Article
Psychiatry
Alaa El-Cheikh Mohamad, Dorit Mohrle, Faraj L. Haddad, Anton Rose, Brian L. Allman, Susanne Schmid
Summary: Many neurodevelopmental disorders are associated with changes in sensory processing and sensorimotor gating; acoustic startle response and prepulse inhibition are widely used measures for assessing these changes; Cntnap2 knockout rats have been proven to be a valid model for ASD and a novel method was used to assess startle and PPI in these rats.
TRANSLATIONAL PSYCHIATRY
(2023)
Review
Clinical Neurology
Lena-Luise Becker, Alexander Gratopp, Christine Prager, Christian E. Elger, Angela M. Kaindl
Summary: Status epilepticus is a life-threatening neurological emergency in childhood, particularly in the first 5 years of life, with high mortality and morbidity rates. Current treatments for convulsive status epilepticus in children may be delayed and inadequate. This study summarizes the current knowledge on treatment options and proposes a treatment algorithm. Benzodiazepines are recommended as first-line treatment, and for refractory status epilepticus, no superiority was found in fosphenytoin, levetiracetam, or phenobarbital. Limited data is available on third-line treatments for refractory status epilepticus lasting >30 min. The proposed algorithm aims to address treatment delay and reduce potential permanent neuronal damage.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Theodore Sheehan, Marta Amengual-Gual, Alejandra Vasquez, Nicholas S. Abend, Anne Anderson, Brian Appavu, Ravindra Arya, Cristina Barcia Aguilar, J. Nicholas Brenton, Jessica L. Carpenter, Kevin E. Chapman, Justice Clark, Raquel Farias-Moeller, William D. Gaillard, Marina Gainza-Lein, Tracy A. Glauser, Joshua L. Goldstein, Howard P. Goodkin, Rejean M. Guerriero, Linda Huh, Michele Jackson, Kush Kapur, Robert Kahoud, Yi-Chen Lai, Tiffani L. McDonough, Mohamad A. Mikati, Lindsey A. Morgan, Edward J. Novotny, Adam P. Ostendorf, Eric T. Payne, Katrina Peariso, Juan Piantino, Latania Reece, James J. Riviello, Tristan T. Sands, Kumar Sannagowdara, Renee Shellhaas, Garnett Smith, Robert C. Tasker, Dmitry Tchapyjnikov, Alexis A. Topjian, Mark S. Wainwright, Angus Wilfong, Korwyn Williams, Bo Zhang, Tobias Loddenkemper
Summary: This study evaluated benzodiazepine administration patterns in pediatric patients with refractory convulsive status epilepticus before transitioning to non-Benzodiazepine antiseizure medication (ASM). Findings include over one-third of patients receiving more than two benzodiazepines before transitioning, and delays in treatment initiation reducing the likelihood of multiple benzodiazepine doses. Patients with out-of-hospital seizure onset were more likely to receive additional benzodiazepines beyond 30 and 45 minutes, and intermittent SE was a risk factor for more benzodiazepines administered beyond 45 minutes.
Article
Chemistry, Multidisciplinary
Mingxia Song, Wennan Zhao, Yangnv Zhu, Wenli Liu, Xianqing Deng, Yushan Huang
Summary: This study discovered a new triazolopyrimidine compound (6d) with good antiepileptic activity and a high protection index. The compound showed significant anticonvulsant effects in multiple epilepsy models and exerted its action through interaction with GABA receptors.
FRONTIERS IN CHEMISTRY
(2022)
Article
Biochemistry & Molecular Biology
Ping Han, Xia Wang, Tianqiu Zhou, Jinmei Cheng, Chengniu Wang, Fei Sun, Xi Zhao
Summary: Expression of Nrf2 in sperm is reduced in oligospermia patients, but the mechanism is unclear. This study found that Nrf2 and GPX4 protein expressions in sperm of oligospermia men were significantly decreased. Animal experiments showed that Nrf2 knockout in mice led to the occurrence of ferroptosis in spermatogenic cells, resulting in oligospermia. Treatment with a ferroptosis inhibitor reversed the decrease in sperm concentration and motility.
FREE RADICAL BIOLOGY AND MEDICINE
(2022)
Article
Clinical Neurology
Dennis L. Cheung, Matthew J. Cooke, Chelsea S. Goulton, Chanchanok Chaichim, Louis F. Cheung, Ashor Khoshaba, Junichi Nabekura, Andrew J. Moorhouse
Summary: The study suggests that addressing impaired Cl- homeostasis can significantly improve the efficacy of GABA inhibition during sustained seizures, and targeting both KCC2 and GABA(A) receptors simultaneously may be a pathway to improve current anticonvulsant therapeutic strategies.
Article
Neurosciences
Vanessa L. Breton, Mark S. Aquilino, Srinivasarao Repudi, Afifa Saleem, Shanthini Mylvaganam, Sara Abu-Swai, Berj L. Bardakjian, Rami Aqeilan, Peter L. Carlen
Summary: Loss of function mutations of the WWOX gene are associated with severe pediatric epileptic encephalopathy. Mouse models show that WWOX plays a crucial role in regulating neuronal excitability.
