Lennox–Gastaut syndrome

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Lennox gastaut syndrome, review of the literature and a case report

Lennox gastaut syndrome, review of the literature and a case report

Background: Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that is defined by generalized multiple type seizures, slowness of intellectual growth, and a specific EEG disturbance. Children affected might previously have infantile spasms or underlying brain disorder but etiology can be idiopathic. In South Africa, the incidence of secondary epilepsy is higher than what is found in developed countries resulting in higher incidence of the disease. LGS seizures are often treatment resistant and the long term prognosis is poor.

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<p>Adjunctive Rufinamide in Children with Lennox-Gastaut Syndrome: A Literature Review</p>

<p>Adjunctive Rufinamide in Children with Lennox-Gastaut Syndrome: A Literature Review</p>

Abstract: Lennox-Gastaut syndrome (LGS) is a severe, childhood-onset, developmental epileptic encephalopathy, with different etiologies and co-morbidities. Seizure treatment in LGS represents a major challenge; new antiepileptic drugs (AEDs) are developed to especially address seizures resulting in high morbidity and mortality, such as drop seizures. Ru fi namide (RFN) is one of the latest AEDs licensed for patients with LGS. Its mechanism of action involves sodium channels in a way that is unrelated to other AEDs. Here we discuss the use of adjunctive RFN in children and adolescents with LGS and its ef fi cacy and safety pro fi le, based on a systematic literature review. RFN shows a very favorable pro fi le in terms of adverse events and drug-interactions in children. It is particularly effective on tonic-atonic seizures and spasms, impacting on the quality of life of the patients. Further studies are needed to clarify the interaction pro fi le with the newest AEDs for LGS and to assess correlations between the etiology of LGS and drug response to individualize treatment and maximize ef fi cacy.
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Dental Considerations of a 4-year-old Girl with Lennox-Gastaut Syndrome. Case Report and Literature Review

Dental Considerations of a 4-year-old Girl with Lennox-Gastaut Syndrome. Case Report and Literature Review

We present the developmental, oral, clinical, radiographic findings and oral treatment of a 4-year- old girl presenting with Lennox-Gastaut syndrome (LGS), which is a severe disabling childhood epilepsy disease that is treated with one or multiple anti-epileptic drugs (AEDs). The child was wheel-chair bound, developmentally delayed, gastrostomy tube (G-tube) fed, and suffered from multiple seizures and infantile spasms. The child’s medical history included an under-developed pituitary gland, gastro esophageal reflux disease, vision and hearing impairment, history of chronic aspiration pneumonia, and allergies. Although the oral findings included no carious lesions, heavy calculus accumulation, spontaneous bleeding from the gingiva, generalized gingival hyperplasia (GH) and abnormal increased mobility in several deciduous teeth. This report describes the comprehensive radiographic and clinical examination and the treatment under general anesthesia.
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Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions

Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions

Abstract: Lennox-Gastaut syndrome is a severe, childhood-onset electroclinical syndrome com- prised of multiple seizure types, intellectual and behavioral disturbances and characteristic findings on electroencephalogram of slow spike and wave complexes and paroxysmal fast frequency activ- ity. Profound morbidity often accompanies a common and severe seizure type, the drop attack. Seizures often remain refractory, or initial treatment efficacy fades. Few individuals are seizure free despite the development of multiple generations of antiseizure medications over decades and high-level evidence on several choices. Approved medications such as lamotrigine, topiramate, rufinamide, felbamate and clobazam have demonstrated efficacy in reducing seizure burden. Can- nabidiol has emerged as a promising investigational therapy with vast social interest yet lacks a standard, approved formulation. Palliative surgical procedures, such as vagal nerve stimulation and corpus callosotomy may provide reduction in total seizures and drop attacks. Emerging evidence suggests that complete callosotomy provides greater improvement in seizures without additional side effects. Etiologies such as dysplasia or hypothalamic hamartoma may be amenable for focal resection and thus offer potential to reverse this devastating epileptic encephalopathy.
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Role of rufinamide in the management of Lennox-Gastaut syndrome (childhood epileptic encephalopathy)

Role of rufinamide in the management of Lennox-Gastaut syndrome (childhood epileptic encephalopathy)

