulsive central apnea (PCCA).

Methods

Seventy‐three patients with focal impaired awareness seizures without FBTC seizures (FBTCneg group) and 30 with FBTCS (FBTCpos group) recorded during …

Deep Histopathology Genotype-Phenotype Analysis Of Focal Cortical Dysplasia Type Ii Differentiates Between The Gator1-Altered Autophagocytic Subtype Iia And Mtor-Altered Migration Deficient Subtype Iib, Jonas Honke, Lucas Hoffmann, Roland Coras, Katja Kobow, Costin Leu, Tom Pieper, Till Hartlieb, Christian G Bien, Friedrich Woermann, Thomas Cloppenborg, Thilo Kalbhenn, Ahmed Gaballa, Hajo Hamer, Sebastian Brandner, Karl Rössler, Arnd Dörfler, Stefan Rampp, Johannes R Lemke, Sara Baldassari, Stéphanie Baulac, Dennis Lal, Peter Nürnberg, Ingmar Blümcke

Faculty, Staff and Student Publications

Focal cortical dysplasia type II (FCDII) is the most common cause of drug-resistant focal epilepsy in children. Herein, we performed a deep histopathology-based genotype–phenotype analysis to further elucidate the clinico-pathological and genetic presentation of FCDIIa compared to FCDIIb. Seventeen individuals with histopathologically confirmed diagnosis of FCD ILAE Type II and a pathogenic variant detected in brain derived DNA whole-exome sequencing or mTOR gene panel sequencing were included in this study. Clinical data were directly available from each contributing centre. Histopathological analyses were performed from formalin-fixed, paraffin-embedded tissue samples using haematoxylin–eosin and immunohistochemistry for NF-SMI32, NeuN, pS6, p62, and vimentin. Ten …

A Case Of Osteogenesis Imperfecta In A Patient Diagnosed In Adulthood, Khalid Sheikh, M. Lastovica, N. S. Appareddy, R. Wagner, E. Luna

Research Symposium

Background: Osteogenesis imperfecta (OI) is an extremely rare inherited connective tissue disorder, with an incidence of 1 in 20,000 births in the United States. Our aim is to assist other providers in diagnosing and managing patients with OI and concurrent medical morbidities.

Case Presentation: A 21 year old male with a BMI of 34 and a lifetime history of over 10 fractures presented to the Emergency department after a complex seizure at home. The seizure was witnessed by his mother and lasted less than one minute. He had another seizure two weeks prior to presentation. In the ED, the patient …

Interictal Respiratory Variability Predicts Severity Of Hypoxemia After Generalized Convulsive Seizures, Rup K Sainju, Deidre N Dragon, Harold B Winnike, Laura Vilella, Xiaojin Li, Samden Lhatoo, Patrick Ten Eyck, Linder H Wendt, George B Richerson, Brian K Gehlbach

Faculty, Staff and Student Publications

Objective: Severe respiratory dysfunction induced by generalized convulsive seizures (GCS) is now thought to be a common mechanism for sudden unexpected death in epilepsy (SUDEP). In a mouse model of seizure-induced death, increased interictal respiratory variability was reported in mice that later died of respiratory arrest after GCS. We studied respiratory variability in epilepsy patients as a predictive tool for severity of postictal hypoxemia, a potential biomarker for SUDEP risk. We then explored the relationship between respiratory variability and central CO2 drive, measured by the hypercapnic ventilatory response (HCVR).

Methods: We reviewed clinical, video-electroencephalography, and respiratory (belts, airflow, pulse oximeter, …

