Treatment of Lennox-Gastaut syndrome: overview and recent findings

Rijckevorsel, Kenou van

doi:10.2147/ndt.s1668

Cited by 84 publications

(132 citation statements)

References 213 publications

(223 reference statements)

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“…Boys are affected more frequently than girls (5,9). In this study, 15% of the patients were diagnosed as LGS at the age of 1-3 years, 40% were diagnosed at the age of 3-5 years and 45% were diagnosed at the age of 5-8 years.…”

Section: Discussionmentioning

confidence: 50%

Lennox-Gastaut sendromlu hastaların klinik özellikleri ve uzun süreli seyri

Ekici¹,

Yarar²,

Yakut³

et al. 2012

tpa

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Aim:The aim of this study was to retrospectively evaluate clinical features, treatment and prognosis of 20 patients followed up as Lennox-Gastaut Syndrome in ESOGU in the Division of Pediatric Neurology. Material and Method: Multiple different types of seizure, diffuse spike-wave pattern(<3 Hz) on electroencephalogram, psychomotor retardation were the diagnostic criteria for Lennox-Gastaut Syndrome. The patients who had no etiologic reason were described as the idiopathic type. Prenatal, natal and postnatal risk factors, seizure and clinical features, neurodevelopmental status, treatment and prognostic features were evaluated. Results: Thirteen patients were female, seven were male; male/female ratio was 1,9. Nineteen patients were described as the symptomatic type. Hypoxic ischemic encephalopthy was the most common risk factor. Sixty percent of the patients had a history of infantile spasms. Generalized tonic, generalized tonic clonic and atonic drop attacks were the most common seizure types. Speech impairment and microcephaly were the most frequently seen neurologic findings. Diffuse spike-wave activity (1-3 Hz/s) on EEG, bioelectrical status epilepticus and 9-14 Hz/s fast activity discharges were the most commonly seen discharges. The most common neuroimaging finding was cerebral atrophy. Sixty percent of the patients suffered from a large number of seizures per day despite multiple antiepileptic drugs. The most commonly used antiepileptic drugs were valproate sodium, clobazam, lamotrigine and topiramate. Conclusions: The prognosis of Lennox-Gestaut Syndrome is poor due to either recurrent seizures or mental deterioration. Seizure control can not be provided despite the use of multiple anti-epileptic drugs. New antiepileptic drugs are needed. (Turk Arch Ped 2012; 47: 49-54)

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Section: Discussionmentioning

confidence: 50%

Lennox-Gastaut sendromlu hastaların klinik özellikleri ve uzun süreli seyri

Ekici¹,

Yarar²,

Yakut³

et al. 2012

tpa

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“…Accordingly, CBD represents a highly desirable treatment alternative for patients with earlyonset, severe epilepsy such as DS and LGS. In addition to pharmacoresistant seizures, these patients suffer from severe neurodevelopmental delay, intellectual disability, autism, motor impairments, and significant morbidity and mortality [265,266]. As patients with DS and LGS require effective and better-tolerated therapies and represent relatively homogeneous populations, they stand out as candidates for an initial trial of CBD safety and efficacy.…”

Section: Phase I Clinical Trial For Cbd In Treatment-resistant Epilepsymentioning

confidence: 99%

Cannabinoids and Epilepsy

et al. 2015

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Cannabis has been used for centuries to treat seizures. Recent anecdotal reports, accumulating animal model data, and mechanistic insights have raised interest in cannabisbased antiepileptic therapies. In this study, we review current understanding of the endocannabinoid system, characterize the pro-and anticonvulsive effects of cannabinoids [e.g., Δ9-tetrahydrocannabinol and cannabidiol (CBD)], and highlight scientific evidence from pre-clinical and clinical trials of cannabinoids in epilepsy. These studies suggest that CBD avoids the psychoactive effects of the endocannabinoid system to provide a well-tolerated, promising therapeutic for the treatment of seizures, while whole-plant cannabis can both contribute to and reduce seizures. Finally, we discuss results from a new multicenter, open-label study using CBD in a population with treatment-resistant epilepsy. In all, we seek to evaluate our current understanding of cannabinoids in epilepsy and guide future basic science and clinical studies.

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“…28 Data on the use of vigabatrin to treat seizures associated with LGS are inconsistent. 6,30 Besides these pharmacologic treatments, several nonpharmacologic treatments may be used in the treatment of LGS (Table 2). Based on the clinical experience of the authors, the ketogenic diet should be implemented as early as possible, as this diet is easier to control in younger children.…”

Section: Treatment For Lgsmentioning

confidence: 99%

“…[3][4][5] Patients with LGS experience frequent seizures, usually during sleep, with 10-20 Hz fast rhythms. 6,7 LGS is thought to comprise as much as 10% of all childhood epilepsies. 4,8,9 Onset of LGS usually occurs before 8 years of age, and peaks between ages 3 and 5 years.…”

Section: Introduction: Lennox-gastaut Syndromementioning

confidence: 99%

“…In addition, the frequency of each type of seizure tends to fluctuate, sometimes being brought on by the treatments themselves. 6,16 A correct diagnosis of LGS must include a detailed medical history, along with waking and sleeping EEGs and may require years of follow-up to confirm. 16 Misdiagnosis of LGS could lead to futile attempts to treat patients with drugs that are not effective for their actual diagnosis.…”

Section: Introduction: Lennox-gastaut Syndromementioning

confidence: 99%

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Rufinamide in patients with Lennox-Gastaut syndrome

Clark¹,

Gibson²

2016

NRR

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Objectives: Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy with an onset at the age of ~4 years.LGS is notoriously difficult to manage, as most patients experience multiple seizures per day, despite their concomitant use of several antiepileptic drugs (AEDs). Rufinamide (BANZEL ® ) is an AED approved by the US Food and Drug Administration for the adjunctive treatment of seizures associated with LGS in pediatric patients aged ≥1 year and in adults. The expert care of nurses knowledgeable in the treatment options for LGS is valuable to patients and caregivers. This review summarizes the existing knowledge on LGS and data from clinical and real-world studies on the use of rufinamide in patients with LGS. Methods: Recent review articles and information from the Epilepsy Foundation Website were reviewed for data on LGS treatment. Primary articles on rufinamide were also selected for review. Results and conclusion: The efficacy and safety of rufinamide have been evaluated in children and adults by using double-blind, open-label, and observational studies. In general, these studies indicate that rufinamide effectively reduces the frequency and severity of multiple seizure types associated with LGS and has tolerable side effects, the most common being vomiting and somnolence. Dosing modifications based on age, weight, and concomitant AED usage are recommended for patients using rufinamide.

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Treatment of Lennox-Gastaut syndrome: overview and recent findings

Cited by 84 publications

References 213 publications

Lennox-Gastaut sendromlu hastaların klinik özellikleri ve uzun süreli seyri

Lennox-Gastaut sendromlu hastaların klinik özellikleri ve uzun süreli seyri

Cannabinoids and Epilepsy

Rufinamide in patients with Lennox-Gastaut syndrome

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