244:. This gene is located on the long (q) arm of chromosome 2 at position 24.3 and code for the alpha subunit of the transmembrane sodium channel protein. A mutation this gene will cause an individual to develop dysfunctional sodium channel Nav 1.1, which is crucial in the pathway for sending chemical signals in the brain, causing the phenotypic display of myoclonic epilepsy from the individual. A properly functioning channel would respond to a voltage difference across the membrane and form a pore through which only sodium ions can pass. The influx of sodium induces the generation of action potential by temporarily changing the electrochemical equilibrium of the cell. When the gene is mutated, the eventually translated protein improperly folds its pore segment within the cell membrane because it has different amino acid chemistry, which renders the channel inactive. It is also possible for a mutation to reduce the number of channels produced by an individual, which leads to the development of Dravet syndrome. Both disfunctions, incorrect folding or transcribing less amount of protein, can cause Dravet syndrome.
271:- PCDH19: This gene, located on the X chromosome, encodes protocadherin 19, a protein that helps neurons adhere to each other as they migrate to form networks and recognize other cells. Because males only possess one copy of the X chromosome, even if this mutation occurs in males, it creates a type of cells containing functional protocadherin 19, so no problems occur. However, it is believed that females (who have two X chromosomes) are affected when one copy is mutated and the other is normal. Therefore, two different populations of cells containing protocadherin 19 are generated, and their abnormal interactions are believed to cause the disease's symptoms. Epilepsy with Mental Retardation limited to Females (EFMR) is its own syndrome, primarily affecting females, although it mimics and resembles Dravet syndrome in several aspects. Seizure onset is later in this epilepsy (an average of about 11 months versus the average of 6 months in Dravet syndrome), photosensitivity is less common, seizure clusters are more frequent and respond to steroids, an approach not used in Dravet syndrome.
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to pass away before 10 years of age. The
International Dravet Syndrome Epilepsy Action League (IDEA League) conducted a study in which they concluded that 31 of 833 DS patients passed away within 10 years. The average death age was 4.6 years, with 19 of 31 deaths because of SUDEP, 10 from status epilepticus, 1 from ketoacidosis, and 1 from an accident. It is unclear what duration generally these patients are projected to live. A prospective study of 37 individuals showed that, by reducing status epilepticus from occurring at a young age, the prognosis for seizures and mental impairment in DS patients can be improved.
401:(VPA) is recommended in both published recommendations. The American recommendations provide clobazam (CLB) monotherapy as an alternative; however, very few European facilities would use it. It is a prevalent misconception that since the first seizures are typically hemiclonic (focal), antiseizure medicine (ASM) can be a good choice for focal seizures. However, using sodium channel blockers is not recommended as it is can lead to fatal results over an extended period.
280:- STXBP1: This gene encodes the syntaxin-binding protein 1, which is involved in the vesicle fusion process (sacs containing substances like neurotransmitters) of the cell with the membrane. Therefore, mutations in this gene can affect the cell's ability to release neurotransmitters. Mutations have been found in patients with Ohtahara syndrome, West syndrome, and non-specific epilepsies with variable components of intellectual disability and movement disorders.
438:, as well as Dravet's syndrome, supplemented with clobazam and valproate was approved in Europe in 2007 as a therapy for Dravet syndrome and has been found to reduce overall seizure rate by 70%. In cases with more drug-resistant seizures, topiramate and the ketogenic diet are used as alternative treatments. A Cochrane review first published in 2014 and updated 2022 called for larger, randomized, well controlled trials to be able to draw conclusions.
274:- GABRA1: GABA is the primary neurotransmitter. The receptors on neurons that accept this neurotransmitter are called "GABR" (R for receptor) and are divided into two groups: A and B. GABRA1 encodes the alpha-1 receptor, and mutations are found in several epilepsies, including Childhood Absence Epilepsy, Juvenile Myoclonic Epilepsy, and Genetic Generalized Epilepsy. Some cases of Dravet syndrome are associated with mutations in the GABRA1 gene.
262:- SCN8A: This gene encodes the alpha-8 subunit (Nav1.6) and is primarily expressed in excitatory neurons (unlike SCN1A, which is inhibitory). The clinical presentation is distinct from Dravet syndrome, and patients sometimes experience epileptic spasms (not typically observed in Dravet syndrome), are less susceptible to fever-related seizures, generally do not have myoclonic seizures, and often respond to sodium channel blockers.
479:(ASOs) that can modify gene expression in the nervous system. The FDA approved ASOs for treatment of ten genetic disorders. The technique consists of targeted augmentation of nuclear gene output which allows to selectively boost expression only in tissues where the protein is normally expressed. STK-001 can increase the level of productive SCN1A mRNA and consequently increase the expression of sodium channel gene SCN1A.
