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Book: Epilepsy

Rho, Jong M.

scientific foundations of clinical practice

(Neurological disease and therapy ; 64)

2004

Author's details	ed. by Jong M. Rho
Series title	Neurological disease and therapy ; 64
Collection
Language	English
Size	XII, 510 S. : Ill.
Publisher	Dekker
Publishing place	New York u.a.
Publishing country	United States
Document type	Book
HBZ-ID	HT013928052
ISBN	0-8247-5043-8 ; 978-0-8247-5043-5
Database	Catalogue ZB MED Medicine, Health

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Article ; Online: The metabolic basis of epilepsy.

Rho, Jong M / Boison, Detlev

Nature reviews. Neurology

2022 Volume 18, Issue 6, Page(s) 333–347

Abstract: The brain is a highly energy-demanding organ and requires bioenergetic adaptability to balance normal activity with pathophysiological fuelling of spontaneous recurrent seizures, the hallmark feature of the epilepsies. Recurrent or prolonged seizures ... ...

Abstract	The brain is a highly energy-demanding organ and requires bioenergetic adaptability to balance normal activity with pathophysiological fuelling of spontaneous recurrent seizures, the hallmark feature of the epilepsies. Recurrent or prolonged seizures have long been known to permanently alter neuronal circuitry and to cause excitotoxic injury and aberrant inflammation. Furthermore, pathological changes in bioenergetics and metabolism are considered downstream consequences of epileptic seizures that begin at the synaptic level. However, as we highlight in this Review, evidence is also emerging that primary derangements in cellular or mitochondrial metabolism can result in seizure genesis and lead to spontaneous recurrent seizures. Basic and translational research indicates that the relationships between brain metabolism and epileptic seizures are complex and bidirectional, producing a vicious cycle that compounds the deleterious consequences of seizures. Metabolism-based treatments such as the high-fat, antiseizure ketogenic diet have become mainstream, and metabolic substrates and enzymes have become attractive molecular targets for seizure prevention and recovery. Moreover, given that metabolism is crucial for epigenetic as well as inflammatory changes, the idea that epileptogenesis can be both negatively and positively influenced by metabolic changes is rapidly gaining ground. Here, we review evidence that supports both pathophysiological and therapeutic roles for brain metabolism in epilepsy.
MeSH term(s)	Brain/pathology ; Energy Metabolism/physiology ; Epilepsy ; Humans ; Seizures/drug therapy ; Seizures/etiology ; Status Epilepticus
Language	English
Publishing date	2022-03-31
Publishing country	England
Document type	Journal Article ; Review ; Research Support, N.I.H., Extramural
ZDB-ID	2491514-2
ISSN	1759-4766 ; 1759-4758
ISSN (online)	1759-4766
ISSN	1759-4758
DOI	10.1038/s41582-022-00651-8
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Article ; Online: How does the ketogenic diet induce anti-seizure effects?

Rho, Jong M

Neuroscience letters

2017 Volume 637, Page(s) 4–10

Abstract: The high-fat ketogenic diet (KD) is a remarkably effective treatment for medically intractable epilepsy and has been part of the clinical armamentarium for nearly a century. However, the mechanisms underlying the KD's actions have remained elusive. Over ... ...