NEUROBIOLOGY OF DISEASE
(2021)
Article
Biochemistry & Molecular Biology
Philippe Liere, Guo-Jun Liu, Antoine Pianos, Ryan J. J. Middleton, Richard B. B. Banati, Yvette Akwa
Summary: This study found that complete deletion of the Tspo gene in male mice does not impair de novo steroidogenesis in vivo.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Sai Surthi Konduru, Yu-Zhen Pan, Eli Wallace, Jesse A. Pfammatter, Mathew Jones, Rama K. Maganti
Summary: Sleep deprivation worsens seizures and survival in epileptic mice, possibly due to reductions in GABAergic tonic inhibition.
ANNALS OF NEUROLOGY
(2021)
Article
Medicine, Research & Experimental
Anna D. Manis, Oleg Palygin, Elena Isaeva, Vladislav Levchenko, Peter S. LaViolette, Tengis S. Pavlov, Matthew R. Hodges, Alexander Staruschenko
Summary: The K(ir)5.1 channel plays a crucial role in the brain, and its knockout leads to seizures and mortality. SSKcnj16-/- rats showed increased light sensitivity and reproducible audiogenic seizures, highlighting a distinct role for K(ir)5.1 channels in the brain.
Article
Biochemistry & Molecular Biology
Duk-Shin Lee, Tae-Hyun Kim, Hana Park, Tae-Cheon Kang
Summary: In chronic epilepsy rats, the non-competitive alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptor antagonist PER can improve the astroglial TASK-1 upregulation in responders, but has no effect on non-responders to PER.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Multidisciplinary Sciences
Masako Tachibana, Toshiya Inada, Masaru Ichida, Norio Ozaki
Summary: This study aimed to explore factors affecting delirium in cases where hallucinations are prominent and found that alcohol drinking, benzodiazepine withdrawal, and the use of angiotensin II receptor blockers or dopamine receptor agonists were significantly associated with hallucinations in delirium patients.
SCIENTIFIC REPORTS
(2021)
Article
Pharmacology & Pharmacy
Caterina De Sarro, Martina Tallarico, Maria Pisano, Luca Gallelli, Rita Citraro, Giovambattista De Sarro, Antonio Leo
Summary: The hormone GLP-1 has been identified as a potential target for the treatment of neurological diseases such as epilepsy. In this study, the effects of acute and chronic treatment with LIRA on epilepsy-prone rats were investigated. It was found that chronic treatment with LIRA had a modest antiseizure effect, while acute treatment did not show significant results. Additionally, chronic use of diazepam led to the development of tolerance, but when combined with LIRA, a reduced development of tolerance and an enhanced antiseizure effect were observed. Overall, LIRA as an add-on therapy showed superior benefits compared to single antiseizure medications in treating epilepsy.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2022)
Article
Pharmacology & Pharmacy
Ke-Ting Pan, I-Hsun Li, Hui-Han Kao, Yi-Hsien Chen, Pei-Xun Zhong, Li-Ting Kao
Summary: This study found that among patients with psoriasis, the use of BZRAs may exacerbate mild symptoms, especially with high doses, while only slightly worsening symptoms in mild cases with low doses. However, low-dose BZRA exposure led to more improvements in psoriasis severity among patients with severe psoriasis. Clinicians should carefully consider the risks and benefits of BZRA usage in psoriasis patients.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Immunology
Yiying Zhang, Zhangyang Wang, Rongrong Wang, Lu Xia, Yiying Cai, Fangchao Tong, Yanqin Gao, Jing Ding, Xin Wang
Summary: Conditional knockout of ASK1 in microglia/macrophages reduces seizure severity, improves neurobehavioral impairments, and mitigates histological damage, suggesting that ASK1 in microglia/macrophages is a potential therapeutic target for inflammatory responses in epilepsy.
JOURNAL OF NEUROINFLAMMATION
(2022)
Article
Biochemistry & Molecular Biology
Themasap A. Khan, Omer Revah, Aaron Gordon, Se-Jin Yoon, Anna K. Krawisz, Carleton Goold, Yishan Sun, Chul Hoon Kim, Yuan Tian, Min-Yin Li, Julia M. Schaepe, Kazuya Ikeda, Neal D. Amin, Noriaki Sakai, Masayuki Yazawa, Leila Kushan, Seiji Nishino, Matthew H. Porteus, Judith L. Rapoport, Jonathan A. Bernstein, Ruth O'Hara, Carrie E. Bearden, Joachim F. Hallmayer, John R. Huguenard, Daniel H. Geschwind, Ricardo E. Dolmetsch, Sergiu P. Pasca
Editorial Material
Clinical Neurology
John R. Huguenard
Article
Biology
Adam C. Lu, Christine Kyuyoung Lee, Max Kleiman-Weiner, Brian Truong, Megan Wang, John R. Huguenard, Mark P. Beenhakker
Article
Multidisciplinary Sciences
Anna K. Koster, Austin L. Reese, Yuri Kuryshev, Xianlan Wen, Keri A. McKiernan, Erin E. Gray, Caiyun Wu, John R. Huguenard, Merritt Maduke, J. Du Bois
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2020)
Article
Neurosciences
Aaron Gordon, Se-Jin Yoon, Stephen S. Tran, Christopher D. Makinson, Jin Young Park, Jimena Andersen, Alfredo M. Valencia, Steve Horvath, Xinshu Xiao, John R. Huguenard, Sergiu P. Pasca, Daniel H. Geschwind
Summary: Research shows that three-dimensional human cortical organoids can reach stages of postnatal brain development after being cultured for 250 to 300 days, paralleling in vivo development. These findings provide important insights and tools for modeling neurodevelopmental diseases.
