Anti-seizure medications for Lennox-Gastaut syndrome

Brigo, Francesco; Jones, Katherine; Eltze, Christin; Matricardi, Sara

doi:10.1002/14651858.cd003277.pub4

Cited by 19 publications

(26 citation statements)

References 67 publications

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“…CBD is non-psychoactive and has been reported to have anti-inflammatory and antioxidant effects ( 49 ). Previous research has primarily focused on CBD’s effects for treating epilepsy and seizure ( 50 – 52 ). A recent report suggests that CBD can inhibit SARS-CoV-2 in lung cells by the induction of ER stress and innate immune response ( 13 ).…”

Section: Discussionmentioning

confidence: 99%

Cannabidiol modulates expression of type I IFN response genes and HIV infection in macrophages

Tomer

Suryawanshi

et al. 2022

Front. Immunol.

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Cannabis (Cannabis sativa) is a widely used drug in the United States and the frequency of cannabis use is particularly high among people living with HIV (PLWH). One key component of cannabis, the non-psychotropic (−)-cannabidiol (CBD) exerts a wide variety of biological actions, including anticonvulsive, analgesic, and anti-inflammatory effects. However, the exact mechanism of action through which CBD affects the immune cell signaling remains poorly understood. Here we report that CBD modulates type I interferon responses in human macrophages. Transcriptomics analysis shows that CBD treatment significantly attenuates cGAS-STING-mediated activation of type I Interferon response genes (ISGs) in monocytic THP-1 cells. We further showed that CBD treatment effectively attenuates 2’3-cGAMP stimulation of ISGs in both THP-1 cells and primary human macrophages. Interestingly, CBD significantly upregulates expression of autophagy receptor p62/SQSTM1. p62 is critical for autophagy-mediated degradation of stimulated STING. We observed that CBD treated THP-1 cells have elevated autophagy activity. Upon 2’3’-cGAMP stimulation, CBD treated cells have rapid downregulation of phosphorylated-STING, leading to attenuated expression of ISGs. The CBD attenuation of ISGs is reduced in autophagy deficient THP-1 cells, suggesting that the effects of CBD on ISGs is partially mediated by autophagy induction. Lastly, CBD decreases ISGs expression upon HIV infection in THP-1 cells and human primary macrophages, leading to increased HIV RNA expression 24 hours after infection. However, long term culture with CBD in infected primary macrophages reduced HIV viral spread, suggesting potential dichotomous roles of CBD in HIV replication. Our study highlights the immune modulatory effects of CBD and the needs for additional studies on its effect on viral infection and inflammation.

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Section: Discussionmentioning

confidence: 99%

Cannabidiol modulates expression of type I IFN response genes and HIV infection in macrophages

Tomer

Suryawanshi

et al. 2022

Front. Immunol.

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“…Consequently, there is an urgent need to develop up-to-date treatment guidelines for CDD ( 4 , 57 ). A lag between completion/publication of clinical research and the incorporation of their key findings into disease specific guidelines is expected, and this is reflected in the absence of identified guidelines for DS that include recommendations for the drug fenfluramine, which is the latest treatment approved for this indication ( 58 , 59 ). However with the recent emergence of novel treatments for DS, LGS and CDD ( 3 , 59 – 61 ), and for other diseases in general, it is hoped that this lag will become as short as possible.…”

Section: Discussionmentioning

confidence: 99%

Treatment Guidelines for Rare, Early-Onset, Treatment-Resistant Epileptic Conditions: A Literature Review on Dravet Syndrome, Lennox-Gastaut Syndrome and CDKL5 Deficiency Disorder

et al. 2021

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Background: Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS) and CDKL5 deficiency disorder (CDD) are rare epileptic conditions, characterised by drug-resistant seizures. Seizure management in these patients requires careful therapy selection. This targeted literature review (TLR) aimed to collate and synthesise information from country-specific and international treatment guidelines for DS, LGS and CDD.Methods: A TLR was performed between 25th January and 11th March 2021. Online rare diseases and guideline databases were manually searched in addition to websites of national health technology assessment bodies for the following countries: Australia, Canada, France, Germany, Israel, Italy, Japan, Spain, Switzerland, UK and US, as defined by pre-specified eligibility criteria. Search terms, developed for each condition, were translated into local languages where appropriate. Descriptive analyses were performed to examine the geographical distribution of included guidelines; methodologies used to develop guidelines; cross-referencing of treatment recommendations made within other guidelines; patterns of treatment recommendations. An author map was created using R version 3.5.1, to visualise the extent of collaboration between authors.Results: Forty total guidelines were included, of which 29, 34 and 0 contained recommendations for DS, LGS and CDD, respectively (some provided recommendations for ≥1 condition). Most were country-specific, with guideline authors predominantly publishing in regional groups. Five guidelines were classified as “International” and displayed connections between author groups in the US, UK, France and Italy. Reported guideline development processes were lacking [43% (17 guidelines) had unclear/absent literature review methodologies] and those reported were variable, including both systematic and targeted literature reviews. Use of expert consultation was also variable. A high degree of heterogeneity was observed in the availability of treatment recommendations across disorders, with 271 and 190 recommendations for LGS and DS, respectively, and contradictory positive and negative treatment recommendations for several drugs in each indication [35% (11/31) and 22% (6/27) in LGS and DS, respectively].Conclusions: This review highlights the need for further high-quality international consensus-based treatment guidelines for LGS, DS, and particularly for CDD (for which no treatment guidelines were identified). Supra-national consensus guidance based on findings from a wider geographical range may improve resource allocation and establish an improved world-wide standard of care.

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“…Zonisamide, LEV, perampanel (PER), and FFA may have a possible efficacy in this syndrome, although the level of evidence is not yet consistent ( 71 ). On the other hand, CBD has obtained a good class of evidence of efficacy in LGS as in DS; hence its use should be encouraged whenever other treatments have failed ( 72 , 73 ).…”

Section: Pharmacological Therapy In Drug-resistant Epilepsymentioning

confidence: 99%

The Pharmacoresistant Epilepsy: An Overview on Existent and New Emerging Therapies

et al. 2021

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Epilepsy is one of the most common neurological chronic disorders, with an estimated prevalence of 0. 5 – 1%. Currently, treatment options for epilepsy are predominantly based on the administration of symptomatic therapy. Most patients are able to achieve seizure freedom by the first two appropriate drug trials. Thus, patients who cannot reach a satisfactory response after that are defined as pharmacoresistant. However, despite the availability of more than 20 antiseizure medications (ASMs), about one-third of epilepsies remain drug-resistant. The heterogeneity of seizures and epilepsies, the coexistence of comorbidities, and the broad spectrum of efficacy, safety, and tolerability related to the ASMs, make the management of these patients actually challenging. In this review, we analyze the most relevant clinical and pathogenetic issues related to drug-resistant epilepsy, and then we discuss the current evidence about the use of available ASMs and the alternative non-pharmacological approaches.

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Anti-seizure medications for Lennox-Gastaut syndrome

Cited by 19 publications

References 67 publications

Cannabidiol modulates expression of type I IFN response genes and HIV infection in macrophages

Cannabidiol modulates expression of type I IFN response genes and HIV infection in macrophages

Treatment Guidelines for Rare, Early-Onset, Treatment-Resistant Epileptic Conditions: A Literature Review on Dravet Syndrome, Lennox-Gastaut Syndrome and CDKL5 Deficiency Disorder

The Pharmacoresistant Epilepsy: An Overview on Existent and New Emerging Therapies

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