Abstract	The high-fat ketogenic diet (KD) is a remarkably effective treatment for medically intractable epilepsy and has been part of the clinical armamentarium for nearly a century. However, the mechanisms underlying the KD's actions have remained elusive. Over the past decade, there has been phenomenal international growth of clinical centers offering metabolism-based therapies for epilepsy, and rapidly expanding research into the cellular and biochemical effects induced by the KD. At present, there are many hypotheses regarding KD action, and while each is uniquely compelling, it is becoming more apparent that the KD likely works through multiple mechanisms that target fundamental biochemical pathways linked to cellular substrates (e.g., ion channels) and mediators responsible for neuronal hyperexcitability. This is not altogether surprising given the complexity of the epileptic brain, and the many different pathophysiologic mechanisms that underlie seizure genesis and epileptogenicity. The scientific literature involving the KD strongly supports the notion that epilepsy may indeed in part represent a "metabolic disease", and that this concept could serve as a novel framework for the development of more effective anti-seizure drugs.
MeSH term(s)	Animals ; Diet, High-Fat ; Diet, Ketogenic ; Humans ; Oxidative Stress/physiology ; Potassium Channels/metabolism ; Seizures/drug therapy ; Treatment Outcome
Chemical Substances	Potassium Channels
Language	English
Publishing date	2017--10
Publishing country	Ireland
Document type	Journal Article ; Review
ZDB-ID	194929-9
ISSN	1872-7972 ; 0304-3940
ISSN (online)	1872-7972
ISSN	0304-3940
DOI	10.1016/j.neulet.2015.07.034
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Article ; Online: Metabolic epilepsies amenable to ketogenic therapies: Indications, contraindications, and underlying mechanisms.

Gavrilovici, Cezar / Rho, Jong M

Journal of inherited metabolic disease

2020 Volume 44, Issue 1, Page(s) 42–53

Abstract: Metabolic epilepsies arise in the context of rare inborn errors of metabolism (IEM), notably glucose transporter type 1 deficiency syndrome, succinic semialdehyde dehydrogenase deficiency, pyruvate dehydrogenase complex deficiency, nonketotic ... ...

Abstract	Metabolic epilepsies arise in the context of rare inborn errors of metabolism (IEM), notably glucose transporter type 1 deficiency syndrome, succinic semialdehyde dehydrogenase deficiency, pyruvate dehydrogenase complex deficiency, nonketotic hyperglycinemia, and mitochondrial cytopathies. A common feature of these disorders is impaired bioenergetics, which through incompletely defined mechanisms result in a wide spectrum of neurological symptoms, such as epileptic seizures, developmental delay, and movement disorders. The ketogenic diet (KD) has been successfully utilized to treat such conditions to varying degrees. While the mechanisms underlying the clinical efficacy of the KD in IEM remain unclear, it is likely that the proposed heterogeneous targets influenced by the KD work in concert to rectify or ameliorate the downstream negative consequences of genetic mutations affecting key metabolic enzymes and substrates-such as oxidative stress and cell death. These beneficial effects can be broadly grouped into restoration of impaired bioenergetics and synaptic dysfunction, improved redox homeostasis, anti-inflammatory, and epigenetic activity. Hence, it is conceivable that the KD might prove useful in other metabolic disorders that present with epileptic seizures. At the same time, however, there are notable contraindications to KD use, such as fatty acid oxidation disorders. Clearly, more research is needed to better characterize those metabolic epilepsies that would be amenable to ketogenic therapies, both experimentally and clinically. In the end, the expanded knowledge base will be critical to designing metabolism-based treatments that can afford greater clinical efficacy and tolerability compared to current KD approaches, and improved long-term outcomes for patients.
MeSH term(s)	Amino Acid Metabolism, Inborn Errors/diet therapy ; Animals ; Contraindications ; Diet, Ketogenic ; Energy Metabolism ; Epilepsy/diet therapy ; Humans ; Ketone Bodies/biosynthesis ; Ketone Bodies/therapeutic use ; Treatment Outcome
Chemical Substances	Ketone Bodies
Language	English
Publishing date	2020-08-11
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	438341-2
ISSN	1573-2665 ; 0141-8955
ISSN (online)	1573-2665
ISSN	0141-8955
DOI	10.1002/jimd.12283
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Article ; Online: Intermittent vs continuous ketogenic diet: Impact on seizures, gut microbiota, and mitochondrial metabolism.

Shearer, Jane / Scantlebury, Morris H / Rho, Jong M / Tompkins, Thomas A / Mu, Chunlong

Epilepsia

2023 Volume 64, Issue 8, Page(s) e177–e183

Abstract: We have shown previously that the ketogenic diet (KD) is effective in reducing seizures associated with infantile spasms syndrome (ISS) and that this benefit is related to alterations in the gut microbiota. However, it remains unclear whether the ... ...

