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Department of Neurology Faculty Papers

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Full-Text Articles in Medical Specialties

Optimized Acute Treatment Of Migraine Is Associated With Greater Productivity In People With Migraine: Results From The Chronic Migraine Epidemiology And Outcomes (Cameo) Study., Dawn C Buse, Stephanie J. Nahas, Walter Buzz F Stewart, Cynthia E Armand, Michael L Reed, Kristina M Fanning, Aubrey Manack Adams, Richard B Lipton Apr 2023

Optimized Acute Treatment Of Migraine Is Associated With Greater Productivity In People With Migraine: Results From The Chronic Migraine Epidemiology And Outcomes (Cameo) Study., Dawn C Buse, Stephanie J. Nahas, Walter Buzz F Stewart, Cynthia E Armand, Michael L Reed, Kristina M Fanning, Aubrey Manack Adams, Richard B Lipton

Department of Neurology Faculty Papers

OBJECTIVE: This study aimed to ascertain whether level of optimization of acute treatment of migraine is related to work productivity across the spectrum of migraine.

METHODS: Data were from the Chronic Migraine Epidemiology and Outcomes (CaMEO) Study, an internet-based longitudinal survey. Respondents with migraine who reported full-time employment and use of ≥1 acute prescription medication for migraine were included. We determined relationships among lost productive time (LPT; measured with the Migraine Disability Assessment Scale), acute treatment optimization (Migraine Treatment Optimization Questionnaire- ), and monthly headache days (MHDs).

RESULTS: There was a direct relationship between LPT and MHD category. Greater acute …


A Learned Map For Places And Concepts In The Human Medial Temporal Lobe, Nora A. Herweg, Lukas Kunz, Daniel Schonhaut, Armin Brandt, Paul A. Wanda, Ashwini D. Sharan, Michael R. Sperling, Andreas Schulze-Bonhage, Michael J. Kahana Mar 2023

A Learned Map For Places And Concepts In The Human Medial Temporal Lobe, Nora A. Herweg, Lukas Kunz, Daniel Schonhaut, Armin Brandt, Paul A. Wanda, Ashwini D. Sharan, Michael R. Sperling, Andreas Schulze-Bonhage, Michael J. Kahana

Department of Neurology Faculty Papers

Distinct lines of research in both humans and animals point to a specific role of the hippocampus in both spatial and episodic memory function. The discovery of concept cells in the hippocampus and surrounding medial temporal lobe (MTL) regions suggests that the MTL maps physical and semantic spaces with a similar neural architecture. Here, we studied the emergence of such maps using MTL microwire recordings from 20 patients (9 female, 11 male) navigating a virtual environment featuring salient landmarks with established semantic meaning. We present several key findings. The array of local field potentials in the MTL contains sufficient information …


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 Mar 2023

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 …


B Cells And T Cells Abnormalities In Patients With Selective Iga Deficiency, Yasser Bagheri, Tannaz Moeini Shad, Shideh Namazi, Farzaneh Tofighi Zavareh, Gholamreza Azizi, Fereshteh Salami, Somayeh Sadani, Ali Hosseini, Mohsen Saeidi, Salar Pashangzadeh, Samaneh Delavari, Babak Mirminachi, Nima Rezaei, Hassan Abolhassani, Asghar Aghamohammadi, Reza Yazdani Mar 2023

B Cells And T Cells Abnormalities In Patients With Selective Iga Deficiency, Yasser Bagheri, Tannaz Moeini Shad, Shideh Namazi, Farzaneh Tofighi Zavareh, Gholamreza Azizi, Fereshteh Salami, Somayeh Sadani, Ali Hosseini, Mohsen Saeidi, Salar Pashangzadeh, Samaneh Delavari, Babak Mirminachi, Nima Rezaei, Hassan Abolhassani, Asghar Aghamohammadi, Reza Yazdani

Department of Neurology Faculty Papers

BACKGROUND: Selective IgA deficiency (SIgAD) is the most prevalent inborn errors of immunity with almost unknown etiology. This study aimed to investigate the clinical diagnostic and prognostic values of lymphocyte subsets and function in symptomatic SIgAD patients.

METHODS: A total of 30 available SIgAD patients from the Iranian registry and 30 age-sex-matched healthy controls were included in the present study. We analyzed B and T cell peripheral subsets and T cell proliferation assay by flow cytometry in SIgAD patients with mild and severe clinical phenotypes.