460:
their experiences with management and health services. According to reports, only a tiny percentage of adult patients receive treatment with sodium channel blockers, even though many of them had already been exposed to this class of ASM. It has been shown that certain DS patients may respond to sodium channel blockers, especially LTG, with a greater frequency of seizures noted upon halting the medication.
286:- CHD2: This gene encodes the chromodomain helicase DNA-binding protein 2, which modifies gene expression. All patients diagnosed as Dravet syndrome with CHD2 mutations began their epilepsy later than usual (ages 1, 2, and 3 years), which generally seems to be a common feature of CHD2 mutations. It has also been described in patients with Jeavons syndrome, Lennox-Gastaut syndrome, and other epilepsies.
265:- SCN9A: This gene encodes the alpha-9 subunit (Nav1.7), expressed in cells of the dorsal root ganglia, neuroendocrine cells, and smooth muscle. Mutations in this gene cause sensory disorders, including an abnormal response to pain. Some Dravet syndrome patients have been found to have mutations in the SCN9A gene, but there is likely a more polygenic cause of Dravet syndrome in these cases.
259:- SCN2A: This gene encodes the alpha-2 subunit of the sodium ion channel (Nav1.2). The expression of this gene increases throughout childhood (unlike SCN1A, which peaks at 7â9 months) and is primarily produced in hippocampal neurons. Mutations in the SCN2A gene have been found in patients with various syndromes, and unlike SCN1A mutations, patients often respond to sodium channel blockers.
180:. The seizures experienced by people with Dravet syndrome become worse as the patient ages, as the disease is not very observable when symptoms first appear. This coupled with the range of severity differing between each individual diagnosed and the resistance of these seizures to drugs has made it challenging to develop treatments.
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associated with seizures in persons with Dravet syndrome. Some of the patients who put forth vaccine injury claims from encephalopathy were later found, upon testing, to actually have Dravet syndrome. Some patients who filed vaccine injury claims for encephalopathy were subsequently diagnosed with Dravet syndrome upon testing.
268:- SCN1B: This gene encodes the beta-1 subunit of the sodium ion channel, which regulates sodium channel entry on the outer side of the cell membrane. Mutations in the SCN1B gene have been found in several patients with Generalized Epilepsy with Febrile Seizures Plus (GEFS+), but very few with Dravet syndrome.
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serotype 9 (AAV9) SCN1A gene regulation therapy. It has been designed to target transgene expression to GABAergic inhibitory neurons and reduce off-target expression within excitatory cells. In this case, the treatment would be administered as a single dose intracerebroventricularly. This company has
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Regarding the care of adult DS patients, no particular guidelines are available. The usage of VPA, CLB, and TPM continued through childhood, adolescence, and adulthood, although that of STP decreased with age (31% in adults), according to the findings of a survey of caretakers of patients with DS on
183:
Dravet syndrome appears during the first year of life, often beginning around six months of age with frequent febrile seizures (fever-related seizures). Children with Dravet syndrome typically experience a lagged development of language and motor skills, hyperactivity and sleep difficulties, chronic
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Numerous research studies have been performed to evaluate DS prognosis. According to two studies, status epilepticus and sudden unexpected death in epilepsy (SUDEP) are the two most frequent causes of premature fatality among DS patients. Between ten and twenty percent of people with DS are thought
404:
Children may experience fewer seizures and less severe (longer-lasting) seizures as a result of first-line therapy with VPA; however, it is uncommon for them to live a life free of seizures. According to the recommendations, second-line options include the ketogenic diet (KD), topiramate (TPM), and
251:
in either the S5 or S6 segment of the sodium channel pore results in a loss of channel function and the development of Dravet syndrome. A heterozygous inheritance of an SCN1A mutation is all that is necessary to develop a defective sodium channel; patients with Dravet syndrome will still have one
482:
Stoke
Therapeutics is currently evaluating the long-term safety and tolerability of repeated doses of STK-001 in patients with Dravet syndrome. Change in seizure frequency, overall clinical status and quality of life will be measured as secondary endpoints in this open-label study. Recently, the
393:
As there are still very few randomized controlled trials (RCTs) available, evidence-based therapy remains difficult: RCTs are now only available for the drugs fenfluramine (FFA), cannabidiol (CBD), and stiripentol (STP). A North
American consensus panel and, more subsequently, a European expert
187:
Febrile seizures are divided into two categories known as simple and complex. A febrile seizure would be categorized as complex if it has occurred within 24 hours of another seizure or if it lasts longer than 15 minutes. A febrile seizure lasting less than 15 minutes would be considered simple.
