It is a debilitating and early progressive neurological disorder, resulting in intractable seizures and severe mental retardation.
[ncbi.nlm.nih.gov]
OHTAHARA SYNDROME Overview Ohtahara syndrome (also known as early infantile epileptic encephalopathy, EIEE) is a syndrome characterized by frequent intractable seizures and
[epilepsydiagnosis.org]
It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe intellectual disabilities.
[en.wikipedia.org]
Lennox-Gastaut syndrome is a severe childhood epilepsy disorder characterized by encephalopathy and multiple, often intractable, seizure types.
[ncbi.nlm.nih.gov]
[…] wave discharge and intermittent <3-Hz generalized spike-and-wave discharges lasting 1-8 seconds.
[pediatricneurosciences.com]
Abstract The West syndrome and the Lennox-Gas-taut syndrome are characterized by their onset in infancy and early childhood, intractable seizures occurring almost daily, severe
[pediatrics.aappublications.org]
EARLY MYOCLONIC ENCEPHALOPATHY Overview Early myoclonic encephalopathy is a syndrome characterized by frequent intractable seizures and severe early encephalopathy resulting
[epilepsydiagnosis.org]
[…] activity. Multi-form,intractable and often aggressive seizures. Severe cognitive, behavioural and neurological deficits. Poor prognosis with occ. early death. 3.
[slideshare.net]
Epileptic encephalopathies are severe brain disorders of early age that manifest with (1) EEG activity that is often aggressive, (2) seizures that are usually multiform and
[fortheloveofbubbe.weebly.com]
Prognosis Prognosis is variable, ranging from normal neurodevelopmental outcome and complete seizure remission after several years, to severe intellectual disability and intractable
[orpha.net]
[…] years of age; (3) presence of generalized spike- or polyspike-wave EEG discharges at 2-3 Hz, without focal spike discharges; and (4) exclusion of severe and benign myoclonic
[ncbi.nlm.nih.gov]
[…] years; EEG findings of generalized spikes or polyspike-wave discharges at 2-3 Hz with an absence of focal spikes; Exclusion of other syndromes, specifically, cryptogenic Lennox-Gastaut
[symptoma.com]
Recent study has shown that mutations in the alpha-II-spectrin (SPTAN1) gene cause early onset intractable seizures, severe developmental delay, diffuse hypomyelination, and
[ncbi.nlm.nih.gov]
It is characterized by intractable seizures occurring almost daily, severe psychomotor retardation, poor prognosis and EEG abnormalities, known as hypsarrhythmia.
[ncbi.nlm.nih.gov]
Abstract The West syndrome and the Lennox-Gas-taut syndrome are characterized by their onset in infancy and early childhood, intractable seizures occurring almost daily, severe
[pediatrics.aappublications.org]
SCN1A gene have been identified in probands affected by the syndrome of intractable childhood epilepsy with generalized clonic tonic seizures (ICEGTC).
[neurologyindia.com]
[…] complexes of 3-4 Hz, or faster frequency, superimposed on a normal EEG background [6–8].
[hindawi.com]
Mutations in the genes encoding sodium channel α and β1 subunits have been associated with genetic forms of epilepsy. [37],[51],[52],[53],[54] Several missense mutations in
[neurologyindia.com]
These epilepsies are also among the most severe with dire consequences including intractable seizures and severe cognitive dysfunction.
[doi.org]
[…] is often profound; medical intractability; and severe consequences for a normal development.
[doi.org]
These epilepsies share several important characteristics: diverse causes; severe and frequent seizures; diffusely abnormal background activity on electroencephalograms that
[doi.org]
PLUS, TYPE 2 SEVERE MYOCLONIC EPILEPSY OF INFANCY FAMILIAL HEMIPLEGIC MIGRAINE, TYPE 3 INTRACTABLE CHILDHOOD EPILEPSY, WITH GENERALIZED TONIC-CLONIC SEIZURES FAMILIAL FEBRILE
[neuromab.ucdavis.edu]
EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum
[slideshare.net]
Childhood, With Generalized Tonic-Clonic Seizures, Included Myoclonic Epilepsy, Severe, Of Infancy Severe Myoclonic Epilepsy Of Infancy EIEE6 EIEE6 SME SMEI 607208 Genetic
[ukgtn.nhs.uk]
Plus, SCN1A-Related Intractable Childhood Epilepsy with Generalized Tonic-Clonic Seizures, SCN1A-Related Myoclonic-Astatic Epilepsy, SCN1A-Related Severe Myoclonic Epilepsy
[genetests.org]
Their EEG was characterized by generalized discharges of 3~6 Hz bilaterally symmetrical polyspikes and slow waves induced by eye closure and photosensitivity.
[dict.cnki.net]
The ictal EEG pattern is described as 10 to 16 hertz (Hz) polyspikes, while the interictal EEG is a generalized (throughout the whole brain) 3 to 6 Hz spike and wave pattern
[verywellhealth.com]
Several states have approved medical marijuana to treat seizures or seizure disorders.
[medicalmarijuanainc.com]
An EEG in untreated individuals is typically abnormal with a specific EEG pattern, known as a 3-6 Hz generalized polyspike and wave discharge.
[epilepsy.com]
[…] epilepsy), Utah (intractable epilepsy), Virginia (intractable epilepsy), West Virginia, Wisconsin (seizure disorders), and Wyoming (intractable epilepsy).
