Early infantile epileptic encephalopathy with burst-suppression;
Early Infantile Developmental & Epileptic Encephalopathy
Specialty
Neurology
Ohtahara syndrome (OS), also known as Early Infantile Developmental & Epileptic Encephalopathy (EIDEE)[2] is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life,[3] and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG). It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe intellectual disabilities. No single cause has been identified, although in many cases structural brain damage is present.[4]
^Berg AT, Berkovic SF, Brodie MJ, et al. (April 2010). "Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009". Epilepsia. 51 (4): 676–85. doi:10.1111/j.1528-1167.2010.02522.x. PMID 20196795.
^National Institute of Neurological Disorders and Stroke (5 December 2008). "NINDS Ohtahara Syndrome Information Page". Archived from the original on 28 February 2009. Retrieved 2009-03-10.
Ohtaharasyndrome (OS), also known as Early Infantile Developmental & Epileptic Encephalopathy (EIDEE) is a progressive epileptic encephalopathy. The...
Zandoná, Denise Isabel (June 2008). "Proteus syndrome associated with hemimegalencephaly and Ohtaharasyndrome: Report of two cases". Seizure. 17 (4): 378–382...
avoidance of seizure precipitants.[citation needed] Ohtaharasyndrome is a rare but severe epilepsy syndrome usually starting in the first few days or weeks...
neurotransmitters. Mutations have been found in patients with Ohtaharasyndrome, West syndrome, and non-specific epilepsies with variable components of intellectual...
during early development, or pathological, as in diseases such as Ohtaharasyndrome. The burst suppression pattern was first observed by Derbyshire et...
(deprecated abbreviation) Ohtaharasyndrome, a brain disorder Overall survival rate, a cancer survival statistic Oneiroid syndrome, a dreamlike fantastic...
human diseases, including Sotos syndrome (2002), Marfan syndrome type II (2004), Ohtaharasyndrome (2008), West syndrome (2010), Microphthalmia with limb...
combination of cerebral palsy and a form of severe epilepsy called Ohtaharasyndrome, requiring round-the-clock care. Recalling the receipt of this news...
discovered in 2008 as cause for this severe form of epilepsy also called Ohtaharasyndrome. Since then it has become one of the most prominent genes for epileptic...
may occur with or without severe epileptic encephalopathy (Ohtaharasyndrome, West syndrome, or early myoclonic epilepsy) in addition to severe-to-profound...
(August 2006). "Are early myoclonic encephalopathy (EME) and the Ohtaharasyndrome (EIEE) independent of each other?". Epilepsy Research. 70 Suppl 1:...
Electroclinical syndromes are primarily classified by age of onset, in which MMPSI is preceded by Ohtaharasyndrome and succeeded by West syndrome, which onset...
discovered that mutations in the PIGQ and CBL genes are a cause of Ohtaharasyndrome, a rare and fatal form of early-onset epilepsy. Hudspith completed...
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