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The timing of the first signs and symptoms in Dravet syndrome occur about the same time as normal childhood vaccinations, leading some to believe the vaccine was the cause. However, this is likely a non-specific response to fever, as vaccination often induces fever, and fever is known to be
283:- HCN1: This gene encodes a non-selective positive ion channel (allowing the passage of calcium, potassium, and other positive ions), and mutations generally result in a gain of function. In some Dravet patients with HCN1 mutation, the presentation is similar to classic Dravet syndrome.
289:- KCNA2: This gene encodes a delayed potassium channel that helps a neuron repolarize after activation. Patients believed to have Dravet syndrome with this mutation managed to remain seizure-free in adulthood, a result that is often not achieved in classic Dravet syndrome.
184:
infection, growth and balance issues, and difficulty relating to others. The effects of this disorder do not diminish over time, and children diagnosed with Dravet syndrome require fully committed caretakers with tremendous patience and the ability to closely monitor them.
172:. It is characterized by cognitive impairment, behavioural disorders, and motor deficits. Behavioural deficits often include hyperactivity and impulsiveness, and in more rare cases, autistic-like behaviours. Dravet syndrome is also associated with sleep disorders including
255:
Dravet syndrome is generally associated with mutations in the SCN1A gene, but it can also be found in patients with other mutations. Likewise, the presence of a mutation in the SCN1A gene does not necessarily mean that the patient has Dravet syndrome.
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Prior to the age of 20, photosensitivity and pattern sensitivity each have a tendency to vanish; nevertheless, some individuals still displayed light sensitivity. Consequently, for those who are older, triggering variables ought to be minimized.
444:(CBD) was approved in United States for treatment of Dravet syndrome in 2018. A 2017 study showed that the frequency of seizures per month decreased from 12 to 6 with the use of cannabidiol, compared with a decrease from 15 to 14 with placebo.
1986:
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549:, who was diagnosed as having Dravet syndrome, was the focus of a cause célÚbre to provide a means for use of cannabidiol for persons with intractable seizures. She died from pneumonia, possibly caused by COVID-19, in April, 2020.
1924:
Charlotte Figi, the
Colorado Springs girl who, as a gleeful and fragile child, launched a movement that led to sweeping changes in marijuana laws across the globe, has died from complications possibly related to the new
371:. Although diet adjustment can help, it does not eliminate the symptoms. Until a better form of treatment or cure is discovered, those with this disease will have myoclonic epilepsy for the rest of their lives.
277:- GABRG2: This gene encodes the GABA gamma-2 receptor, and mutations have been found in patients with Generalized Epilepsy with Febrile Seizures Plus (GEFS+), as well as in some Dravet syndrome patients.
188:
Sometimes modest hyperthermic stressors like physical exertion or a hot bath can provoke seizures in affected individuals. However, any seizure uninterrupted after 5 minutes, without a resumption of
44:
Severe myoclonic epilepsy of infancy, severe polymorphic epilepsy of infancy, borderland SMEI (SMEB), borderline SMEI, intractable childhood epilepsy with generalised tonic clonic seizures (ICEGTCS)
204:
In most cases the mutations in Dravet syndrome are not hereditary and the mutated gene is found for the first time in a single family member. In 70â90% of patients, Dravet syndrome is caused by
367:. Because the course of the disorder varies from individual to individual, treatment protocols may vary. A diet high in fats and low in carbohydrates may also be beneficial, known as a
141:. It often begins before one year of age, with six months being the age that seizures, characterized by prolonged convulsions and triggered by fever, usually begin.
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Treatments include cognitive rehabilitation through psychomotor and speech therapy. In addition, valproate is often administered to prevent recurrence of febrile seizures and a
405:
STP combined with VPA and CLB. The more current
European recommendations now mention CBD and FFA as potential second-line treatments (in Europe, CLB and CBD are combined).
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Certain anticonvulsant medications that are classed as sodium channel blockers are now known to make seizures worse in most Dravet patients. These medications include
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started a clinical trial in phase 1 and 2 to evaluate the safety and efficacy of ETX101 in participants with SCN1A-positive Dravet syndrome aged 6 to 36 months.
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is recommended if there is any doubt. Due to drug-refractory epilepsy in DS, many other therapies are being explored to prolong the life expectancy of patients.
1403:
Devinsky O, Cross JH, Laux L, Marsh E, Miller I, Nabbout R, et al. (May 2017). "Trial of
Cannabidiol for Drug-Resistant Seizures in the Dravet Syndrome".
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Modifying therapeutics are those which seek to correct the underlying cause of the disease. These types of treatments are into the known âadvanced therapiesâ.