RESULTS: Our results indicated a significant increase in naïve and transitional B cells and a …


Sleep Duration, Hypnotic Drug Use, And Risk Factors: Cross- Sectional Study, Nazanin Jalali, Parvin Khalili, Zahra Jamali, Zahra Jalali, Amir Moghadam-Ahmadi, Alireza Vakilian, Fatemeh Ayoobi Mar 2023

Sleep Duration, Hypnotic Drug Use, And Risk Factors: Cross- Sectional Study, Nazanin Jalali, Parvin Khalili, Zahra Jamali, Zahra Jalali, Amir Moghadam-Ahmadi, Alireza Vakilian, Fatemeh Ayoobi

Department of Neurology Faculty Papers

Both short sleep duration (SSD) and long sleep duration (LSD) are associated with an increased risk of morbidity and mortality. Here, we aimed to assess the prevalence of sleep duration disturbances among adults in association with demographic, medication use, personal habits, and chronic diseases, while also considering the impact of hypnotic drug use. We performed a cross-sectional study of 9991 adult participants of the Rafsanjan Cohort Study (RCS), as part of the Prospective epidemiological research studies in Iran (PERSIAN). Multivariate logistic regression analyses were conducted to assess the association between short (< 6 h) and long (> 9 h) sleep duration with demographic and lifestyle …


Repurposing Ms Immunotherapies For Cidp And Other Autoimmune Neuropathies: Unfulfilled Promise Or Efficient Strategy?, Felix Kohle, Marinos Dalakas, Helmar C Lehmann Jan 2023

Repurposing Ms Immunotherapies For Cidp And Other Autoimmune Neuropathies: Unfulfilled Promise Or Efficient Strategy?, Felix Kohle, Marinos Dalakas, Helmar C Lehmann

Department of Neurology Faculty Papers

Despite advances in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and other common autoimmune neuropathies (AN), still-many patients with these diseases do not respond satisfactorily to the available treatments. Repurposing of disease-modifying therapies (DMTs) from other autoimmune conditions, particularly multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD), is a promising strategy that may accelerate the establishment of novel treatment choices for AN. This approach appears attractive due to homologies in the pathogenesis of these diseases and the extensive post-marketing experience that has been gathered from treating MS and NMOSD patients. The idea is also strengthened by a number …


Work Difficulties, Work Restrictions, And Disability Benefits In People With Functional Seizures: A Survey Study, Jared Woodward, A A Asadi-Pooya, Bridget Mildon, Benjamin Tolchin Jan 2023

Work Difficulties, Work Restrictions, And Disability Benefits In People With Functional Seizures: A Survey Study, Jared Woodward, A A Asadi-Pooya, Bridget Mildon, Benjamin Tolchin

Department of Neurology Faculty Papers

Purpose: Functional seizures (FS) cause significant long-term disability and clinicians offer differing views on proper work restrictions and qualifications for disability benefits in this population. We assess the views and perspectives of experienced disability and work limitations in people living with functional seizures. Methods: Between (4/29/2020–1/13/2021) an open-access 21-item internet survey was conducted via FNDHope.org; allowing for people living with self-reported functional seizures to remark on topics of work difficulties, work restrictions, qualifications for disability benefits, and driving. Demographic information was collected, and univariate and multivariate logistic regressions were used to evaluate potential predictors of current employment status. Results: One …


Association Of Human Leukocyte Antigen Alleles With Carbamazepine-Or Lamotrigine-Induced Stevens-Johnson Syndrome And Toxic Epidermal Necrolysis In An Iranian Population: A Case-Control Study, Ladan Dastgheib, Farima Rostami, Behrouz Gharesi-Fard, Ali Akbar Asadi-Pooya, Saba Namjoo, Foroozan Tahmasebi, Maryam Hadibarhaghtalab Jan 2023

Association Of Human Leukocyte Antigen Alleles With Carbamazepine-Or Lamotrigine-Induced Stevens-Johnson Syndrome And Toxic Epidermal Necrolysis In An Iranian Population: A Case-Control Study, Ladan Dastgheib, Farima Rostami, Behrouz Gharesi-Fard, Ali Akbar Asadi-Pooya, Saba Namjoo, Foroozan Tahmasebi, Maryam Hadibarhaghtalab

Department of Neurology Faculty Papers

Background: Genetic diversity in human leukocyte antigen (HLA) alleles across populations is a significant risk factor for drug-induced severe cutaneous adverse reactions (SCARs), e.g., carbamazepine (CBZ)- and lamotrigine (LTG)-induced StevensJohnson syndrome (SJS), and toxic epidermal necrolysis (TEN). The present study aimed to investigate the frequency of different HLA alleles in Iranian patients with CBZ- and LTG-induced SJS/TEN.