Monoallelic Variation In Dhx9, The Gene Encoding The Dexh-Box Helicase Dhx9, Underlies Neurodevelopment Disorders And Charcot-Marie-Tooth Disease, Daniel G Calame, Tianyu Guo, Chen Wang, Lillian Garrett, Angad Jolly, Moez Dawood, Alina Kurolap, Noa Zunz Henig, Jawid M Fatih, Isabella Herman, Haowei Du, Tadahiro Mitani, Lore Becker, Birgit Rathkolb, Raffaele Gerlini, Claudia Seisenberger, Susan Marschall, Jill V Hunter, Amanda Gerard, Alexis Heidlebaugh, Thomas Challman, Rebecca C Spillmann, Shalini N Jhangiani, Zeynep Coban-Akdemir, Seema Lalani, Lingxiao Liu, Anya Revah-Politi, Alejandro Iglesias, Edwin Guzman, Evan Baugh, Nathalie Boddaert, Sophie Rondeau, Clothide Ormieres, Giulia Barcia, Queenie K G Tan, Isabelle Thiffault, Tomi Pastinen, Kazim Sheikh, Suur Biliciler, Davide Mei, Federico Melani, Vandana Shashi, Yuval Yaron, Mary Steele, Emma Wakeling, Elsebet Østergaard, Lusine Nazaryan-Petersen, Undiagnosed Diseases Network, Francisca Millan, Teresa Santiago-Sim, Julien Thevenon, Ange-Line Bruel, Christel Thauvin-Robinet, Denny Popp, Konrad Platzer, Pawel Gawlinski, Wojciech Wiszniewski, Dana Marafi, Davut Pehlivan, Jennifer E Posey, Richard A Gibbs, Valerie Gailus-Durner, Renzo Guerrini, Helmut Fuchs, Martin Hrabě De Angelis, Sabine M Hölter, Hoi-Hung Cheung, Shen Gu, James R Lupski

Faculty, Staff and Student Publications

DExD/H-box RNA helicases (DDX/DHX) are encoded by a large paralogous gene family; in a subset of these human helicase genes, pathogenic variation causes neurodevelopmental disorder (NDD) traits and cancer. DHX9 encodes a BRCA1-interacting nuclear helicase regulating transcription, R-loops, and homologous recombination and exhibits the highest mutational constraint of all DDX/DHX paralogs but remains unassociated with disease traits in OMIM. Using exome sequencing and family-based rare-variant analyses, we identified 20 individuals with de novo, ultra-rare, heterozygous missense or loss-of-function (LoF) DHX9 variant alleles. Phenotypes ranged from NDDs to the distal symmetric polyneuropathy axonal Charcot-Marie-Tooth disease (CMT2). Quantitative Human Phenotype Ontology (HPO) …

Use Of Video Alone For Differentiation Of Epileptic Seizures From Non-Epileptic Spells: A Systematic Review And Meta-Analysis, Cemal Karakas, Liam D Ferreira, Zulfi Haneef

Faculty, Staff and Students Publications

Introduction: Review of videos (without electroencephalography) to differentiate epileptic seizures (ES) from non-epileptic spells (NES) may be helpful where epilepsy monitoring is not feasible. Previous studies of video-based diagnosis have suffered from variable accuracy, sensitivity, and specificity.

Methods: We systematically reviewed relevant literature in PubMed, Embase, and Web of Science from inception to September 2022, identifying articles that reported on the video-based diagnosis of ES and NES. In primary analysis, for each study, the most expert group was chosen when different groups of reviewers classified the videos (e.g., epilepsy specialists and general neurologists). In secondary analysis, we compared the diagnostic …

Genome-Wide Identification And Phenotypic Characterization Of Seizure-Associated Copy Number Variations In 741,075 Individuals, Ludovica Montanucci, David Lewis-Smith, Ryan L Collins, Lisa-Marie Niestroj, Shridhar Parthasarathy, Julie Xian, Shiva Ganesan, Marie Macnee, Tobias Brünger, Rhys H Thomas, Michael Talkowski, Epi25 Collaborative, Ingo Helbig, Costin Leu, Dennis Lal

Faculty, Staff and Student Publications

Copy number variants (CNV) are established risk factors for neurodevelopmental disorders with seizures or epilepsy. With the hypothesis that seizure disorders share genetic risk factors, we pooled CNV data from 10,590 individuals with seizure disorders, 16,109 individuals with clinically validated epilepsy, and 492,324 population controls and identified 25 genome-wide significant loci, 22 of which are novel for seizure disorders, such as deletions at 1p36.33, 1q44, 2p21-p16.3, 3q29, 8p23.3-p23.2, 9p24.3, 10q26.3, 15q11.2, 15q12-q13.1, 16p12.2, 17q21.31, duplications at 2q13, 9q34.3, 16p13.3, 17q12, 19p13.3, 20q13.33, and reciprocal CNVs at 16p11.2, and 22q11.21. Using genetic data from additional 248,751 individuals with 23 neuropsychiatric phenotypes, …

Pulvinar Neuromodulation For Seizure Monitoring And Network Modulation In Temporal Plus Epilepsy, Yash Shashank Vakilna, Ganne Chaitanya, Muhammad Ubaid Hafeez, Adeel Ilyas, Manojkumar Saranathan, Jay Gavvala, Nitin Tandon, Sandipan Pati