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Currently, the SCN1A gene is the most clinically relevant; the largest number of DS-related mutations characterized thus far occur in this gene. Typically, a
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1.1. In mouse models, these loss-of-function mutations have been observed to result in a decrease in sodium currents and impaired excitability of GABAergic
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Selmer KK, Eriksson AS, Brandal K, Egeland T, Tallaksen C, Undlien DE (October 2009). "Parental SCN1A mutation mosaicism in familial Dravet syndrome".
2404:
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1018:"A missense mutation of the Na+ channel alpha II subunit gene Na(v)1.2 in a patient with febrile and afebrile seizures causes channel dysfunction"
543:, France, in 1978; the name was later changed to Dravet syndrome in 1989. Similar descriptions were given by Bernardo Dalla Bernardina in Verona.
1379:"Press Announcements - FDA approves first drug comprised of an active ingredient derived from marijuana to treat rare, severe forms of epilepsy"
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infection, recent publications have shown that affected individuals and their families have suffered some indirect consequences during the
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company announced positive results of MONARCH and ADMIRAL in which patients received 3 doses of STK-001 and were observed for 6 months.
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The genotypic explanation of the disorder has been located on the specific voltage-gated sodium channel genes known as
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Aledo-Serrano Ă, Mingorance A, JimĂ©nez-Huete A, Toledano R, GarcĂa-Morales I, Anciones C, Gil-Nagel A (June 2020).
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Seizures in Dravet syndrome can be difficult to manage but may be reduced by anticonvulsant medications such as
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846:"Oil-in-water emulsion adjuvants for pediatric influenza vaccines: a systematic review and meta-analysis"
301:, the diagnostic criteria for DS requires the patient to present with several of the following symptoms:
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gene resulting in a non-functional protein. This gene codes for neuronal voltage-gated sodium channel Na
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in children. It is a rare genetic disorder that affects an estimated 1 in every 20,000â40,000 births.
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was performed and this medication showed efficacy in trials. It acts as a GABAergic agent and as a
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Dravet syndrome is a severe form of epilepsy. It accounts for roughly 10% of cases of epileptic
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1.1 channels was sufficient to cause the epilepsy and premature death seen in Dravet syndrome.
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Normal initial development followed by slow development during the first few years of life
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Although it is not clear whether people with Dravet syndrome are specially vulnerable to
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192:(more normal; recovery-type; after-seizure) consciousness can lead to potentially fatal
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Characterizing the role of sodium channels in mouse models of Dravet
Syndrome
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Ankle pronation and flat feet and/or development of a crouched gait with age
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first described severe myoclonic epilepsy of infancy in Centre Saint Paul,
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was approved for the medical treatment in the
European Union and the USA.
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Initial seizures are typically prolonged and are generalized or unilateral
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Onset of seizures in the first year of life in an otherwise healthy infant
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within the first year of a child's life. This disease progresses to other
133:, with prolonged seizures that are often triggered by hot temperatures or
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Seizures are the most common form of DS. DS is diagnosed clinically and
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528:, such as healthcare barriers, loss of therapies or economic issues.
169:
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Seizures in response to strong lighting or certain visual patterns
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833:(Ph.D. thesis). Seattle, Washington: University of Washington.
1075:
Steel D, Symonds JD, Zuberi SM, Brunklaus A (November 2017).
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Seizures associated with fever due to illness or vaccinations
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like myoclonic and partial seizures, psychomotor delay, and
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Seizures induced by prolonged exposure to warm temperatures
75:
66:
1893:"Bernardo Dalla Bernardina | University of Verona (UNIVR)"
1755:"Early diagnosis of severe myoclonic epilepsy in infancy"
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Presence of other seizure types (i.e. myoclonic seizures)
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969:"A plethora of SCN1A mutations: what can they tell us?"
745:"Comprehensive care of children with Dravet syndrome"
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Dravet syndrome has been characterized by prolonged
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486:In parallel, Encoded Therapeutics is developing an
327:and later EEGs with slowing and severe generalized
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572:Oxford textbook of epilepsy and epileptic seizures
1426:20.500.11820/2ed5e0db-7de9-4411-93d7-3a5c388006b9
418:was the only medication for which a double-blind
394:committee both established treatment guidelines.
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917:"Vaccination and the onset of dravet syndrome"
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1480:FDA Approves New Therapy for Dravet Syndrome
1198:"NICE: Epilepsies: diagnosis and management"
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475:Stoke Therapeutics developed STK-001 is an
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575:. Oxford: Oxford Univ. Press. p. 13.
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2488:Citizens United for Research in Epilepsy
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1172:"Dravet Syndrome Foundation: Treatment"
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224:. The researchers found that loss of Na
2569:Syndromes with intellectual disability
2564:Syndromes affecting the nervous system
805:. Seattle: University of Washington.