Abstract	We have shown previously that the ketogenic diet (KD) is effective in reducing seizures associated with infantile spasms syndrome (ISS) and that this benefit is related to alterations in the gut microbiota. However, it remains unclear whether the efficacy of the KD persists after switching to a normal diet. Employing a neonatal rat model of ISS, we tested the hypothesis that the impact of the KD would diminish when switched to a normal diet. Following epilepsy induction, neonatal rats were divided into two groups: continuous KD for 6 days; and a group fed with KD for 3 days and then a normal diet for 3 days. Spasms frequency, mitochondrial bioenergetics in the hippocampus, and fecal microbiota were evaluated as major readouts. We found that the anti-epileptic effect of the KD was reversible, as evidenced by the increased spasms frequency in rats that were switched from the KD to a normal diet. The spasms frequency was correlated inversely with mitochondrial bioenergetic function and a set of gut microbes, including Streptococcus thermophilus and Streptococcus azizii. These findings suggest that the anti-epileptic and metabolic benefits of the KD decline rapidly in concert with gut microbial alterations in the ISS model.
MeSH term(s)	Rats ; Animals ; Gastrointestinal Microbiome ; Diet, Ketogenic ; Seizures ; Epilepsy ; Spasms, Infantile/drug therapy ; Anticonvulsants/therapeutic use ; Spasm
Chemical Substances	Anticonvulsants
Language	English
Publishing date	2023-06-29
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	216382-2
ISSN	1528-1167 ; 0013-9580
ISSN (online)	1528-1167
ISSN	0013-9580
DOI	10.1111/epi.17688
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Article ; Online: A clinically relevant selective ERK-pathway inhibitor reverses core deficits in a mouse model of autism.

Murari, Kartikeya / Abushaibah, Abdulrahman / Rho, Jong M / Turner, Ray W / Cheng, Ning

EBioMedicine

2023 Volume 91, Page(s) 104565

Abstract: Background: Extracellular signal-regulated kinase (ERK/MAPK) pathway in the brain is hypothesized to be a critical convergent node in the development of autism spectrum disorder. We reasoned that selectively targeting this pathway could reverse core ... ...

Abstract	Background: Extracellular signal-regulated kinase (ERK/MAPK) pathway in the brain is hypothesized to be a critical convergent node in the development of autism spectrum disorder. We reasoned that selectively targeting this pathway could reverse core autism-like phenotype in animal models. Methods: Here we tested a clinically relevant, selective inhibitor of ERK pathway, PD325901 (Mirdametinib), in a mouse model of idiopathic autism, the BTBR mice. Findings: We report that treating juvenile mice with PD325901 reduced ERK pathway activation, dose and duration-dependently reduced core disease-modeling deficits in sociability, vocalization and repetitive behavior, and reversed abnormal EEG signals. Further analysis revealed that subchronic treatment did not affect weight gain, locomotion, or neuronal density in the brain. Parallel treatment in the C57BL/6J mice did not alter their phenotype. Interpretation: Our data indicate that selectively inhibiting ERK pathway using PD325901 is beneficial in the BTBR model, thus further support the notion that ERK pathway is critically involved in the pathophysiology of autism. These results suggest that a similar approach could be applied to animal models of syndromic autism with dysregulated ERK signaling, to further test selectively targeting ERK pathway as a new approach for treating autism. Funding: This has beenwork was supported by Alberta Children's Hospital Research Foundation (JMR & NC), University of Calgary Faculty of Veterinary Medicine (NC), Kids Brain Health Network (NC), and Natural Sciences and Engineering Research Council of Canada (NC).
MeSH term(s)	Mice ; Animals ; Autistic Disorder/drug therapy ; Autistic Disorder/metabolism ; Autism Spectrum Disorder/metabolism ; Mice, Inbred C57BL ; MAP Kinase Signaling System ; Mice, Inbred Strains ; Disease Models, Animal
Chemical Substances	mirdametinib (86K0J5AK6M)
Language	English
Publishing date	2023-04-21
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	2851331-9
ISSN	2352-3964
ISSN (online)	2352-3964
DOI	10.1016/j.ebiom.2023.104565
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Article ; Online: Inhibition of Lactate Dehydrogenase to Treat Epilepsy.

