該文獻由北京癲癇病醫院（整理
infants and children with severe neocortical epilepsy syndrome clinical manifestation
typically, the involvement of children in the prior to the onset of normal. usually with focal motor seizures, partial status epilepticus, generalized tonic-clonic seizures ( gtcs ) or with automatism in complex partial seizures onset. won continuous state may occur in 20% of patients.
in 50% of cases the blood ring attack in the previous 6 months, with upper respiratory tract infections, otitis media or tonsillitis history.
典型的，受累兒童在發病前正常。通常以局灶性運動性發作、部分性癲癇持續狀態、全面性強直陣攣發作〔gtcs)或不伴自動症的複雜部分性發作起病。won持續狀態可能出現於20%的患者中。
50%的病例在血響發作前6個月左右，有上呼吸道感染、中耳炎或扁桃體炎史。
when the disease, focal motor seizures involve the distal muscles ( a thumb, finger, mouth or eyes ), but as time advances, the affected area spread to adjacent areas, h**ing a walk and a more diffuse, and sleep in the persistence of. along with the progress of. there are a variety of seizures, seizure
frequency, and gradually increased during episodes of prolonged, often accompanied by seizures after hemiplegia. partial epilepsy to state in 60% patients, and can last for several days, often mixed with laterality tonic-clonic seizures, but also can develop as a generalized tonic-clonic seizure. appears in episodes after initial hemiparesis, short duration, but gradually develop persistent.
in addition, there are reported in the literature in histologically confirmed kozhevvikov rssmussen syndrome cases, but without oar attack.
發病時，局灶性運動性發作主要累及遠端少數肌群(拇指、手指、嘴角或眼角)，但是隨著時間進展，受累部位擴散至周圍相鄰區域，具有遊走性和更加瀰散，並且在睡眠中也持續存在。隨著病程的進展。出現多種發作，並且發作頻率逐漸增加、發作期延長，經常伴有發作後偏癱。
部分性癲癇待續狀態見於60%的患者，並可以持續數天，經常混有偏側強直一陣攣發作，也可發展為全面性強直一陣攣發作。出現於發作後的偏癱，最初持續時間短暫，但逐步發展為永續性。另外，也有文獻報進了經組織學確診的kozhevvikov一rssmussen症候群病例，但是不伴有oar發作。
the clinical course is divided into three stages
臨床病程分三個階段
the first stage : the key to ****** motion or somatosensory as feature. the situation is relatively rare to partial status epilepticus, not with automatism in complex partial seizures or secondary gtcs onset, may also represent the seizure type combination.
after a few weeks or months, gradually become frequent episodes.
第一階段:主要以簡單運動或軀體感覺發作為特徵。比較少見的情況是以部分性癲癇持續狀態、不伴自動症的複雜部分性發作或繼發性gtcs起病，也可能表現為這些發作型別的組合。
經過數週或數月，發作逐漸地變得頻繁。
second stage: episodes of deterioration and the intact hemisphere neurological symptoms progress to feature. this phase usually begins about the occurrence of seizures after 3 months, but can also be delayed until the disease was first symptoms after 10 years.
in this phase, attacks become
more frequent, the affected area is more extensive. episodes of longer duration. at the same time, as the disease progression in children with sharp, lasting psychomotor defects, including associated with cognitive and language impaired ( aphasia and dysarthria ) of hemiparesis, hemihypesthesia, hemianopia.
most of the brain damage occurred in 8 after a 12 month.
第二階段:以發作的惡化和受累半球的神經系統症狀進展為特徵。這一階段通常大約開始於出現癲癇發作3個月後，但也可延遲至該病首次症狀後10年之久。
在此階段，發作變得更加頻繁，受累部位更加廣泛。發作持續時間更長。同時，隨著病情急劇進展，患兒出現持久的精神運動缺陷，包括伴有認知和語言受損(失語和構音障礙)的輕偏癱、偏側感覺減退、偏盲。
大部分腦損害發生於起病後的8一12個月。
progressive neurological and psychomotor function degradation, either with or without delusion seizure deterioration, koehevnikov is a typical rssmusrxn syndrome.
進行性神經系統和精神運動功能退化，無論伴有或不伴有痴癇發作的惡化，是koehevnikov一rssmusrxn症候群的典型特徵。
the third stage: the disease showed the frequency of seizures and decreased severity of neurological deficits, progress is slow, but remnants in patients with severe cognitive impairment.
第三階段:該病表現出癲癇發作的頻率和嚴重性降低，神經功能缺損的進展也減緩，但是患者遺留嚴重的神經認知功能損害。
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