Methods:A case-control study was conducted from 2011 to 2018 at various hospitals affiliated with Shiraz University of Medical Sciences (Shiraz, Iran). A total of 31 patients receiving anticonvulsant drugs (CZB or LTG) were recruited and divided into two groups. The drug-induced group …


Safety And Tolerability Results Of Atogepant For The Preventive Treatment Of Episodic Migraine From A 40-Week, Open-Label Multicenter Extension Of The Phase 3 Advance Trial, Brad C Klein, Rosa Miceli, Lawrence Severt, Peter Mcallister, Laszlo Mechtler, Jennifer Mcvige, Merle Diamond, Michael J. Marmura, Hua Guo, Michelle Finnegan, Joel M Trugman Jan 2023

Safety And Tolerability Results Of Atogepant For The Preventive Treatment Of Episodic Migraine From A 40-Week, Open-Label Multicenter Extension Of The Phase 3 Advance Trial, Brad C Klein, Rosa Miceli, Lawrence Severt, Peter Mcallister, Laszlo Mechtler, Jennifer Mcvige, Merle Diamond, Michael J. Marmura, Hua Guo, Michelle Finnegan, Joel M Trugman

Department of Neurology Faculty Papers

Background: Atogepant is a United States Food and Drug Administration-approved oral calcitonin gene-related peptide receptor antagonist for the preventive treatment of episodic migraine. The study objective was to evaluate the long-term safety and tolerability of atogepant in participants who completed the phase 3 ADVANCE trial (NCT03777059).

Methods: This 40-week, open-label extension trial (NCT03939312) monitored safety in participants receiving oral atogepant 60 mg once daily, followed by a four-week safety follow-up period.

Results: Of the 685 participants taking at least one dose of atogepant, the treatment period was completed by 74.6% of participants with a mean (standard …


How To Manage The Initiation Of Apomorphine Therapy Without Antiemetic Pretreatment: A Review Of The Literature, Stuart H. Isaacson, Richard B. Dewey, Rajesh Pahwa, Daniel E. Kremens Dec 2022

How To Manage The Initiation Of Apomorphine Therapy Without Antiemetic Pretreatment: A Review Of The Literature, Stuart H. Isaacson, Richard B. Dewey, Rajesh Pahwa, Daniel E. Kremens

Department of Neurology Faculty Papers

Introduction

Pretreatment with the antiemetic trimethobenzamide has been recommended practice in the United States (US) to address the risk of nausea and vomiting during initiation of apomorphine treatment. However, trimethobenzamide is no longer being manufactured in the US, and despite the recent update to the US prescribing information, there may be uncertainty regarding how to initiate apomorphine.

Methods

To better understand why antiemetic pretreatment was recommended and if it is necessary when initiating apomorphine therapy, we performed a literature review of subcutaneous apomorphine therapy initiation with and without antiemetic pretreatment in patients with PD.

Results

Three studies were identified as …


Gata1 Controls Numbers Of Hematopoietic Progenitors And Their Response To Autoimmune Neuroinflammation, Daniel Hwang, Larissa Ishikawa, Maryam S. Seyedsadr, Elisabeth R. Mari, Ezgi Kasimoglu, Ziver Sahin, Alexandra Boehm, Soohwa Jang, Javad Rasouli, Courtney Vaccaro, Michael Gonzalez, Hakon Hakonarson, Mohamad Rostami, Guang-Xian Zhang, Bogoljub Ciric Dec 2022

Gata1 Controls Numbers Of Hematopoietic Progenitors And Their Response To Autoimmune Neuroinflammation, Daniel Hwang, Larissa Ishikawa, Maryam S. Seyedsadr, Elisabeth R. Mari, Ezgi Kasimoglu, Ziver Sahin, Alexandra Boehm, Soohwa Jang, Javad Rasouli, Courtney Vaccaro, Michael Gonzalez, Hakon Hakonarson, Mohamad Rostami, Guang-Xian Zhang, Bogoljub Ciric

Department of Neurology Faculty Papers

GATA-binding factor 1 (GATA1) is a transcription factor that governs the development and function of multiple hematopoietic cell lineages. GATA1 is expressed in hematopoietic stem and progenitor cells (HSPCs) and is essential for erythroid lineage commitment; however, whether it plays a role in hematopoietic stem cell (HSC) biology and the development of myeloid cells, and what that role might be, remains unclear. We initially set out to test the role of eosinophils in experimental autoimmune encephalomyelitis (EAE), a model of central nervous system autoimmunity, using mice lacking a double GATA-site (ΔdblGATA), which lacks eosinophils due to the deletion of the …


The Spectral Sensitivity Of Human Circadian Phase Resetting And Melatonin Suppression To Light Changes Dynamically With Light Duration, Melissa A. St Hilaire, María L. Ámundadóttir, Shadab A. Rahman, Shantha M. W. Rajaratnam, Melanie Rüger, George C. Brainard, Charles A. Czeisler, Marilyne Andersen, Joshua J. Gooley, Steven W. Lockley Dec 2022

The Spectral Sensitivity Of Human Circadian Phase Resetting And Melatonin Suppression To Light Changes Dynamically With Light Duration, Melissa A. St Hilaire, María L. Ámundadóttir, Shadab A. Rahman, Shantha M. W. Rajaratnam, Melanie Rüger, George C. Brainard, Charles A. Czeisler, Marilyne Andersen, Joshua J. Gooley, Steven W. Lockley