428:positive allosteric modulator of GABA
137:. It is very difficult to treat with
7:
2405:Dentatorubralâpallidoluysian atrophy
1845:"The core Dravet syndrome phenotype"
129:which causes a catastrophic form of
113:severe myoclonic epilepsy of infancy
18:Severe myoclonic epilepsy of infancy
2452:Sudden unexpected death in epilepsy
1405:The New England Journal of Medicine
2549:Neurological disorders in children
2382:Complex partial status epilepticus
1724:10.1111/j.1528-1157.1990.tb05494.x
25:
2467:Psychogenic non-epileptic seizure
2339:Benign familial neonatal seizures
2291:Sleep-related hypermotor epilepsy
434:. Stiripentol, which can improve
1862:10.1111/j.1528-1167.2011.02994.x
1300:10.1111/j.1528-1167.2011.03007.x
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762:10.1111/j.1528-1167.2011.03011.x
703:10.1111/j.1399-0004.2009.01208.x
339:Unstable gait and balance issues
56:
2427:Early myoclonic encephalopathy
2400:Progressive myoclonus epilepsy
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397:As a first-line medication,
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424:randomized controlled trial
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1910:Ingold J (April 8, 2020).
1498:Developmental Neuroscience
1146:Dravet Syndrome Foundation
933:10.5698/1535-7511-11.4.120
902:"What is Dravet Syndrome?"
870:10.1038/s41467-019-14230-x
299:Dravet Syndrome Foundation
139:anticonvulsant medications
2500:Epilepsy Action Australia
1584:10.1177/10738584221088244
477:antisense oligonucleotide
436:focal refractory epilepsy
252:normal copy of the gene.
2462:LandauâKleffner syndrome
2296:Panayiotopoulos syndrome
2064:dravet-syndrome-spectrum
743:Granata T (April 2011).
336:Some degree of hypotonia
27:Genetic form of epilepsy
2344:LennoxâGastaut syndrome
2226:Epilepsy and employment
1843:Dravet C (April 2011).
1759:Brain & Development
671:10.1126/science.aab2456
111:), previously known as
2276:Temporal lobe epilepsy
2196:Electroencephalography
1043:10.1073/pnas.111065098
488:adeno-associated virus
2281:Frontal lobe epilepsy
1417:10.1056/NEJMoa1611618
1142:"Diagnostic Criteria"
850:Nature Communications
607:The Lancet. Neurology
2517:Epilepsy Research UK
2301:Vertiginous epilepsy
2221:Epilepsy and driving
2172:Epilepsy in children
1772:10.1093/brain/aws151
1675:(10216): 2203â2204.
2505:Epilepsy Foundation
2472:Epilepsy in animals
2152:Aura (warning sign)
1635:10.7759/cureus.5006
1178:on 28 December 2015
1034:2001PNAS...98.6384S
862:2020NatCo..11..315L
2392:Myoclonic epilepsy
2369:Status epilepticus
2039:External resources
1914:. The Colorado Sun
1571:The Neuroscientist
1546:clinicaltrials.gov
1470:, on ema.europa.eu
1458:, on ema.europa.eu
1294:(Suppl 2): 72â75.
1246:10.1002/epi4.12569
967:Wallace R (2005).
755:(Suppl 2): 90â94.
206:nonsense mutations
194:status epilepticus
152:Signs and symptoms
2531:
2530:
2445:Related disorders
2440:
2439:
2286:Rolandic epilepsy
2095:
2094:
1812:10.1111/epi.16537
1542:"CTG Labs - NCBI"
1510:10.1159/000517686
1411:(21): 2011â2020.
1094:10.1111/epi.13889
1087:(11): 1807â1816.
1028:(11): 6384â6389.
973:Epilepsy Currents
921:Epilepsy Currents
691:Clinical Genetics
582:978-0-19-965904-3
526:COVID-19 pandemic
323:Initially normal
297:According to the
249:missense mutation
102:
101:
30:Medical condition
16:(Redirected from
2576:
2522:Epilepsy Society
2511:Epilepsy Outlook
2354:Epileptic spasms
2266:Gelastic seizure
2241:
2167:Neonatal seizure
2122:
2115:
2108:
2099:
1940:
1928:
1927:
1921:
1919:
1912:"Charlotte Fiji"
1907:
1901:
1900:
1889:
1883:
1882:
1864:
1855:(Suppl 2): 3â9.
1840:
1834:
1833:
1823:
1806:(6): 1312â1314.
1791:
1785:
1784:
1774:
1750:
1744:
1743:
1707:
1701:
1700:
1664:
1658:
1657:
1647:
1637:
1613:
1607:
1606:
1596:
1586:
1562:
1556:
1555:
1553:
1552:
1538:
1532:
1531:
1521:
1504:(3â4): 247â252.