[medicalmarijuanainc.com]
OMIM : 56 Mutations in the KCTD7 gene cause a severe neurodegenerative phenotype characterized by onset of intractable myoclonic seizures before age 2 years and accompanied
[malacards.org]
EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum
[slideshare.net]
Childhood, With Generalized Tonic-Clonic Seizures, Included Myoclonic Epilepsy, Severe, Of Infancy Severe Myoclonic Epilepsy Of Infancy EIEE6 EIEE6 SME SMEI 607208 Genetic
[ukgtn.nhs.uk]
PLUS, TYPE 2 SEVERE MYOCLONIC EPILEPSY OF INFANCY FAMILIAL HEMIPLEGIC MIGRAINE, TYPE 3 INTRACTABLE CHILDHOOD EPILEPSY, WITH GENERALIZED TONIC-CLONIC SEIZURES FAMILIAL FEBRILE
[neuromab.ucdavis.edu]
The EEG is characterised by generalised polyspike and wave discharges at 4-6 Hz. (05 Mar 2000) MeSH(Medical Subject Headings) 맞춤 검색 (http://www.nlm.nih.gov) 결과 : 3 페이지: 1
[kmle.co.kr]
seizures, 90% cognitive impairment, 5% death Severe epilepsy with MISF In spectrum of IS and LGS Onset 4-7 years EEG shows MISF Mostly intractable seizures, especially tonic
[emilytam.com]
[…] activity. Multi-form,intractable and often aggressive seizures. Severe cognitive, behavioural and neurological deficits. Poor prognosis with occ. early death. 3.
[slideshare.net]
Could present normal interictal EEG with occasional frontally dominant 3-6 Hz s/w or ps/w. Simple Febrile Normal interictally. Shows Sz type (usually TC).
[studystack.com]
Severe brain disorders in which the epileptic electrical discharges may contribute to progressive psychomotor dysfunction. Early age of presentation. EEG paroxysmal
[slideshare.net]
PLUS, TYPE 2 SEVERE MYOCLONIC EPILEPSY OF INFANCY FAMILIAL HEMIPLEGIC MIGRAINE, TYPE 3 INTRACTABLE CHILDHOOD EPILEPSY, WITH GENERALIZED TONIC-CLONIC SEIZURES FAMILIAL FEBRILE
[neuromab.ucdavis.edu]
EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum
[slideshare.net]
Hz generalized spike-and-wave or polyspike-and-waves but no focal epileptiform discharges (except for fragments of generalized discharges), (3) no epileptogenic lesion on
[frontiersin.org]
PLUS, TYPE 2 SEVERE MYOCLONIC EPILEPSY OF INFANCY FAMILIAL HEMIPLEGIC MIGRAINE, TYPE 3 INTRACTABLE CHILDHOOD EPILEPSY, WITH GENERALIZED TONIC-CLONIC SEIZURES FAMILIAL FEBRILE
[neuromab.ucdavis.edu]
6 Hz generalized polyspike and wave activity JME is a lifelong condition that typically improves with age Lennox-Gastaut syndrome Syndrome of severe, refractory seizures that
[straighthealthcare.com]
Severe myoclonic epilepsy in infancy ( Dravet syndrome). Intractable childhood epilepsy with generalized tonic-clonic seizures (ICE-GTC).
[slideplayer.com]
A 7-year-old boy presented with intractable head nodding and atonic seizures since the age of 3 years and severe behavior problems unsuitable for video EEG monitoring.
[ncbi.nlm.nih.gov]
EEG demonstrates 3–6 Hz generalised polyspike and wave activity, with photosensitivity in more than 30% of people.
[nice.org.uk]
Surgery is only a consideration for very severe, intractable cases in which medical treatment doesn’t work.
[healthline.com]
6 Hz generalized polyspike and wave activity JME is a lifelong condition that typically improves with age Lennox-Gastaut syndrome Syndrome of severe, refractory seizures that
[straighthealthcare.com]
The outlook for someone who has had a focal seizure varies depending on the underlying cause.
[healthline.com]
Although the epileptic seizures were controlled by multiple antiepileptic drugs, her cortical myoclonus remained intractable, which severely interfered her activity of daily
[pesquisa.bvsalud.org]
EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum
[slideshare.net]
seizures, 90% cognitive impairment, 5% death Severe epilepsy with MISF In spectrum of IS and LGS Onset 4-7 years EEG shows MISF Mostly intractable seizures, especially tonic
[emilytam.com]
These discharges are common in juvenile myoclonic epilepsy and usually appear as repetitive 4-6 Hz polyspike-waves without any associated myoclonus.
[aibolita.com]
[…] febrile status epi-lepticus 3 years after the first status episode (patient 10).She had several brief febrile seizures between these twoepisodes of status epilepticus.
[docslide.com.br]
Childhood, With Generalized Tonic-Clonic Seizures, Included Myoclonic Epilepsy, Severe, Of Infancy Severe Myoclonic Epilepsy Of Infancy EIEE6 EIEE6 SME SMEI 607208 Genetic
[ukgtn.nhs.uk]
EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum
[slideshare.net]
Genetic Test Registry Epileptic Encephalopathy, Early Infantile, 5 EIEE5 613477 Genetic Test Registry Epileptic Encephalopathy, Early Infantile, 6 Dravet Syndrome Epilepsy, Intractable
[ukgtn.nhs.uk]
Compared with its hemizygous male counterpart, the heterozygous female disease has less severe ID, but is more often associated with a severe and intractable myoclonic epilepsy
[ncbi.nlm.nih.gov]
Women had similar interictal EEG findings, that is, normal background but generalized 3–6 Hz spike/polyspike waves.
[ncbi.nlm.nih.gov]
Electroencephalogram (EEG) ictal accompaniments were generalized 3-6 Hz polyspikes and slow waves [Figure 2].
[neurologyindia.com]