Department of Neurology Faculty Papers

Human circadian, neuroendocrine, and neurobehavioral responses to light are mediated primarily by melanopsin-containing intrinsically-photosensitive retinal ganglion cells (ipRGCs) but they also receive input from visual photoreceptors. Relative photoreceptor contributions are irradiance- and duration-dependent but results for long-duration light exposures are limited. We constructed irradiance-response curves and action spectra for melatonin suppression and circadian resetting responses in participants exposed to 6.5-h monochromatic 420, 460, 480, 507, 555, or 620 nm light exposures initiated near the onset of nocturnal melatonin secretion. Melatonin suppression and phase resetting action spectra were best fit by a single-opsin template with lambdamax at 481 and 483 …


Impact Of Solid State Roadway Lighting On Melatonin In Humans, Ronald B Gibbons, Rajaram Bhagavathula, Benjamin Warfield, George Brainard, John P Hanifin Nov 2022

Impact Of Solid State Roadway Lighting On Melatonin In Humans, Ronald B Gibbons, Rajaram Bhagavathula, Benjamin Warfield, George Brainard, John P Hanifin

Department of Neurology Faculty Papers

Introduction: In 2009, the World Health Organization identified vehicle crashes, both injury-related and fatal, as a public health hazard. Roadway lighting has long been used to reduce crashes and improve the safety of all road users. Ocular light exposure at night can suppress melatonin levels in humans. At sufficient light levels, all visible light wavelengths can elicit this response, but melatonin suppression is maximally sensitive to visible short wavelength light. With the conversion of roadway lighting to solid state sources that have a greater short wavelength spectrum than traditional sources, there is a potential negative health impact through suppressed melatonin …


Delta Oscillation Coupled Propagating Fast Ripples Precede Epileptiform Discharges In Patients With Focal Epilepsy, Shennan A. Weiss, Laurent Sheybani, Nitish Seenarine, Itzhak Fried, Chengyuan Wu, Ashwini Sharan, Jerome Engel Jr., Michael R. Sperling, Yuval Nir, Richard J. Staba Nov 2022

Delta Oscillation Coupled Propagating Fast Ripples Precede Epileptiform Discharges In Patients With Focal Epilepsy, Shennan A. Weiss, Laurent Sheybani, Nitish Seenarine, Itzhak Fried, Chengyuan Wu, Ashwini Sharan, Jerome Engel Jr., Michael R. Sperling, Yuval Nir, Richard J. Staba

Department of Neurology Faculty Papers

Epileptiform spikes are used to localize epileptogenic brain tissue. The mechanisms that spontaneously trigger epileptiform discharges are not yet elucidated. Pathological fast ripple (FR, 200–600 Hz) are biomarkers of epileptogenic brain, and we postulated that FR network interactions are involved in generating epileptiform spikes. Using macroelectrode stereo intracranial EEG (iEEG) recordings from a cohort of 46 patients we found that, in the seizure onset zone (SOZ), propagating FR were more often followed by an epileptiform spike, as compared with non-propagating FR (p < 0.05). Propagating FR had a distinct frequency and larger power (p < 1e-10) and were more strongly phase coupled to the peak of iEEG delta oscillation, which likely correspond with the DOWN states during non-REM sleep (p < 1e-8), than non-propagating FR. While FR propagation was rare, all FR occurred with the highest probability within +/− 400 msec of epileptiform spikes with superimposed high-frequency oscillations (p < 0.05). Thus, a sub-population of epileptiform spikes in the SOZ, are preceded by propagating FR that are coordinated by the DOWN state during non-REM sleep.


Headache Characteristics Among Patients With Epilepsy And The Association With Temporal Encephaloceles, Patricia Graese, Milad Yazdani, Zeke Campbell Nov 2022

Headache Characteristics Among Patients With Epilepsy And The Association With Temporal Encephaloceles, Patricia Graese, Milad Yazdani, Zeke Campbell

Department of Neurology Faculty Papers

PURPOSE: Our aim was to determine if headaches characteristic of possible Idiopathic Intracranial Hypertension (IIH) and in general were more prevalent in patients with versus without temporal encephaloceles (TEs) among patients with epilepsy.

METHODS: Electronic medical records were reviewed retrospectively. Among 474 patients with epilepsy, 103 patients (21.7%) had at least one TE diagnosed on initial MRI or on retrospective review by a board-certified neuroradiologist, while 371 patients had no TE present. The patients were grouped into one of four categories depending on their headache characteristics (IIH-like, peri-ictal, other, or no headaches). Analysis of the categories was performed using a …