Abstract: Rufinamide, a triazole derivative that is structurally distinct from currently marketed antiepileptic drugs (AEDs), is in development for the adjunctive treatment of Lennox-Gastaut syndrome (LGS) in children and adults. Rufinamide is well absorbed after oral administration, demonstrates low protein binding, and is metabolized by enzymatic hydrolysis without involvement of cytochrome P450 enzymes, conferring a low drug interaction potential. In a randomized, double-blind trial involving 138 adult and pediatric patients with LGS, compared with placebo, rufinamide 45 mg/kg/day resulted in significantly superior reductions in drop attacks (median change –42.5% vs +1.4% with placebo) and total seizures (–32.1% vs – 11.7% with placebo), accompanied by significantly higher responder rates. These results are comparable with findings reported for other AEDs in randomized, controlled clinical trials in patients with LGS. Rufinamide produced statistically significant seizure reduction which was maintained during long-term therapy and accompanied by good tolerability. The most frequently reported adverse events from a pooled safety database evaluating short- and long- term therapy were headache (22.9% and 29.5%), dizziness (15.5% and 22.5%) and fatigue (13.6% and 17.7%). Rufinamide therefore presents a favorable efficacy and tolerability profile and is a promising candidate for the adjunctive therapy of LGS.
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Adjunctive use of electroconvulsive therapy in patients with Lennox-Gastaut syndrome and drug-resistant epilepsy: A pilot study

Adjunctive use of electroconvulsive therapy in patients with Lennox-Gastaut syndrome and drug-resistant epilepsy: A pilot study

For many years, investigators and physi- cians have addressed the problem of medically-refractory epilepsy by investigat- ing and developing new AEDs (11). How- ever, no study has demonstrated that the new AEDs have greater potency than more established AEDs. Therefore, it would be desirable to explore novel methods in hop- ing of developing more effective ways to treat epilepsy. Electroconvulsive therapy (ECT) has been proposed as a treatment option for refractory epilepsy in a few an- ecdotal reports (6-8). However, in this small pilot clinical trial adjunctive use of an intensive ECT program in patients with Lennox-Gastaut syndrome (LGS) and drug- resistant seizures was not efficacious in de- creasing their seizure frequency. In contrast to previous suggestions, we did not observe any short-term or long-term benefits after delivering ECT to patients with LGS, with regard to seizure reduction; repeated in- duced-seizures given over several days did not have any beneficial effects. None of the patients showed any clear and meaningful improvement. Of course, this negative
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Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome

Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome

Background: Autism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15 – 35% of the patients with epilepsy, more frequently in those with intellectual disability (ID). Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are two typical forms of intractable epileptic encephalopathy associated with ID. We previously reported that ASD was diagnosed in 24.3% of patients with DS, higher in those with profound ID. Given the severe epilepsy and high frequency of ID in LGS, it is necessary to know whether ASD is a common psychomotor co-morbidity of LGS. This study evaluated the autistic behaviors and intelligence in patients with LGS and further compared that between LGS and DS, aiming to understand the complex pathogenesis of epilepsy-ASD-ID triad. Methods: A total of 50 patients with LGS and 45 patients with DS were enrolled and followed up for at least 3 years. The clinical characteristics were analyzed, and evaluations of ASD and ID were performed.
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Lennox Gastaut Syndrome in Mitochondrial Disease

Lennox Gastaut Syndrome in Mitochondrial Disease

table seizures among children with early age at first seizure. Several studies have shown that the younger the age, the worse the prognosis. When mitochondrial disease is diag- nosed at an early age, it can be converted to intractable epi- lepsy, and close monitoring is required. On the diagnostic side, cases with normal muscle biopsy were significantly higher in the group with a history of West syndrome. The reason for the high rate of normal muscle biopsy results in cases with a his- tory of West syndrome is believed to be due to examinations done at a younger age. The normal ranges of muscle biopsy according to age group are not clearly established yet. There- fore, it is not easy to correct by age even if the result were ab- normal. As West syndromes present at relatively earlier age, muscle biopsies tend to be performed at an earlier age. There could be a possibility of this affecting our results.
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Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome

Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome

Early and intensive investigations to determine underlying focal etiology are important for deciding surgical candidates, and careful preoperative investigations can be used to stee[r]

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Rufinamide in patients with Lennox-Gastaut syndrome

Rufinamide in patients with Lennox-Gastaut syndrome

The safety and efficacy of rufinamide adjunctive therapy in adult and pediatric patients with LGS (aged 4–30 years, n = 139) were evaluated in a double-blind, randomized, placebo-cont[r]

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Resective Pediatric Epilepsy Surgery in Lennox-Gastaut Syndrome

Resective Pediatric Epilepsy Surgery in Lennox-Gastaut Syndrome

We described here that resective pedi- atric epilepsy surgery for LGS with abundant generalized and multire- gional EEG abnormalities may be suc- cessful for selected patients with or ev[r]

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West Syndrome and Lennox-Gastaut Syndrome: A Survey of Natural History

West Syndrome and Lennox-Gastaut Syndrome: A Survey of Natural History

taut syndrome are characterized by their onset in infancy and early childhood, intractable seizures occurring almost daily, severe psychomotor retardation, and poor prog-.. nosis.[r]

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Update on the management of Lennox-Gastaut syndrome with a focus on rufinamide