Faculty, Staff and Student Publications

Deep brain stimulation (DBS) is a promising treatment for drug-refractory epilepsies (DRE) when targeting the anterior nuclei of thalamus (ANT). However, targeting other thalamic nuclei, such as the pulvinar, shows therapeutic promise. Our pioneering case study presents the application of ambulatory seizure monitoring using spectral fingerprinting (12.15-17.15 Hz) recorded through Medtronic Percept DBS implanted bilaterally in the medial pulvinar thalami. This technology offers unprecedented opportunities for real-time monitoring of seizure burden and thalamocortical network modulation for effective seizure reduction in patients with bilateral mesial temporal and temporal plus epilepsies that are not suitable for resection.

Actigraphic Correlates Of Neuropsychiatric Symptoms In Adults With Focal Epilepsy, Mark A Abboud, Jessica L Kamen, John S Bass, Lu Lin, Jay R Gavvala, Sindhu Rao, Stephen F Smagula, Vaishnav Krishnan

Faculty, Staff and Student Publications

Objectives:

Disability in patients with epilepsy (PWEs) is multifactorial: beyond seizure frequency/severity, PWEs are prone to a range of neuropsychiatric, cognitive, and somatic comorbidities that significantly affect quality of life. Here, we explored how variations in seizure severity and the burden of self-reported somatic/neuropsychiatric symptoms correlate with disruptions to 24 h activity patterns (rest-activity rhythms [RARs]), determined through wrist accelerometry/actigraphy.

Methods:

Multiday wrist-actigraphy recordings were obtained from 59 adult patients with focal epilepsy (44% male, ages 18–72), who contemporaneously responded to validated psychometric instruments to measure anxiety, depression, sleepiness, and somatic symptoms. We conducted a similar in silico psychometric-actigraphic correlation …

The Ethical And Validity Conundrum In Epilepsy Research In Lmic Settings, Pauline Samia, Adeel Shah, Archana Patel, Philip Olielo, Lionel Mudave, Samson Gwer

Brain and Mind Institute

In the last few decades, research in epilepsy has significantly improved understanding of risk factors and etiologies associated with epilepsy, promoting greater access to interventions and medications that have improved health-related outcomes for patients. However, these advances and benefits are not being felt evenly on a global scale due to significant inequalities in access to and utilization of research resources and expertise in Low-and Middle-Income Countries (LMICs).

To promote effective research output, and advance evidence-based practices; the context, disease burden, and challenges that hinder good research need to be re-defined and addressed. This is key in facilitating implementation of coherent …

Use Of Integrative, Complementary, And Alternative Medicine In Children With Epilepsy: A Global Scoping Review, Zahra Zhu, Daniela Dluzynski, Nouran Hammad, Deepika Pugalenthi, Sarah A. Walser, Rea Mittal, Debopam Samanta, Melanie L. Brown, Ali A. Asadi-Pooya, Angelina Kakooza-Mwesige, Alberto Spalice, Martina Capponi, Alain Lekoubou, Ashutosh Kumar, Sita Paudel, Paul R. Carney, Gayatra Mainali, Sunil Naik

Department of Neurology Faculty Papers

(1) Background: Epilepsy is one of the most common chronic neurological disorders in childhood. Complementary and alternative medicine (CAM) use is highly prevalent in patients with epilepsy. Despite CAM’s widespread and increasing popularity, its prevalence, forms, perceived benefits, and potential risks in pediatric epilepsy are rarely explored. (2) Methods: We performed a scoping review of the available literature on the use of CAM in pediatric epilepsy. (3) Results: Overall, global cross-sectional studies showed a variable degree of CAM usage among children with epilepsy, ranging from 13 to 44% in prevalence. Popular types of CAMs reported were supplements, cannabis products, aromatherapy, …

Hyperphosphorylated Tau In Mesial Temporal Lobe Epilepsy: A Neuropathological And Cognitive Study, Eliana C B Toscano, Érica L M Vieira, Lea T Grinberg, Natalia P Rocha, Joseane A S Brant, Regina S Paradela, Alexandre V Giannetti, Claudia K Suemoto, Renata E P Leite, Ricardo Nitrini, Milene A Rachid, Antonio L Teixeira