Individualised Prediction Of Drug Resistance And Seizure Recurrence After Medication Withdrawal In People With Juvenile Myoclonic Epilepsy: A Systematic Review And Individual Participant Data Meta-Analysis, Remi Stevelink, Dania Al-Toma, Floor E. Jansen, Herm J. Lamberink, Ali A. Asadi-Pooya, Mohsen Farazdaghi, Gonçalo Cação, Sita Jayalakshmi, Anuja Patil, Çiğdem Özkara, Şenay Aydın, Joanna Gesche, Christoph P. Beier, Linda J. Stephen, Martin J. Brodie, Gopeekrishnan Unnithan, Ashalatha Radhakrishnan, Julia Höfler, Eugen Trinka, Roland Krause, Emanuele Cerulli Irelli, Carlo Di Bonaventura, Jerzy P. Szaflarski, Laura E. Hernández-Vanegas, Monica L. Moya-Alfaro, Yingying Zhang, Dong Zhou, Nicola Pietrafusa, Nicola Specchio, Giorgi Japaridze, Sándor Beniczky, Mubeen Janmohamed, Patrick Kwan, Marte Syvertsen, Kaja K. Selmer, Bernd J. Vorderwülbecke, Martin Holtkamp, Lakshminarayanapuram G. Viswanathan, Sanjib Sinha, Betül Baykan, Ebru Altindag, Felix Von Podewils, Juliane Schulz, Udaya Seneviratne, Alejandro Viloria-Alebesque, Ioannis Karakis, Wendyl J. D'Souza, Josemir W. Sander, Bobby P. C. Koeleman, Willem M. Otte, Kees P. J. Braun Nov 2022

Individualised Prediction Of Drug Resistance And Seizure Recurrence After Medication Withdrawal In People With Juvenile Myoclonic Epilepsy: A Systematic Review And Individual Participant Data Meta-Analysis, Remi Stevelink, Dania Al-Toma, Floor E. Jansen, Herm J. Lamberink, Ali A. Asadi-Pooya, Mohsen Farazdaghi, Gonçalo Cação, Sita Jayalakshmi, Anuja Patil, Çiğdem Özkara, Şenay Aydın, Joanna Gesche, Christoph P. Beier, Linda J. Stephen, Martin J. Brodie, Gopeekrishnan Unnithan, Ashalatha Radhakrishnan, Julia Höfler, Eugen Trinka, Roland Krause, Emanuele Cerulli Irelli, Carlo Di Bonaventura, Jerzy P. Szaflarski, Laura E. Hernández-Vanegas, Monica L. Moya-Alfaro, Yingying Zhang, Dong Zhou, Nicola Pietrafusa, Nicola Specchio, Giorgi Japaridze, Sándor Beniczky, Mubeen Janmohamed, Patrick Kwan, Marte Syvertsen, Kaja K. Selmer, Bernd J. Vorderwülbecke, Martin Holtkamp, Lakshminarayanapuram G. Viswanathan, Sanjib Sinha, Betül Baykan, Ebru Altindag, Felix Von Podewils, Juliane Schulz, Udaya Seneviratne, Alejandro Viloria-Alebesque, Ioannis Karakis, Wendyl J. D'Souza, Josemir W. Sander, Bobby P. C. Koeleman, Willem M. Otte, Kees P. J. Braun

Department of Neurology Faculty Papers

BACKGROUND: A third of people with juvenile myoclonic epilepsy (JME) are drug-resistant. Three-quarters have a seizure relapse when attempting to withdraw anti-seizure medication (ASM) after achieving seizure-freedom. It is currently impossible to predict who is likely to become drug-resistant and safely withdraw treatment. We aimed to identify predictors of drug resistance and seizure recurrence to allow for individualised prediction of treatment outcomes in people with JME.

METHODS: We performed an individual participant data (IPD) meta-analysis based on a systematic search in EMBASE and PubMed - last updated on March 11, 2021 - including prospective and retrospective observational studies reporting on …


Uncovering The Biological Basis Of Control Energy: Structural And Metabolic Correlates Of Energy Inefficiency In Temporal Lobe Epilepsy, Xiaosong He, Lorenzo Caciagli, Linden Parkes, Jennifer Stiso, Teresa M. Karrer, Jason Z. Kim, Zhixin Lu, Tommaso Menara, Fabio Pasqualetti, Michael R. Sperling, Joseph I. Tracy, Dani S. Bassett Nov 2022

Uncovering The Biological Basis Of Control Energy: Structural And Metabolic Correlates Of Energy Inefficiency In Temporal Lobe Epilepsy, Xiaosong He, Lorenzo Caciagli, Linden Parkes, Jennifer Stiso, Teresa M. Karrer, Jason Z. Kim, Zhixin Lu, Tommaso Menara, Fabio Pasqualetti, Michael R. Sperling, Joseph I. Tracy, Dani S. Bassett

Department of Neurology Faculty Papers

Network control theory is increasingly used to profile the brain's energy landscape via simulations of neural dynamics. This approach estimates the control energy required to simulate the activation of brain circuits based on structural connectome measured using diffusion magnetic resonance imaging, thereby quantifying those circuits' energetic efficiency. The biological basis of control energy, however, remains unknown, hampering its further application. To fill this gap, investigating temporal lobe epilepsy as a lesion model, we show that patients require higher control energy to activate the limbic network than healthy volunteers, especially ipsilateral to the seizure focus. The energetic imbalance between ipsilateral and …