1489:
1483:
1477:
1471:
1465:
1459:
1453:
1447:
1446:
1428:
1400:
1394:
1393:
1391:
1389:
1375:
1369:
1368:
1358:
1326:
1320:
1319:
1283:
1268:
1267:
1257:
1225:
1210:
1209:
1207:
1205:
1194:
1188:
1187:
1185:
1183:
1174:. Archived from
1168:
1162:
1161:
1159:
1157:
1152:on 17 March 2015
1148:. Archived from
1138:
1132:
1131:
1129:
1128:
1113:
1107:
1106:
1096:
1072:
1066:
1065:
1055:
1045:
1013:
1007:
1006:
996:
964:
955:
954:
944:
912:
906:
905:
898:
892:
891:
881:
841:
835:
834:
826:
815:
814:
796:
783:
782:
764:
740:
723:
722:
686:
675:
674:
658:
649:
648:
622:
598:
587:
586:
566:
537:Charlotte Dravet
510:encephalopathies
160:and non-febrile
127:genetic disorder
82:
81:
78:
77:
74:
71:
68:
65:
62:
33:
21:
2584:
2583:
2579:
2578:
2577:
2575:
2574:
2573:
2534:
2533:
2532:
2527:
2494:Epilepsy Action
2476:
2436:
2386:
2363:
2359:Febrile seizure
2324:Absence seizure
2305:
2261:Complex partial
2230:
2213:Personal issues
2207:
2192:Investigations
2188:Anticonvulsants
2176:
2162:Epileptogenesis
2157:Postictal state
2135:
2126:
2096:
2091:
2090:
2075:dravet-syndrome
2034:
2033:
1951:
1937:
1932:
1931:
1917:
1915:
1909:
1908:
1904:
1891:
1890:
1886:
1842:
1841:
1837:
1793:
1792:
1788:
1752:
1751:
1747:
1709:
1708:
1704:
1666:
1665:
1661:
1615:
1614:
1610:
1564:
1563:
1559:
1550:
1548:
1540:
1539:
1535:
1491:
1490:
1486:
1478:
1474:
1466:
1462:
1454:
1450:
1402:
1401:
1397:
1387:
1385:
1377:
1376:
1372:
1341:(9): CD009887.
1328:
1327:
1323:
1285:
1284:
1271:
1227:
1226:
1213:
1203:
1201:
1196:
1195:
1191:
1181:
1179:
1170:
1169:
1165:
1155:
1153:
1140:
1139:
1135:
1126:
1124:
1115:
1114:
1110:
1074:
1073:
1069:
1015:
1014:
1010:
966:
965:
958:
914:
913:
909:
900:
899:
895:
843:
842:
838:
828:
827:
818:
798:
797:
786:
742:
741:
726:
688:
687:
678:
660:
659:
652:
600:
599:
590:
583:
568:
567:
560:
555:
534:
518:
506:
497:
470:
457:
431:
349:
295:
238:
227:
215:
202:
154:
146:genetic testing
105:Dravet syndrome
86:
59:
55:
36:Dravet syndrome
31:
28:
23:
22:
15:
12:
11:
5:
2582:
2580:
2572:
2571:
2566:
2561:
2559:Rare syndromes
2556:
2551:
2546:
2536:
2535:
2529:
2528:
2526:
2525:
2519:
2514:
2508:
2502:
2497:
2491:
2484:
2482:
2478:
2477:
2475:
2474:
2469:
2464:
2459:
2457:Todd's paresis
2454:
2448:
2446:
2442:
2441:
2438:
2437:
2435:
2434:
2429:
2424:
2423:
2422:
2420:Lafora disease
2417:
2415:MERRF syndrome
2412:
2407:
2396:
2394:
2388:
2387:
2385:
2384:
2379:
2373:
2371:
2365:
2364:
2362:
2361:
2356:
2351:
2346:
2341:
2336:
2331:
2329:Atonic seizure
2326:
2321:
2315:
2313:
2307:
2306:
2304:
2303:
2298:
2293:
2288:
2283:
2278:
2273:
2269:
2268:
2263:
2258:
2256:Simple partial
2253:
2249:
2247:
2238:
2232:
2231:
2229:
2228:
2223:
2217:
2215:
2209:
2208:
2206:
2205:
2203:Epileptologist
2200:
2199:
2198:
2190:
2184:
2182:
2178:
2177:
2175:
2174:
2169:
2164:
2159:
2154:
2149:
2143:
2141:
2137:
2136:
2127:
2125:
2124:
2117:
2110:
2102:
2093:
2092:
2089:
2088:
2077:
2066:
2055:
2043:
2042:
2040:
2036:
2035:
2032:
2031:
2020:
2009:
1998:
1983:
1968:
1952:
1947:
1946:
1944:
1943:Classification
1936:
1935:External links
1933:
1930:
1929:
1902:
1884:
1835:
1786:
1765:(5): 299â303.