Update on the management of Lennox-Gastaut syndrome with a focus on rufinamide

Some general treatment considerations include recom- mendations to use as few anticonvulsants concurrently as possible to avoid side effects from polytherapy, avoid excessive drowsiness which exacerbates several of the seizure types in this syndrome, and consider the cognitive and psychological comorbidities which result from both LGS and its treatment. Clearly, a multidisciplinary approach is required to address the medical and psychosocial aspects of LGS. At present, authorities recommend valproic acid as the first line medication, followed by one or two of the second-line agents (lamotrigine, rufinamide, topiramate, clobazam, felbamate, levetiracetam). 5,12 If those therapies fail
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A VALIDATED REVERSED PHASE HIGH PERFORMANCE LIQUID CHROMATOGRAPHY METHOD FOR SIMULTANEOUS DETERMINATION OF FIVE ANTIEPILEPTIC DRUGS USED IN THE TREATMENT OF LENNOX–GASTAUT SYNDROME IN THEIR PHARMACEUTICAL DOSAGE FORMS

A VALIDATED REVERSED PHASE HIGH PERFORMANCE LIQUID CHROMATOGRAPHY METHOD FOR SIMULTANEOUS DETERMINATION OF FIVE ANTIEPILEPTIC DRUGS USED IN THE TREATMENT OF LENNOX–GASTAUT SYNDROME IN THEIR PHARMACEUTICAL DOSAGE FORMS

LennoxGastaut syndrome (LGS) is a severe form of childhood epileptic encephalopathy with multiple etiologies, whether genetic, structural, metabolic, or unknown. LGS could be diagnosed by EEG, usually demonstrating high voltage, bifrontal 1.5–2.5 Hz spikes, and wave complexes. Several seizure types are associated with LGS including sudden tonic–atonic seizures (drop attacks), atypical absence (the most common), myoclonic seizures, generalized tonic–clonic seizures, and partial onset seizures. The optimum treatment for LGS has yet to be established [1-4]. Treatment is aimed at reducing seizure burden using the least number of medications while minimizing side effects. However, seizure freedom is rare, and multiple anticonvulsants are often required. Surveys have shown that valproate is often the preferred drug for initial therapy. Either lamotrigine (LAM) or topiramate or rufinamide (RUF) is often the second-line choice for monotherapy if valproate is not efficacious. BZDs have been used as parenteral or rectal agent (diazepam [DIA], lorazepam, and midazolam) in acute cases, while for chronic oral use, clobazam, clonazepam [CLO], clorazepate, and nitrazepam were used. BZDs remain, in most guidelines, the treatment of choice for acute or subacute seizures [5-15].
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Comprehensive overview: efficacy, tolerability, and cost-effectiveness of clobazam in Lennox&ndash;Gastaut syndrome

Comprehensive overview: efficacy, tolerability, and cost-effectiveness of clobazam in Lennox&ndash;Gastaut syndrome

Abstract: Clobazam is the newest medication approved by the US Food and Drug Administration (FDA) for the treatment of LennoxGastaut syndrome (LGS) in patients at least 2 years of age, although the medication has been available in countries around the world to treat epilepsy and anxiety disorders for many years. Though classified as a benzodiazepine, the drug differs structur- ally from other drugs in the class as it possesses nitrogen atoms at the 1 and 5 positions within the heterocyclic ring rather than at the 1 and 4 positions. This difference and the classification of clobazam as a partial agonist are believed to be responsible for the decreased incidence of sedative effects compared to other benzodiazepines. Adverse events associated with clobazam use in clinical trials have generally been mild to moderate in nature. Data from an open-label extension trial have confirmed that clobazam is efficacious for the treatment of seizures associ- ated with LGS, particularly atonic seizures (drop seizures), over the long term. Tolerance to the drug’s antiepileptic effects does not seem to be a common occurrence. The drug has proven to be a cost-effective option for therapy, particularly due to its ability to decrease the number of seizures that require medical treatment. Clobazam represents a welcome addition to the treat- ment options for LGS.
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Treating Lennox&ndash;Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide

Treating Lennox&ndash;Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide

Abstract: LennoxGastaut syndrome (LGS) is a rare but debilitating pediatric epileptic encephalopathy characterized by multiple intractable seizure types. Treatment of LGS is challenging because of the small number of antiepileptic drugs (AEDs) which are effective for this syndrome, as well as the need for polytherapy in the majority of patients. This review focuses on the treatment of LGS with rufinamide, a recently approved third-generation AED with reported efficacy as adjunctive therapy for LGS. All relevant papers identified through a PubMed search on the treatment of LGS with rufinamide were reviewed. To date, the literature suggests improvements in seizure frequency for pediatric patients with LGS on rufinamide. Rufinamide appears to be especially effective for atonic or drop attack seizures. Rufinamide also displays a favorable adverse event profile compared with the older anticonvulsants, as well as a minimal number of drug interactions, making it a promising option for the adjunctive treatment of seizures associated with LGS.
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Lennox&ndash;Gastaut syndrome: impact on the caregivers and families of patients