Future Opportunities For Research In Rescue Treatments, James W. Wheless, Daniel Friedman, Gregory L. Krauss, Vikram R. Rao, Michael R Sperling, Enrique Carrazana, Adrian L. Rabinowicz Sep 2022

Future Opportunities For Research In Rescue Treatments, James W. Wheless, Daniel Friedman, Gregory L. Krauss, Vikram R. Rao, Michael R Sperling, Enrique Carrazana, Adrian L. Rabinowicz

Department of Neurology Faculty Papers

Clinical studies of rescue medications for seizure clusters are limited and are designed to satisfy regulatory requirements, which may not fully consider the needs of the diverse patient population that experiences seizure clusters or utilize rescue medication. The purpose of this narrative review is to examine the factors that contribute to, or may influence the quality of, seizure cluster research with a goal of improving clinical practice. We address five areas of unmet needs and provide advice for how they could enhance future trials of seizure cluster treatments. The topics addressed in this article are: (1) unaddressed end points to …


Long-Term Effectiveness Of Ivig Maintenance Therapy In 36 Patients With Gad Antibody-Positive Stiff-Person Syndrome., Jessica Yi, Marinos Dalakas Sep 2022

Long-Term Effectiveness Of Ivig Maintenance Therapy In 36 Patients With Gad Antibody-Positive Stiff-Person Syndrome., Jessica Yi, Marinos Dalakas

Department of Neurology Faculty Papers

BACKGROUND AND OBJECTIVES: IVIg has been the preferred immunotherapy in stiff-person syndrome (SPS) based on a 3-month controlled trial, but whether it is also effective in inducing long-term benefits or arresting disease progression is unknown. The information is needed because SPS is a progressively disabling disease and IVIg is liberally used as chronic therapy without efficacy data. The present study explores the long-term effects of IVIg in the largest cohort of well-characterized patients with SPS followed by the same clinicians over 10 years.

METHODS: Data of 36 patients (32 glutamic acid decarboxylase [GAD] positive), diagnosed and treated with monthly maintenance …


Effect Of The Covid-19 Pandemic On Seizure Control Status In Patients With Epilepsy, A A Asadi-Pooya, Seyed Ali Nabavizadeh, Mohsen Farazdaghi Aug 2022

Effect Of The Covid-19 Pandemic On Seizure Control Status In Patients With Epilepsy, A A Asadi-Pooya, Seyed Ali Nabavizadeh, Mohsen Farazdaghi

Department of Neurology Faculty Papers

Background: Previous studies have shown that patients with epilepsy (PWE) perceived significant disruption in the quality and provision of care due to the coronavirus disease 2019 (COVID-19) pandemic. The present study aimed to investigate the effect of this pandemic on seizure control status and changes in seizure frequency in PWE.

Methods:A consecutive sample of adult PWE registered in the database of Shiraz Epilepsy Center (Shiraz, Iran) was included in the study. In July 2021, phone interviews were conducted with all selected patients. Information such as age, sex, last seizure, seizure type, and frequency during the 12 months before the …


Parenteral Nsaids For Acute Treatment Of Migraine: Adherence To The Ihs Guidelines For Controlled Trials, John G. Curran, John Waters, Hsiangkuo Yuan Jul 2022

Parenteral Nsaids For Acute Treatment Of Migraine: Adherence To The Ihs Guidelines For Controlled Trials, John G. Curran, John Waters, Hsiangkuo Yuan

Department of Neurology Faculty Papers

Background: Parenteral non-steroidal anti-inflammatory drugs (NSAIDs) are important alternatives to oral NSAIDs, especially in patients with severe migraine who have emesis or gastroparesis. With increasing research on using parenteral NSAIDs for acute migraine, it is critical to examine the quality of these studies. Our goal was to assess the adherence of these trials to the International Headache Society (IHS) controlled trial guidelines for acute treatment of migraine. Methods: We queried PubMed for clinical trials investigating parenteral NSAIDs for acute treatment of migraine in adult patients. We developed a 14-point scoring system based on the essential components of the IHS guidelines. …


Transcription Factor Runx3 Mediates Plasticity Of Thgm Cells Toward Th1 Phenotype, Javad Rasouli, Giacomo Casella, Weifeng Zhang, Dan Xiao, Gaurav Kumar, Paolo Fortina, Guang-Xian Zhang, Bogoljub Ciric, Abdolmohamad Rostami Jul 2022

Transcription Factor Runx3 Mediates Plasticity Of Thgm Cells Toward Th1 Phenotype, Javad Rasouli, Giacomo Casella, Weifeng Zhang, Dan Xiao, Gaurav Kumar, Paolo Fortina, Guang-Xian Zhang, Bogoljub Ciric, Abdolmohamad Rostami