Rho, Jong M

The New England journal of medicine

2015 Volume 373, Issue 2, Page(s) 187–189

MeSH term(s)	Animals ; Anticonvulsants/pharmacology ; Anticonvulsants/therapeutic use ; Dioxolanes/pharmacology ; Dioxolanes/therapeutic use ; Enzyme Inhibitors/pharmacology ; Enzyme Inhibitors/therapeutic use ; Epilepsy/drug therapy ; Epilepsy/metabolism ; Humans ; L-Lactate Dehydrogenase/antagonists & inhibitors ; L-Lactate Dehydrogenase/metabolism ; Safrole/pharmacology ; Safrole/therapeutic use
Chemical Substances	Anticonvulsants ; Dioxolanes ; Enzyme Inhibitors ; L-Lactate Dehydrogenase (EC 1.1.1.27) ; stiripentol (R02XOT8V8I) ; Safrole (RSB34337V9) ; isosafrole (W6337429LF)
Language	English
Publishing date	2015-07-09
Publishing country	United States
Document type	Journal Article
ZDB-ID	207154-x
ISSN	1533-4406 ; 0028-4793
ISSN (online)	1533-4406
ISSN	0028-4793
DOI	10.1056/NEJMcibr1503558
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Article ; Online: Epigenetics and epilepsy prevention: The therapeutic potential of adenosine and metabolic therapies.

Boison, Detlev / Rho, Jong M

Neuropharmacology

2019 Volume 167, Page(s) 107741

Abstract: Prevention of epilepsy and its progression remains the most urgent need for epilepsy research and therapy development. Novel conceptual advances are required to meaningfully address this fundamental challenge. Maladaptive epigenetic changes, which ... ...

Abstract	Prevention of epilepsy and its progression remains the most urgent need for epilepsy research and therapy development. Novel conceptual advances are required to meaningfully address this fundamental challenge. Maladaptive epigenetic changes, which include methylation of DNA and acetylation of histones - among other mechanisms, are now well recognized to play a functional role in the development of epilepsy and its progression. The methylation hypothesis of epileptogenesis suggests that changes in DNA methylation are implicated in the progression of the disease. In this context, global DNA hypermethylation is particularly associated with chronic epilepsy. Likewise, acetylation changes of histones have been linked to epilepsy development. Clinical as well as experimental evidence demonstrate that epilepsy and its progression can be prevented by metabolic and biochemical manipulations that target previously unrecognized epigenetic functions contributing to epilepsy development and maintenance of the epileptic state. This review will discuss epigenetic mechanisms implicated in epilepsy development as well as metabolic and biochemical interactions thought to drive epileptogenesis. Therefore, metabolic and biochemical mechanisms are identified as novel targets for epilepsy prevention. We will specifically discuss adenosine biochemistry as a novel therapeutic strategy to reconstruct the DNA methylome as antiepileptogenic strategy as well as metabolic mediators, such as beta-hydroxybutyrate, which affect histone acetylation. Finally, metabolic dietary interventions (such as the ketogenic diet) which have the unique potential to prevent epileptogenesis through recently identified epigenetic mechanisms will be reviewed. This article is part of the special issue entitled 'New Epilepsy Therapies for the 21st Century - From Antiseizure Drugs to Prevention, Modification and Cure of Epilepsy'.
MeSH term(s)	Adenosine/metabolism ; Animals ; Anticonvulsants/administration & dosage ; DNA Methylation/physiology ; Diet, Ketogenic/methods ; Diet, Ketogenic/trends ; Epigenesis, Genetic/drug effects ; Epigenesis, Genetic/physiology ; Epilepsy/genetics ; Epilepsy/metabolism ; Epilepsy/therapy ; Histones/metabolism ; Humans
Chemical Substances	Anticonvulsants ; Histones ; Adenosine (K72T3FS567)
Language	English
Publishing date	2019-08-13
Publishing country	England
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	218272-5
ISSN	1873-7064 ; 0028-3908
ISSN (online)	1873-7064
ISSN	0028-3908
DOI	10.1016/j.neuropharm.2019.107741
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Article: Selective Probiotic Treatment Positively Modulates the Microbiota-Gut-Brain Axis in the BTBR Mouse Model of Autism.