Lennox&ndash;Gastaut syndrome: impact on the caregivers and families of patients

Abstract: LennoxGastaut syndrome (LGS) has a major impact on the health-related quality of life (HRQL) of the affected children as well as their caregivers. The primary caregiver in the family is generally the mother, with support from the father and siblings. The burden of care and the effects of the disease on the child necessitate adjustments in virtually all aspects of the lives of their family. These adjustments inevitably affect the physical, emotional, social, and financial health of the whole family. Numerous sources of support for families can help to ease the burden of care. Improvements in the treatment of LGS, in addition to helping the child with LGS, would likely help improve the HRQL of the family members. This pilot par- ent survey was designed to explore the impact of epilepsy on caregiver HRQL. Parents of children with epilepsy who had contacted the Epilepsy Information Service at the Wake Forest University School of Medicine, Winston-Salem, NC, USA, were sent questionnaires compris- ing open- and closed-ended questions. A total of 200 surveys were distributed, with a return rate of 48%. The results revealed that 74% of the parents believed that having a child with epilepsy brought them and their partner closer together. However, when the parents were asked to explain the manner in which epilepsy affected their families, answers included continuous stress, major financial distress, and lack of time to spend with other children. Information and resources for the families of children with LGS could help improve the HRQL of both the patients and their relatives.
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Clobazam as an adjunctive therapy in treating seizures associated with Lennox&ndash;Gastaut syndrome

Clobazam as an adjunctive therapy in treating seizures associated with Lennox&ndash;Gastaut syndrome

Abstract: LennoxGastaut syndrome (LGS) is a devastating childhood epilepsy syndrome characterized by the occurrence of multiple types of seizures and cognitive decline. Most chil- dren suffer from frequent seizures that are refractory to current medical management. Recent clinical trials have suggested that addition of clobazam may improve the clinical outcome for some LGS patients. Although clobazam has been available for over five decades, it has only recently been approved by the US Food and Drug Administration for this indication. As a 1,5-benzodiazepine, clobazam is structurally related to the widely used 1,4-benzodiazepines, which include diazepam. Clobazam has been shown to modulate GABAergic neurotransmission by positive allosteric modulation of GABA A receptors, and to increase expression of transport- ers for both GABA and glutamate. The active metabolite n-desmethylclobazam (norclobazam) also modulates GABA A receptors, and the relative importance of these two compounds in the clinical effectiveness of clobazam remains an open question. Clinical trials involving clobazam as an addon therapy in a variety of pediatric epilepsy populations have found a significant improvement in seizure control. In patients with LGS, clobazam may have greatest efficacy for drop seizures. Longstanding clinical experience suggests that clobazam is a safe and well tolerated antiepileptic drug with infrequent and mild adverse effects. These results suggest that adjunctive treatment with clobazam may be a reasonable option for LGS patients, particularly those who are treatment-resistant.
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A Dendroarchaeological Study of Wood from Fort Lennox National Historic Site, Île-aux-Noix, Québec

A Dendroarchaeological Study of Wood from Fort Lennox National Historic Site, Île-aux-Noix, Québec

match growth patterns. Ring widths were measured using a Velmex micrometer (precision: 2 µm), and measurements were recorded and transformed into skeleton plots in the DENDRO2009 software program (Young- Vigneault 2010). Cross-dating between individual series was tested visually and statistically using COFECHA software to detect any missing or false rings (Holmes 1983; Holmes et al. 1986; Grissino-Mayer 2001). COFECHA also was used to place the series in a relative sequence, and Pearson’s r and t–tests were used to evaluate the correlation between these series. Each white pine and white cedar ring- width series was standardized using 32-year and 64-year spline functions with ARSTAN software (Cook and Holmes 1997). The expressed population signal (EPS) was used to assure homogeneity among the samples (Briffa and Jones 1989). The Fort Lennox white- pine series was then cross-dated and dated using the Champlain reference chronology (Delwaide and Filion 1999), while the white
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Clinical profile of seizure disorder in hospitalized patients

Clinical profile of seizure disorder in hospitalized patients

Study done on adult onset epilepsy by Basu PK, out of 50 patients of generalized seizure 74% were having abnormal CT scan. 17 Gastaut in his conclusion on CT scan in epilepsy have conclusion that it is best method for detecting cerebral lesion responsible for determining their nature and exact location. 18 In present study the CT head abnormality was found in 51 patients (51%). In focal seizure out of 22 patients 19 have abnormal CT scan findings.

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