Department of Neurology Faculty Papers

GM-CSF-producing T helper (Th) cells play a crucial role in the pathogenesis of autoimmune diseases such as multiple sclerosis (MS). Recent studies have identified a distinct population of GM-CSF-producing Th cells, named ThGM cells, that also express cytokines TNF, IL-2, and IL-3, but lack expression of master transcription factors (TF) and signature cytokines of commonly recognized Th cell lineages. ThGM cells are highly encephalitogenic in a mouse model of MS, experimental autoimmune encephalomyelitis (EAE). Similar to Th17 cells, in response to IL-12, ThGM cells upregulate expression of T-bet and IFN-γ and switch their phenotype to Th1. Here we show that …


B- And T-Cell Subset Abnormalities In Monogenic Common Variable Immunodeficiency, Saba Fekrvand, Shaghayegh Khanmohammadi, Hassan Abolhassani, Reza Yazdani Jun 2022

B- And T-Cell Subset Abnormalities In Monogenic Common Variable Immunodeficiency, Saba Fekrvand, Shaghayegh Khanmohammadi, Hassan Abolhassani, Reza Yazdani

Department of Neurology Faculty Papers

Common variable immunodeficiency (CVID) is a heterogeneous group of inborn errors of immunity characterized by reduced serum concentrations of different immunoglobulin isotypes. CVID is the most prevalent symptomatic antibody deficiency with a broad range of infectious and non-infectious clinical manifestations. Various genetic and immunological defects are known to be involved in the pathogenesis of CVID. Monogenic defects account for the pathogenesis of about 20-50% of CVID patients, while a variety of cases do not have a defined genetic background. Deficiencies in molecules of B cell receptor signaling or other pathways involving B-cell development, activation, and proliferation could be associated with …


Efficacy Of Cenobamate For Uncontrolled Focal Seizures In Patients With Previous Epilepsy-Related Surgery: Post Hoc Analysis Of A Phase 3, Multicenter, Open-Label Study, Bassel Abou-Khalil, Sami Aboumatar, Pavel Klein, Gregory L Krauss, Michael R Sperling, William E Rosenfeld May 2022

Efficacy Of Cenobamate For Uncontrolled Focal Seizures In Patients With Previous Epilepsy-Related Surgery: Post Hoc Analysis Of A Phase 3, Multicenter, Open-Label Study, Bassel Abou-Khalil, Sami Aboumatar, Pavel Klein, Gregory L Krauss, Michael R Sperling, William E Rosenfeld

Department of Neurology Faculty Papers

Objective: This post hoc analysis of 10 US study sites from a long-term open-label phase 3 study of adjunctive cenobamate evaluated the efficacy of cenobamate in patients with prior epilepsy-related surgery.

Methods: Patients with uncontrolled focal seizures despite taking stable doses of 1-3 concomitant antiseizure medications (ASMs) received increasing doses of cenobamate (12.5, 25, 50, 100, 150, 200 mg/day) at 2-week intervals over 12 weeks (target dose, 200 mg/day). Further increases up to 400 mg/day using biweekly 50-mg/day increments were allowed during the maintenance phase. Dose adjustments of cenobamate and concomitant ASMs were allowed. Data were assessed until the last …


Functional (Psychogenic) Seizures Are Associated With Thyroid Disorders, Ali A. Asadi-Pooya, Mohsen Farazdaghi May 2022

Functional (Psychogenic) Seizures Are Associated With Thyroid Disorders, Ali A. Asadi-Pooya, Mohsen Farazdaghi

Department of Neurology Faculty Papers

Objective: We investigated medical comorbidities in patients with functional seizures (FS) and those with epilepsy (idiopathic generalized epilepsies [IGEs] or temporal lobe epilepsy [TLE]). We hypothesized that the nature of medical comorbidities differs between these three groups. This might be helpful to postulate on the pathophysiology of FS. Materials and Methods: In a retrospective study, all adult patients with a diagnosis of IGE, TLE, or FS were recruited at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Iran, from 2008 until 2020. The three groups of patients were matched with regard to their age. Age, sex, and medical …


Peripheral Neuropathy Evaluations Of Patients With Prolonged Long Covid., Anne Louise Oaklander, Alexander J Mills, Mary Kelley, Lisa S Toran, Bryan Smith, Marinos Dalakas, Avindra Nath May 2022

Peripheral Neuropathy Evaluations Of Patients With Prolonged Long Covid., Anne Louise Oaklander, Alexander J Mills, Mary Kelley, Lisa S Toran, Bryan Smith, Marinos Dalakas, Avindra Nath

Department of Neurology Faculty Papers

Background and objectives: Recovery from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection appears exponential, leaving a tail of patients reporting various long COVID symptoms including unexplained fatigue/exertional intolerance and dysautonomic and sensory concerns. Indirect evidence links long COVID to incident polyneuropathy affecting the small-fiber (sensory/autonomic) axons.