Faculty, Staff and Student Publications

Temporal lobe epilepsy (TLE) often courses with cognitive deficits, but its underlying neuronal basis remains unclear. Confluent data suggest that epilepsy share pathophysiological mechanisms with neurodegenerative diseases. However, as most studies analyze subjects 60 years old and older, it is challenging to rule out that neurodegenerative changes arise from age-related mechanisms rather than epilepsy in these individuals. To fill this gap, we conducted a neuropathological investigation of the hippocampal formation of 22 adults with mesial TLE and 20 age- and sex-matched controls (both younger than 60 years). Moreover, we interrogated the relationship between these neuropathological metrics and cognitive performance. Hippocampal …

Pre-Surgical Features Of Intrinsic Brain Networks Predict Single And Joint Epilepsy Surgery Outcomes, Walter Hinds, Shilpi Modi, Ankeeta Ankeeta, Michael R Sperling, Dorian Pustina, Joseph I Tracy

Department of Neurology Faculty Papers

Despite the effectiveness of surgical interventions for the treatment of intractable focal temporal lobe epilepsy (TLE), the substrates that support good outcomes are poorly understood. While algorithms have been developed for the prediction of either seizure or cognitive/psychiatric outcomes alone, no study has reported on the functional and structural architecture that supports joint outcomes. We measured key aspects of pre-surgical whole brain functional/structural network architecture and evaluated their ability to predict post-operative seizure control in combination with cognitive/psychiatric outcomes. Pre-surgically, we identified the intrinsic connectivity networks (ICNs) unique to each person through independent component analysis (ICA), and computed: (1) the …

Poor Seizure Control Among Children Attending A Tertiary Hospital In South Western Uganda – A Retrospective Study, Martha Sajatovic

Faculty Scholarship

Background: Seizure control among children with epilepsy (CWE) receiving anti-seizure medications (ASMs) remains a challenge in low-resource settings. Uncontrolled seizures are significantly associated with increased morbidity and mortality among CWE. This negatively impacts their quality of life and increases stigma. Aim: This study determined seizure control status and described the factors associated among CWE receiving ASMs at Mbarara Regional Referral Hospital (MRRH). Methods: In a retrospective chart review study, socio-demographic and clinical data were obtained from 112 medical records. CWE receiving ASMs for at least six months and regularly attending the clinic were included in the study. Physical or telephone …

Bayesian Non-Homogeneous Hidden Markov Model With Variable Selection For Investigating Drivers Of Seizure Risk Cycling, Emily T Wang, Sharon Chiang, Zulfi Haneef, Vikram R Rao, Robert Moss, Marina Vannucci

Faculty, Staff and Students Publications

A major issue in the clinical management of epilepsy is the unpredictability of seizures. Yet, traditional approaches to seizure forecasting and risk assessment in epilepsy rely heavily on raw seizure frequencies, which are a stochastic measurement of seizure risk. We consider a Bayesian non-homogeneous hidden Markov model for unsupervised clustering of zero-inflated seizure count data. The proposed model allows for a probabilistic estimate of the sequence of seizure risk states at the individual level. It also offers significant improvement over prior approaches by incorporating a variable selection prior for the identification of clinical covariates that drive seizure risk changes and …

Association Between Characteristics Of National Association Of Epilepsy Centers And Reported Utilization Of Specific Surgical Techniques, Kristen H Arredondo, Stephanie M Ahrens, Anto I Bagić, Shasha Bai, Kevin E Chapman, Michael A Ciliberto, Dave F Clarke, Mariah Eisner, Nathan B Fountain, Jay R Gavvala, M Scott Perry, Kyle C Rossi, Lily C Wong-Kisiel, Susan T Herman, Adam P Ostendorf, Naec Center Director Study Group

Faculty, Staff and Student Publications

Background and Objective

Nearly one-third of persons with epilepsy will continue having seizures despite trialing multiple antiseizure medications. Epilepsy surgery may be beneficial in these cases, and evaluation at a comprehensive epilepsy center is recommended. Numerous palliative and potentially curative approaches exist, and types of surgery performed may be influenced by center characteristics. This article describes epilepsy center characteristics associated with epilepsy surgery access and volumes in the United States.