Pochakom, Angela / Mu, Chunlong / Rho, Jong M / Tompkins, Thomas A / Mayengbam, Shyamchand / Shearer, Jane

Brain sciences

2022 Volume 12, Issue 6

Abstract: Recent studies have shown promise for the use of probiotics in modulating behaviour through the microbiota-gut-brain axis. In the present study, we assessed the impact of two probiotic strains in mitigating autism-related symptomology in the ... ...

Abstract	Recent studies have shown promise for the use of probiotics in modulating behaviour through the microbiota-gut-brain axis. In the present study, we assessed the impact of two probiotic strains in mitigating autism-related symptomology in the BTBR
Language	English
Publishing date	2022-06-14
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2651993-8
ISSN	2076-3425
ISSN	2076-3425
DOI	10.3390/brainsci12060781
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Article ; Online: A clinically relevant selective ERK-pathway inhibitor reverses core deficits in a mouse model of autismResearch in context

Kartikeya Murari / Abdulrahman Abushaibah / Jong M. Rho / Ray W. Turner / Ning Cheng

EBioMedicine, Vol 91, Iss , Pp 104565- (2023)

2023

Abstract: Summary: Background: Extracellular signal-regulated kinase (ERK/MAPK) pathway in the brain is hypothesized to be a critical convergent node in the development of autism spectrum disorder. We reasoned that selectively targeting this pathway could reverse ... ...

Abstract	Summary: Background: Extracellular signal-regulated kinase (ERK/MAPK) pathway in the brain is hypothesized to be a critical convergent node in the development of autism spectrum disorder. We reasoned that selectively targeting this pathway could reverse core autism-like phenotype in animal models. Methods: Here we tested a clinically relevant, selective inhibitor of ERK pathway, PD325901 (Mirdametinib), in a mouse model of idiopathic autism, the BTBR mice. Findings: We report that treating juvenile mice with PD325901 reduced ERK pathway activation, dose and duration-dependently reduced core disease-modeling deficits in sociability, vocalization and repetitive behavior, and reversed abnormal EEG signals. Further analysis revealed that subchronic treatment did not affect weight gain, locomotion, or neuronal density in the brain. Parallel treatment in the C57BL/6J mice did not alter their phenotype. Interpretation: Our data indicate that selectively inhibiting ERK pathway using PD325901 is beneficial in the BTBR model, thus further support the notion that ERK pathway is critically involved in the pathophysiology of autism. These results suggest that a similar approach could be applied to animal models of syndromic autism with dysregulated ERK signaling, to further test selectively targeting ERK pathway as a new approach for treating autism. Funding: : This has beenwork was supported by Alberta Children’s Hospital Research Foundation (JMR & NC), University of Calgary Faculty of Veterinary Medicine (NC), Kids Brain Health Network (NC), and Natural Sciences and Engineering Research Council of Canada (NC).
Keywords	Autism ; ERK ; Selective inhibitor ; PD325901/Mirdametinib ; Repurposed drug ; EEG ; Medicine ; R ; Medicine (General) ; R5-920
Subject code	616
Language	English
Publishing date	2023-05-01T00:00:00Z
Publisher	Elsevier
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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