Methods: We analyzed cross-sectional and longitudinal data from patients with World Health Organization (WHO)-defined long COVID without prior neuropathy history or risks who were referred for peripheral neuropathy evaluations. We captured standardized symptoms, examinations, objective neurodiagnostic test results, and outcomes, tracking participants for 1.4 years on average.

Results: Among 17 patients (mean age …


Role Of Atogepant In The Treatment Of Episodic Migraines: Clinical Perspectives And Considerations, Fred Cohen, Hsiangkuo Yuan Apr 2022

Role Of Atogepant In The Treatment Of Episodic Migraines: Clinical Perspectives And Considerations, Fred Cohen, Hsiangkuo Yuan

Department of Neurology Faculty Papers

Advances in molecular biology and neuroscience have led to the discovery of calcitonin gene-related peptide (CGRP), a 37 amino-acid neuropeptide that plays a critical role in the pathogenesis of migraine. CGRP receptor antagonist, also known as gepant, is an oral medication that inhibits the CGRP-related nociceptive signaling pathway. To date, three gepants are approved by the FDA for migraine treatment. Atogepant is a 2nd-generation gepant that non-competitively antagonizes CGRP receptors inhibiting neurogenic inflammation and pain sensitization. With its long half-life and minimal cardiovascular or liver toxicity, it is the first in its class approved primarily for migraine prevention. This article …


Neuromotor Prosthetic To Treat Stroke-Related Paresis: N-Of-1 Trial, Mijail D. Serruya, Alessandro Napoli, Nicholas Satterthwaite, Joe Kardine, Joseph Mccoy, Namrata Grampurohit, Kiran Talekar, Devon M. Middleton, Feroze Mohamed, Michael Kogan, Ashwini Sharan, Chengyuan Wu, Robert H. Rosenwasser Apr 2022

Neuromotor Prosthetic To Treat Stroke-Related Paresis: N-Of-1 Trial, Mijail D. Serruya, Alessandro Napoli, Nicholas Satterthwaite, Joe Kardine, Joseph Mccoy, Namrata Grampurohit, Kiran Talekar, Devon M. Middleton, Feroze Mohamed, Michael Kogan, Ashwini Sharan, Chengyuan Wu, Robert H. Rosenwasser

Department of Neurology Faculty Papers

Background: Functional recovery of arm movement typically plateaus following a stroke, leaving chronic motor deficits. Brain-computer interfaces (BCI) may be a potential treatment for post-stroke deficits

Methods: In this n-of-1 trial (NCT03913286), a person with chronic subcortical stroke with upper-limb motor impairment used a powered elbow-wrist-hand orthosis that opened and closed the affected hand using cortical activity, recorded from a percutaneous BCI comprised of four microelectrode arrays implanted in the ipsilesional precentral gyrus, based on decoding of spiking patterns and high frequency field potentials generated by imagined hand movements. The system was evaluated in a home setting for 12 weeks …


Csf-1 Maintains Pathogenic But Not Homeostatic Myeloid Cells In The Central Nervous System During Autoimmune Neuroinflammation, Daniel Hwang, Maryam S Seyedsadr, Larissa Ishikawa, Alexandra Boehm, Ziver Sahin, Giacomo Casella, Soohwa Jang, Michael V Gonzalez, James P Garifallou, Hakon Hakonarson, Weifeng Zhang, Dan Xiao, A M Rostami, Guang-Xian Zhang, Bogoljub Ciric Apr 2022

Csf-1 Maintains Pathogenic But Not Homeostatic Myeloid Cells In The Central Nervous System During Autoimmune Neuroinflammation, Daniel Hwang, Maryam S Seyedsadr, Larissa Ishikawa, Alexandra Boehm, Ziver Sahin, Giacomo Casella, Soohwa Jang, Michael V Gonzalez, James P Garifallou, Hakon Hakonarson, Weifeng Zhang, Dan Xiao, A M Rostami, Guang-Xian Zhang, Bogoljub Ciric

Department of Neurology Faculty Papers

SignificanceMultiple sclerosis (MS) and its animal model, experimental autoimmune encephalomyelitis (EAE), are autoimmune diseases characterized by accumulation of myeloid cells in the central nervous system (CNS). Both harmful and beneficial myeloid cells are present in EAE/MS, and a goal of MS therapy is to preferentially remove harmful myeloid cells. The receptor for CSF-1 (CSF-1R) is found on myeloid cells and is important for their survival. CSF-1R can bind two ligands, CSF-1 and IL-34, but it is not known whether their functions in EAE/MS differ. We found that blocking CSF-1 depleted only harmful myeloid cells in the CNS and suppressed EAE, …


Improved Cotard Delusion And Motor Function In Parkinson’S Disease Following Electroconvulsive Therapy (Ect), Sunihidi Ramesh, Jeffrey Ratliff Apr 2022

Improved Cotard Delusion And Motor Function In Parkinson’S Disease Following Electroconvulsive Therapy (Ect), Sunihidi Ramesh, Jeffrey Ratliff

Department of Neurology Faculty Papers

No abstract provided.