Seizures and epilepsy: an overview for neuroscientists

CE Stafstrom, L Carmant - Cold Spring …, 2015 - perspectivesinmedicine.cshlp.org
Epilepsy is one of the most common and disabling neurologic conditions, yet we have an
incomplete understanding of the detailed pathophysiology and, thus, treatment rationale for …

The system epilepsies: a pathophysiological hypothesis

G Avanzini, P Manganotti, S Meletti, SL Moshé… - …, 2012 - Wiley Online Library
We postulate that “system epilepsies”(SystE) are due to an enduring propensity to generate
seizures of functionally characterized brain systems. Data supporting this hypothesis—that …

[HTML][HTML] Epileptic encephalopathies: new genes and new pathways

SE Nieh, EH Sherr - Neurotherapeutics, 2014 - Elsevier
Epileptic encephalopathies represent a group of devastating epileptic disorders that occur
early in life and are often characterized by pharmaco-resistant epilepsy, persistent severe …

Increased electroencephalography connectivity precedes epileptic spasm onset in infants with tuberous sclerosis complex

PE Davis, K Kapur, R Filip‐Dhima, SK Trowbridge… - …, 2019 - Wiley Online Library
Objective To identify whether abnormal electroencephalography (EEG) connectivity is
present before the onset of epileptic spasms (ES) in infants with tuberous sclerosis complex …

Mechanisms of infantile epileptic spasms syndrome: What have we learned from animal models?

ACH Ng, A Choudhary, KT Barrett, C Gavrilovici… - …, 2024 - Wiley Online Library
The devastating developmental and epileptic encephalopathy of infantile epileptic spasms
syndrome (IESS) has numerous causes, including, but not limited to, brain injury, metabolic …

Pathophysiology of epileptic encephalopathies

FA Lado, G Rubboli, P Capovilla, G Avanzini… - …, 2013 - Wiley Online Library
The application of metabolic imaging and genetic analysis, and now the development of
appropriate animal models, has generated critical insights into the pathogenesis of epileptic …

APC conditional knock-out mouse is a model of infantile spasms with elevated neuronal β-catenin levels, neonatal spasms, and chronic seizures

A Pirone, J Alexander, LA Lau, D Hampton… - Neurobiology of …, 2017 - Elsevier
Infantile spasms (IS) are a catastrophic childhood epilepsy syndrome characterized by
flexion-extension spasms during infancy that progress to chronic seizures and cognitive …

Detailed magnetic resonance imaging (MRI) analysis in infantile spasms

C Harini, S Sharda, AM Bergin… - Journal of child …, 2018 - journals.sagepub.com
Purpose: To evaluate initial magnetic resonance imaging (MRI) abnormalities in infantile
spasms, correlate them to clinical characteristics, and describe repeat imaging findings …

[HTML][HTML] Decreased abundance of Akkermansia after adrenocorticotropic hormone therapy in patients with West syndrome

L Xu, D Chen, C Zhao, L Jiang, S Mao, C Song, F Gao - BMC microbiology, 2021 - Springer
Background Infants suffer from a severe epileptic encephalopathy known as West syndrome
(WS). Treatment with adrenocorticotropic hormone (ACTH) indicates the involvement of the …

Vigabatrin therapy implicates neocortical high frequency oscillations in an animal model of infantile spasms

JD Frost Jr, JT Le, CL Lee, C Ballester-Rosado… - Neurobiology of …, 2015 - Elsevier
Abnormal high frequency oscillations (HFOs) in EEG recordings are thought to be reflections
of mechanisms responsible for focal seizure generation in the temporal lobe and neocortex …