NATURE NEUROSCIENCE
(2021)
Article
Multidisciplinary Sciences
Yuan Pan, Jared D. Hysinger, Tara Barron, Nicki F. Schindler, Olivia Cobb, Xiaofan Guo, Belgin Yalcin, Corina Anastasaki, Sara B. Mulinyawe, Anitha Ponnuswami, Suzanne Scheaffer, Yu Ma, Kun-Che Chang, Xin Xia, Joseph A. Toonen, James J. Lennon, Erin M. Gibson, John R. Huguenard, Linda M. Liau, Jeffrey L. Goldberg, Michelle Monje, David H. Gutmann
Summary: Neurons play an essential role in promoting the growth of certain types of brain tumors, such as optic pathway gliomas driven by mutations in the NF1 tumor suppressor gene. Inhibition of neuronal activity through light deprivation can prevent tumor formation and progression, highlighting a potential therapeutic strategy and the importance of Nf1 mutation-mediated dysregulation of neuronal signaling pathways in cancer predisposition syndromes.
Article
Multidisciplinary Sciences
Anna Elleman, Gabrielle Devienne, Christopher D. Makinson, Allison L. Haynes, John R. Huguenard, J. Du Bois
Summary: The researchers have found that a synthetic saxitoxin derivative affixed to a photocleavable protecting group can control Na channels and have a precise impact on neurons.
NATURE COMMUNICATIONS
(2021)
Article
Neurosciences
Juliet K. Knowles, Haojun Xu, Caroline Soane, Ankita Batra, Tristan Saucedo, Eleanor Frost, Lydia T. Tam, Danielle Fraga, Lijun Ni, Katlin Villar, Sydney Talmi, John R. Huguenard, Michelle Monje
Summary: This study demonstrates that activity-dependent myelination resulting from absence seizures contributes to the progression of epilepsy, suggesting that maladaptive myelination may be pathogenic in certain forms of epilepsy and other neurological diseases.
NATURE NEUROSCIENCE
(2022)
Correction
Multidisciplinary Sciences
Anna V. Elleman, Gabrielle Devienne, Christopher D. Makinson, Allison L. Haynes, John R. Huguenard, J. Du Bois
NATURE COMMUNICATIONS
(2022)
Article
Biology
Brielle Ferguson, Cameron Glick, John R. Huguenard
Summary: Absence seizures are a type of epilepsy characterized by brief periods of unconsciousness and accompanying motor function lapses. These seizures can occur frequently throughout the day and are often accompanied by treatment-resistant attention impairments. In this study, researchers used various techniques to examine the role of prefrontal cortex dysfunction in attention impairments in a mouse model of absence epilepsy. They found altered activity in a specific type of interneuron in the prefrontal cortex, as well as reductions in gamma power during cue presentation. Optogenetic stimulation of these interneurons improved attention performance in the mice, suggesting a potential therapeutic target for cognitive comorbidities in absence epilepsy.
Article
Multidisciplinary Sciences
Eric T. Zhao, Jacob M. Hull, Nofar Mintz Hemed, Hasan Ulusan, Julian Bartram, Anqi Zhang, Pingyu Wang, Albert Pham, Silvia Ronchi, John R. Huguenard, Andreas Hierlemann, Nicholas A. Melosh
Summary: We developed an electrode connector that allows for multithousand channel counts at a millimeter scale by using an ultra-conformable thin-film electrode array. This connector successfully measured action potentials and seizure propagation trajectories in epileptic mice.
Editorial Material
Clinical Neurology
John R. Huguenard
Meeting Abstract
Oncology
Yuan Pan, Jared Hysinger, Tara Barron, Nicki Schindler, Olivia Cobb, Xiaofan Guo, Belgin Yalcin, Corina Anastasaki, Sara Mulinyawe, Anitha Ponnuswami, Suzanne Scheaffer, Yu Ma, Kun-Che Chang, Xin Xia, Joseph Toonen, James Lennon, Erin Gibson, John Huguenard, Linda Liau, Jeffrey Goldberg, Michelle Monje, David Gutmann
Meeting Abstract
Neurosciences
Brielle Ferguson, John Huguenard
NEUROPSYCHOPHARMACOLOGY
(2020)
Meeting Abstract
Clinical Neurology
Ricardo L. Azevedo-Pereira, Tanya Weerakkody, Jennifer Vu, Fang Du, Liang Xibin, John Huguenard, Tonya M. Bliss, Gary K. Steinberg