1745:
1718:(4): 397â400.
1702:
1659:
1608:
1577:(6): 732â750.
1557:
1533:
1484:
1472:
1460:
1448:
1395:
1370:
1321:
1269:
1234:Epilepsia Open
1211:
1189:
1163:
1133:
1108:
1067:
1008:
956:
927:(4): 120â122.
907:
893:
836:
816:
784:
724:
697:(4): 398â403.
676:
650:
588:
581:
557:
556:
554:
551:
547:Charlotte Figi
533:
530:
517:
514:
505:
502:
496:
493:
469:
466:
456:
453:
429:
410:benzodiazepine
369:ketogenic diet
348:
345:
344:
343:
340:
337:
334:
331:
321:
318:
315:
312:
309:
306:
294:
291:
237:
234:
225:
213:
201:
198:
153:
150:
100:
99:
94:
88:
87:
85:
84:
52:
50:
46:
45:
42:
38:
37:
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2581:
2570:
2567:
2565:
2562:
2560:
2557:
2555:
2552:
2550:
2547:
2545:
2542:
2541:
2539:
2523:
2520:
2518:
2515:
2512:
2509:
2506:
2503:
2501:
2498:
2495:
2492:
2489:
2486:
2485:
2483:
2481:Organizations
2479:
2473:
2470:
2468:
2465:
2463:
2460:
2458:
2455:
2453:
2450:
2449:
2447:
2443:
2433:
2430:
2428:
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2421:
2418:
2416:
2413:
2411:
2408:
2406:
2403:
2402:
2401:
2398:
2397:
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2393:
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2383:
2380:
2378:
2375:
2374:
2372:
2370:
2366:
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2357:
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2352:
2350:
2347:
2345:
2342:
2340:
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2327:
2325:
2322:
2320:
2317:
2316:
2314:
2312:
2308:
2302:
2299:
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2289:
2287:
2284:
2282:
2279:
2277:
2274:
2271:
2270:
2267:
2264:
2262:
2259:
2257:
2254:
2251:
2250:
2248:
2246:
2242:
2239:
2237:
2236:Seizure types
2233:
2227:
2224:
2222:
2219:
2218:
2216:
2214:
2210:
2204:
2201:
2197:
2194:
2193:
2191:
2189:
2186:
2185:
2183:
2179:
2173:
2170:
2168:
2165:
2163:
2160:
2158:
2155:
2153:
2150:
2148:
2147:Seizure types
2145:
2144:
2142:
2138:
2134:
2130:
2123:
2118:
2116:
2111:
2109:
2104:
2103:
2100:
2087:
2083:
2082:
2078:
2076:
2072:
2071:
2067:
2065:
2061:
2060:
2056:
2054:
2050:
2049:
2045:
2044:
2041:
2037:
2030:
2026:
2025:
2021:
2019:
2015:
2014:
2010:
2008:
2004:
2003:
1999:
1997:
1993:
1992:
1988:
1984:
1982:
1978:
1977:
1973:
1969:
1967:
1963:
1962:
1958:
1954:
1953:
1950:
1945:
1941:
1934:
1926:
1913:
1906:
1903:
1898:
1894:
1888:
1885:
1880:
1876:
1872:
1868:
1863:
1858:
1854:
1850:
1846:
1839:
1836:
1831:
1827:
1822:
1817:
1813:
1809:
1805:
1801:
1797:
1790:
1787:
1782:
1778:
1773:
1768:
1764:
1760:
1756:
1749:
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1741:
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1733:
1729:
1725:
1721:
1717:
1713:
1706:
1703:
1698:
1694:
1690:
1686:
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1678:
1674:
1670:
1663:
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1655:
1651:
1646:
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1631:
1627:
1623:
1619:
1612:
1609:
1604:
1600:
1595:
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1585:
1580:
1576:
1572:
1568:
1561:
1558:
1547:
1543:
1537:
1534:
1529:
1525:
1520:
1515:
1511:
1507:
1503:
1499:
1495:
1488:
1485:
1481:
1476:
1473:
1469:
1464:
1461:
1457:
1452:
1449:
1444:
1440:
1436:
1432:
1427:
1422:
1418:
1414:
1410:
1406:
1399:
1396:
1384:
1380:
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1371:
1366:
1362:
1357:
1352:
1348:
1344:
1340:
1336:
1332:
1325:
1322:
1317:
1313:
1309:
1305:
1301:
1297:
1293:
1289:
1282:
1280:
1278:
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1274:
1270:
1265:
1261:
1256:
1251:
1247:
1243:
1239:
1235:
1231:
1224:
1222:
1220:
1218:
1216:
1212:
1199:
1193:
1190:
1177:
1173:
1167:
1164:
1151:
1147:
1143:
1137:
1134:
1122:
1118:
1112:
1109:
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1095:
1090:
1086:
1082:
1078:
1071:
1068:
1063:
1059:
1054:
1049:
1044:
1039:
1035:
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1027:
1023:
1019:
1012:
1009:
1004:
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986:
982:
978:
974:
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961:
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948:
943:
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934:
930:
926:
922:
918:
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908:
903:
897:
894:
889:
885:
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871:
867:
863:
859:
855:
851:
847:
840:
837:
832:
825:
823:
821:
817:
812:
808:
804:
803:
795:
793:
791:
789:
785:
780:
776:
772:
768:
763:
758:
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750:
746:
739:
737:
735:
733:
731:
729:
725:
720:
716:
712:
708:
704:
700:
696:
692:
685:
683:
681:
677:
672:
668:
664:
657:
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651:
646:
642:
638:
634:
630:
626:
621:
616:
612:
608:
604:
597:
595:
593:
589:
584:
578:
574:
573:
565:
563:
559:
552:
550:
548:
544:
542:
538:
531:
529:
527:
523:
515:
513:
511:
503:
501:
494:
492:
489:
484:
480:
478:
473:
467:
465:
461:
454:
452:
450:
445:
443:
439:
437:
433:
425:
421:
417:
413:
411:
406:
402:
400:
399:valproic acid
395:
391:
389:
385:
381:
377:
376:carbamazepine
372:
370:
366:
362:
358:
354:
346:
341:
338:
335:
332:
330:
326:
322:
319:
316:
313:
310:
307:
304:
303:
302:
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292:
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257:
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245:
243:
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233:
229:
223:
219:
211:
207:
199:
197:
195:
191:
185:
181:
179:
175:
171:
167:
166:seizure types
163:
159:
151:
149:
147:
142:
140:
136:
132:
128:
125:
122:
118:
114:
110:
106:
98:
95:
93:
89:
80:
54:
53:
51:
49:Pronunciation
47:
43:
39:
34:
19:
2319:Tonicâclonic
2079:
2068:
2057:
2046:
2022:
2011:
2000:
1985:
1970:
1955:
1925:coronavirus.
1923:
1916:. Retrieved
1905:
1897:ResearchGate
1896:
1887:
1852:
1848:
1838:
1803:
1799:
1789:
1762:
1758:
1748:
1715:
1711:
1705:
1672:
1668:
1662:
1628:(6): e5006.
1625:
1621:
1611:
1574:
1570:
1560:
1549:. Retrieved
1545:
1536:
1501:
1497:
1487:
1482:, on fda.gov
1475:
1463:
1451:
1408:
1404:
1398:
1386:. Retrieved
1382:
1373:
1338:
1334:
1324:
1291:
1287:
1240:(1): 11â26.
1237:
1233:
1202:. Retrieved
1192:
1180:. Retrieved
1176:the original
1166:
1154:. Retrieved
1150:the original
1145:
1136:
1125:. Retrieved
1123:(in Spanish)
1120:
1111:
1084:
1080:
1070:
1025:
1021:
1011:
979:(1): 17â20.
976:
972:
924:
920:
910:
896:
853:
849:
839:
830:
801:
752:
748:
694:
690:
662:
613:(6): 592â8.
610:
606:
571:
545:
535:
519:
507:
504:Epidemiology
498:
485:
481:
474:
471:
462:
458:
449:fenfluramine
446:
440:
422:-controlled
414:
407:
403:
396:
392:
373:
350:
296:
288:
285:
282:
279:
276:
273:
270:
267:
264:
261:
258:
254:
246:
239:
230:
218:interneurons
203:
186:
182:
155:
143:
116:
112:
108:
104:
103:
2311:Generalised
2048:GeneReviews
1383:www.fda.gov
442:Cannabidiol
416:Stiripentol
384:lamotrigine
357:stiripentol
222:hippocampus
41:Other names
2538:Categories
2334:Automatism
2181:Management
2013:DiseasesDB
1551:2023-10-27
1127:2023-10-27
856:(1): 315.
553:References
380:gabapentin
361:topiramate
329:polyspikes
174:somnolence
2029:230437002
2024:SNOMED CT
1918:April 13,
1849:Epilepsia
1800:Epilepsia
1712:Epilepsia
1697:209394550
1288:Epilepsia
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