Methods

We analyzed National Association of Epilepsy Centers 2019 annual report and supplemental survey data obtained with responses from 206 adult epilepsy center directors and 136 pediatric epilepsy center directors …

Hippocampal Δfosb Expression Is Associated With Cognitive Impairment In A Subgroup Of Patients With Childhood Epilepsies, Chia-Hsuan Fu, Jason C You, Carrie Mohila, Robert A Rissman, Daniel Yoshor, Angela N Viaene, Jeannie Chin

Faculty, Staff and Students Publications

Epilepsy is a chronic neurological disorder characterized by recurrent seizures, and is often comorbid with other neurological and neurodegenerative diseases, such as Alzheimer's disease (AD). Patients with recurrent seizures often present with cognitive impairment. However, it is unclear how seizures, even when infrequent, produce long-lasting deficits in cognition. One mechanism may be seizure-induced expression of ΔFosB, a long-lived transcription factor that persistently regulates expression of plasticity-related genes and drives cognitive dysfunction. We previously found that, compared with cognitively-intact subjects, the activity-dependent expression of ΔFosB in the hippocampal dentate gyrus (DG) was increased in individuals with mild cognitive impairment (MCI) and …

Behavioral Improvements Following Lesion Resection For Pediatric Epilepsy: Pediatric Psychosurgery?, Huy Dang, Abdul Basit Khan, Nisha Gadgil, Himanshu Sharma, Cristina Trandafir, Fatema Malbari, Howard L Weiner

Faculty, Staff and Students Publications

Introduction: Resection of brain lesions associated with refractory epilepsy to achieve seizure control is well accepted. However, concurrent behavioral effects of these lesions such as changes in mood, personality, and cognition and the effects of surgery on behavior have not been well characterized. We describe 5 such children with epileptogenic lesions and significant behavioral abnormalities which improved after surgery.

Case descriptions: Five children (ages 3-14 years) with major behavioral abnormalities and lesional epilepsy were identified and treated at our center. Behavioral problems included academic impairment, impulsivity, self-injurious behavior, and decreased social interaction with diagnoses of ADHD, oppositional defiant disorder, and …

Epilepsy Center Characteristics And Geographic Region Influence Presurgical Testing In The United States, Stephanie M Ahrens, Kristen H Arredondo, Anto I Bagić, Shasha Bai, Kevin E Chapman, Michael A Ciliberto, Dave F Clarke, Mariah Eisner, Nathan B Fountain, Jay R Gavvala, M Scott Perry, Kyle C Rossi, Lily C Wong-Kisiel, Susan T Herman, Adam P Ostendorf, Naec Center Director Study Group

Faculty, Staff and Student Publications

bjective

Persons with drug‐resistant epilepsy may benefit from epilepsy surgery and should undergo presurgical testing to determine potential candidacy and appropriate intervention. Institutional expertise can influence use and availability of evaluations and epilepsy surgery candidacy. This census survey study aims to examine the influence of geographic region and other center characteristics on presurgical testing for medically intractable epilepsy.

Methods

We analyzed annual report and supplemental survey data reported in 2020 from 206 adult epilepsy center directors and 136 pediatric epilepsy center directors in the United States. Test utilization data were compiled with annual center volumes, available resources, and US Census …

Stereo-Eeg-Guided Network Modulation For Psychiatric Disorders: Surgical Considerations, Sameer A Sheth, Ben Shofty, Anusha Allawala, Jiayang Xiao, Joshua A Adkinson, Raissa K Mathura, Victoria Pirtle, John Myers, Denise Oswalt, Nicole R Provenza, Nisha Giridharan, Angela M Noecker, Garrett P Banks, Ron Gadot, Ricardo A Najera, Adrish Anand, Ethan Devara, Huy Dang, Eleonora Bartoli, Andrew Watrous, Jeffrey Cohn, David Borton, Sanjay J Mathew, Cameron C Mcintyre, Wayne Goodman, Kelly Bijanki, Nader Pouratian

Faculty, Staff and Students Publications

BACKGROUND: Deep brain stimulation (DBS) and other neuromodulatory techniques are being increasingly utilized to treat refractory neurologic and psychiatric disorders.

OBJECTIVE: /Hypothesis: To better understand the circuit-level pathophysiology of treatment-resistant depression (TRD) and treat the network-level dysfunction inherent to this challenging disorder, we adopted an approach of inpatient intracranial monitoring borrowed from the epilepsy surgery field.

METHODS: We implanted 3 patients with 4 DBS leads (bilateral pair in both the ventral capsule/ventral striatum and subcallosal cingulate) and 10 stereo-electroencephalography (sEEG) electrodes targeting depression-relevant network regions. For surgical planning, we used an interactive, holographic visualization platform to appreciate the 3D anatomy …